Psychiatry Test Preparation and Review Manual: Expert Consult, 2nd Ed.

Explanations - Test Number Two

Question 1. E. There are several life-cycle theorists that are important to know. Freud’s theory of development involving oral, anal, and phallic stages is important. Carl Jung felt that development of the personality occurs through experiences that teach a person who they intrinsically are. For him, libido included sexual energy, but also spiritual urges and a drive to understand the meaning of life. Harry Stack Sullivan focused on social interaction. In his theory he defined each stage of life through the need to interact with certain individuals. These interactions shaped the development of the personality.

  Erik Erikson developed a life cycle from childhood to adulthood. It consists of:

• Stage 1: Trust vs mistrust. Trust is shown by ease of feeding and depth of sleep, and depends on consistency of experience provided by the caretaker. If trust is strong, the child develops self-confidence. (Birth–1 year old)

• Stage 2: Autonomy vs shame and doubt. This stage includes the child learning to walk and feed himself. There is a need for outer control. Shame happens through excessive punishment, and self-doubt occurs if the child is made to feel ashamed of his actions. (Age 1–3)

• Stage 3: Initiative vs guilt. In this stage the child initiates both motor and intellectual activities. If reinforced, his intellectual curiosity is satisfied. If he is made to feel inadequate, he will develop guilt about self-initiated activities. (Age 3–5 years)

• Stage 4: Industry vs inferiority. In this stage the child is busy building, creating, and accomplishing. If he is inferior to his peers with use of tools and skills, he will have less status and develop a sense of inadequacy and inferiority. (Age 6–11 years)

• Stage 5: Ego identity vs role confusion. There is a struggle to develop a sense of inner sameness and continuity. The adolescent shows preoccupation with appearance, hero worship, and ideology. There are dangers of role confusion, and doubts about his sexual orientation and vocational identity. (Age 11 years–end of adolescence)

• Stage 6: Intimacy vs isolation. Intimacy is marked by formation of life-long attachments and self-abandonment. Separation occurs if the individual is isolates and views others as dangerous. (Age 21–40 years)

• Stage 7: Generativity vs stagnation. Generativity is marked by raising children, altruism, creativity, and guiding the next generation. Stagnation occurs if there is isolation, excessive self-concern, and an absence of intimacy. (Age 40–65 years)

• Stage 8: Ego identity vs despair. Integrity is a feeling of satisfaction that life has been worthwhile, along with an acceptance of one’s place in life. Despair is the feeling of loss of hope, disgust, and fear of death. (Age 65+ years)

  Piaget developed a theory of cognitive development. It consists of four stages: sensorimotor, preoperational thought, concrete operations, and formal operations. The work of Freud, Jung and Erikson was the result of observations of children, not of carefully crafted studies. Their theories are a framework to understand development, but are not intended to describe objective reality. Their work has been followed by more scientific longitudinal studies of development, and more neurobiological understandings of human behavior.

  Human Development

  K&S Ch. 2

Question 2. D. The clinical picture given is that of a conduction aphasia. Conduction aphasia results from left hemispheric (dominant) lesions particularly in the inferior parietal or superior temporal regions. The area of predilection for this lesion is considered to be the arcuate fasciculus that connects Wernicke’s and Broca’s areas. Conduction aphasia is characterized by inability to repeat, relatively normal spontaneous speech, and the possibility of paraphasic errors and hesitancy. Naming may be impaired, but auditory comprehension is intact. Writing may or may not be impaired. Associated symptoms of a conduction aphasia may include right hemi-paresis, right hemisensory loss, right hemianopsia, and limb apraxia.

  Broca’s aphasia would involve a lesion of Broca’s area in the left posterior inferior frontal gyrus. This results in broken, stuttering, staccato speech, with inability to repeat and phonemic and paraphasic errors. Reading is often impaired, although auditory comprehension is usually intact. Broca’s aphasia is frequently associated with depression and right hemiparesis and hemisensory loss.

  Wernicke’s aphasia results from a lesion in the superior temporal gyrus in Wernicke’s area. Speech is generally fluent, but comprehension is impaired. Speech may also be logorrheic, or overproductive. The speech displays paragrammatism, which involves neologisms, verbal paraphasic errors, and production of jargon. Repetition, naming, and auditory comprehension are impaired. Reading comprehension is impaired. Wernicke’s aphasia is often accompanied by a right homonymous hemianopsia, with a marked absence of motor and sensory signs and symptoms.

  Global aphasia results from large lesions involving the superior temporal, inferior frontal and parietal lobes. The region generally corresponds to the territory of the middle cerebral artery (MCA) and indeed a thrombus at the trifurcation of the MCA can produce a global aphasia. Speech is nonfluent or mute and comprehension is impaired. Naming, reading, repetition, and writing are all poor. Most patients present with a dense right hemiparesis, hemisensory loss and hemianopsia.

  Transcortical aphasias involve the areas around and adjacent to Broca’s and Wernicke’s areas and essentially present with similar features to the two perisylvian aphasias, except that repetition is spared. Transcortical motor aphasia presents with similar features to Broca’s aphasia, with telegraphic, stuttering speech, sparing of comprehension and fluent repetition. The area involved is usually anterior to Broca’s area in the anterior cerebral artery territory. Transcortical sensory aphasia is the analogue of Wernicke’s aphasia and presents with fluent paraphasic speech, paraphasic naming, impaired reading and auditory comprehension, and normal repetition. The lesion usually localizes to the temporo-occipital area. Transcortical mixed aphasia is a rare condition also known as isolation of the speech area. The classic presentation is that of a global aphasia with echolalic repetition only, and no propositional speech or comprehension. The patient can only repeat. The lesion localizes to large watershed areas in the left hemisphere, sparing the perisylvian areas.

  Neurology

  B&D Ch. 12

Question 3. D. Major types of receptors found on neurons include the seven-transmembrane-domain receptor, which requires G proteins to open ion channels, and the ligand-gated ion channel receptor, which actually has an ion channel as part of its structure. The seven-transmembrane-domain receptor has a characteristic NH2 terminal outside the cell, several intracytoplasmic loops, and an intracellular COOH terminal. It is the tyrosine kinase receptor which interacts with nerve growth factor (NGF) and brain derived neurotropic factor (BDNF). Through these interactions the tyrosine kinase receptor is thought to play a large role in neuronal plasticity and the remodeling of synaptic associations. It is also important to recall that hormones and steroids can diffuse into the neuron and bind to cytoplasmic receptors whose effects carry to the nucleus and regulate gene expression.

  Basic Neuroscience

  K&S Ch. 3

Question 4. E. The classic complication of rapid sodium replacement in hyponatremia is central pontine myelinolysis. This can result in a clinical transection of the pons and a locked-in syndrome. Locked-in syndrome is the result of a pontine lesion that is clinically devastating and produces quadriplegia, mutism and a lower cranial nerve palsy with only the ability to move the eyes up and down and blink the eyelids being preserved. Only the upper brainstem function is preserved and patients usually need to be on a respirator. Cognition and comprehension are usually grossly intact and the patient is often quite aware of the predicament. Prognosis is very poor. The locked-in syndrome is most often the result of a ventral pontine infarct as a consequence of basilar artery thrombosis, but can in certain instances result from central pontine myelinolysis, which produces a more central pontine lesion. Other possible causes of locked-in syndrome are acute inflammatory demyelinating polyneuropathy (AIDP or Guillain–Barré syndrome), myasthenia gravis, and neuromuscular blocking agents.

  Neurology

  B&D Ch. 5

Question 5. D. This question contains an example of rationalization. Rationalization is characterized by offering rational explanations in an attempt to justify attitudes, beliefs or behaviors that may otherwise be unacceptable. Projection is perceiving and reacting to unacceptable inner impulses as if they are outside the self. Blocking is temporarily halting thinking. It often occurs in psychosis because of hallucinations and thought disorganization. Externalization is perceiving elements of your own personality in the external world or in external objects. It is a more general term than projection. Denial is avoiding awareness of some painful aspect of reality by negating sensory data. The most primitive of the defenses are the narcissistic defenses (denial, distortion, projection). The least primitive defenses are the mature defenses (altruism, anticipation, asceticism, humor, sublimation, suppression).

  Psychological Theory and Psychometric Testing

  K&S Ch. 6

Question 6. B. The clinical condition depicted in this question is that of pseudotumor cerebri, also called benign intracranial hypertension. Classically a problem seen in young obese African-American women, the exact etiology is unknown. Risk factors include hypervitaminosis A, high-dose corticosteroid therapy, tetracycline therapy, oral contraceptive use and head trauma. Patients typically present with a waxing and waning headache and intermittent visual obscurations. Neurologic examination can reveal papilledema on funduscopic examination and enlargement of the blind spot on visual field testing. Brain imaging is usually normal, although some scans reveal slit-like ventricles. The diagnosis is established by lumbar puncture with measurement of the opening pressure, which is elevated over 20 cmH2O. The treatment modality of choice is pharmacologic, with acetazolamide 500 mg once or twice daily or with prednisone 20–40 mg daily. In certain cases patients opt for repeated lumbar punctures to siphon off fluid to maintain normal cerebrospinal fluid pressure. Surgical options for treatment include ventriculoperitoneal shunting, or lumboperitoneal shunting if the ventricles are too small, and optic nerve sheath fenestration, which can siphon off cerebrospinal fluid. ESR would be used as a diagnostic test for temporal arteritis. Serum prolactin level is sometimes drawn following a seizure to determine if it is epileptic or nonepileptic. The prolactin level would be expected to be elevated greater than twice normal within an hour after a true epileptic seizure.

  Neurology

  B&D Ch. 46

Question 7. C. The National Alliance for the Mentally Ill (NAMI) is an advocacy group made up of families of the mentally ill that works at local, state, and federal levels to improve services for the mentally ill. They are involved with lawmakers, outreach and education. The other answer choices are distracters. The most important organization to know is NAMI.

  Public Policy

  K&S Ch. 4

Question 8. E. In 1988, the International Headache Society (IHS) established strict criteria for migraine with and without aura. The criteria for migraine without aura are as follows: at least five headache attacks lasting 4 to 72 hours (untreated or unsuccessfully treated), which have at least two out of four characteristics – unilateral location; pulsating quality; moderate or severe intensity; or aggravated by walking stairs or similar routine physical activity. During the headache at least one of the two following symptoms occur: phonophobia and photophobia; nausea and/or vomiting.

  Migraine with aura involves at least two attacks with at least three of the following: one or more fully reversible aura symptoms indicating focal cerebral cortical and/or brainstem functions; At least one aura symptom develops gradually over more than 4 minutes, or two or more symptoms occur in succession; no aura symptom lasts more than 60 minutes; or headache follows aura with free interval of at least 60 minutes (it may also simultaneously begin with the aura). At least one of the following aura features establishes a diagnosis of migraine with typical aura: homonymous visual disturbance; unilateral paresthesias and/or numbness; unilateral weakness; or aphasia or unclassifiable speech difficulty.

  Neurology

  B&D Ch. 69

Question 9. C. The lifetime prevalence of schizophrenia is 1%. It is estimated that there are somewhere in the vicinity of 2 million patients with schizophrenia in the United States. The other answer choices are distracters.

  Psychotic Disorders

  K&S Ch. 4

Question 10. A. Recombinant tissue plasminogen activator (r-TPA) is FDA-approved for acute thrombolysis of ischemic cerebrovascular accident within 3 hours of the occurrence of the event. Inclusion criteria for intravenous r-TPA administration include acute ischemic stroke with a clear time of onset less than or equal to 3 hours prior to desired r-TPA administration, neurologic deficit measurable on the NIH stroke scale and CT scan of the head demonstrating an absence of intracranial hemorrhage. Exclusion criteria are: rapidly improving or minor stroke symptoms or deficits, seizure at the onset of stroke, prior intracranial hemorrhage, pretreatment blood pressure greater than 185/110 mmHg, major surgery within the previous 14 days, a prior stroke or head injury within the last 3 months, intestinal or urinary bleeding within 3 weeks prior to the stroke, subarachnoid hemorrhage, blood glucose level <50 mg/dL or >400 mg/dL, recent myocardial infarction, current use of oral anticoagulants (PT > 15 or INR > 1.7) or heparin use within the last 48 hours, a platelet count of <100 000 per mL, or arterial puncture at a noncompressible site within the last 7 days.

  Neurology

  B&D Ch. 51

Question 11. B. Assertive community treatment is based on the model whereby teams of psychiatrists and other mental health workers go out into the community to patients’ homes to maintain contact with them, monitor their status, and encourage medication compliance. The goal is to prevent decompensation of severely mentally ill patients and catch them before they severely decompensate and require hospitalization.

  Public Policy

  K&S Ch. 4

Question 12. C. The condition described in this question is that of restless legs syndrome (RLS). The only FDA-approved treatment is ropinorole (Requip), a dopamine agonist, which can improve symptoms at low doses starting at 0.5 mg prior to bedtime. Other dopamine agonists, pergolide, pramipexole and bromocriptine can be useful, but are not specifically FDA approved. Other helpful agents include benzodiazepines, opiate analgesics, gabapentin and levodopa/carbidopa. Restless legs syndrome has a prevalence rate of about 5% and is most often seen in middle to older-aged patients. It is usually idiopathic, but has been associated with polyneuropathy, uremia, and iron deficiency. The classic symptoms of RLS are crawling or creeping sensations (paresthesias or dysesthesias) of the lower extremities that are worse when lying down or in bed and occur most often at the onset of sleep. There is an urge to move the legs during rest, or when lying down or sitting. The urge to move is generally relieved by movement, like walking or stretching. Symptoms are worse at night or only occur in the evening or at night. Family history can be positive in 40–50% of patients, which suggests an autosomal dominant pattern of inheritance.

  Neurology

  B&D Ch. 68

Question 13. D. The best method to diagnose depression is the standard psychiatric interview. Psychiatric interviews serve two functions: to find and classify symptoms, and to find psychological determinants of behavior. Interviews can either be insight or symptom oriented. The other answer choices all have major flaws. The MMPI is a self-report inventory used to assess personality traits, and as such is not appropriate to the task. The scholastic achievement test is completely unrelated to symptom identification for depression. The dexamethasone suppression test is used to demonstrate abnormal activity of the hypothalamic–pituitary–adrenal axis which can be found in 50% of major depression patients. However, the test has limited clinical usefulness because of the frequency of false positive and negative test results. Interviewing the patient’s teacher is a good idea, but in no way can it replace a face-to-face meeting with the child or be the sole basis for diagnosing depression.

  Diagnostic and Treatment Procedures in Psychiatry

  K&S Chs 1&15

Question 14. B. This question describes a left lateral medullary syndrome (also called a Wallenberg’s syndrome). The Wallenberg syndrome is a brainstem stroke syndrome usually caused by occlusion of one of the vertebral arteries, or less commonly, one of the posterior inferior cerebellar arteries. The area of infarction is the lateral medulla. The clinical syndrome involves an ipsilateral Horner’s syndrome, ipsilateral loss of pain and temperature sensation in the face, cerebellar ataxia and weakness of the vocal cords, pharynx and palate. There is contralateral loss of pain and temperature sensation to the hemibody. The visual system is not affected with this syndrome.

  Neurology

  B&D Ch. 51

Question 15. C. Decreased levels of serotonin in the cerebrospinal fluid (CSF) have been shown to be linked to higher levels of aggression in patients. In general, dopamine seems to promote aggression, whereas norepinephrine and serotonin seem to inhibit it, as does GABA. Rapid declines in serotonin levels have been linked to irritability and aggression, and low CSF serotonin has been linked to increased frequency of suicide. Corticotropin-releasing hormone (CRH) is a hormone that may increase in major depression, anorexia, and anxiety disorders. It is produced by the hypothalamus. It has no relation to aggression. Growth hormone is also unrelated to aggression.

  Basic Neuroscience

  K&S Ch. 4

Question 16. D. Metachromatic leukodystrophy (MLD) is an autosomal recessive metabolic disorder of myelin that results in a deficiency in arylsulfatase A (ASA). The result is an abnormal accumulation of sulfatides in the brain and peripheral nerves. High sulfatide levels lead to progressive demyelination. The ASA gene is located on chromosome 22q13. Gait disorder with hypotonia and lower limb areflexia are early manifestations of the disease and often precedes central nervous system involvement in infantile and juvenile forms of the disease. Adult-onset MLD tends to present with progressive dementia and behavioral problems. Nerve conduction velocities (NCV) are slowed in both juvenile and adult patients. Delayed visual and somatosensory evoked potential latencies are noted more often in adult cases. The classic neuropathologic findings are segmental nerve demyelination on nerve biopsy and metachromatic inclusions within Schwann cells and macrophages. Although nerve biopsy is diagnostic of MLD, MRI of the brain combined with urine assay for increased sulfatide excretion and abnormal ASA enzyme assay in leukocytes is less invasive and the preferred diagnostic method today. Treatment by bone marrow transplantation may increase brain ASA levels sufficiently to slow or stop disease progression.

  Tay–Sachs disease is the severe infantile form of the autosomal recessive gangliosidosis that results in hexosaminidase A deficiency. Hexosaminidase A codes to chromosome 15q23–q24. The classic infantile picture is that of developmental retardation, paralysis, dementia, and blindness with death in the second or third year of life. The classic finding is a “cherry-red spot” on funduscopic examination. Babies with Tay–Sachs disease have a persistent hypersensitivity to loud noise with a marked hyperreactivity of startle response.

  Krabbe’s disease, also called globoid cell leukodystrophy, is an autosomal recessive disease caused by a deficiency in lysosomal enzyme galactocerebrosidase β-galactosidase. The gene has been localized to chromosome 14q31. Multinucleated macrophages in central white matter are accompanied by extensive central and peripheral demyelination. Infants present with rapid deterioration in motor and intellectual development, hypertonicity, optic atrophy, opisthotonic posture, and seizures. Stem cell transplantation can improve central nervous system manifestations by providing a source of missing enzyme.

  Neurology

  B&D Ch. 76

Question 17. C. Seasonal affective disorder is a non-DSM term used to describe a seasonal specifier added to the diagnoses of bipolar I & II disorders, and major depressive disorder. It is associated with depressive symptoms that occur at a certain time of year, with complete remission of symptoms at other times of the year. One must show a pattern of two episodes during the same season of the previous 2 years to make the diagnosis. In addition, the seasonal depressive episodes must substantially outnumber any non-seasonally related depressive episodes during the patient’s lifetime. The treatment is light therapy.

  Depressive Disorders

  K&S Ch. 15

Question 18. D. The parachute response can persist far longer than 6 months. The Moro reflex is elicited by startle and is usually present in the normal infant up to 6 months of age. The child extends the arms symmetrically when the head is rapidly but gently dropped a few centimeters into the examiner’s hand with the baby in a supine position. The tonic neck reflex is normally present from birth to about 3 months of age. Grasp and rooting reflexes usually disappear at or about the 6 month of age mark. Any persistence of these reflexes beyond this period or recurrence later in life, would be considered abnormal. Positive grasp and rooting reflexes in the adult would be considered frontal release signs and would signal extensive frontal white matter damage or disease.

  Neurology

  B&D Ch. 27

Question 19. D. Answer choice D describes pseudocyesis, which is listed in the DSM under somatoform disorders NOS. It involves a false belief that one is pregnant, and can involve physical signs associated with pregnancy, such as those described. However, the patient is not pregnant and there is no endocrine disorder present to explain the findings. Other patients that fall under the heading of somatoform disorder NOS include those with other somatic symptoms that do not meet the time criteria for other diagnoses. Answer choice A describes pain disorder, in which a patient experiences pain that can not be explained by a medical condition and is thought to be significantly mediated by psychological factors. Answer choice B describes hypochondriasis, in which a patient is convinced that he has a serious disease, based on misinterpretation of bodily symptoms, and despite the reassurance of doctors. Choice C describes conversion disorder, in which the patient develops neurological symptoms with no medical explanation that are thought to be mediated by psychological factors. Choice E describes somatization disorder, in which the patient has physical complaints spanning several organ systems that have no medical explanation and are thought to be associated with psychological factors. To make the diagnosis of somatization disorder, the patient must exhibit at any time during the disturbance, four pain symptoms, two gastrointestinal symptoms, one sexual symptom, and one pseudoneurological symptom.

  Somatic Symptom Disorders

  K&S Ch. 17

Question 20. A. Cytomegalovirus (CMV) is the most common offending infectious agent in AIDS-related retinopathy. It accounts for 30% of cases of HIV-related retinopathy. Toxoplasmosis is less common, likely involved in about 5% of retinitis cases. Tuberculosis is a rare cause of AIDS-related retinitis. Cryptococcus neoformans is usually responsible for a fungal meningitis and not a retinitis in AIDS patients with low CD4 counts. JC virus is the offending agent in progressive multifocal leukoencephalopathy in AIDS patients.

  Neurology

  B&D Ch. 53

Question 21. D. The most common hallucinations in schizophrenia are auditory. Visual, olfactory and tactile hallucinations are also possible. Schizophrenia was first discovered by Morel who called it “démence precoce”. Later Kraepelin used “dementia praecox” to describe a group of illnesses that started in adolescence and ended in dementia. Bleuler coined the term “schizophrenia.” The disorder is chronic and has a prodromal, active, and residual phase.

  Psychotic Disorders

  K&S Ch. 13

Question 22. B. Night-time awakening is not considered to be part of the clinical picture of narcolepsy. Narcolepsy is a lifelong sleep disorder that is believed to have a hereditary component. In the United States, the prevalence is about 3 to 6 in 10 000. One to two percent of first-degree relatives of narcoleptic patients manifest the illness compared to 0.02–0.18% of the population at large. Narcolepsy is linked to the dysfunction of the hypocretin (orexin) peptide system.

  The hallmark of the disease is excessive daytime sleepiness and sleep attacks. Sleep attacks manifest as the irresistible desire to fall asleep. Attacks can occur at any waking moment and last for a few minutes up to thirty minutes’ duration. Performance at school, work, and in social situations usually suffers.

  The second major manifestation of narcolepsy is cataplexy. Cataplexy is the sudden loss of muscle tone in the voluntary muscles, with sparing of the respiratory and ocular muscles. Attacks of cataplexy involve the patient falling to the ground following rapid complete loss of muscle tone. Consciousness is preserved during cataplexic attacks. Anywhere from 60–100% of narcoleptic patients suffer from cataplexy.

  Sleep paralysis is the third major symptom of narcolepsy. It can be noted in one-quarter to one-half of narcoleptic patients. These attacks are characterized by hypnagogic or hypnopompic sudden bilateral or unilateral limb paralysis. Consciousness is preserved during these attacks, but the patient cannot move or speak.

  Hypnagogic hallucination is the fourth major symptom of narcolepsy. These can occur at onset of sleep or during early morning awakening. About 20–40% of narcoleptic patients experience these hallucinations. Other major symptoms of narcolepsy include disturbance of night sleep in about 70–80% of patients and automatic behavior that can resemble a fugue-like state, which can occur in 20–40% of narcoleptic patients.

  The only medications FDA-approved for narcolepsy are modafinil and sodium oxybate. Modafinil, which can be given in a single dose of 100–200 mg daily, modulates the hypocretin (orexin) neurons in the brainstem and is a non-amphetamine stimulant. Sodium oxybate (Xyrem) is a drug that was initially FDA-approved specifically for cataplexy, but now has an approval for narcolepsy as well.

  Sleep Wake Disorders

  B&D Ch. 68

Question 23. B. Logorrhea is uncontrollable, excessive talking. Alexithymia is a difficulty in recognizing and describing one’s emotions. Echolalia is the imitative repetition of the speech of another. Flight of ideas is rapid shifting from one topic to another. Stilted speech is a formal stiff speech pattern.

  Diagnostic and Treatment Procedures in Psychiatry

  K&S Ch. 8

Question 24. C. Intramuscular phenytoin is a poor choice for treatment of status epilepticus because of its erratic rate of absorption. Useful status epilepticus treatments include rectal or intravenous diazepam, intravenous lorazepam, intravenous phenytoin or phosphenytoin, intravenous valproic acid, oxygen by nasal cannula and airway protection, and intravenous phenobarbital. Phenobarbital is acceptable treatment, but not the best choice because of the narrow therapeutic window and possibility of overdose leading to respiratory depression and possible death.

  Neurology

  B&D Ch. 67

Question 25. A. Of the statements listed, the only correct one is answer choice A. Ziprasidone is an agonist at 5-HT-1A and does inhibit reuptake of serotonin and norepinephrine. It is also an antagonist at 5-HT-1D, 2A, and 2C, as well as D2 and D3. It has low affinity for histaminic, muscarinic and alpha-2 receptors. Because of its action on serotonin and norepinephrine, it has been postulated to be of benefit not only in psychosis, but also in anxiety and depression. Risperidone is a similar blocker of D2 when compared to haldol, and as such carries one of the highest risks for EPS among the atypicals. Quetiapine is known for its low incidence of EPS, as it has only a moderate affinity for the D2 receptor. It has a high affinity for 5-HT types 2 and 6, H1, and alpha 1 and 2. It has low affinity for muscarinic receptors. Olanzapine has been associated with weight gain in many patients. It is very anticholinergic and carries with it the corresponding side effects. Clozapine has been shown to be associated with a decreased risk of suicide in schizophrenic patients. Major side effects of clozapine include seizures and agranulocytosis, among others. And while we’re on the topic of anticholinergic side effects, the treatment of choice for urinary retention is urecholine (Bethanechol).

  Psychopharmacology

  K&S Ch. 35

Question 26. A. Divalproex sodium (valproic acid) has the broadest spectrum of coverage and indication of all the anticonvulsant medications. It has proven efficacy in primary-generalized tonic–clonic seizures, absence seizures, and myoclonic seizures. Its likely mechanism of action is by blockade of voltage-dependent sodium channels and GABA enhancement. Phenytoin is not indicated for absence seizures and has no place in their treatment. It is indicated for treatment of partial and generalized tonic-clonic seizures. Its mechanism of action is by blockade of voltage-dependent sodium channels. Oxcarbazepine and carbamazepine are indicated for partial complex and secondary generalized seizures, but both can worsen absence seizures. Oxcarbazepine does not produce autoinduction of its own metabolism in the way that carbamazepine does. Ethosuximide is indicated only in uncomplicated absence seizures and has no place in the treatment of partial complex or secondary generalized tonic–clonic seizures. Its mechanism of action is by lowering voltage-dependent calcium conductance in thalamic neurons.

  Neurology

  B&D Ch. 67

Question 27. E. Constipation is a common side effect of the tricyclic antidepressants as a result of the anticholinergic activity of these drugs. Other tricyclic side effects include dry mouth, blurry vision, sweating, orthostatic hypotension, sedation, lethargy, agitation, tremor, slowed cardiac conduction (as evidenced by prolonged PR and QRS intervals), and tachycardia.

  Psychopharmacology

  K&S Ch. 36

Question 28. A. Foot flexion (also called dorsiflexion; the bending of the foot upwards) involves the tibialis anterior muscle innervated by nerves that emanate from the L5 motor nerve root. L5 also controls the extension (bending upwards) of the big toe (extensor hallucis longus). A lesion at L5 often causes a foot drop from dorsiflexor muscle weakness. Foot extension (pushing down on the gas pedal) involves the gastrocnemius (calf) and soleus muscles innervated by nerves that emanate from the S1 nerve root predominantly. Leg extension is a function of the quadriceps muscles (anterior thigh) which are innervated by nerves emanating from the L3 and L4 nerve roots. Hip flexion (raising of the knee in the air) is a function of the iliopsoas muscles which are innervated by the L1, L2 and L3 nerve roots. The Achilles or ankle jerk motor reflex is a function of the S1 motor nerve root.

  Neurology

  B&D Ch. 23

Question 29. D. Amitriptyline can be used for gastric ulcer because of its strong histamine blockade. Other drugs that can also be used for this purpose include doxepin and trimipramine.

  Psychopharmacology

  K&S Ch. 36

Question 30. A. The condition noted in this question is that of the classic Brown–Séquard syndrome due to a hemisection of the spinal cord. The correct combination of deficits is that of loss of motor control and posterior column function ipsilateral to and below the level of the lesion coupled with contralateral loss of pain and temperature sensation one to two dermatomal levels below the level of the lesion. Frequent causes include disk herniation, penetrating trauma, spinal fracture and radiation injury. The cervical spinal cord is most commonly affected.

  Neurology

  B&D Ch. 24

Question 31. B. This question focuses on Piaget’s stages of cognitive development. In the sensorimotor stage children respond to stimuli in the environment, learning during the process. They eventually develop object permanence (objects exist independent of the child’s awareness of their existence), and begin to understand symbols (as in the use of words to express thoughts).

  During the preoperational stage there is a sense that punishment for bad deeds is unavoidable (immanent justice). There is a sense of egocentrism, and phenomenalistic causality (the thought that events that occur together cause one another). Animistic thinking (inanimate objects are given thoughts and feelings) is also seen.

  In the concrete operations stage, egocentric thought changes to operational thought where another’s point of view can be taken into consideration. In concrete operations children can put things in order and group objects according to common characteristics. They develop the understanding of conservation (a tall cup and a wide cup can both hold equal volumes of water) and reversibility (ice can change to water and back to ice again).

  During formal operations children can think abstractly, reason deductively, and define abstract concepts. Trust vs mistrust is one of Erik Erikson’s stages and is a distracter in this question.

  Human Development

  K&S Ch. 2

Question 32. D. The anterior cerebral artery (ACA) territory is the area affected by this stroke. The ACA irrigates the medial frontal lobes and therefore when affected causes preferential leg greater than arm and face weakness contralateral to the side of the lesion. The destruction of the nearby frontal eye fields causes loss of tonic opposition of the gaze to the opposite side so the eyes will often be noted to look toward the side of the lesion. Sphincteric incontinence is sometimes seen if the cortical bowel and bladder areas are affected. Patients often suffer from abulia, a loss of will power or the lack of ability to do things independently. Bilateral ACA territory damage can result in akinetic mutism.

  Neurology

  B&D Ch. 51

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Question 33. A. Norepinephrine is made in the locus ceruleus. Serotonin is made in the dorsal raphe nuclei. Dopamine is made in the substantia nigra. Acetylcholine is made in the nucleus basalis of Meynert.

  Basic Neuroscience

  K&S Ch. 3

Question 34. E. Pure motor hemiparesis is one of the classic lacunar stroke syndromes and would be expected from an infarct in the area of the internal capsule, the basis pontis, or the corona radiata. Lacunar strokes are characterized as ischemic strokes resulting from small vessel lipohyalinosis that is caused by hypertension and diabetes as the two major risk factors. Lacunar infarcts by definition are small and range from 0.5 mm to 1.5 cm in diameter. Microembolism may also be a mechanism of lacunar strokes.

  Other lacunar syndromes include pure sensory stroke, usually from a small lesion in the ventroposterolateral nucleus of the thalamus; sensorimotor stroke, from a stroke to the internal capsule and thalamus, or the posterior limb of the internal capsule; ataxic hemiparesis, which results from a lacunar infarct to either the basis pontis or posterior limb of the internal capsule; and the dysarthria-clumsy hand syndrome, resulting from a stroke to the deep areas of the basis pontis.

  Neurology

  B&D Ch. 51

Question 35. D. Of the receptors listed, it is the histamine receptor that is associated with weight gain and sedation. The M1 receptor is associated with constipation, blurred vision, dry mouth, and drowsiness. Alpha 1 receptors are associated with dizziness and decreased blood pressure. The 5-HT-1A receptor is a presynaptic autoreceptor involved in the response of neurons to the SSRIs. The 5-HT-2A receptor is one of the post-synaptic serotonin receptors involved in the neuron’s response to the SSRIs. The serotonin receptors are associated with modulation of depression and anxiety, but not with weight gain or sedation.

  Basic Neuroscience

  K&S Ch. 3

Question 36. A. The contralateral subthalamic nucleus of Luys is most often the area where a lesion will produce hemiballismus. Hemiballismus is a dramatic, flinging movement of the proximal extremities. It can affect both the upper and lower limbs and is most often unilateral. Most frequently, the cause of hemiballismus is an acute stroke.

  Neurology

  B&D Ch. 71

Question 37. B. The six biogenic amine neurotransmitters are dopamine, epinephrine, norepinephrine, acetylcholine, histamine, and serotonin. Of these, dopamine, norepinephrine, and epinephrine are all synthesized from the precursor tyrosine, and are known as a group as the catecholamines. GABA is an amino acid neurotransmitter, not a biogenic amine. Of note is that cocaine works by blocking the reuptake of the biogenic amines, more specifically serotonin, norepinephrine, and dopamine.

  Basic Neuroscience

  K&S Ch. 3

Question 38. D. Reserpine interferes with the magnesium and ATP-dependent uptake of biogenic amines which results in the depletion of dopamine, norepinephrine and serotonin neurotransmitters. In this way, it can relieve symptoms of dystonia and can also cause depression, sedation, and a Parkinson’s like syndrome.

  Neurology

  B&D Ch. 71

Question 39. A. Projection is perceiving and reacting to unacceptable inner impulses as though they were outside the self. On a psychotic level it takes the form of delusions about external realities that are usually persecutory in nature. One’s own impulses and hostilities are projected onto another who is now assumed to have intentions to persecute you. The other answer choices are distracters that have nothing to do with projection.

  Psychological Theory and Psychometric Testing

  K&S Ch. 6

Question 40. E. The arachnoid (pacchionian) granulations are the major sites for drainage of cerebrospinal fluid (CSF) into the blood. These granulations protrude through the dura into the superior sagittal sinus and act as one way valves or siphons for the CSF. Equilibrium is maintained by the arachnoid granulations, for if CSF pressure drops below a certain level, absorption stops. If the CSF pressure increases, more fluid is absorbed. The choroid plexuses that protrude into the ventricles are responsible for the majority of CSF production in humans. In humans, the rate of CSF production is about 0.35 mL/min. Drugs that can temporarily reduce production of CSF and ease increased intracranial pressure include acetazolamide and mannitol. States of hypothermia, hypocarbia, hypoxia and hyperosmolality can also temporarily decrease CSF production. Virchow–Robin spaces act as a duct for substances in the subarachnoid space to enter the brain. The other answer choices are distracters.

  Neurology

  B&D Ch. 59

Question 41. D. Regression analysis is a method of predicting the value of one variable in relation to another variable based on observed data. Probability is the likelihood that an event will occur. A probability of 0 means it will not occur. A probability of 1 means it will definitely occur. Point prevalence is the number of people with a disorder at a specific point in time divided by the total population at that point in time. Incidence is the number of new cases of a disease over a given time divided by the total number of people at risk during that time. Central tendency is a central value in a distribution around which other values are arranged. Examples of central tendency are the mean, median, and mode. The mean is the average. The median is the middle value in a series of values. The mode is the value that appears most frequently in a series of measurements. Kappa is a variable used to indicate a constant value that does not change. Sensitivity is the ability of a test to detect that which is being tested for.

  Statistics

  K&S Ch. 4

Question 42. D. Noncontrast head CT is the best immediate test of choice in the emergency room if a lobar hemorrhage is suspected. MRI of the brain can add precision, but in the hyperacute stage may not be able to reveal acute bleeding as well as the CT scan. The most common location of intracranial hemorrhage is from the putamen, which accounts for about 35% of cases. Lobar hemorrhage is second to putaminal hemorrhage in frequency and accounts for about one-quarter of cases. Hypertension remains the most frequent cause of intracranial hemorrhage, but arteriovenous malformations, cerebral amyloid angiopathy and sympathomimetic agents can also account for quite a number of cases.

  Emergency room management of suspected intracranial hemorrhage begins with stabilization of vital signs and airway protection. Intubation is indicated if the level of consciousness drops to a Glasgow Coma Scale score of 8 or less. CT scan of the head must next be done to determine the location and size of the hemorrhage. Neurosurgical consultation may be indicated if the hemorrhage is large and increased intracranial pressure is suspected.

  Blood work checking the coagulation panel is very important. If the patient is on oral or parenteral anticoagulation therapy, it is imperative to consider reversing anticoagulation by protamine sulfate for those on heparin or with parenteral vitamin K or fresh frozen plasma for those patients on warfarin therapy.

  Bedside EEG is not indicated unless there is pervasive coma or seizures. Lumbar puncture is usually contraindicated if intracranial or lobar hemorrhage is suspected, because it can trigger uncal herniation and eventual death if the patient is already in a state of increased intracranial pressure. The lumbar puncture with CSF xanthochromia assay is useful only if a subarachnoid hemorrhage is suspected.

  Neurology

  B&D Ch. 51

Question 43. E. Outcomes in psychodynamic therapy have been found to be closely associated with the empathy provided by the therapist. The goal is for the therapist to be in tune with the patient’s internal state in an empathic way. When patients feel that therapists understand their internal world they are more likely to accept interpretations made by the therapist. The other answer choices, while important are not the most important. Some of these distracters may have impacts on the therapeutic relationship, but will not be the universally most important factor in the outcome of therapy.

  Psychotherapy

  K&S Ch. 35

Question 44. A. Melatonin is believed to be released principally by the pineal gland and there is a feedback loop between the pineal gland and the suprachiasmatic nucleus in the hypothalamus that helps with sleep regulation. Melatonin is secreted predominantly at night and levels peak between 3:00 am and 5:00 am, and decrease to lower levels during the day. Melatonin is a modulator of human circadian rhythm for entrainment by the light–dark cycle.

  Basic Neuroscience

  B&D Ch. 68

Question 45. D. The Minnesota Multiphasic Personality Inventory (MMPI) is an objective psychological test. It is a self report inventory used to find areas of pathological personality structure. The other tests listed are projective tests. In projective tests the patient is given a picture or incomplete piece of information and asked to fill in the details or complete the unfinished task. The answers that the patient gives reveal aspects of his personality, thought content, thought structure, and psychological makeup. In the Rorschach test the patient is shown ink blots and asked to share what they thinks the ink blots look like. In the sentence completion test the patient is asked to finish incomplete sentences. In the thematic apperception test the patient is shown a series of pictures and asked to make up stories based on the pictures. In the draw a person test the patient is asked to draw a person of the same and opposite sex. The person of the same sex is thought to represent the patient. Interpretations are made based on the details the patient puts into the drawings.

  Psychological Theory and Psychometric Testing

  K&S Ch. 5

Question 46. A. Subacute sclerosing panencephalitis (SSPE) is the result of a persistent and nonproductive viral infection of the neurons and glia that is caused by the measles virus. It is a late complication of measles. Children 5 to 10 years of age are affected most often. Clinical manifestations include personality and cognitive changes, myoclonic seizures, spasticity, choreoathetoid movements, difficulty swallowing, eventually leading to coma and death. The three tests of choice are lumbar puncture for cerebrospinal fluid (CSF) assay, electroencephalogram (EEG) and brain biopsy. CSF reveals an elevated measles antibody titer, oligoclonal bands, absence of pleocytosis, normal glucose and normal to elevated protein. Brain biopsy may reveal neuronal and glial nuclear and cytoplasmic viral inclusion bodies. EEG reveals a characteristic pattern of periodic bursts of generalized slow-wave complexes. There is no specific accepted treatment, but studies have revealed that intraventricular interferon-α combined with oral isoprinosine can be helpful. Prognosis is generally poor and often death can be expected within twelve months if response to treatment is poor. There is no evidence that SSPE results from measles vaccination in children.

  Neurology

  B&D Ch. 53

Question 47. E. Attention deficit–hyperactivity disorder (ADHD) is diagnosed by six or more symptoms of inattention or six or more symptoms of hyperactivity–impulsivity that persist for 6 months or more. Some symptoms that cause impairment should be present before age 7 years. Impairment must be present in more than one setting to make the diagnosis. Symptoms of inattention involve failure to pay close attention to tasks, failure to sustain attention, not listening, not following through on tasks, problems organizing tasks, forgetfulness, and being easily distracted by extraneous stimuli. Symptoms of hyperactivity–impulsivity include fidgeting, inability to remain seated when expected, running or climbing excessively, difficulty playing quietly, acting as if driven by a motor, talking excessively, blurting out answers, difficulty awaiting turn, and interrupting others.

  Disruptive, Impulse Control, Conduct Disorders, and ADHD

  K&S Ch. 42

Question 48. E. Lesch–Nyhan syndrome is an X-linked recessive hereditary disorder of purine and pyrimidine metabolism. Hyperuricemia results from a deficiency in hypoxanthine-guanine phosphoribosyltransferase (HGPRT). Clinical symptoms and signs of the syndrome include choreoathetosis, hyperreflexia, hypertonia, dysarthria, behavioral disturbances, cognitive impairment and self-mutilatory behavior. Neurologic signs and symptoms are likely a result of diminished dopamine concentrations in the cerebrospinal fluid and basal ganglia.

  Metachromatic leukodystrophy is an autosomal recessive disorder which is caused by arylsulfatase-A (ASA) deficiency. There is an accumulation of excess sulfatides in the nervous system which leads to progressive demyelination. The disorder localizes to chromosome 22q13.

  Tay–Sachs is a recessive disorder localizing to chromosome 15q23-24. It is caused by a deficiency in hexosaminidase A. The adult form presents as progressive weakness in the proximal muscles of the upper and lower extremities. Associated symptoms may involve spasticity, dysarthria, cognitive and psychiatric impairment.

  Krabbe disease, also called globoid cell leukodystrophy, is an autosomal recessive disease that localizes to chromosome 14q31. It is a result of a deficiency in lysosomal enzyme galactocerebroside β-galactosidase. Generalized central and peripheral demyelination is the hallmark of the disorder, as well as the presence of multinucleated macrophages (globoid cells) in cerebral white matter. Infantile arrest of motor and cognitive development is noted, with seizures, hypertonicity, optic atrophy and opisthotonic posturing. Stem cell transplantation may reverse the neurologic deficits by providing the missing enzyme.

  Gaucher’s disease is an autosomal recessive disorder resulting from β-glucosidase deficiency. It localizes to chromosome 1q21. There are three identified types. Type I presents with the characteristic findings of hematologic anomalies, hypersplenism, bone lesions, skin pigmentation and ocular pingueculae.

  Neurology

  B&D Chs 62&76

Question 49. B. In order to diagnose schizophrenia, active phase symptoms must be present for a 1 month period only. It may be diagnosed if other symptoms (i.e., negative) are present over a 6 month period, but at least one month of that 6 months must have contained active symptoms. All other answer choices are part of the characteristic active phase symptoms for schizophrenia. Social isolation is a bad prognostic sign for schizophrenia. Good prognostic signs include late age of onset, acute onset, and the presence of an affective component.

  Psychotic Disorders

  K&S Ch. 13

Question 50. D. Botulinum toxin type A is FDA-approved for the treatment of cervical dystonia, blepharospasm, hemifacial spasm, strabismus, and axillary hyperhidrosis, and cosmetically for hyperactive glabellar lines of the procerus muscles of the forehead. Although not FDA-approved for spasticity related to multiple sclerosis, it is an excellent treatment for that particular disorder. Botulinum toxin type A is also used off-label for the treatment of migraine. The mechanism of action of botulinum toxin type A is through blockade of neuromuscular transmission via blockade of presynaptic acetylcholine release. Botulinum toxin type A has no place in the treatment of restless legs syndrome.

  Neurology

  B&D Ch. 48

Question 51. D. This question is a clear description of a delusional disorder. In a delusional disorder, the patient usually presents with a nonbizarre delusion (i.e., something that could happen in real life). There are no other psychotic symptoms present. His ability to function in his daily life is preserved. There is not a significant mood component to the disease. The question says nothing about memory impairment, therefore dementia is not correct. The patient does not meet criteria for active phase schizophrenic symptoms, therefore schizophrenia and schizoaffective disorder are ruled out. Brief psychotic disorder lasts less than a month, and this patient has had symptoms for 5 months, so this answer choice is incorrect.

  Psychotic Disorders

  K&S Ch. 14

Question 52. A. The clinical presentation described in this question is that of classic tension-type or muscle contraction headache. These headaches can result from both muscular and emotional tension and stress. These headaches are not well understood and muscle tension may not fully explain their pathogenesis. The hypothalamic–mamillothalamic tract may be implicated in the generation of the pain, but studies have yet to investigate this thoroughly. Often treatment with rest, sleep, or simple analgesics is sufficient to bring relief. These headaches can begin at any age, are most often bilateral and are described as band-like around the head. Patients describe squeezing, pressure or burning types of pain. The pain can wax and wane throughout the day and can be present, if left untreated, from a period of hours to days. This headache type is usually free of any associated or autonomic symptoms, which helps to differentiate it from migraine. Treatment of tension-type headache usually begins with aspirin or acetaminophen. If headaches are more severe, combination drugs like acetaminophen-butalbital-caffeine (Fioricet) may be helpful, or non-steroidal anti-inflammatory agents such as naproxen or ibuprofen.

  Migraine is differentiated from tension-type headache mainly by its stereotyped features and associated autonomic symptoms. Migraine is generally unilateral, pulsatile and is accompanied by photophobia, phonophobia, or osmophobia. Other features can include nausea and vomiting, anorexia, blurred vision, and lightheadedness. The aura that can precede a migraine involves transient visual, motor, or focal neurologic deficits or symptoms. The scintillating scotoma is the most common visual manifestation of aura. The patient can perceive a shimmering arc or zigzag pattern of light in the peripheral visual field that can gradually enlarge. Sensory aura can involve transient numbness and tingling in the extremities that can last from seconds up to a half an hour’s duration. Motor aura is rare and can manifest as a transient motor weakness or hemiparesis, in much the same way as the sensory deficits. The patient may therefore misinterpret these symptoms as the onset of a stroke.

  Basilar migraine usually presents with occipital headache and neurologic symptoms attributable to the brainstem. The aura can involve visual and sensory phenomena, but these are often accompanied by vertigo, dysarthria, tinnitus and speech deficits. Loss of consciousness may occur if the reticular activating system of the pons is involved. The patient will often experience severe occipital pain after awakening from the aura. These attacks resemble stroke-like symptoms in the vertebrobasilar territory.

  Paroxysmal hemicrania is a special headache entity that falls into the category of indomethacin-responsive headache syndromes. This headache type begins early in life and is more common in women than in men (2:1 ratio). The hemicrania can be episodic or chronic. Pain is unilateral and brief, occurring generally in the periorbital or temporal areas. Attacks are frequent, usually five or more daily and each lasts about 20 minutes. Each attack is accompanied by autonomic features such as conjunctival injection, lacrimation, ptosis, rhinorrhea, ipsilateral to the side of the headache. Response to indomethacin 25–100 mg daily in divided doses is usually dramatic. The mechanism of indomethacin’s efficacy in this headache variety is not well understood.

  Neurology

  B&D Ch. 69

Question 53. E. In major depressive disorder libido is decreased. Increased libido is often found in mania.

  Depressive Disorders

  K&S Ch. 15

Question 54. B. The best answer to this question is the trigeminal nucleus caudalis (TNC). Many theories exist to try to explain the pathogenesis of migraine, and unfortunately there is no perfect unified theory. It is generally believed that abnormal intracranial and extracranial vascular reactivity accounts for the pulsatile nature of migraine by the abnormal dilatation of these vessels. It is believed that the aura of migraine is caused by a phenomenon called spreading oligemia or spreading depression. This refers to a phase of oligemia that spreads over the occipital cortex at a rate of about 2–3 mm/min and causes the scintillating scotoma noted during aura. This was described by Lashley in 1941, by his analysis of his own aura. Abnormal neuronal impulses from the trigeminal nucleus caudalis in the pons send messages along the trigeminal ophthalmic branches to nerve terminals that release substance P, neurokinin A and calcitonin gene-related peptide (CGRP). These polypeptides in turn activate a cascade of sterile neurogenic perivascular inflammation in the brain. Vessels then dilate and by vascular endothelial activation, there is an extravasation of plasma proteins that triggers an inflammatory response. Ergot alkaloid medications and triptan medication effectively block neuronal firing in the TNC and can abort an attack. The reticular activating system (RAS) refers to the ascending tracts in the pons that are responsible for maintenance of wakefulness. Bilateral pontine lesions affecting the RAS can result in impairment of consciousness. The suprachiasmatic nuclei (SCN) in the hypothalamus are thought to be the generator of the human biological clock that regulates circadian rhythms. Melatonin is the neurohormone produced by the pineal gland that acts at melatonin receptor sites in the SCN by a feedback loop. The SCN has no known role in migraine. Muscular contraction of the scalp and cranial musculature is not believed to be associated with migraine, but may play a role in the manifestation of tension-type headache.

  Neurology

  B&D Chs 61&69

Question 55. C. This is a case of seasonal affective disorder, which is a depression that sets in during the fall and winter and resolves during the spring and summer. It is often characterized by hypersomnia, hyperphagia, and psychomotor slowing. Treatment involves exposure to bright artificial light for 2–6 hours each day during the fall and winter months. It is thought to be related to abnormal melatonin metabolism. All other answer choices given are distracters and are unrelated to the patient’s primary problem.

  Depressive Disorders

  K&S Ch. 15

Question 56. C. Carbidopa serves a simple function: it inhibits dopa decarboxylase and thereby prevents the peripheral metabolism of levodopa to dopamine before it can cross the blood–brain barrier. The dopamine agonists such as ropinirole, pramipexole, pergolide and bromocriptine are direct postsynaptic dopamine receptor agonists indicated for Parkinson’s disease treatment. Selegiline is the selective monamine oxidase type B (MAO-B) inhibitor that blocks dopamine degradation by MAO-B and can potentiate the action of levodopa in the central nervous system. Catechol-O-methyltransferase (COMT) inhibitors such as tolcapone and entacapone prevent peripheral degradation of levodopa and central metabolism of levodopa and dopamine, thereby increasing central levodopa and dopamine levels. COMT inhibitors are usually taken simultaneously with doses of levodopa-carbidopa. Anticholinergic agents such as trihexyphenidyl and benztropine can reduce tremor in Parkinson’s disease by blockade of post-synaptic muscarinic receptors. These agents carry the danger of toxic side effects that include confusion, blurred vision, urinary retention, constipation and dry mouth.

  Neurology

  B&D Ch. 71

Question 57. E. This question includes common symptoms found in a panic attack. Others include trembling, choking sensations, dizziness, fear of losing control, fear of death, paresthesias, chills, or hot flushes. The patient in question does not present with the characteristic signs and symptoms of a manic episode. Myxedema madness is a depressed and psychotic state found in some patients with hypothyroidism. Mad Hatter’s syndrome presents as manic symptoms resulting from chronic mercury intoxication. The patient describes no psychotic symptoms, so psychotic disorder NOS is clearly the wrong choice.

  Anxiety Disorders

  K&S Ch. 16

Question 58. E. Apoptosis refers to programmed neuronal cell death. This concept is believed to account for the pathophysiology of several neurodegenerative diseases. These diseases include spinal muscular atrophy, Parkinson’s disease, amyotrophic lateral sclerosis, and Alzheimer’s dementia.

  Neurology

  B&D Ch. 60

Question 59. B. Amoxapine and maprotiline are tetracyclic, not tricyclic antidepressants. Amoxapine has significant dopamine blocking activity, and can produce side effects similar to those found with many antipsychotics, such as tardive dyskinesia and dystonia. Maprotiline is one of the most selective inhibitors of norepinephrine reuptake. It has mild sedative and anticholinergic side effects. Notably there is an increased incidence of seizure associated with its use. It has a long half life of 43 hours.

  Psychopharmacology

  K&S Ch. 36

Question 60. B. Sturge–Weber syndrome is a neurocutaneous disorder that is sporadic and not genetically inherited. The hallmark is the presence of a facial cutaneous angioma (port-wine nevus) usually with a brain angioma ipsilateral to the skin lesion. Other characteristics can include contralateral hemiparesis, mental retardation and homonymous hemianopia. Glaucoma is common and if untreated can lead to blindness. Seizures develop in over 70% of Sturge–Weber patients and present most often as motor seizures or generalized tonic–clonic seizures. Treatment of seizures is achieved with anticonvulsant medication, which if proven ineffective, may lead to the consideration of surgical intervention such as hemispherectomy to excise the offending brain tissue.

  Von Hippel–Lindau syndrome (VHL) is a heritable neurocutaneous disorder that is transmitted by autosomal dominant pattern of inheritance. The VHL gene is a tumor-suppressor gene on chromosome 3. Prevalence is about 1 in 40 000 to 100 000. The predominant clinical presentation is characterized by retinal and CNS hemangioblastomas and visceral cysts and tumors. Hemangioblastomas are slow- growing vascular tumors that are benign but can bleed and cause local mass effect. The most common CNS sight of this tumor type is the cerebellum in about 50% of cases. Renal cysts occur in about half of patients with VHL. Pheochromocytoma occurs in about 10–20% of patients. Frequent follow-up and screening for tumors is the management of choice for patients with VHL.

  Ataxia–telangiectasia is a neurodegenerative neurocutaneous disorder that is inherited by autosomal recessive pattern of inheritance. Prevalence is about 1 in 40 000 to 100 000. The hallmark of the disorder is the development of early-onset ataxia in childhood. Ataxia tends to develop at around 12 months of age, when the child begins to walk. Children typically are wheelchair-bound by 12 years of age. Telangiectases (small dilated blood vessels) tend to develop later, from about age 3–6 years, and often affect the earlobes, nose and sclerae. There is also a higher risk of lymphoma and leukemia in patients with the disorder than in the general population at large. Abnormal eye movements are common in children with the disorder. These include nystagmus, ocular motility impairment and gaze apraxia.

  Fabry’s disease is an X-linked lysosomal storage disease resulting from deficiency in α-galactosidase A. Stigmata of the disease include the development of asymptomatic red or purple papules that occur around the umbilicus, hips, thighs and scrotum area. Other characteristics include corneal deposits, painful dysesthesias of the distal extremities, cerebral thrombosis or hemorrhage, and eventual vascular narrowing from deposition of glycolipids in the arterial endothelium. Renal failure is a common cause of death due to renal vascular compromise. Treatments are disappointing in Fabry’s disease. Enzyme repletion does not help the clinical problems. Plasmapheresis is also not particularly effective. Renal transplantation can delay systemic complications and alleviate renal failure, but is not curative.

  Tuberous sclerosis is discussed elsewhere in this volume in great detail.

  Neurology

  B&D Ch. 65

Question 61. A. The doctor who takes Medicare must accept Medicare’s maximum fee. This fee includes any appropriate copays set forth in the patient’s policy. The physician can not bill the patient or another party for the difference between what Medicare allows and what you want to charge.

  Public Policy

  K&S Ch. 4

Question 62. E. Reflex sympathetic dystrophy is one of the so-called complex regional pain syndromes. It is the result of regional pain and sensory changes following trauma or a noxious event. There may or may not be a diagnosable underlying nerve lesion to the problem. Soft tissue injury is the trigger in about 40% of cases and bony fracture in about 25% of cases.

  There are three stages to the disorder. Stage one (acute) is associated with pain that is out of proportion to the initial injury. There is usually hypersensitivity to painful stimuli or physical contact with the extremity. Stage two (dystrophic) is associated with tissue edema and skin that is cool, cyanotic, hyperhidrotic, with livedo reticularis. Pain is constant and increases with any physical contact to the affected extremity. Stage three (atrophic) is associated with paroxysmal pain and irreversible tissue damage. The skin can become thin and shiny and the fascia can become thickened or contractured. Treatments begin with intense physical therapy which can be helpful by improving mobility of the affected extremity. Steroids may be helpful in certain cases. Phenoxybenzamine, which is a sympathetic blocking agent, can also be useful, if tolerated. Some patients require invasive anesthesia by regional block, which can be helpful in certain patients.

  Neurology

  B&D Ch. 29

Question 63. B. In the situation where you are presented with an unknown psychotic or paranoid patient, the first step is to make sure that patient and staff are safe. All other options are valid, but would be taken after an initial assessment of the patient’s safety has been made. When given a question such as this on an examination, maintenance of safety comes first, and diagnosis and treatment come later. You would not automatically give sedation to the patient if they were not agitated or dangerous, so answer choice A is not the correct first move. Remember that past violence is the best predictor of future violence.

  Management in Psychiatry

  K&S Ch. 33

Question 64. B. Carbamazepine is an inducer of cytochrome P450 2C19 and 3A4. It can therefore induce the metabolism of any substrate of the 2C19 and 3A4 systems. The 3A4 substrates that can be induced by carbamazepine include erythromycin, clarithromycin, alprazolam, diazepam, midazolam, cyclosporine, indinavir, ritonavir, saquinavir, diltiazem, nifedipine, amlodipine, verapamil, atorvastatin, simvastatin, aripiprazole, buspirone, haloperidol, tamoxifen, trazodone, propranolol, zolpidem, zaleplon, methadone, estradiol (oral contraceptives), progesterone, testosterone, and fentanyl, to name but a few. The 2C19 substrates that can be induced by carbamazepine include amitriptyline, citalopram, clomipramine, imipramine, R-warfarin, propranolol, and primidone. Carbamazepine can lower lamotrigine levels by induction of glucuronidation enzyme 1A4. Clozapine is a substrate of cytochrome P450 1A2 and is not affected by carbamazepine.

  Neurology

  Psychopharmacology

  B&D Chs 8,52&67

Question 65. E. The standard of substituted judgment holds that a surrogate decision maker will make decisions based on what the patient would have wanted, and implies that the decision maker be familiar with the patient’s values and attitudes. The best interest principle, which was the past, but not current standard, states that a decision maker will decide which option would be in the patient’s best interests. Patients do have the right to refuse treatment that they feel would lessen their quality of life. Advanced directives and the living will are ways for patients to preserve their wishes in writing such that the correct decisions are made for them should they become incapacitated. The state will follow the course that preserves human life should a suitable surrogate decision maker not be present. Surrogate decision makers can be appointed by the patient, courts, or the hospital. In many cases this person is the patient’s next of kin.

  Forensic Psychiatry

  K&S Ch. 56

Question 66. D. Tobacco smoking is a potent inducer of cytochrome P450 1A2. As such it can significantly lower levels of amitriptyline, fluvoxamine, clozapine, olanzapine, haloperidol, and imipramine. Risperidone is a substrate of cytochrome P450 2D6 and its levels can be lowered by 2D6 inducers such as dexamethasone and rifampin. Risperidone levels can be significantly increased by 2D6 inhibitors such as bupropion, citalopram, clomipramine, doxepin, duloxetine, escitalopram, fluoxetine, paroxetine, sertraline, and perphenazine. Risperidone levels are not affected by tobacco smoking.

  Psychopharmacology

  B&D Ch. 9

Question 67. E. One cannot treat what is assumed to be denial before actually knowing that it is denial. Often in medical settings things are not clearly explained to patients in language they can understand. As such it is first necessary to explain what is going on to the patient clearly and in language they can follow. Confronting the patient’s denial forcefully, and aggressively removing his defenses against overwhelming emotion, is potentially more harmful than helpful. Playing along with the denial can lead to noncompliance and failed treatment outcomes and is a bad idea. Neuropsychological testing is not necessary if a patient is in denial. If he had a neurocognitive deficit that precluded his understanding of the material presented to him it could be considered, but those were not the circumstances described in this question. Meeting the family is important, but not more important than making sure the patient has had the situation explained properly. The patient is the primary person who has to understand. Informing the family while the patient is left in the dark is not the best approach.

  Psychological Theory and Psychometric Testing

  K&S Ch. 28

Question 68. A. This is a classic case of Sheehan’s syndrome, which is essentially a postpartum pituitary infarction or apoplexy. The classic symptoms are those similar to a pituitary hemorrhage. The pituitary infarction can cause chiasmal compression that can lead to bitemporal hemianopsia from bilateral medial optic nerve compression. Hypotension can occur due to pre-existing adrenocorticotropic hormone deficiency. The clinical picture can resemble a subarachnoid hemorrhage due to the rupture of a berry aneurysm or an arteriovenous malformation. This would account for the severe acute headache and cranial nerve palsy. The syndrome can also result in meningeal irritation with positive Kernig’s and Brudzinski’s signs and a stiff neck. The diagnosis can often be confirmed by a CT or MRI scan of the brain. Treatment is generally supportive and only in certain cases is it necessary to provide corticosteroid replacement or conduct a surgical decompression.

  Cushing’s disease is caused by endogenous overproduction of adrenocorticotropic hormone from the anterior pituitary gland. Cushing’s syndrome is the result of exposure to either excessive exogenous or endogenous corticosteroids. The clinical picture is that of hypertension, truncal obesity, impaired glucose tolerance or diabetes mellitus, menstrual irregularities, hirsutism, acne, purplish abdominal striae, osteoporosis, thin skin with excessive bruising, and proximal myopathy. The diagnostic test of choice is the dexamethasone suppression test.

  Subarachnoid hemorrhage is most often the result of an intracranial arterial aneurysmal rupture. The acute presentation very often consists of a sudden explosive headache (“the worst headache of my life”), meningeal signs, nausea, vomiting, photophobia, and obtundation. Head CT scan is the imaging test of choice as it reveals acute blood better than MRI. CT scans can only reveal subarachnoid blood in about 90–95% of patients within 24 hours of the hemorrhage. This sensitivity drops to about 80% after 72 hours. A negative CT scan should therefore be followed by a lumbar puncture with centrifuging of CSF looking for characteristic xanthochromia (from lysed red blood cells). If either CT or lumbar puncture is positive for subarachnoid hemorrhage, then cerebral angiography should be performed as soon as possible to determine the location of the aneurysm and best method of intervening. The most popular interventions for intracranial aneurysms are endovascular coiling, microsurgical clipping, and balloon embolization. Saccular or berry aneurysms are the most common kind of intracranial aneurysms. Between 80 and 85% of aneurysms stem from the anterior cerebral circulation, most often arising from the anterior communicating artery, posterior communicating artery, or the trifurcation of the middle cerebral artery. Between 15 and 20% of aneurysms originate in the posterior circulation, most often from the origin of the posterior inferior cerebellar artery or at the bifurcation of the basilar artery.

  Bacterial meningitis presents in adults as a febrile illness involving headache, stiff neck, and signs of cerebral dysfunction, which are present in over 85% of patients. Associated signs and symptoms include nausea, vomiting, photophobia, and myalgia. Often Kernig’s and Brudzinski’s signs can be elicited. Cerebral dysfunction can involve delirium, confusion, and decreased level of consciousness that ranges from lethargy to coma. Seizures can occur in about 40% of cases. Cranial nerve palsies can be seen in 10–20% of patients. Increased intracranial pressure may result in the appearance of bilateral VI nerve palsies. Lumbar puncture and blood cultures clinch the diagnosis. Cerebrospinal fluid (CSF) opening pressure is high (200–500 mmH2O) and protein is also high (100–500 mg/dL, normal = 15–45 mg/dL), with decreased glucose and marked pleocytosis (100–10 000 WBC/mL, normal = <5), with 60% or more polymorphonuclear leukocytes. CSF cultures are positive in about 75% of cases. In the United States, the predominant causative organism in children ages 2 to 18 years is N. meningitidis and in adults, S. pneumoniae. Intravenous ampicillin, penicillin-G and third-generation cephalosporins (ceftriaxone, cefotaxime, ceftazidime) are the usual agents of first-line treatment.

  Familial hemiplegic migraine (FHM) is a rare autosomal dominant migraine subtype in which the aura is accompanied by hemiplegia. FHM has been mapped to chromosome 19p13 that codes for the α1-subunit of brain-specific voltage-gated P/Q-type calcium channel. There is some notion that because FHM has similar symptoms to traditional migraine with aura that this implies that migraine with aura may also be genetically linked to chromosome 19.

  Neurology

  B&D Chs 42&53

Question 69. C. The question stem accurately describes agoraphobia. The other answer choices are distracters. Agonothete is the judge of games in ancient Greece. Agoniada is the bark of a South American shrub. Agora is the market place in ancient Greece. Agouara is a South American wild dog or a crab-eating raccoon. Needless to say, the only one that will show up on the boards is agoraphobia.

  Anxiety Disorders

  K&S Ch. 16

Question 70. C. Normal-pressure hydrocephalus (NPH) presents with the triad of dementia, incontinence, and gait disturbance. The cause, in up to one-third of cases, is undetermined. NPH can be caused by trauma, infection, or subarachnoid hemorrhage. Brain CT scan or MRI reveal enlargement of the third, fourth, and lateral ventricles. NPH causes an apraxic, or magnetic gait, making it difficult for the patient to raise the legs off the ground. The dementia is considered to be subcortical, and results in slowing of verbal and motor functioning while the cortical functions remain intact. Abulia, apathy and depression are common in NPH. Urinary incontinence occurs early in the course of the illness, particularly when there is prominent gait disturbance. Lumbar puncture reveals a normal opening pressure. Serial lumbar punctures with drainage of cerebrospinal fluid can improve the symptoms and support the diagnosis of NPH. Ventriculoperitoneal shunting is the treatment of choice and is successful in up to 80% of cases. Shunts fail about one-third of the time and complications of shunting include subdural hematoma and infection.

  Diffuse Lewy-body disease, or dementia with Lewy bodies is the second-most prevalent dementia after Alzheimer’s disease. The symptomatic triad is that of dementia, parkinsonism, and visual hallucinations. A hallmark of the disease is extreme sensitivity to dopamine receptor antagonists which can result in severe parkinsonism when treatment with neuroleptics is undertaken. Dopaminergic therapy is generally not that helpful. Hallucinations are ideally treated with the newer antipsychotics, particularly quetiapine, clozapine, and ziprasidone. Rivastigmine and donepezil may be useful cholinergic therapy to try to improve cognition.

  Wernicke’s encephalopathy presents with the acute triad of mental confusion, ophthalmoplegia and gait ataxia, predominantly in alcoholic patients. On autopsy, the neuropathologic hallmark is multiple small hemorrhages in the periventricular gray matter, mainly around the aqueduct and the third and fourth ventricles. MRI of the brain often reveals abnormalities in the periaqueductal regions, bilateral mamillary bodies, and medial thalami.

  Sydenham’s chorea (SC) is a result of rheumatic fever. It is extremely rare these days because of the widespread availability of antistreptococcal therapy. The disorder is more frequent in girls, generally between 5 and 15 years of age. Chorea begins insidiously over a period of weeks and can last up to 6 months. Behavioral manifestations can include irritability, obsessive–compulsive traits and restlessness. Enlargement of the basal ganglia can be noted on MRI. Symptoms are generally self-limited, lasting up to about 6 months. Antibasal ganglia antibodies can be detected by Western blot testing and these antibodies seem to account for the mechanism of the disorder. Valproic acid is a useful therapy for the chorea.

  Neurology

  B&D Chs 66&71

Question 71. E. Cyclothymic disorder does not involve psychotic symptoms, although these symptoms may be found in bipolar disorder. Cyclothymia is a less severe form of bipolar with alternation between hypomania and moderate depression. Symptoms must exist for two years to make the diagnosis. It is equally common in men and women. Substance use often coexists. The onset is usually insidious and occurs in late adolescence or early adulthood.

  Bipolar Disorders

  K&S Ch. 15

Question 72. B. Depression is not considered a stroke risk factor. Common risk factors for stroke include older age, male gender, low socioeconomic status, diabetes mellitus, obesity, cigarette smoking, excessive alcohol consumption, and family history. Other important risk factors are arterial hypertension, prior stroke or transient ischemic attack, asymptomatic carotid bruit, dyslipidemia, hyperhomocysteinemia and oral contraceptive use. Hereditary blood dyscrasias also elevate stroke risk, such as protein C or S deficiency, antithrombin III deficiency and factor V Leiden deficiency.

  Neurology

  B&D Ch. 51

Question 73. D. This question gives a classic description of the appearance of a patient in the manic phase of bipolar disorder. They are often very bizarre, colorful, seductive and erratically behaved. A depressed patient would be apathetic and psychomotor retarded, not hyperexcited. There was no mention made in the question of psychosis or delusions so brief psychotic disorder and delusional disorder are incorrect. There is also no mention of abnormally perceived body image so body dysmorphic disorder is incorrect as well. There is an equal prevalence of bipolar disorder in women versus men. Bipolar I disorder in women most often starts with depression.

  Bipolar Disorders

  K&S Ch. 15

Question 74. A. Ataxia is indicative of a cerebellar lesion and the cerebellum is perfused by the vertebrobasilar arterial system. Symptoms indicative of a carotid territory transient ischemic attack or stroke would be: transient ipsilateral monocular blindness (amaurosis fugax), contralateral body weakness or sensory loss, aphasia with dominant hemisphere involvement, contralateral homonymous visual field deficits. Symptoms that suggest a vertebrobasilar territory stroke or transient ischemic attack include: bilateral, shifting, or crossed weakness or sensory loss (ipsilateral face with contralateral body), bilateral or contralateral homonymous visual field defects or binocular visual loss, two or more of vertigo, diplopia, dysphagia, dysarthria and ataxia.

  Neurology

  B&D Ch. 51

Question 75. E. Suicidal ideation is part of the thought content of the depressed patient. It is possible to find it in the manic bipolar patient, but is more likely during the depressed phase of the illness and is not part of the thought process.

  Diagnostic and Treatment Procedures in Psychiatry

  K&S Ch. 15

Question 76. D. Essential tremor (ET) is one of the most common movement disorders. It has been noted in up to 5% of patients over 60 years of age. It is defined as a postural and kinetic tremor of the forearms and hands (sometimes with other body parts) that gradually increases in amplitude over time. ET is believed to be a monosymptomatic illness, without other neurologic deficits. As many as two-thirds of patients have a positive family history of ET. The disease is likely heterogeneous with an autosomal dominant pattern of inheritance.

  The mainstay of treatment is the use of β-adrenergic blocking agents and primidone. Propranolol is the β-blocker of choice and can be given in doses of 120–320 mg daily in divided doses. This helps reduce tremor amplitude in roughly half of patients. Primidone can be given as a single nighttime dose of 25 mg and subsequently titrated to a therapeutic dose of 50–350 mg daily. Benzodiazepine sedative–hypnotic agents, like clonazepam and lorazepam are also frequently used and can be helpful. Botulinum toxin type A can be injected intramuscularly for head and hand tremor and can be effective in certain cases. Desipramine and the tricyclic antidepressants are not useful in ET and may in fact worsen tremor. If oral therapy fails, surgical intervention can be contemplated if symptoms are serious. Stereotactic thalamotomy has been shown to reduce contralateral tremor by 75% in as many as 90% of cases. Thalamic deep brain stimulation has also been demonstrated to be effective in treating ET and can improve symptoms in up to 80% of cases.

  Neurology

  B&D Ch. 71

Question 77. B. To qualify for a diagnosis of major depressive disorder, symptoms must be present for at least two weeks. Symptoms can include depressed mood, diminished interest in activities, weight loss or gain, insomnia or hypersomnia, psychomotor retardation, fatigue, feelings of worthlessness, decreased concentration, recurrent thoughts of death, and recurrent suicidal ideation.

  Depressive Disorders

  K&S Ch. 15

Question 78. B. The thymus gland is believed to play a role in the pathogenesis of myasthenia gravis (MG). MG is an autoimmune disorder and the thymus gland is involved in tolerance to self-antigens. Ten per cent of patients with MG have a thymic tumor and 70% have cellular hyperplasia of the thymus indicative of an active immune response. About 20% of patients with MG who develop symptoms between ages 30 and 60 years have a thymoma. The thymus contains myoid cells that express the AChR (acetylcholine receptor) antigen, antigen presenting cells and immunocompetent T cells. The thymus is believed to produce AChR subunits that act as autoantigens in the sensitization of the patient against the AChR. Most thymomas in MG patients are benign and are amenable to easy surgical resection which may in certain cases improve symptomatology. The other tumor types mentioned above are not typically associated with MG.

  Neurology

  B&D Ch. 78

Question 79. B. Prevalence for schizophrenia is higher in urban than in rural areas, as are morbidity and severity of presentation. The lifetime prevalence is about 1%. The male to female ratio is 1:1. Onset is usually between 15 and 35 years, with onset before 10 years and after 40 years being rare. There is a higher incidence of cases in babies born in the winter and early spring.

  Psychotic Disorders

  K&S Ch. 13

Question 80. A. Friedrich’s ataxia (FA) is an autosomal recessive disease that localizes to chromosome 9q13-21.1. It is the result of an unstable expansion of a trinucleotide repeat (GAA). Onset is typically noted in adolescence with gait ataxia, loss of lower extremity proprioception, and absence of deep tendon reflexes, more often in the lower extremities. There is also predominant central nervous system involvement with noted dysarthria, presence of Babinski signs and eye movement anomalies. The natural progression of the disease is towards a complete loss of ambulation ability and ultimately death due to hypertrophic cardiomyopathy in about 50% of cases. Death occurs on average by late in the fourth decade.

  Myotonic dystrophy (MD) type I is an autosomal dominantly inherited disease with trinucleotide repeat expansion of CTG that codes to chromosome 19q13.3. The incidence is 1 in 8000 live births. Classic symptoms and signs include ptosis, bifacial weakness, frontal baldness, triangular drooping facies. Motor weakness is greater distally than proximally. Myotonia is a classic sign, which is the inability to relax a contracted muscle or group of muscles. For example, a patient is unable to let go after shaking hands. Percussion myotonia can also be demonstrated, particularly in the thenar and hypothenar hand muscles. This is involuntary contraction of muscles after percussion with a reflex hammer. Fibrotic or infiltrative cardiomyopathy is a frequent associated problem in myotonic dystrophy. DNA testing by serum polymerase chain reaction is the diagnostic modality of choice for MD. Electromyography reveals myopathic features and myotonic discharges (“dive-bomber” sound after muscular relaxation). Perifascicular muscle fiber atrophy is the classic histopathologic finding on muscle biopsy. Please note that the other three answer choices are explained in questions elsewhere in this volume and are all diseases inherited by autosomal dominant transmission.

  Neurology

  B&D Chs 72&79

Question 81. D. Disinhibited behavior is more characteristic of mania than it is of depression. All of the other symptoms are somatic or sleep complaints that are frequently associated with depression. Patients with depression have disrupted REM sleep, including shortened REM latency, increased percentage of REM sleep, and a shift in REM distribution from the last half to the first half of the night. Acetylcholine is associated with the production of REM sleep.

  Depressive Disorders

  K&S Ch. 15

Question 82. E. It is believed that migraine maps to several regions on chromosome 19, because of the entity FHM (familial hemiplegic migraine) that has been associated with this gene locus. FHM is thought to be a channelopathy involving a brain-specific calcium channel α-1 subunit gene that has been mapped to chromosome 19p13. Given the similarity between FHM and typical migraine, researchers believe that chromosome 19 may well be the locus that links both disorders.

  Neurology

  B&D Ch. 69

Question 83. A. Obsessions are part of thought content, as are delusions, ideas of reference, phobias, suicidal or homicidal thought, depersonalization, derealization, and neologisms, to name a few. Thought process would include word salad, flight of ideas, circumstantiality, tangentiality, clang associations, perseveration, and goal directed ideas.

  Diagnostic and Treatment Procedures in Psychiatry

  K&S Ch. 7

Question 84. B. CNS cysticercosis involves parenchymal invasion of the brain by the larval stage pork tapeworm Taenia solium. Cysticercosis occurs by ingestion of undercooked pork containing cysticerci. The infection occurs most frequently in Central and South America where poor hygiene and sanitation lead to unsanitary conditions. After ingestion of tainted pork, the tapeworm eggs hatch in the gastric tract, develop to larval stage and eventually penetrate the bowel to migrate to host tissue, most often in the CNS. Clinical presentation ranges from epilepsy, focal neurological deficits, hydrocephalus, cognitive decline, meningitis, or myelopathy. Diagnosis is confirmed by brain MRI demonstrating the parasites in the brain and from confirmatory serology and/or CSF enzyme-linked immunosorbent assay (ELISA) tests. ELISA on CSF is more sensitive and specific than serologic tests. Treatment is with albendazole or praziquantel oral therapy, coupled with parenteral steroids. Trichinella spiralis infection causes trichinosis which is also caused by ingestion of poorly cooked pork or other game meats in endemic areas. Echinococcus granulosus are the larval tapeworms that cause CNS echinococcosis. Humans generally acquire echinococci through contact with dogs that are infected. E. granulosus tends to cause solitary CNS spherical cysts without edema. Treatment can involve surgical resection of the cyst or conservative medical management with albendazole if the cysts are not resectable. Leishmania major is a protozoan that causes leishmaniasis. The vector is a sandfly bite. Visceral disease can be accompanied by inflammatory neuropathies that resemble Guillain–Barré syndrome. Treatment is by parenteral administration of antimony or amphotericin B. Toxoplasma gondii is the intracellular protozoan responsible for toxoplasmosis infection. Vectors include migratory birds and cats and so the infection can be found worldwide. Toxoplasmosis rarely occurs in immunocompetent patients, but is more often seen in AIDS patients with low CD4 counts. CNS lesions present as ring enhancing on both CT and MRI. CSF titers may contain T. gondii DNA detectable by PCR. Treatment is usually a combined regimen of pyrimethamine and sulfadiazine with folinic acid. Ventricular shunting may be required if lesions are space-occupying and cause hydrocephalus.

  Neurology

  B&D Ch. 53

Question 85. C. The effects of alcohol can be seen in several lab tests. The GGT will be elevated in 80% of alcoholic patients, while MCV is increased in 60% of alcoholic patients. Uric acid, triglycerides, AST and ALT can also be elevated. The other lab values given in the question are unrelated to alcohol abuse.

  Laboratory Tests in Psychiatry

  K&S Ch. 12

Question 86. C. Alcoholic neuropathy is the most common neurologic manifestation of chronic alcoholism. Up to 75% of alcoholic patients are diagnosed with this disorder. Most patients are chronic alcohol abusers between 40 and 60 years of age. This type of neuropathy is a mixed motor and sensory disorder. Symptoms usually begin gradually, symmetrically in the feet.

  Alcoholic dementia refers to older patients with a lifelong history of heavy alcohol use, who experience an insidious decline in their cognitive functioning. Nearly 20% of older alcoholics have some form of dementia, but this is complicated by the presence of other comorbidities such as liver abnormalities, head trauma, malnutrition and stroke. Alcoholic dementia results in more of a predominance of fine motor control and verbal fluency deficits than is seen in patients with dementia of the Alzheimer type.

  Marchiafava–Bignami disease is a rare demyelinating disease preferentially affecting the corpus callosum in chronic alcoholics. The exact etiology of the disorder is not well-understood. It may be related to nutritional factors or direct toxic effects of alcohol on the cerebral white matter, but this is unclear. The most common neurologic manifestation is that of frontal lobe damage and dementia noted on neurologic examination. The central portion of the corpus callosum is more often affected than the anterior or posterior portions. Treatment is directed at nutritional support and alcoholic rehabilitation.

  Alcoholic-nutritional cerebellar degeneration is seen more often in men than in women. The disorder presents in longstanding alcoholics as unsteadiness in walking evolving over weeks to months. The most common presentation on examination is truncal ataxia, with a wide-based gait and difficulty with tandem walking. Pathologically, the disorder results from preferential atrophy of the superior and anterior cerebellar vermis, with lesser involvement of the cerebellar hemispheres. Wernicke’s encephalopathy is explained extensively elsewhere in this volume.

  Neurology

  B&D Chs 57&76

Question 87. B. A cohort study is when a group from a well-known population is chosen and followed over a long period of time. These studies give us estimates of risk based on suspected causative factors for a given disease. Case history studies look back on people with a given disease. Cross-sectional studies give information on prevalence of a disease in a population at a given point in time. Retrospective studies are based on past data, as opposed to prospective studies which are based on observing things as they occur.

  Statistics

  K&S Ch. 4

Question 88. C. The Miller–Fisher syndrome is a variant of Guillain–Barré syndrome (GBS, acute inflammatory demyelinating polyneuropathy). The classic triad of symptoms is gait ataxia, areflexia and ophthalmoplegia. This accounts for about 5% of GBS cases. Motor strength is usually intact. Serum IgG antibodies to the ganglioside GQ1b can be detected in serum early in the course of the Miller–Fisher variant of GBS. F-wave latencies may be intact on EMG with reduced or absent SNAP amplitudes. CSF protein is elevated without pleocytosis about one week into the course of the illness. Dementia, parkinsonism and psychosis make up the triad of dementia with Lewy bodies. The clinical picture is that of dementia, extrapyramidal symptoms, fluctuations and visual hallucinations. Visual hallucinations occur in as many as 80% of cases. Neuroleptic sensitivity is another diagnostic characteristic. The classic pathologic finding is the Lewy bodies which are diffuse throughout the cortex and are eosinophilic inclusions with a core halo on hematoxylin and eosin stain. Treatment with newer atypical antipsychotics, particularly clozapine, is usually best and cholinesterase inhibitors such as donepezil may also be helpful. The triad of gait ataxia, urinary incontinence and dementia is classic for normal pressure hydrocephalus.

  Neurology

  B&D Ch. 76

Question 89. C. Konrad Lorenz is known best for his work with imprinting. Imprinting is the phenomenon whereby during a critical period of early development a young animal will attach to their parent, or whatever surrogate is in the parent’s place. From then on the presence of that parent or surrogate will elicit a specific behavior pattern even when the animal is much older. In Lorenz’s case newborn goslings imprinted on him instead of their mother and followed him around as if he were their mother. This has correlates in psychiatry because it is evidence of the link between early experiences and later behaviors. Lorenz also studied aggression in animals and worked on the need for aggression in humans given the pressures of natural selection. All the other answer choices in this question are distracters which have nothing to do with the work of Lorenz.

  Human Development

  K&S Ch. 4

Question 90. B. This case depicts a classic case of acute inflammatory demyelinating polyneuropathy (Guillain–Barré syndrome or GBS). The annual incidence is 1.8 in 100 000. About 65% of patients report a prior “insult” before the onset of symptoms. This often takes the form of a gastrointestinal infection, an upper respiratory infection, surgery, or immunization a few weeks before symptoms appear. The most commonly identified organism responsible for prodromal infection is Campylobacter jejuni. C. jejuni can be detected in stool cultures and serologic studies. The clinical presentation of GBS can vary from case to case. Typically patients present with symmetrical lower extremity weakness, with paresthesias and possibly with sensory symptoms. The paralysis is usually an ascending paralysis and the most worrisome outcome is paralysis of the muscles of respiration which can prove fatal in some cases. Deep tendon reflexes are usually greatly diminished or absent. Diagnostic testing reveals the classic nerve conduction abnormalities of conduction block and prolonged F-wave latencies, which are pathognomonic of GBS. CSF studies reveal cytoalbuminergic dissociation: elevated protein with an acellular fluid. In certain cases, nerve biopsy can reveal complement fixing antibodies to peripheral nerve myelin. Treatment should take place in the hospital and in an intensive care unit setting if respiratory compromise is imminent. Two treatments of choice are high dose intravenous immunoglobulin administration and plasmapheresis. These have been proven to be equally efficacious in numerous recent studies.

  Neurology

  B&D Ch. 76

Question 91. A. Second messengers are molecules that work within the cell to carry on the message delivered by the neurotransmitter on the cell surface. IP3, cGMP, Ca2+, cAMP, DAG, NO, and CO are all common second messenger molecules. Adenylyl cyclase is not a second messenger itself, but rather is the enzyme that makes cAMP from ATP. Adenylyl cyclase is turned on or off by G proteins depending on the need for cAMP. Binding cAMP to transcription factors regulates gene transcription, including the machinery to make certain neurotransmitters. Calcium plays a number of roles within the cell, and excess Ca2+ is linked to production of NO and cell death through excitotoxicity. One of the major functions of IP3 is to cause the release of intracellular Ca2+ from the endoplasmic reticulum.

  Basic Neuroscience

  K&S Ch. 3

Question 92. A. The cortical dementias such as Alzheimer’s disease generally produce a gradual decline in cognitive function, with normal speed of cognition and the presence of aphasia, dyspraxia, and agnosia. Depression is less common in cortical dementia than in subcortical disease. Motor abnormalities are typically absent in cortical dementia, unless the disease is in the terminal stages. Subcortical dementia, as exemplified in Parkinson’s disease, typically presents with dysarthria and extrapyramidal motor abnormalities. Apathy and depression are often present. Frontal memory impairment, with recall aided by cues, is often noted. Speed of cognition in subcortical dementia is slow.

  Neurocognitive Disorders

  B&D Ch. 66

Question 93. B. This question focuses on Piaget’s stages of cognitive development. In the sensorimotor stage children respond to stimuli in the environment, and learn during this process. They eventually develop object permanence (objects exist independent of the child’s awareness of their existence), and begin to understand symbols (as in the use of words to express thoughts).

  During the preoperational stage there is a sense that punishment for bad deeds is unavoidable (immanent justice). There is a sense of egocentrism, and phenomenalistic causality (the thought that events that occur together cause one another). Phenomenalistic causality is the subject of this question. Animistic thinking (inanimate objects are given thoughts and feelings) is also seen.

  In the concrete operations stage, egocentric thought changes to operational thought where another’s point of view can be taken into consideration. In concrete operations children can put things in order and group objects according to common characteristics. They develop the understanding of conservation (a tall cup and a wide cup can both hold equal volumes of water) and reversibility (ice can change to water and back to ice again).

  During formal operations children can think abstractly, reason deductively, and define abstract concepts. Latency is a distracter. It occurs in Freud’s model after the genital phase and before puberty.

  Human Development

  K&S Ch. 2

Question 94. C. T4 is the dermatome at the level of the nipples. T10 is the dermatome at the level of the umbilicus. Other important dermatomes to remember include: C2 – back of head, C4 – above collar bone, C6 – thumb, C7 – middle fingers, C8 – little finger, L1 – groin, L2 – lateral thigh, L3 – medial thigh, L4 – medial leg, L5 – lateral leg, big toe, S1 – little toe, sole of foot, S5 – perianal area.

  Neurology

  B&D Ch. 24

Question 95. D. The three receptor types associated with glutamate are AMPA, kainate, and NMDA. Acetylcholine is associated with the nicotinic and muscarinic receptors. Norepinephrine is associated with the alpha 1, alpha 2, and beta receptors. Serotonin is associated with the various 5-HT receptors. GABA is associated with the GABA receptor. Opioids are associated with the mu and delta receptors. Dopamine is associated with the D1, D2 etc. receptors.

  Basic Neuroscience

  K&S Ch. 3

Question 96. B. Buspirone is a serotonin 1A agonist or partial agonist. It is indicated for the treatment of anxiety disorders, in particular, generalized anxiety disorder. It does have activity at the serotonin 2 and dopamine 2 receptor sites, but its significance at those sites is not well understood. It may have mild dopamine 2 agonistic and antagonistic effects, but this is not its predominant mechanism of action. Buspirone takes 2–3 weeks to exert therapeutic effects. The initial dose is 15 mg daily in two or three divided doses. Therapeutic effects are usually not seen until a dose of 30 mg or above is reached. The maximum approved daily dose is 60 mg. Buspirone can increase blood levels of haloperidol. Buspirone cannot be used with monoamine oxidase inhibitors (MAOI) and a two-week washout needs to happen after an MAOI is stopped before buspirone can be started. Buspirone levels can be increased by nefazodone, erythromycin, itraconazole and grapefruit juice by their inhibition of cytochrome P450 3A4 in the liver.

  Psychopharmacology

  K&S Ch. 36

Question 97. E. Excitatory neurotransmitters open cation channels that depolarize the cell membrane and increase the likelihood of generating an action potential. These neurotransmitters elicit excitatory post-synaptic potentials or EPSPs.

  Basic Neuroscience

  K&S Ch. 3

Question 98. C. Phencyclidine (PCP) is a potent N-methyl-D-aspartate (NMDA) receptor antagonist. The NMDA receptor is a subtype of the glutamate receptor. PCP has calcium channel binding properties and prevents the influx of calcium into neurons. PCP also has dopaminergic properties that would seem to explain the reinforcing effects of the drug.

  Tolerance can occur with PCP, but it is generally held that PCP does not cause a physical dependence. There is psychological dependence to the agent and users can become dependent to its euphoric effects in this way. PCP intoxication is characterized by maladaptive behavioral changes, such as violence, impulsivity, belligerence, agitation, impaired judgment. Symptoms of intoxication include nystagmus, hypertension, decreased pain responsiveness, ataxia, dysarthria, muscle rigidity, seizures, coma, and hyperacusis. PCP can induce a delirium with agitated, bizarre, or violent behavior. It can also cause an acute psychotic disorder with delusions and hallucinations that can persist for several weeks following ingestion of the drug. PCP can remain in the blood and urine for more than a week. Treatment of the behavioral abnormalities related to PCP is best undertaken with benzodiazepines and dopamine antagonists.

  Substance Abuse and Addictive Disorders

  K&S Ch. 12

Question 99. C. Sublimation is one of the mature defenses. It is characterized by obtaining gratification by transforming a socially unacceptable aim or object into an acceptable one. Sublimation allows instincts to be channeled in an acceptable direction rather than blocked. All other choices are immature defenses. Hypochondriasis is exemplified by exaggerating an illness for the purpose of evasion or regression. Introjection is demonstrated by internalizing the qualities of an outside object. It is an important part of development but can also be used as an unproductive defense. The classic example is identification with an aggressor. Regression is characterized by attempting to return to an earlier libidinal phase of functioning to avoid the tension and conflict of the current level of development. Passive aggression takes the form of expressing anger towards others through passivity, masochism, and turning against the self. Manifestations can include failure and procrastination.

  Psychological Theory and Psychometric Testing

  K&S Ch. 6

Question 100. E. Phenelzine is a monoamine oxidase inhibitor and is not likely to worsen the movement disorder symptoms of Parkinson’s disease. The other four agents are antagonists of dopamine D2 receptors and can of course worsen symptoms of Parkinson’s disease and cause drug-induced parkinsonism. The pathophysiology involves D2 receptor antagonism in the caudate. Patients who are elderly and female are at greatest risk for neuroleptic-induced parkinsonism. More than half of patients exposed to neuroleptics on a long-term basis have been noted to develop this unwanted adverse effect. Amoxapine (Asendin) is a dibenzoxazepine tetracyclic antidepressant that has strong D2 antagonistic properties because it is a chemical derivative of the neuroleptic loxapine (Loxitane). Because of its unique structure and chemical properties, amoxapine can also cause akathisia, dyskinesia and infrequently, neuroleptic malignant syndrome.

  Psychopharmacology

  K&S Ch. 36

Question 101. D. Ana Freud wrote The Ego and Mechanisms of Defense and was the first to give a comprehensive study of the defense mechanisms. She maintained that all people, healthy or neurotic, use a number of defense mechanisms. Freud was the father of psychoanalysis whose work focused on the importance of libido, aggression, and the oedipal complex, among other concepts. Kohut was the father of self psychology. Fromm defined five character types common to western culture. Jung went beyond Freud’s work and founded the school of analytic psychology. It focused on the growth of the personality through one’s experiences.

  Psychological Theory and Psychometric Testing

  K&S Ch. 6

Question 102. B. In the case of Dusky vs United States, the US Supreme Court determined that, in order to have the competence to stand trial, a criminal defendant must be able to have the ability to consult his lawyer with a reasonable degree of rational understanding and he must have a reasonable and rational understanding of the proceedings against him. The McGarry instrument is a clinical guide that identifies thirteen areas of functioning that must be demonstrated by a criminal defendant in order to be declared competent to stand trial. Answer choices A, C, D, and E are included in these 13 areas, as well as the ability to plan legal strategy, the ability to appraise the roles of participants in courtroom procedure, capacity to challenge prosecution witnesses realistically, capacity to testify relevantly, ability to appraise the likely outcome, and understanding the possible penalties, among several others.

  Forensic Psychiatry

  K&S Ch. 57

Question 103. D. Paranoid schizophrenia is characterized by systematized delusions or frequent auditory hallucinations related to a single theme. None of the other answer choices are characteristic of paranoid schizophrenia, but may be found in other types of schizophrenia.

  Psychotic Disorders

  K&S Ch. 13

Question 104. E. Studies have indicated that depression tends to be a chronic, relapsing disorder. The percentage of patients who recover following repeated episodes diminishes over time. About one-quarter of patients have a recurrence within the first 6 months after initial treatment. This figure rises to about 30–50% in the first 2 years and even higher to about 50–75% within 5 years. It has been proven than ongoing antidepressant prophylaxis helps to lower relapse rates. As a patient experiences more depressive episodes over time, the time between episodes decreases and the severity of the episodes worsens.

  Depressive Disorders

  K&S Ch. 15

Question 105. D. Dysthymic disorder is characterized by decreased mood over a period of 2 years with poor appetite or over eating, sleep problems, fatigue, low self esteem, poor concentration, and feelings of hopelessness. Hallucinations are not considered part of dysthymia, although it is possible to have hallucinations as part of a major depressive episode.

  Depressive Disorders

  K&S Ch. 15

Question 106. D. Tarasoff vs regents of the University of California is the landmark case from 1976 in which the California Supreme Court ruled that any psychotherapist who believes that a patient could injure or kill someone must notify the potential victim, the victim’s relatives or friends, or the authorities. In 1982, the same court issued a second ruling that broadened Tarasoff to include the duty to protect, not only to warn the intended victim.

  The Durham Rule was determined by the ruling in the case of Durham vs the United States in 1954 by Judge Bazelon. This rule stipulates that a defendant cannot be found criminally responsible if the criminal act was the product of a mental illness or defect. In 1972 the District of Columbia Court of Appeals in the ruling United States vs Brawner, discarded the Durham Rule.

  In 1976 in the ruling of O’Connor vs Donaldson, the United States Supreme Court ruled that harmless mentally ill patients cannot be confined involuntarily without treatment if they can survive outside an institution.

  Clites vs State was a landmark case pertaining to a ruling in favor of a patient and his family who sued for damages resulting from chronic neuroleptic exposure that resulted in tardive dyskinesia. The appellate court ruled that the defendants deviated from the usual standards of care by failing to conduct physical examinations and routine laboratory tests and failed to intervene at the first signs of tardive dyskinesia.

  Forensic Psychiatry

  K&S Ch. 57

Question 107. C. All of the drugs listed are tertiary amines except desipramine, which is a secondary amine. The other tertiary amine is doxepin. The secondary amines include desipramine, nortriptyline, and protriptyline. During metabolism the tertiary amines are converted into secondary amines.

  Psychopharmacology

  K&S Ch. 35

Question 108. C. Surgical approaches to Parkinson’s disease include deep brain stimulation, surgical stereotactic ablation of overactive brain areas and cellular implantation of dopaminergic neuronal cells. Stereotactic thalamotomy can reduce tremor and rigidity contralaterally in about 75–85% of patients. There is little effect on bradykinesia. Bilateral thalamotomy can cause cognitive and speech deficits. Thalamic deep brain stimulation is better tolerated and results are similar to thalamotomy without the risks involved with the ablative procedure. Stereotactic posteroventral pallidotomy can improve dopa-induced dyskinesia and akinesia in about 70% of patients. Pallidal deep brain stimulation has also been shown to be almost as effective as pallidotomy. Subthalamic deep brain stimulation is the most preferred of the surgical procedures and has demonstrated 40–50% improvement in the motor fluctuations of Parkinson’s disease. Human fetal substantia nigra transplantation into the putamen has been shown to be modestly effective on an experimental basis. Injection of glial-derived neurotrophic factor (GDNF) into brain parenchyma may be of future utility, but up until recently has only been studied in animal models. The superior colliculus is not an area that is targeted in neurosurgical intervention for Parkinson’s disease.

  Neurology

  B&D Ch. 71

Question 109. A. Amoxapine has a 7-hydroxymetabolite that has potent dopamine blocking activity. This can lead to antipsychotic-like side effects that result from the drug’s use.

  Psychopharmacology

  K&S Ch. 35

Question 110. C. Right–left disorientation is a result of a lesion to the dominant angular gyrus and is one of the symptoms of Gerstmann’s syndrome. A large stroke in the right middle cerebral artery territory can result in hemi-neglect, visual and tactile extinction, impaired speech prosody (loss of musical and emotional inflection), anosognosia (not knowing that you have a deficit or problem), and behavioral problems such as delirium and confusion. The patient may not recognize that the affected left arm and/or hand is his own and may have limb apraxia. A contralateral homonymous hemianopia or inferior quadrantanopia can also be noted in nondominant hemispheric strokes.

  Neurology

  B&D Ch. 51

Question 111. D. The metabolism of tricyclic antidepressants by CYP2D6 is an important topic that has led to an FDA-recommended precaution. Tricyclics are metabolized by CYP 2D6, and as such anything that decreases the activity of CYP 2D6 will increase the plasma level of the tricyclic, even into the toxic range. Some people are naturally “poor metabolizers” who have decreased CYP 2D6 activity, and as such, higher plasma levels. Cimetidine inhibits CYP 2D6 and as such will increase tricyclic levels. The same holds true for quinidine. Several drugs are substrates for CYP 2D6 and will decrease its ability to clear tricyclics when present. These include fluoxetine, sertraline, paroxetine, carbamazepine, phenothiazines, propafenone, and flecainide.

  Psychopharmacology

  K&S Ch. 35

Question 112. A. This question depicts a classic diabetic third nerve palsy. The differential diagnosis of greatest importance with an isolated third nerve palsy is diabetes (benign) versus that of an internal carotid artery (ICA) aneurysm (potentially fatal). Aneurysms that most commonly affect third nerve functioning are those originating at the ICA near the origin of the posterior communicating artery. A third nerve palsy resulting from an aneurysm is usually associated with a dilated pupil and eyelid ptosis, as contrasted with a diabetic third nerve palsy that usually spares the pupillary function.

  Neurology

  B&D Ch. 51

Question 113. A. Residual schizophrenia is characterized by the absence of prominent hallucinations, delusions, incoherence, and disorganized behavior. Two or more of the residual symptoms may be present (i.e., negative symptoms or active phase symptoms present in a clearly attenuated form). Posturing is a characteristic symptom of catatonic schizophrenia.

  Psychotic Disorders

  K&S Ch. 13

Question 114. E. Severe motor tics in Tourette’s syndrome are best treated by neuroleptics, in particular haloperidol and pimozide. Recently the atypical neuroleptics have come to be used more readily because of their superior safety profiles, in particular risperidone, quetiapine, olanzapine, ziprasidone and clozapine. Fluphenazine, molindone, and other conventional antipsychotics are also acceptable treatment choices. Clonidine is also a frequently used and effective treatment of tics and is particularly favored by pediatric neurologists for its excellent safety profile. Botulinum toxin type A can be effective for blepharospasm, eyelid motor tics, and is in fact FDA-approved for this indication. Protriptyline and the other antidepressants may be effective for associated obsessive–compulsive symptoms, but these agents are not useful for treatment of tics.

  Disruptive, Impulse Control, Conduct Disorders, and ADHD

  B&D Ch. 71

Question 115. E. Grossly inappropriate affect is a characteristic of disorganized schizophrenia, along with disorganized behavior, incoherence, and loosening of associations. In addition to the symptoms listed in the question, catatonic schizophrenia can also present with stupor, purposeless excitement, posturing, and echopraxia.

  Psychotic Disorders

  K&S Ch. 13

Question 116. C. The middle cerebral artery territory includes the optic radiations. A lesion of these tracts results in either a contralateral homonymous hemianopsia or a contralateral inferior quadrantanopsia (“pie on the floor”). Bitemporal hemianopsia is of course caused by invasion of the optic chiasm, often by a sellar lesion such as a pituitary macroadenoma. Left monocular blindness would result from an ipsilateral central retinal artery occlusion.

  Neurology

  B&D Ch. 51

Question 117. E. The process by which neurotransmitter is released into the synaptic cleft is called exocytosis. Neurotransmitters are synthesized in the presynaptic neuron, and both their synthesis and release are mediated by Ca2+ influx into the cell. Feedback receptors exist on the presynaptic membranes of many cells, a good example being the alpha 2 receptor on the noradrenergic neuron which participates in a negative feedback loop to stop the release of norepinephrine. Neurotransmitters such as dopamine, norepinephrine, and serotonin will remain active until they diffuse out of the cleft or are removed by reuptake mechanisms. Degradation of recycled neurotransmitters is done via monoamine oxidases (MAOs), with MAO type A degrading NE and serotonin and MAO type B degrading dopamine.

  Basic Neuroscience

  K&S Ch. 3

Question 118. B. Lesch–Nyhan syndrome is an X-linked recessive disorder. Hyperuricemia results from a deficit in hypoxanthine-guanine phosphoribosyltransferase. Clinical hallmarks of the disorder include cognitive impairment, self-mutilatory behavior, hypertonia, hyperreflexia, dysarthria, developmental delay and choreoathetotic movements. Low dopamine concentrations in the basal ganglia and cerebrospinal fluid may be a cause of the movement disorder noted in Lesch–Nyhan syndrome.

  Ornithine transcarbamylase deficiency is an X-linked inborn error of metabolism of the urea cycle that causes hyperammonemia, encephalopathy and respiratory alkalosis. Treatment with low-protein diet and dietary arginine supplementation can be effective.

  Carbamoylphosphate synthase deficiency is an autosomal recessive inborn error of metabolism of the urea cycle that causes hyperammonemia, encephalopathy and respiratory alkalosis. There are two forms of the disorder, type I an early fatal form and type II a delayed-onset form which may only present later in childhood or early adulthood. Treatment with dietary arginine repletion may be helpful in certain cases.

  Arginase deficiency results in hyperammonemia as well. The disorder is usually caused by a point-mutation or deletion on chromosome 6q23 that codes for the ARG1 gene. The incidence of the disorder is less than 1 in 100 000.

  Adenylosuccinase deficiency results in autism, growth retardation, and psychomotor delay. It is believed to be an autosomal recessive cause of autism. Seizures may also arise as a result of this disorder.

  Neurology

  B&D Ch. 62

Question 119. D. This question focuses on Piaget’s stages of cognitive development. In the sensorimotor stage children respond to stimuli in the environment, and learn during this process. They eventually develop object permanence (objects exist independent of the child’s awareness of their existence), and begin to understand symbols (as in the use of words to express thoughts).

  During the preoperational stage there is a sense that punishment for bad deeds is unavoidable (immanent justice). There is a sense of egocentrism, and phenomenalistic causality (the thought that events that occur together cause one another). Animistic thinking (inanimate objects are given thoughts and feelings) is also seen.

  In the concrete operations stage, egocentric thought changes to operational thought where another’s point of view can be taken into consideration. In concrete operations children can put things in order and group objects according to common characteristics. They develop the understanding of conservation (a tall cup and a wide cup can both hold equal volumes of water) and reversibility (ice can change to water and back to ice again.)

  During formal operations children can think abstractly, reason deductively, and define abstract concepts. Symbiosis is one of Mahler’s stages of separation–individuation. It is unrelated to Piaget.

  Human Development

  K&S Ch. 2

Question 120. D. Post-traumatic dementia most often results in frontotemporal cognitive dysfunction from diffuse subcortical axonal shear that disrupts cortical and subcortical circuitry. Slowing of mental processing and difficulty with executive functioning, set-shifting, organization and planning, are the most frequent cognitive deficits noted. Dementia pugilistica can occur in patients with repeated head trauma and is not simply limited to boxers. Clinical features include severe memory and attentional deficits and extrapyramidal signs. ApoE4 carriers (which resides on chromosome 19) are at increased risk of post-traumatic dementia as well as Alzheimer’s disease.

  Neurology

  B&D Ch. 68

Question 121. E. Doxepin is the tricyclic with the most antihistaminic activity.

  Psychopharmacology

  K&S Ch. 35

Question 122. A. Bupropion is neither indicated nor effective in the treatment of obsessive–compulsive disorder (OCD). The selective serotonin reuptake inhibitors (SSRIs) have demonstrated proven efficacy in treating OCD. Trials of 4–6 weeks’ duration are usually needed to produce results, but often trials need to be extended to 8–16 weeks to achieve maximal therapeutic benefit. The standard of care is to start either an SSRI or clomipramine which generally generate a response in 50–70% of cases. Four of the SSRIs have been FDA-approved for OCD, fluoxetine, fluvoxamine, paroxetine and sertraline. Higher doses may be needed in order to achieve a response. Clomipramine (Anafranil) is the most serotonergic of the tricyclic and tetracyclic antidepressant agents, which are more noradrenergic in their nature. Clomipramine was the first agent specifically FDA-approved for OCD. It needs to be titrated upwards gradually over several weeks in order to minimize gastrointestinal and anticholinergic side effects. The best results are achieved with a combination of drug and cognitive behavioral therapy.

  Psychopharmacology

  K&S Ch. 16

Question 123. A. Giving tricyclic antidepressants leads to a decrease in the number of α-adrenergic and 5-HT receptors. This downregulation of receptors is what correlates most closely with the time to clinical improvement in patients.

  Psychopharmacology

  K&S Ch. 35

Question 124. E. Enuresis should first be treated with the bell-and-pad behavioral conditioning method before pharmacotherapy is instituted. The principle is simple: a bell awakens the child when the mattress becomes wet. Tricyclic antidepressants, such as amitriptyline and imipramine can reduce the frequency of enuresis in about 60% of patients. Desmopressin (DDAVP) is effective in about half of patients. Tricyclic antidepressants are to be given about one hour before bedtime. The response to therapy can be as rapid as a few days. Desmopressin is administered intranasally in doses of 10–40 mg daily. Children who respond completely to any of these pharmacological agents should continue the therapy for several months to prevent relapse. Olanzapine and the antipsychotic medications are not generally used in the treatment of enuresis.

  Elimination Disorders

  K&S Ch. 53

Question 125. D. This question stem is referring to Ca2+. During an action potential, the first ion channel to open is the Na+ channel. This lets Na+ flow into the neuron. Next Ca2+ channels open allowing more positively charged ions to enter and contribute to the action potential. Once inside, Ca2+ ions act as second messengers involved in protein–protein interactions and gene regulation. Calcium ions are critical to the release of neurotransmitter and also activate the opening of potassium ion channels that then put a stop to the action potential through the after-hyperpolarization of the membrane. With regard to receptors, also keep in mind that the GABA receptor is a chloride ion channel.

  Basic Neuroscience

  K&S Ch. 3

Question 126. B. The most worrisome side effects of the tricyclic antidepressant agents are cardiac conduction abnormalities, because of course these side effects can lead to fatal cardiac arrhythmias if the medication is taken in overdose. These agents can cause flattened T waves, tachycardia, prolonged QT intervals and depressed ST segments on electrocardiograms. The tricyclics are also noted for causing orthostatic hypotension by α-1 adrenergic blockade and they can of course cause sedation and the lowering of the seizure threshold. The tricyclics can also cause anticholinergic side effects, which consist of dry mouth, constipation, blurred vision and urinary retention. In the male patient suffering from benign prostatic hypertrophy, the anticholinergic load can lead to severe urinary retention and even anuria, which can be very problematic. Bethanechol 25–50 mg three to four times daily can reduce urinary hesitancy and retention. Tricyclic antidepressants can actually help with migraine and neuropathic pain prophylaxis, particularly at lower dosages. These are off-label uses for these agents.

  Psychopharmacology

  K&S Ch. 36

Question 127. D. Inhibitory neurotransmitters open chloride channels that hyperpolarize the membrane and decrease the likelihood of an action potential being generated. They cause inhibitory post-synaptic potentials or IPSPs.

  Basic Neuroscience

  K&S Ch. 3

Question 128. C. This question addresses different types of behavior therapy for a simple phobia of spiders (arachnophobia). Flooding (implosion) involves exposing the patient to the feared stimulus in vivo immediately, without a gradual buildup, as would be expected in therapeutic graded exposure. The goal of flooding is immediate exposure and response prevention until the patient can tolerate the anxiety and gain a sense of mastery of the anxiety. Therapeutic graded exposure is similar to systematic desensitization, except that relaxation techniques are not involved and the technique is carried out in real situations. Progress is graded and at first the patient may be exposed to pictures of the spiders only, and then later exposed to the real spiders themselves. Participant modeling involves the patient learning a new behavior by observation first, then eventually by doing it, often accompanied by the therapist. This model can work particularly well with agoraphobia. Aversion therapy is the use of a noxious stimulus or punishment to suppress an undesired behavior. Using a bad-tasting nail polish to prevent nail-biting, or taking disulfiram to prevent alcohol use would be excellent examples of aversion therapy. Assertiveness training teaches the patient to develop social and interpersonal skills through responding appropriately in social or occupational situations, expressing opinions acceptably, and achieving personal goals. Techniques such as role modeling, positive reinforcement and desensitization can be used to develop assertiveness.

  Psychotherapy

  K&S Ch. 35

Question 129. C. Disclaimers such as “I know you don’t agree with this but …” or “I don’t know why I think this but…” often precede emotionally charged material. They serve to soften the delivery of such material and relieve anxiety on the part of the person making them. Whenever a disclaimer is made by the patient in therapy the therapist should listen closely to the material that follows as that material is often a window into how the patient truly feels. The other answer choices in this question border on the ridiculous.

  Psychotherapy

  K&S Ch. 35

Question 130. D. This question depicts a patient with an acute delirium. The mainstay of treatment of delirium is to treat the underlying cause if it is identifiable. The other goal of treatment is to give the patient environmental, sensory and physical support. Environmental support can be given by increasing the lighting level in the room, providing a clock and calendar in the room showing the correct time and date, and by dimming the lights and closing the blinds only at night. Physical support can be provided to patients by the presence of friends and relatives in the room, or a constant caregiver figure. This can help prevent falls and other physical accidents. Low-dose haloperidol in either oral or intramuscular form can be helpful to treat psychotic symptoms or agitation. Benzodiazepines, particularly those of high potency and short duration of action like flurazepam (Dalmane) should be avoided, as they can paradoxically agitate and further confuse the delirious patient.

  Neurocognitive Disorders

  K&S Ch. 10

Question 131. D. Abreaction is an emotional release after recalling a painful event. It is part of psychodynamic therapy and is a large part of what Freud thought brought about cure during psychoanalysis of conversion disorder patients during his early work. The other answer choices are part of cognitive behavioral therapy (CBT). In CBT the false belief systems that underlie maladaptive behaviors and mood disturbance are examined through techniques such as those listed in the question. Underlying assumptions that feed false belief systems are also examined. The goal of therapy is to correct the underlying thoughts and assumptions, and in so doing, change the mood and behavior.

  Psychotherapy

  K&S Ch. 35

Question 132. A. Pinpoint pupils are a feature of opioid intoxication or usage and not withdrawal. Opioid withdrawal consists of dysphoric mood, nausea or vomiting, muscle aches or twitches, lacrimation or rhinorrhea, papillary dilatation, piloerection, sweating, diarrhea, yawning, fever and insomnia. Associated symptoms can include hypertension, tachycardia, and temperature dysregulation such as hypothermia or hyperthermia. Other possible symptoms can include depression, irritability, weakness and tremor.

  Substance Abuse and Addictive Disorders

  K&S Ch. 12

Question 133. A. The least sedating tricyclic antidepressants are desipramine and protriptyline. Moderately sedating tricyclics include imipramine, amoxapine, nortriptyline and maprotiline. The most sedating include amitriptyline, trimipramine, and doxepin.

  Psychopharmacology

  K&S Ch. 35

Question 134. B. Secondary gain refers to tangible advantages that a patient may get as a result of being unwell. Examples of secondary gain include getting out of responsibility, getting money, drugs, or other financial entitlements, or controlling other people’s behavior. Getting or seeking medical help, or enjoying playing the sick role for its own sake, would be considered a form of primary gain.

  Somatic Symptom Disorders

  K&S Ch. 17

Question 135. C. In hypothyroidism, one would expect to find an increased TSH and a low free T4 level. Subclinical cases of hypothyroidism will present with a high TSH and a normal T4 level. Other answer choices are distracters, and are not the most important lab values to look for when trying to diagnose hypothyroidism.

  Laboratory Tests in Psychiatry

  K&S Ch. 7

Question 136. E. There are no absolute contraindications to electroconvulsive shock therapy (ECT). Pregnancy is not a contraindication for ECT. Fetal monitoring is only considered important if the pregnancy is high risk or complicated. Brain tumors increase the risk of ECT, especially of brain edema and herniation after ECT. If the tumor is small, complications can be minimized by administration of dexamethasone prior to ECT and close monitoring of blood pressure during the treatment. Patients with aneurysms, vascular malformations or increased intracranial pressure are at greater risk during ECT because of increased blood flow during the induction of the seizure. This risk can be decreased by careful control of blood pressure during the seizure. Epilepsy and prior neuroleptic malignant syndrome are not problematic with the administration of ECT. Recent myocardial infarction is another risk factor, but the risk decreases markedly 2 weeks after the infarction and even further 3 months after the infarction. Hypertension, if controlled and stabilized with antihypertensive medication, does not pose an increased risk during ECT.

  Diagnostic and Treatment Procedures in Psychiatry

  K&S Ch. 36

Question 137. E. Carbamazepine has many drug–drug interactions because of its effects on the cytochrome P450 system. Of importance is the fact that it lowers concentrations of oral contraceptives, leading to breakthrough bleeding and uncertain protection against pregnancy. It has a long list of other drugs with which it interacts including lithium, some antipsychotics, and certain cardiac medications. It should not be combined with MAOIs. Combining clozapine with carbamazepine will increase the risk of bone marrow suppression.

  Psychopharmacology

  K&S Ch. 36

Question 138. B. Alfred Binet developed the idea of mental age in 1905 as the average intellectual level of a particular age. Intelligence quotient (IQ) is simply the quotient of mental age divided by chronological age multiplied by 100. An IQ of 100 is therefore considered to be the average, that is, when mental age and chronological age are equal. An IQ of 100 represents the 50th percentile in intellectual ability for the general population.

  Psychological Theory and Psychometric Testing

  K&S Ch. 5

Question 139. B. Open-ended questions are those that allow the patient to express what he is thinking and do not direct the patient to speak about a specific topic that the doctor chooses. Ideally an interview should begin with open ended questions and become more specific and close-ended as it continues. Closed-ended questions can often be answered in one or few words. Open-ended questions often require the patient to explain and provide information.

  Psychotherapy

  K&S Ch. 1

Question 140. A. The Karolinska Institute has shown in numerous studies that diminished central serotonin plays a role in suicidal behavior. This group was the first to demonstrate that low levels of cerebrospinal fluid (CSF) 5-hydroxyindoleacetic acid (5-HIAA) is associated with suicidal behavior. It has also been shown that low 5-HIAA levels predict future suicidal behavior and that low 5-HIAA levels have been shown in the CSF of adolescents who kill themselves.

  Depressive Disorders

  K&S Ch. 34

Question 141. E. Symptoms of lithium toxicity include ataxia, tremor, nausea, vomiting, nephrotoxicity, muscle weakness, convulsions, coma, lethargy, confusion, hyperreflexia, and nystagmus. Non-specific T-wave changes can also be seen at high lithium concentrations. Jaundice is a result of hepatic dysfunction. Lithium damages the kidneys, not the liver. Lithium induced polyuria is the result of lithium antagonism to the effects of ADH (antidiuretic hormone).

  Psychopharmacology

  K&S Ch. 36

Question 142. B. Obsessive–compulsive disorder (OCD) is believed to be a result of an imbalance in the serotonin system. Data show that serotonergic drugs are more effective in relieving symptoms of OCD than drugs that work on other neurotransmitter systems. Studies are unclear as to whether serotonin actually is involved in the cause of OCD. Cerebrospinal fluid (CSF) assays of 5-hydroxyindoleacetic acid (5-HIAA) have reported variable findings in patients with OCD. In one study CSF levels of 5-HIAA decreased after treatment with clomipramine, leading researchers to the conclusion that the serotonin system is involved in OCD symptom genesis.

  Obsessive Compulsive and Related Disorders

  K&S Ch. 16

Question 143. E. Amoxapine is the most likely of the tricyclic antidepressants to cause parkinsonian symptoms. It can also cause akathisia or dyskinesia. This is because its metabolites have dopamine blocking activity. It can even cause NMS in rare cases. Tricyclics are contraindicated in patients with cardiac conduction deficits. They should be stopped before elective surgery because of the risk of hypertension when tricyclics are given concomitantly with anesthetics. Fludrocortisone can help some patients with orthostatic hypotension. Myoclonic twitches and tremors can be seen in patients on desipramine and protriptyline.

  Psychopharmacology

  K&S Ch. 35

Question 144. D. Vascular dementia, formerly known as multi-infarct dementia, is generally the result of one or more cerebrovascular accidents that are the consequence of ischemic or embolic risk factors for cerebrovascular events. Vascular dementia occurs more frequently in men, particularly those with known cerebrovascular risk factors, which include smoking, hypertension and diabetes. Symptoms are generally abrupt in onset and a pattern of step-wise decline in cognition and functioning can often be noted. There is often a presence of demonstrable focal neurologic deficits. Vascular dementia is generally poorly responsive to cholinergic repletion therapy by acetylcholinesterase inhibitors.

  Neurocognitive Disorders

  K&S Ch. 10

Question 145. B. Clomipramine is the most serotonin-selective of the tricyclic antidepressants.

  Psychopharmacology

  K&S Ch. 35

Question 146. E. The psychiatrist is the member of the team who is held legally responsible for the team’s decisions. It stems from the concept that the highest person in the hierarchy is responsible for the actions of those he or she supervises. The psychiatrist is considered the head of this team. As such, the attending psychiatrist is responsible for the actions of the residents he or she supervises, and is responsible for the actions of the team.

  Forensic Psychiatry

  K&S Ch. 57

Question 147. E. The tricyclic antidepressants have many cardiac side effects that are worsened in overdose. They act as type 1A antiarrhythmics. As such they can terminate ventricular fibrillation, and increase collateral blood supply to ischemic heart tissue. In overdose they can be highly cardiotoxic and will cause decreased myocardial contractility, tachycardia, hypotension, and increased myocardial irritability. Also important to note is that nortriptyline is unique in that it has a therapeutic window. Blood levels should be obtained and the therapeutic range is 50–150 ng/mL. Levels above 150 ng/mL may reduce its efficacy.

  Psychopharmacology

  K&S Ch. 35

Question 148. C. Past suicidal behavior is the best predictor of future suicidal behavior. It is a better predictor than any of the other answer choices given in this question. As such, one of the most important questions to ask a patient who has suicidal thoughts is about their history of past suicide attempts. Other risk factors for suicide are as follows: men are more likely than women, divorced or single more likely than married, elderly are less likely to attempt, but more likely to succeed when they do, whites are more likely than other ethnic groups, and the higher the social status the higher the risk.

  Management in Psychiatry

  K&S Ch. 34

Question 149. D. Clomipramine has been shown to be particularly useful in treating obsessive–compulsive disorder in comparison to the other tricyclics. Its efficacy when compared to the SSRIs for OCD is equal or better, depending on the study.

  Obsessive Compulsive and Related Disorders

  K&S Ch. 35

Question 150. E. Imipramine is often used to treat childhood enuresis. Desmopressin is also useful in 50% of patients. Improvement can range from cessation of enuresis to leaking less urine. Drugs should be given 1 hour before bedtime and results are usually evident within days. Patients who achieve full dryness should take the medication for several months to prevent relapse.

  Elimination Disorders

  K&S Ch. 35