Comprehensive Review in Clinical Neurology: A Multiple Choice Question Book for the Wards and Boards

Chapter 12. Cognitive and Behavioral Neurology

Question

Questions 1–2

  1. A 77-year-old man presents to the office with a complaint of difficulty remembering things such as names and directions. His wife has noticed this more than he has. He denies any other cognitive complaints and his memory impairment has not interfered with his daily activities. On examination, you note that he recalls zero out of three objects on the Mini-Mental Status Examination at 5 minutes, and his Mini-Mental Status Examination score is 27. Which of the following is the most likely diagnosis?

      a.  Alzheimer’s disease

      b.  Mild cognitive impairment

      c.  FTD

      d.  Normal aging

      e.  DLB

  2. Your suspected diagnosis for the patient depicted in question 1 is defined by impairment in:

      a.  One or more cognitive domains

      b.  Two or more cognitive domains

      c.  Three or more cognitive domains

      d.  Four or more cognitive domains

      e.  Five or more cognitive domains

Questions 3–6

  3. A 60-year-old man is brought to your office by his daughter and wife because of prominent memory loss, several recent automobile accidents near his home, and lack of insight regarding these recent events. There is a family history of early dementia in his uncle. You decide to proceed with an evaluation. According to the American Academy of Neurology guidelines, which of the following is not recommended in the routine evaluation of patients presenting with dementia?

      a.  Vitamin B12

      b.  Complete blood count

      c.  VDRL

      d.  Depression screening

      e.  Electrolytes

  4. You suspect that the patient depicted in question 3 has Alzheimer’s disease. With that in mind, which chromosome is linked to presenilin-2?

      a.  14

      b.  1

      c.  21

      d.  19

      e.  2

  5. Which of the following is not a structure involved in the formation of new memories within the circuit of Papez?

      a.  Hippocampus

      b.  Mamillary bodies

      c.  Fornix

      d.  Mediodorsal nucleus of the thalamus

      e.  Entorhinal cortex

  6. Which of the following symptoms is typically the earliest symptom of Alzheimer’s disease?

      a.  Immediate memory impairment

      b.  Recent memory impairment

      c.  Remote memory impairment

      d.  Procedural memory impairment

      e.  Apraxia

Questions 7–9

  7. A 74-year-old woman is brought to your office for memory problems and neuropsychiatric symptoms. An FDG-PET scan was completed, which is shown in Figure 12.1. What diagnosis do you suspect on the basis of this?

FIGURE 12.1 Axial and sagittal FDG-PET (Courtesy of Dr. Guiyun Wu). Shown also in color plates

      a.  Parkinson’s disease

      b.  Alzheimer’s disease

      c.  DLB

      d.  FTD

      e.  Huntington’s disease

  8. On the basis of your suspected diagnosis in question 7, which of the following would be the least commonly associated histopathologic finding in the brain of this patient?

      a.  Neurofibrillary tangles and neuritic plaques

      b.  Amyloid deposition in cortical and leptomeningeal blood vessel walls

      c.  Lewy bodies

      d.  Granulovacuolar neuronal degeneration

      e.  Hirano bodies

  9. In the neurodegenerative disease suspected in the patient depicted in question 7, there is loss of cholinergic neurons in which of the following structures?

      a.  Locus coeruleus

      b.  Raphe nuclei

      c.  Nucleus accumbens

      d.  Substantia nigra pars reticulata

      e.  Nucleus basalis of Meynert (substantia innominata)

Questions 10–11

10. A 78-year-old man is seen in the office, brought in by nursing home personnel for progressively worsening agitation and other neurologic features. On the basis of your findings and history provided, you diagnose DLB. The three primary clinical findings in DLB are:

      a.  Parkinsonism, impaired memory, visual hallucinations

      b.  Visual hallucinations, aphasia, parkinsonism

      c.  Fluctuating cognitive function, parkinsonism, aggression

      d.  Parkinsonism, fluctuating cognitive function, visual hallucinations

      e.  Visual hallucinations, auditory hallucinations, fluctuating cognitive function

11. Which of the following medications would not be recommended to treat severe agitated psychotic symptoms in this patient?

      a.  Quetiapine

      b.  Risperidone

      c.  Haloperidol

      d.  Olanzapine

      e.  Clozapine

12. Depression is least likely to be seen in:

      a.  Alzheimer’s disease

      b.  Parkinson’s disease

      c.  Huntington’s disease

      d.  Multiple sclerosis

      e.  Pick’s disease

13. Impairment in which of the following structures would be least likely to be associated with personality changes?

      a.  Orbitofrontal cortex

      b.  Temporal lobe

      c.  Dorsolateral frontal lobe

      d.  Occipital lobe

      e.  Caudate nucleus

Questions 14–15

14. A 59-year-old woman is brought to your office by her family for progressively offensive speech, inappropriate behavior, and impaired social functioning over the past year. She pays no attention to her personal hygiene and sometimes urinates in her pants without even noticing anything is wrong. Her family reports that these behaviors are highly unusual for her. Several family members have reportedly been diagnosed with FTD. Given a possible case of familial FTD, which chromosome may be involved?

      a.  14

      b.  17

      c.  19

      d.  21

      e.  1

15. Regarding FTD, which of the following is not commonly associated with one of the FTD variants?

      a.  Anomia

      b.  Motor neuron disease

      c.  Progressive supranuclear palsy

      d.  Corticobasal degeneration

      e.  Visual hallucinations

16. The FDG-PET scan in Figures 12.2 and 12.3 are suggestive of which neurodegenerative disease?

FIGURE 12.2 Axial FDG-PET (Courtesy of Dr. Guiyun Wu). Shown also in color plates

FIGURE 12.3 Sagittal FDG-PET (Courtesy of Dr. Guiyun Wu). Shown also in color plates

      a.  Parkinson’s disease

      b.  Alzheimer’s disease

      c.  DLB

      d.  FTD

      e.  Huntington’s disease

17. A 65-year-old woman presents with abrupt onset akinetic mutism, lack of motivation, apathy, leg weakness, and incontinence. You suspect she may have had a stroke. Which of the following could explain her symptoms?

      a.  Pontine infarct

      b.  Dominant temporal lobe infarct

      c.  Nondominant parietal lobe infarct

      d.  Nondominant temporal lobe infarct

      e.  Bilateral ACA infarcts

18. Lesions in which thalamic nucleus would most likely cause symptoms of abulia, anterograde amnesia, social disinhibition, and motivation loss?

      a.  Anterior nucleus

      b.  Ventral posteromedial nucleus

      c.  Pulvinar nucleus

      d.  Dorsomedial nucleus

      e.  Ventral posterolateral nucleus

19. Akinetic mutism can be caused by lesions to which of the following structures?

      a.  Bilateral globus pallidus interna

      b.  Bilateral globus pallidus externa

      c.  Bilateral putamen

      d.  Bilateral caudate

      e.  Bilateral amygdala

20. Huntington’s disease is a neurodegenerative disease resulting from genetic changes to which chromosome?

      a.  14

      b.  4

      c.  17

      d.  21

      e.  19

21. Features of Kluver-Bucy syndrome may occur as part of which of the following neurodegenerative disorders?

      a.  Alzheimer’s disease

      b.  Schizophrenia

      c.  DLB

      d.  Pick’s disease

      e.  Parkinson’s disease

22. In traumatic brain injury, which of the following signs or symptoms is most likely to persist and interfere with rehabilitation?

      a.  Memory loss

      b.  Motor deficit

      c.  Altered personality

      d.  Sensory deficit

      e.  Language dysfunction

23. You are consulted on a 39-year-old woman for significant memory loss following an uncomplicated medical procedure. You suspect transient global amnesia. This disorder typically affects what aspect of memory?

      a.  Immediate memory

      b.  Procedural memory

      c.  Recent (short-term) memory

      d.  Remote memory

      e.  Personal identity

Questions 24–26

24. A 79-year-old man is brought to your office by his daughter. She says he has been getting increasingly confused, although his periods of confusion seem to fluctuate. Some days he seems much better than others. He often complains that there are squirrels and rodents in his bedroom. On examination, he appears bradykinetic, and you notice a fine resting tremor in both hands. What is your suspected diagnosis?

      a.  Progressive supranuclear palsy

      b.  Alzheimer’s disease

      c.  Pick’s disease

      d.  DLB

      e.  Parkinson’s disease

25. What would you expect to find on an FDG-PET scan in the patient depicted in question 24?

      a.  Occipital hypometabolism > temporoparietal hypometabolism

      b.  Occipital hypometabolism < temporoparietal hypometabolism

      c.  Frontotemporal hypometabolism > temporoparietal hypometabolism

      d.  Frontotemporal hypometabolism > occipital hypometabolism

      e.  Global hypometabolism of cortex and deep brain structures

26. Two years later, the daughter of the patient depicted in question 24 calls you and says that her father died and had an autopsy. On the basis of the histopathology in Figure 12.4, what is your final diagnosis?

FIGURE 12.4 Brain specimen (Courtesy of Dr. Richard A. Prayson). Shown also in color plates

      a.  Alzheimer’s disease

      b.  DLB

      c.  CJD

      d.  Progressive supranuclear palsy

      e.  Pick’s disease

27. A 32-year-old alcoholic woman presents to the emergency department. She says she cannot remember how she got there or even what her name is. On examination, you find that she has intact new learning ability with loss of remote memory, including autobiographical memory. What is your diagnosis?

      a.  Wernicke’s encephalopathy

      b.  Korsakoff’s disease

      c.  Transient global amnesia

      d.  Psychogenic amnesia

      e.  Retrograde amnesia

Questions 28–32

28. A 76-year-old man with a clinical diagnosis of Alzheimer’s disease (AD) is brought to your office by his family. They are here today and have been reading up on different AD medications and have many questions. Which of the following is not an acetylcholinesterase inhibitor used to treat AD?

      a.  Donepezil

      b.  Memantine

      c.  Rivastigmine

      d.  Galantamine

      e.  Tacrine

29. The mechanism of action of donepezil is:

      a.  NMDA receptor antagonist

      b.  Acetylcholinesterase agonist

      c.  Butyrylcholinesterase inhibitor

      d.  Allosteric nicotinic modulator

      e.  Acetylcholinesterase inhibitor

30. The mechanism of action of rivastigmine is:

      a.  Allosteric nicotinic modulator

      b.  Acetylcholinesterase agonist

      c.  Acetylcholinesterase and butyrylcholinesterase inhibitor

      d.  NMDA receptor antagonist

      e.  Acetylcholinesterase antagonist and allosteric nicotinic modulator

31. The mechanism of action of galantamine is:

      a.  Acetylcholinesterase and butyrylcholinesterase inhibitor

      b.  Pure acetylcholinesterase inhibitor

      c.  Acetylcholinesterase inhibitor and allosteric nicotinic modulator

      d.  NMDA receptor antagonist

      e.  NMDA receptor agonist

32. The mechanism of action of memantine is:

      a.  NMDA receptor agonist

      b.  Acetylcholinesterase and butyrylcholinesterase inhibitor

      c.  Acetylcholinesterase inhibitor and allosteric nicotinic modulator

      d.  NMDA receptor antagonist

      e.  Acetylcholinesterase inhibitor

33. Immediate recall (digit span) is preserved in all of the following, except:

      a.  Korsakoff’s encephalopathy

      b.  Mild cognitive impairment

      c.  Transient global amnesia

      d.  Major depression

      e.  Early Alzheimer’s disease

Questions 34–35

34. You are called to the bedside of a previously healthy 79-year-old man who underwent a total knee replacement yesterday. The nurse states his level of alertness has been fluctuating and he has been slurring his speech, has been very drowsy at times, and mentioned seeing a large colorful eye on the wall looking at him. What is the most likely explanation for these symptoms?

      a.  Posterior circulation stroke

      b.  Occipital lobe seizures

      c.  Delirium

      d.  Dementia

      e.  Schizophrenia

35. Which of the following will likely give the most useful information in your evaluation of this patient?

      a.  CT brain

      b.  EEG

      c.  Lumbar puncture

      d.  Psychiatry consult

      e.  Checking the patient’s medications list and laboratory studies

36. Which of the following diseases is considered a synucleinopathy?

      a.  Corticobasal ganglionic degeneration

      b.  Multiple system atrophy

      c.  Progressive supranuclear palsy

      d.  FTD

      e.  Pick’s disease

37. What disease do you suspect on the basis of the histopathologic findings seen in Figure 12.5?

FIGURE 12.5 Brain specimen (Courtesy of Dr. Richard A. Prayson). Shown also in color plates

      a.  Parkinson’s disease

      b.  Alzheimer’s disease

      c.  Pick’s disease

      d.  CJD

      e.  DLB

Questions 38–40

38. Which of the following diseases would you suspect on the basis of the histopathology in Figure 12.6?

FIGURE 12.6 Brain specimen (Courtesy of Dr. Richard A. Prayson). Shown also in color plates

      a.  Rabies

      b.  DLB

      c.  Sphingolipidosis

      d.  CJD

      e.  Alzheimer’s disease

39. The disease that you suspect from the histopathologic slide shown in Figure 12.6, has been associated with which of the following chromosomes?

      a.  1

      b.  19

      c.  14

      d.  20

      e.  21

40. Which of the following is not typically an associated finding on MRI in patients with the histopathologic findings seen in Figure 12.6?

      a.  Cortical ribbon sign

      b.  Pulvinar sign

      c.  Increased T2 signal in the neocortex and thalamus

      d.  Increased T2 signal in the caudate and putamen

      e.  Increased T2 signal in the globus pallidus

Questions 41–43

41. What is the pathologic finding in Figure 12.7?

FIGURE 12.7 Brain specimen (Courtesy of Dr. Richard A. Prayson). Shown also in color plates

      a.  Lewy bodies

      b.  Negri bodies

      c.  Pick bodies

      d.  Bunina bodies

      e.  Neurofibrillary tangles

42. Which of the following has not been suggested to be a risk factor for the disease associated with the pathologic finding in Figure 12.7?

      a.  Low level of education

      b.  Male gender

      c.  Age

      d.  Repeated head trauma

      e.  Family history

43. Which of the following would be the most consistent finding on an FDG-PET scan in patients with the disease associated with the pathologic finding in Figure 12.7?

      a.  Occipital hypometabolism greater than temporoparietal hypometabolism

      b.  Posterior temporal and parietal hypometabolism

      c.  Frontotemporal hypometabolism

      d.  Temporoparietal hypermetabolism

      e.  Global hypometabolism of cortex and deep brain structures

Questions 44–46

44. A 76-year-old woman with a history of successfully-treated breast cancer is brought in by her daughter for progressive memory loss and cognitive difficulties. Her daughter is very concerned by this and also mentions that the patient has difficulty with walking and seems to have a problem with lifting her feet off the floor. She wonders if you can also give a referral to a urologist because her mother has been requiring the use of adult diapers in the last 6 months. On the basis of the history, which of the following is the best next step for diagnosis?

      a.  Formal neuropsychiatric/cognitive testing

      b.  Trial of levodopa

      c.  Lumbar puncture

      d.  MRI brain

      e.  MRI spine

45. A brain MRI was obtained in this patient. What diagnosis do you suspect based on the findings in Figure 12.8?

FIGURE 12.8 Axial FLAIR MRI

      a.  Parkinson’s disease

      b.  Advancing Alzheimer’s disease

      c.  Normal-pressure hydrocephalus

      d.  Metastatic disease to central nervous system

      e.  Transverse myelitis

46. What would be your first choice in the approach and management of this patient?

      a.  Levodopa

      b.  Lumbar puncture

      c.  Schedule a CSF shunt procedure

      d.  Referral to oncology for further evaluation

      e.  A course of intravenous steroids

47. What is the pathologic finding in Figure 12.9?

FIGURE 12.9 Brain specimen (Courtesy of Dr. Richard A. Prayson). Shown also in color plates

      a.  Negri bodies

      b.  Bunina bodies

      c.  Pick bodies

      d.  Granulovacuolar degeneration

      e.  Lewy bodies

48. A woman with Alzheimer’s disease stopped being able to recognize her son after he shaved his moustache. She could recognize her husband only when she heard his voice. The term used to describe this type of agnosia is:

      a.  Topographagnosia

      b.  Prosopagnosia

      c.  Asomatognosia

      d.  Misoplegia

      e.  Somatoparaphrenia

49. A 69-year-old man was noted to have difficulty looking at objects that were brought into his visual field. He could also not reach for objects placed in front of him, though he could see them. When presented with a picture of a complex scene, he could identify items within the picture but could not describe the picture as a whole. This syndrome localizes to:

      a.  Bilateral temporooccipital region

      b.  Bilateral primary visual cortices

      c.  Bilateral parietooccipital region

      d.  Anterior thalamus

      e.  Bilateral anterior cingulate gyrus

50. A 52-year-old woman is brought to the emergency department by her family with disorientation. On further questioning, her family reported that she had been walking into walls and into furniture, and then she would make up absurd excuses as to why this occurred. On examination, she had complete visual loss on confrontation testing and loss of optokinetic nystagmus. She confabulated when asked to name objects or count fingers. She categorically denied there being anything wrong with her vision. This presentation is most consistent with:

      a.  Delusional disorder

      b.  A receptive aphasia

      c.  Anton’s syndrome

      d.  Balint’s syndrome

      e.  Psychogenic blindness

51. An 87-year-old gentleman who is legally blind due to severe macular degeneration reports seeing images of people and colorful animals. He realizes that he is blind and clearly understands that these are hallucinations. This man has:

      a.  Anton’s syndrome

      b.  Balint’s syndrome

      c.  Psychosis with visual hallucinations

      d.  Charles Bonnet syndrome

      e.  Delusional disorder

52. A 63-year-old college professor is brought to a neurologist after she is noted to have difficulty reading her slides during a lecture she was giving. On examination, she has speech hesitation, but speech output is otherwise normal. She is able to read individual letters, but not entire words. She can write a full sentence to dictation, but cannot read what she has written. Her comprehension, repetition, writing, and naming are normal. Visual acuity is normal. On visual field testing to confrontation, she has a right homonymous hemianopia. Cranial nerve, motor, and sensory examinations are normal. Her MRI is shown in Figure 12.10. This patient’s symptoms are consistent with:

FIGURE 12.10 T2-weighted axial MRI

      a.  Wernicke’s aphasia

      b.  Psychogenic dyslexia

      c.  Alexia without agraphia

      d.  A selective neglect syndrome

      e.  Cortical blindness

53. A woman cannot understand others when they speak to her. She can speak normally and her reading comprehension is intact. She has:

      a.  Wernicke’s aphasia

      b.  Anomia

      c.  Conduction aphasia

      d.  Nonverbal auditory agnosia

      e.  Pure word deafness

54. A man is able to write normally, but his speech is fragmented and effortful. He can speak only in brief phrases, and exhibits multiple paraphasic errors. He is unable to repeat. Comprehension is intact. This is most consistent with:

      a.  Aphemia

      b.  A receptive aphasia

      c.  Conduction aphasia

      d.  Transcortical motor aphasia

      e.  Alexia without agraphia

55. A 72-year-old woman with atrial fibrillation has suffered multiple strokes over the past several years. On examination, she is noted to have severe dysarthria and is able to utter only a few unintelligible sounds. She has severe dysphagia and bilateral near-total paralysis of both upper and lower parts of her face. She cannot voluntarily smile, but in response to a joke, will smile symmetrically. During yawning and sneezing, there is no evidence of facial or pharyngeal weakness. This syndrome localizes to:

      a.  Bilateral anterior operculum

      b.  Anterior cingulate

      c.  Bilateral occipital lobes

      d.  Medulla

      e.  Pons

56. A 67-year-old woman is seen in the medical ICU, where she is being treated for sepsis with profound hypotension. On examination, she is speaking fluently but nonsensically, and comprehension is markedly impaired. However, she can repeat, and exhibits significant echolalia throughout the examination. This constellation of findings is consistent with:

      a.  A Broca’s (expressive) aphasia due to a dominant hemisphere posteroinferior frontal gyrus lesion

      b.  A Wernicke’s (receptive) aphasia due to a dominant hemisphere posterosuperior temporal gyrus lesion

      c.  A conduction aphasia due to a lesion to the internal arcuate fasciculus

      d.  A transcortical sensory aphasia due to a watershed infarct involving the MCA-PCA distribution

      e.  Aphemia due to a lesion in the dominant hemisphere anteroinferior frontal gyrus

57. On examination, an 88-year-old man has impaired speech marked by inability to verbalize, except for a few single- or two-word phrases, and he cannot write. His language comprehension is intact, and he can repeat. This aphasic syndrome localizes to:

      a.  The frontal operculum of the dominant hemisphere, involving Broca’s area

      b.  The posterior aspect of the superior temporal gyrus in the dominant hemisphere, involving Wernicke’s area

      c.  The right anterior cingulate gyrus

      d.  The middle frontal gyrus of the nondominant hemisphere

      e.  Dominant hemisphere ACA-MCA watershed territory, involving connections from the supplementary motor area to Broca’s area

58. On examination, a patient is found to have normal speech, normal language comprehension, and normal naming abilities. However, he is unable to repeat. The aphasia this patient exhibits is:

      a.  Broca’s aphasia due to a dominant hemisphere posteroinferior frontal gyrus lesion

      b.  Wernicke’s aphasia due to a dominant hemisphere posterosuperior temporal gyrus lesion

      c.  Conduction aphasia due to a lesion in the internal arcuate fasciculus

      d.  Wernicke’s aphasia due to a dominant hemisphere posterosuperior temporal gyrus lesion

      e.  Thalamic aphasia due to right anterior thalamic infarction

59. A 39-year-old right-handed woman loses the ability to vary her speech according to her emotional state. Her speech becomes monotonous. This localizes to:

      a.  Right posteroinferior frontal gyrus

      b.  Left posteroinferior frontal gyrus

      c.  Caudate

      d.  Superior temporal gyrus

      e.  Internal arcuate fasciculus

Questions 60–61

60. A 58-year-old man with a 20-year history of relapsing-remitting multiple sclerosis presents for follow-up in an outpatient clinic. His wife reports that he has been having episodes of involuntary laughter and crying during which he does not feel associated mirth or sadness. These episodes occur suddenly and are causing significant social embarrassment. He has also developed progressive dysarthria and dysphagia. It can be expected that this man has lesions in the:

      a.  Right occipital lobe

      b.  Bilateral frontal operculum

      c.  Bilateral corticobulbar pathways

      d.  Cerebellum

      e.  Bilateral anterior thalamic nuclei

61. Which of the following medications have been shown in randomized controlled trials to be effective in treating the disorder depicted in question 60?

      a.  Levodopa

      b.  Risperidone

      c.  Dextromethorphan-quinidine

      d.  Prochlorperazine

      e.  Morphine

62. A 68-year-old man with an infarction in the territory of the superior division of the left MCA is undergoing inpatient rehabilitation. Following his infarction, he had significant right arm weakness, could speak only a few words and exhibited marked paraphasic errors, and could not write, but his comprehension was intact. His speech has been steadily improving, as is the strength in his right hand. However, he is noticed to have significant difficulty performing simple, previously known motor tasks on command with his left hand, though his hand is not weak per se and he understands the command. This is explained by:

      a.  A new infarction in the left middle cerebral artery territory

      b.  A disconnection syndrome resulting from impaired transmission from his left receptive speech area to his right-hand motor area

      c.  Neglect of his left hand due to his MCA infarction

      d.  Sympathy weakness, in which his left hand is weak because his right hand is weak

      e.  A receptive aphasia due to a dominant posterosuperior temporal gyrus infarct

63. A 72-year-old woman is brought to a neurology clinic by her family members for memory impairment and a decline in functional abilities. On examination, when she is asked to pantomime brushing her teeth, she uses her finger as the toothbrush, instead of pretending to hold a toothbrush. When asked to show how she would open a letter with a letter opener, she uses her finger as the blade instead of pretending to hold a letter opener. This is consistent with:

      a.  Ideomotor apraxia

      b.  Conduction apraxia

      c.  Ideational apraxia

      d.  Disassociation apraxia

      e.  Conceptual apraxia

64. Dressing apraxia localizes to the

      a.  Left frontal lobe

      b.  Left parietal lobe

      c.  Right parietal lobe

      d.  Anterior cingulate

      e.  Splenium of the corpus callosum

Questions 65–67

65. A 54-year-old woman was brought to a psychiatry clinic by her family with a 2-month history of odd behavior. She was previously a reserved, pious woman, but in recent days, she had been found at a bar singing karaoke in leather pants and had a tattoo placed on her arm. She was also irritable, and had been sleeping less than 4 hours a night. On examination, she seemed distinctly restless and her speech was pressured. She made lewd remarks and joked inappropriately. She would imitate the gestures of the examiner, and pretended to be holding a stethoscope and auscultating the examiner with it. This presentation is most consistent with dysfunction in the:

      a.  Dorsolateral prefrontal cortex

      b.  Dorsomedial prefrontal cortex

      c.  Anterior cingulate

      d.  Caudate

      e.  Orbitofrontal cortex

66. Following an infarct, an 81-year-old previously gregarious and active gentleman became socially withdrawn. He no longer conversed with loved ones or laughed at jokes. He would spend his day sitting in his armchair and staring at the wall. This presentation is most consistent with dysfunction in the:

      a.  Dorsolateral prefrontal cortex

      b.  Dorsomedial prefrontal cortex

      c.  Anterior cingulate

      d.  Caudate

      e.  Orbitofrontal cortex

67. A 72-year-old chief executive officer of a multinational corporation was brought to a neuropsychiatry clinic by his wife. She reported that he had been a highly functioning person for years, was adept at multitasking, and single-handedly built his corporation from a small business over a decade. In the past year, he had become disorganized, and several of his affiliates were concerned because he kept missing scheduled meetings. He had made some poor investments in the prior year and his company was nearing bankruptcy. He did not seem particularly concerned about these mishaps, and had lost interest in social activities. He had previously been a cheese connoisseur, but he seemed to have lost all interest in food and had lost a significant amount of weight. This presentation is most consistent with dysfunction in the:

      a.  Dorsolateral prefrontal cortex

      b.  Dorsomedial prefrontal cortex

      c.  Anterior cingulate

      d.  Caudate

      e.  Orbitofrontal cortex

68. Regarding the utilization of neuropsychologic tests in the evaluation of various cortical functions, which of the following is incorrect:

      a.  The Trail-Making Tests are used to assess attention

      b.  The Wisconsin Card Sorting Test can be used as a measure of prefrontal cortical function

      c.  The Random Cancellation Test can be used to assess attention

      d.  The Clock-Drawing Test is a test of visuospatial function

      e.  The Grooved Pegboard Test is a test of verbal registration

69. A 28-year-old man is brought to a psychiatrist’s office by his fiancée. She reports that he is convinced that the man who “claims to be his father” is in fact a double, identical-looking im-poster. This is known as:

      a.  Fregoli’s syndrome

      b.  Intermetamorphosis

      c.  Pseudocyesis

      d.  Capgras’ syndrome

      e.  Cotard’s delusion

70. A 62-year-old man is brought to the emergency department by his wife because he was acting “funny.” He had been paying bills that morning and could not seem to calculate simple sums. He was also having trouble writing. On examination, he had right-left confusion and finger agnosia. This man’s syndrome localizes to the:

      a.  Nondominant superior parietal lobule

      b.  Dominant inferior parietal lobule

      c.  Nondominant frontal operculum

      d.  Dominant dorsolateral prefrontal cortex

      e.  Nondominant superior temporal gyrus

71. A 52-year-old woman presents with acute left hemiparesis, with predominant involvement of the left face and arm. On examination, she has extinction to double simultaneous visual and tactile stimulation, impaired two-point discrimination, and agraphesthesia. Her lesion is most likely in the:

      a.  Right MCA distribution

      b.  Right internal capsule area with thalamic involvement

      c.  Left pons

      d.  Right pons

      e.  Right ACA-MCA watershed territory

72. A 13-year-old male undergoes resection of a large hemispheric tumor. He is undergoing inpatient rehabilitation, and his therapy is being hindered by his lack of awareness of the left side of his body. He barely acknowledges the presence of weakness on the left side of his body; in fact, he largely does not recognize the left side of his body as being his own. This is consistent with:

      a.  A lesion in the dominant hemisphere parietal region involving the primary somatosensory cortex

      b.  A lesion in the nondominant hemisphere primary visual cortex

      c.  A lesion in the nondominant frontal lobe

      d.  A lesion in the nondominant hemisphere parietal region involving the primary somatosensory cortex

      e.  A lesion in the dominant hemisphere angular gyrus

73. A 52-year-old woman is brought to the emergency department after sudden onset of left hemiparesis. On examination, she has forced gaze deviation to the right that can be overcome by oculocephalic maneuver. Her lesion most likely involves:

      a.  The left frontal eye fields in the middle frontal gyrus

      b.  The right frontal eye fields in the middle frontal gyrus

      c.  The right parapontine reticular formation

      d.  The left parapontine reticular formation

      e.  The right parapontine reticular formation

74. A 73-year-old man suffers from an acute infarction leading to right arm and leg hemianesthesia. Three months later, he presents to an outpatient clinic with severe right-sided hyperesthesia and allodynia. Which of the following would not be a likely explanation for this man’s symptoms?

      a.  A left thalamic lesion

      b.  A lesion in the left parietal operculum

      c.  A lesion in the primary somatosensory cortex

      d.  A lesion in the medial lemniscus

      e.  A lesion in the cervical dorsal columns

75. Regarding the different types of memory, which of the following is incorrect?

      a.  Memory of high school graduation is an example of declarative, or explicit, memory

      b.  Ability to drive a car or ride a bicycle after years of having not done so is an example of nondeclarative, or implicit, memory

      c.  Lesions to the bilateral medial temporal lobes lead largely to loss of declarative (explicit) memory, with relative preservation of nondeclarative (implicit) memory

      d.  Lesions to the bilateral parietal lobes lead to loss of nondeclarative (implicit) memory

      e.  Nondeclarative memory does not localize to one specific area of the cerebral hemispheres

76. An 18-year-old man is admitted to a neurologic ICU with herpes simplex encephalitis. A brain MRI shows hyperintensities on T2 sequences involving extensive areas of the bilateral temporal lobes. He is treated with acyclovir and is discharged to a rehabilitation center for several weeks. As his encephalopathy begins to improve, he is noted to constantly ask for food, place any objects that come into sight into his mouth, and make sexual advances, lewd comments, and obscene gestures to all female staff in the rehabilitation center. He is otherwise calm and easily appeased. At times he is noted to focus for prolonged periods of times on minute visual stimuli, such as a piece of lint on his pajamas. This syndrome is due to:

      a.  A hypothalamic lesion

      b.  Bilateral mamillary body necrosis

      c.  Bilateral medial frontal lesions

      d.  Bilateral amygdala lesions

      e.  Bilateral thalamic lesions

77. A 59-year-old man was brought to a neurology clinic by his family with complaints of abnormal posturing of his right arm. A few months prior to presentation, he had been involved in a motor vehicle accident because he reported his “right hand was driving the steering wheel on its own” and he could not control it. On physical examination, he was found to have rigidity in the right arm with dystonic posturing. Sensory examination showed intact sensation to light touch, but impaired graphesthesia in the right hand. This presentation is most consistent with:

      a.  Progressive supranuclear palsy

      b.  Huntington’s disease

      c.  DLB

      d.  Pick’s variant of frontotemporal dementia

      e.  Corticobasal ganglionic degeneration

78. Which of the following lesions would not lead to a significant alteration in level of consciousness?

      a.  Bilateral hemispheric lesions

      b.  Bilateral midbrain infarct

      c.  Bilateral thalamic infarct

      d.  Bilateral dorsal pontine infarct

      e.  Bilateral occipital infarct

79. An 82-year-old man with moderate Alzheimer’s dementia is brought to the emergency department in respiratory distress due to aspiration pneumonia. He is intubated and mechanically ventilated, and has a prolonged ICU stay due to multiorgan failure in the setting of sepsis, and is now considered to be in a terminal state. After 6 days in the ICU, his physicians approach the family about goals of care. The patient’s best friend, who is his legal durable power of attorney for health care (and has the paperwork to prove it), states that the patient would not want to be on long-term mechanical ventilation and would not want to have a feeding tube. A living will to that effect is available that the patient had made several years earlier, when he had mild memory problems, but was otherwise cognitively normal. The patient’s son, who lives in another country and had flown in when his father was hospitalized, states that he wants “everything done” for the patient. Which of the following statements is most appropriate in the management of this patient?

      a.  The patient’s wishes as communicated by his power of attorney will be upheld though communication with the son in order to reconcile him with his father’s wishes if possible

      b.  Because the patient’s son is his next of kin, the wishes of the son will be followed

      c.  Consultation with a lawyer is necessary because there is discrepancy between the patient’s power of attorney and the family members

      d.  Because the patient had dementia, his living will is not valid even if he was of sound mind when the living will was made

      e.  Decisions regarding this patient’s management will be taken strictly by the medical team because there is disagreement among the patient’s friend and son

Answer Key

1. b

2. a

3. c

4. b

5. d

6. b

7. b

8. c

9. e

10. d

11. c

12. e

13. d

14. b

15. e

16. d

17. e

18. d

19. a

20. b

21. d

22. c

23. c

24. d

25. a

26. b

27. d

28. b

29. e

30. c

31. c

32. d

33. d

34. c

35. e

36. b

37. b

38. d

39. d

40. e

41. e

42. b

43. b

44. d

45. c

46. b

47. d

48. b

49. c

50. c

51. d

52. c

53. e

54. a

55. a

56. d

57. e

58. c

59. a

60. c

61. c

62. b

63. a

64. c

65. e

66. b

67. a

68. e

69. d

70. b

71. a

72. d

73. b

74. c

75. d

76. d

77. e

78. e

79. a

Answers

 1. b, 2. a

Mild Cognitive Impairment (MCI) is defined by cognitive impairment that does not interfere with activities of daily living, is not severe enough to classify the patient as demented, and patients retain general cognitive function. MCI is characterized by memory complaints (especially when noticed by friends and family), objective memory impairment for age and education, intact activities of daily living, and preserved general cognition. Impairments may be in one or more cognitive domains, such as attention, memory, language, executive, or visuospatial function. In general, MCI is classified as amnestic MCI (primarily memory), nonamnestic MCI (cognitive domain other than memory, such as language), or multiple-domain MCI (more than one cognitive domains affected). A gradual decline in cognition can be characteristic of normal aging, but it can often be differentiated from MCI by the degree of cognitive impairment. Amnestic MCI is often an early stage of Alzheimer’s disease, with a conversion rate to dementia of about 10% to 15% per year.

 Petersen RC, Smith GE, Waring SC, et al. Mild cognitive impairment: Clinical characterization and outcome. Arch Neurol. 1999; 56:303–308.

 Petersen RC, Stevens JC, Ganguli M, et al. Practice parameter: Early detection of dementia: Mild cognitive impairment (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2001; 56:1133–1142.

 Tierney MC, Szalai JP, Snow WG, et al. Prediction of probable Alzheimer’s disease in memory-impaired patients: A prospective longitudinal study. Neurology. 1996; 46:661–665.

 3. c, 4. b,  5. d,  6. b

According to the American Academy of Neurology guidelines, routine dementia screening should include assessment for vitamin B12, complete blood count, electrolytes, glucose, blood urea nitrogen, creatinine, liver function tests, thyroid function tests, and depression screening. Further testing including HIV, VDRL, lumbar puncture, and heavy metals are not recommended unless there is a specific clinical indication.

Alzheimer’s disease (AD) is the most common cause of dementia. Most inherited forms of AD are autosomal dominant and typically present before age 65, although these cases account for less than 5% of all cases of AD. Presenilin-2 is in chromosome 1 and leads to early-onset familial AD. Presenilin-1 is in chromosome 14 and accounts for 80% of early-onset aggressive familial cases of AD. Apolipoprotein E4 is in chromosome 19 and triples the risk of AD, as well as leads to an early age of onset, but does not lead to AD per se (only modifies the risk of AD). Amyloid precursor protein (APP) is in chromosome 21. Chromosome 2 is not related to AD.

Injury to any of the structures within the circuit of Papez will interrupt formation of new memories. The mediodorsal nucleus of the thalamus is not part of the circuit of Papez. The circuit of Papez is: entorhinal cortex → hippocampus → fornix → mamillary bodies → anterior nucleus of thalamus → cingulate gyrus → entorhinal cortex → hippocampus. Clinical neurologists often divide memory into stages. Immediate memory is the amount of information someone can keep in conscious awareness without active memorization. Immediate memory can be tested by forward digit span. Normal human beings can retain seven digits in active memory span. Working memory is tested by manipulation of information retained in immediate memory (such as adding two of the digits repeated in a number series). Recent memory involves the ability to register and recall specific items after a delay of minutes or hours. It requires the hippocampus and parahippocampal areas of the medial temporal lobe for storage and retrieval, which is why this type of memory is usually impaired first in early AD and is evident by impaired word recall. Remote memory is tested by asking about historical life events and long-known information.

Memory impairment is the essential and earliest clinical feature of AD. Declarative memory (facts and events) is significantly affected in AD. Of declarative memory, episodic memory (specific events and contexts) is the most impaired in early AD. Within episodic memory, memory for recent events is more prominently impaired in early AD compared with immediate or remote memory. Memory for facts such as vocabulary and concepts (semantic memory) is spared until later. Likewise, procedural memory and motor learning are also spared until later. Loss of visuospatial skills is also often an early feature of AD, which manifests as navigational difficulty in unfamiliar and later familiar areas and misplacement of items. Verbal dysfluency and anomia are also commonly early features and may be the presenting symptom. Other features often associated with later stages of AD include progressive language dysfunction, impaired executive function, lack of insight, neuropsychiatric symptoms, and apraxia. Inability to recognize objects and faces, or visual agnosia and prosopagnosia, respectively (see discussion to question 48), are a late feature.

 Ahmed S, Mitchell J, Arnold R, et al. Memory complaints in mild cognitive impairment, worried well, and semantic dementia patients. Alzheimer Dis Assoc Disord. 2008; 22:227–235.

 Guerin F, Belleville S, Ska B. Characterization of visuoconstructional disabilities in patients with probable dementia of Alzheimer’s type. J Clin Exp Neuropsychol. 2002; 24:1–17.

 Knopman DS, DeKosky ST, Cummings JL, et al. Practice parameter: Diagnosis of dementia (an evidence-based review): Report of the quality standards subcommittee of the American Academy of Neurology. Neurology. 2001; 56:1143–1153.

 Peters F, Collette F, Degueldre C, et al. The neural correlates of verbal short-term memory in Alzheimer’s disease: An fMRI study. Brain. 2009; 132:1833–1846.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

 7. b, 8. c,  9. e

The FDG-PET scan in Figure 12.1 shows bilateral parietotemporal hypometabolism, consistent with Alzheimer’s disease (AD). FDG-PET scanning may reveal decreased glucose metabolism in parietotemporal regions in AD, in the frontal and anterior temporal regions in FTD, in the head of the caudate in Huntington’s disease, and in the occipital regions in DLB. FDG-PET scanning in Parkinson’s disease usually does not show a decrease in metabolism.

All of the listed items in question 8 are commonly associated histopathologic findings seen in AD except for Lewy bodies, which would be the least commonly associated finding. Neuritic plaques and neurofibrillary tangles are the most specific, although granulovacuolar degeneration, amyloid deposition, and Hirano bodies are also commonly seen. Cortical Lewy bodies are classically found primarily in DLB, although there are occasionally overlapping histopathologic features between different types of dementia.

In AD, along with loss of cholinergic neurons in the nucleus basalis of Meynert, there is loss of choline acetyltransferase activity throughout the cortex, which correlates with the severity of memory loss. The locus coeruleus contains noradrenergic neurons, the median and dorsal raphe nuclei contain serotonergic neurons, the nucleus accumbens and ventral tegmental area contain dopaminergic neurons, and the substantia nigra pars reticulata contain GABAergic neurons (as opposed to the pars compacta, which contain dopaminergic neurons).

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Hoffman JM, Welsh-Bohmer KA, Hanson M, et al. FDG PET imaging in patients with pathologically verified dementia. J Nucl Med. 2000; 41:1920–1928.

 Prayson RA, Goldblum JR. Neuropathology, 1st ed. Philadelphia, PA: Elsevier; 2005.

10. d, 11. c

Parkinsonism, fluctuating cognitive impairment, and visual hallucinations make up the classic triad of DLB. Auditory hallucinations are not a typical finding in DLB. DLB is considered the second most common type of degenerative dementia after Alzheimer’s disease. In addition to dementia, other clinical features may include dysautonomia, sleep disorders, and neuroleptic sensitivity. Treatment is generally symptomatic and targeted toward specific disease manifestations. Acetylcholinesterase inhibitors may be of benefit. Typical neuroleptics are generally avoided due to significant sensitivity reactions, such as neuroleptic malignant syndrome, worsening parkinsonism, confusion, or autonomic dysfunction. If absolutely necessary, especially for agitated psychotic symptoms, atypical neuroleptics should be tried cautiously in low doses. Of the choices in question 11, haloperidol is the only typical neuroleptic listed, and should not be used.

 Ballard CG, O’Brien JT, Swann AG, et al. The natural history of psychosis and depression in dementia with Lewy bodies and Alzheimer’s disease: Persistence and new cases over 1 year of follow-up. J Clin Psychiatry. 2001; 62:46–49.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

 Stavitsky K, Brickman AM, Scarmeas N, et al. The progression of cognition, psychiatric symptoms, and functional abilities in dementia with Lewy bodies and Alzheimer disease. Arch Neurol. 2006; 63:1450–1456.

12. e

Pick’s disease is a disorder grouped under the FTDs (see discussion to questions 14–15). Of the listed choices, depression is least likely to be seen in Pick’s disease, although it can occur. Pick’s disease is manifested predominantly by frontal lobe symptoms such as personality changes, behavioral problems, apathy, abulia, and poor judgment. Aphasia can be present with temporal lobe involvement as well as features of Kluver-Bucy syndrome (see discussion to questions 21 and 76). Cognitive decline does occur, but memory impairment is not the most prominent feature, distinguishing the FTDs from Alzheimer’s disease.

 Josephs KA. Frontotemporal lobar degeneration. Neurol Clin. 2007; 25:683–696.

 Mendez MF, Shapira JS. Loss of insight and functional neuroimaging in frontotemporal dementia. J Neuropsychiatry Clin Neurosci. 2005; 17:413–416.

13. d

Lesions in the occipital lobe would not be associated with personality changes. Lesions in the dorsolateral frontal lobe cause symptoms such as personality changes, perseveration, apathy, and depression. Lesions in the orbitofrontal cortex may cause obsessive compulsive disorder traits, disinhibition, hypersexuality, anxiety, depression, impulsiveness, and antisocial behavior. Temporal lobe lesions can cause psychosis and memory disturbances. Caudate nucleus lesions can occasionally affect behavior. The occipital lobe underlies visual processing (see discussion to question 49), and is least likely to be associated with personality change (see discussion to questions 65 to 67 for a more extensive review of the frontal lobe syndromes).

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

14. b, 15. e

Visual hallucinations are not a common finding in FTD. In familial FTD, the most common genetic association is to chromosome 17q21, although chromosomes 3 and 9 have also been implicated in autosomal dominant inheritance. Most sporadic cases of FTD have not been linked to specific chromosomal sites. Linkage to the remaining chromosomes listed has been identified in some patients with familial Alzheimer’s disease.

In FTD, mean age of symptom onset is between 55 and 60 years. There are three major distinct clinical phenotypes of FTD. The behavioral variant FTD is the most common phenotype and symptoms include personality changes, abulia, apathy, social withdrawal, disinhibition, impulsivity, lack of insight, poor personal hygiene, stereotyped or ritual behaviors, change in eating patterns, suddenly new artistic abilities or hobbies, emotional blunting, loss of empathy, mental rigidity, distractibility, impersistence, perseverative behavior, and impaired organizational and executive skills. The patient depicted in question 14 appears to have this phenotype.

The second phenotype is progressive nonfluent aphasia and is characterized in early stages by anomia, word-finding difficulty, impaired object naming, and effortful speech with preserved comprehension. Spontaneous speech becomes increasingly dysfluent and speech errors become frequent. Behavior and social interaction remain unaffected until late in the disease at which point the patient becomes globally aphasic.

The third phenotype is semantic dementia, also called progressive fluent aphasia or the temporal variant of FTD. It is characterized by a progressive speech disturbance with normal fluency, but impaired comprehension, anomia, and semantic paraphasias. It may clinically resemble a transcortical sensory aphasia. There is typically a predominance of left temporal dysfunction, and/or in face and object recognition, reflecting right temporal dysfunction.

In addition, some patients with FTD may develop variant syndromes of motor impairment including motor neuron disease, progressive supranuclear palsy and corticobasal degeneration.

 Josephs KA. Frontotemporal lobar degeneration. Neurol Clin. 2007; 25:683–696.

 Kertesz A. Clinical features and diagnosis of frontotemporal dementia. Front Neurol Neurosci. 2009; 24:140–148.

 Knibb JA, Xuereb JH, Patterson K, et al. Clinical and pathological characterization of progressive aphasia. Ann Neurol. 2006; 59:156–165.

 Liu W, Miller BL, Kramer JH, et al. Behavioral disorders in the frontal and temporal variants of frontotemporal dementia. Neurology. 2004; 62:742–748.

 McKhann GM, Albert MS, Grossman M, et al. Clinical and pathological diagnosis of frontotemporal dementia: Report of the Work Group on Frontotemporal Dementia and Pick’s Disease. Arch Neurol. 2001; 58:1803–1809.

16. d

These FDG-PET scan images are consistent with FTD. FDG-PET reveals decreased glucose metabolism in the frontal and anterior temporal regions in FTD, parietotemporal regions in Alzheimer’s disease, the head of the caudate in Huntington’s disease, and the occipital regions in DLB. FDG-PET scanning in Parkinson’s disease usually does not show a decrease in cortical metabolism.

 Hoffman JM, Welsh-Bohmer KA, Hanson M, et al. FDG PET Imaging in Patients with Pathologically Verified Dementia. J Nucl Med. 2000; 41:1920–1928.

17. e

These symptoms would be most consistent with bilateral ACA infarcts. The key is identifying symptoms of frontal lobe dysfunction (such as those listed in the question) along with ACA territory dysfunction in terms of motor weakness. Recall the vascular distribution and the homunculus in which the legs are supplied by the ACA, where they are represented along the medial frontal cortex. Pontine infarcts can result in leg weakness and incontinence, but not frontal lobe symptoms. Lesions in the dominant temporal lobe could cause amnesia for verbal information and sensory, or Wernicke’s, aphasia. Lesions in the nondominant temporal lobe can cause amnesia for nonverbal and visuospatial information, as well as amusia.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

18. d

The dorsomedial nucleus has projections to dorsolateral prefrontal, orbitofrontal, anterior cingulate gyrus, and temporal lobe/amygdala. Dysfunction of this nucleus can result in the symptoms listed. The anterior nucleus is mostly involved in limbic relay and memory formation (part of the circuit of Papez). The pulvinar is involved in processing visual information and sensory integration. The ventral posterolateral nucleus is involved in sensory relay from the body, whereas the ventral posteromedial nucleus is involved in sensory relay from the face, both of which project to the somatosensory cortex.

 Carrera E, Bogousslavsky J. The thalamus and behavior effects of anatomically distinct strokes. Neurology. 2006; 66:1817–1823.

19. a

Bilateral globus pallidus interna lesions can cause akinetic mutism. In akinetic mutism, the patient generally has preserved awareness with open eyes, but remains immobile and mute and does not respond to commands. The globus pallidus interna is part of the anterior cingulate-frontal-subcortical circuit. Bilateral ACA infarcts and other lesions to the medial frontal lobes are other causes of akinetic mutism.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

20. b

Huntington’s disease is an autosomal dominant trinucleotide repeat disorder resulting from expansion of CAG repeats on chromosome 4p in a region that codes for the Huntingtin protein. The disease is associated with choreoathetosis and dementia. Chromosome 17 has been linked to FTD. Chromosomes 14, 19, and 21 are linked to some familial forms of Alzheimer’s disease.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

21. d

Pick’s disease has been associated with Kluver-Bucy syndrome (KBS), and is discussed in question 12. KBS is caused by lesions to bilateral anterior temporal lobes/amygdala and is characterized by hyperorality (tendency to explore objects with mouth), hypermetamorphosis (preoccupied with minute environmental stimuli), blunted emotional affect, hypersexuality, and visual agnosia (see discussion to question 76 for further review of KBS).

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

22. c

Disorders of higher cortical function often confer more disability than focal neurologic deficits after traumatic brain injury (TBI) and interfere with rehabilitation. Of these, alterations in personality interfere with rehabilitation the most. The other listed options frequently result from TBI, and may affect rehabilitation, but to a lesser degree. In addition, TBI can cause overlapping cognitive, physical, emotional, and behavioral symptoms. TBI is the result of a traumatic external force to the skull, injuring the brain. Causes include motor vehicle accidents, falls, sports injuries, and violence. It is a major cause of death and disability worldwide, especially in children and young adults because they are more likely to be involved in these scenarios. The trauma to the brain can be caused either by direct impact or acceleration, or both, which may lead to alterations in cerebral blood flow and intracranial pressure. Outcome can range from death or permanent disability to complete recovery.

 Brooks N, Mckinlay W, Symington C, et al. Return to work within the first seven years of severe head injury. Brain Inj. 1987; 1:5–19.

 Lezak MD, O’brien K. Longitudinal study of emotional, social, and physical changes after traumatic brain injury. J Learn Disabil. 1988; 21(8):456–463.

23. c

In transient global amnesia (TGA), recent memory is impaired. The pathophysiology of TGA is not well understood, but in at least some cases, it is thought to result from functional alterations in the bilateral medial temporal lobes. It has been associated with migraine, hypertension, medical procedures, and stressful events, among others. It typically lasts 12 to 24 hours and usually resolves without deficit. Clinically, the patients may ask the same questions over and over each time the examiners come into the room. They often forget meeting the examiner if the examiner leaves the room briefly and then returns. Immediate memory is usually spared and can be tested by digit span. Remote and procedural memory and personal identity are also retained. The primary clinical impairment in TGA is in recent (short-term) memory.

 Rae-Grant AD. Neurology for the House Officer. Philadelphia, PA: Lippincott Williams and Wilkins; 2008.

 Sander K, Sander D. New insights into transient global amnesia: Recent imaging and clinical findings. Lancet Neurol. 2005; 4:437–444.

24. d, 25. a, 26. b

This patient exhibits symptoms of DLB. Parkinsonism, fluctuating cognitive impairment, and visual hallucinations make up the classic triad of DLB. This disorder is discussed further in questions 10 and 11.

FDG-PET imaging of DLB reveals bilateral occipital hypometabolism greater than temporoparietal hypometabolism. Progressive supranuclear palsy (PSP) is associated with global metabolic reduction in various regions including the anterior cingulate, basal ganglia (especially caudate and putamen), thalamus, and upper brain stem. FTD shows frontotemporal hypometabolism. Alzheimer’s disease shows temporoparietal hypometabolism. FDG-PET scan is not typically useful in CJD; specific MRI findings are characteristic, making MRI a useful imaging modality in the diagnosis of CJD.

The histopathologic specimen obtained from the brain of this patient shows Lewy bodies, which are seen in DLB. Lewy bodies are cytoplasmic inclusions with anti-ubiquitin and anti-α-synuclein immunohistochemistry. Pick’s disease is defined pathologically by the presence of silver-staining, spherical aggregations of tau protein in neurons (Pick bodies). PSP is characterized by globose neurofibrillary tangles in neurons of subcortical nuclei and tufted astrocytes. The histopathology of Alzheimer’s disease is discussed in questions 37, 41, and 47, and CJD in question 38.

 Gold G. Dementia with Lewy bodies: Clinical diagnosis and therapeutic approach. Front Neurol Neurosci. 2009; 24:107–113.

 Hoffman JM, Welsh-Bohmer KA, Hanson M, et al. FDG PET imaging in patients with pathologically verified dementia. J Nucl Med. 2000; 41:1920–1928.

 Prayson RA, Goldblum JR. Neuropathology, 1st ed. Philadelphia, PA: Elsevier; 2005.

27. d

Psychogenic amnesia has the characteristic finding of loss of autobiographical memory, sometimes with preserved ability for new learning. Wernicke’s encephalopathy, which results from deficiency in thiamine (vitamin B1), as occurs in malnourishment, such as in alcoholism, is defined by the triad of confusion, ataxia, and ophthalmoplegia. Korsakoff’s disease, the chronic phase of thiamine deficiency, presents with anterograde and retrograde amnesia, and is classically associated with confabulation as a result of the poor memory. Transient global amnesia is transient and impairs recent memory while sparing immediate memory and remote memory.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

28. b, 29. e, 30. c, 31. c, 32. d

Memantine is the only listed drug that is not an acetylcholinesterase inhibitor. Memantine is a low-to-moderate affinity noncompetitive NMDA receptor antagonist and is approved for moderate-to-severe dementia in Alzheimer’s disease (AD). The other listed drugs in question 28 are acetylcholinesterase inhibitors. Tacrine is no longer available in United States due to hepatotoxicity.

Patients with AD have reduced cerebral production of choline acetyltransferase, which leads to a decrease in acetylcholine synthesis and impaired cortical cholinergic function. Donepezil, rivastigmine, galantamine, and memantine are all medications used in the treatment of dementia, most commonly AD. Donepezil is a pure acetylcholinesterase inhibitor. Rivastigmine is a combined acetylcholinesterase and butyrylcholinesterase inhibitor, both of which result in limiting the breakdown of acetylcholine. Galantamine is a combined acetylcholinesterase inhibitor and allosteric nicotinic modulator. Memantine is an NMDA receptor antagonist that inhibits glutamate stimulation and thus theoretically limits overactivation and toxicity to remaining cholinergic neurons. Memantine also has some antagonistic action at the 5-HT3 serotonin receptor.

 Qaseem A, Snow V, Cross JT Jr, et al. Current pharmacologic treatment of dementia: A clinical practice guideline from the American College of Physicians and the American Academy of Family Physicians. Ann Intern Med. 2008; 148:370–378.

 Raina P, Santaguida P, Ismaila A, et al. Effectiveness of cholinesterase inhibitors and memantine for treating dementia: Evidence review for a clinical practice guideline. Ann Intern Med. 2008; 149(5):358–359.

33. d

Major depression results in poor attention, and subsequently, immediate recall is often impaired, whereas short-term memory is typically preserved. Early Alzheimer’s disease and Korsakoff’s disease preserve immediate recall and show impaired learning and recall of new information. Transient global amnesia is also associated with retrograde and anterograde amnesia with preserved immediate recall.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Schatzberg AF, Posener JA, DeBattista C, et. al. Neuropsychological deficits in psychotic versus nonpsychotic major depression and no mental illness. Am J Psychiatry. 2000; 157(7):1095–1100.

34. c, 35. e

This elderly man fits the typical clinical scenario of delirium, and checking his medications and recent laboratory testing would be the first step in evaluation. Delirium is classically differentiated from dementia by its waxing and waning course as opposed to the steady cognitive impairment seen in dementia. His age, combined with the stress of major surgery, and probable exposure to postoperative pain medications are all contributors to this common occurrence. Infections such as urinary tract infection and pneumonia, as well as hypoxia (such as from pulmonary embolus), are also potential causes of delirium. Assessing his medications to look for contributors (especially narcotics, antihistamines, and anticholinergics) and routine metabolic studies should be checked. An arterial blood gas should be checked if hypoxia is suspected. Visual hallucinations are most often related to metabolic derangements and medications. Although stroke should be ruled out, it is unlikely on the basis of this constellation of symptoms (especially positive visual hallucinations). Seizures are also unlikely, although occipital lobe seizures can cause colorful geometric shapes to be seen. EEG would most likely show diffuse slowing, which would be typical of delirium. In this case, his symptoms are unlikely to be related to schizophrenia, as there is no prior history. In addition, hallucinations in schizophrenia are typically auditory, rather than visual. Therefore, there is no need for a psychiatry consult at this time. A lumbar puncture is not indicated at this time, although can be considered in the future if symptoms progress or do not improve, despite an unrevealing work-up.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

36. b

Multiple system atrophy (MSA) is considered a synucleinopathy, whereas the other listed choices represent tauopathies. The synucleins are a family of proteins that includes α-synuclein, β-synuclein, and γ-synuclein. Abnormal accumulation of these proteins results in the synucleinopathies and include Parkinson’s disease, DLB, MSA, and neuroaxonal dystrophies. The tauopathies are associated with the microtubule-associated protein, tau. Tau promotes microtubule polymerization and stabilization. Accumulation of this protein results in the tauopathies.

 Dickson DW. Neuropathology of non-Alzheimer degenerative disorders. Int J Clin Exp Pathol. 2010; 3(1):1–23.

 Prayson RA, Goldblum JR. Neuropathology, 1st ed. Philadelphia, PA: Elsevier; 2005.

37. b

The photomicrograph in Figure 12.5 shows histopathology consistent with Alzheimer’s disease (AD). It shows amyloid (neuritic) plaques, which are extracellular collections of amyloid protein deposited on dendrites and axons. They are composed of β-amyloid proteins. Amyloid plaques are a characteristic finding in AD. The other histopathologic findings in AD include intraneuronal neurofibrillary tangles (paired helical filaments made up of abnormally hyperphosphorylated tau protein), granulovacuolar degeneration (neuronal intracytoplasmic granule-containing vacuoles), amyloid angiopathy (amyloid deposition in the walls of small- and medium-sized arteries), and Hirano bodies (cytoplasmic inclusions composed mainly of actin and actin-associated proteins).

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Prayson RA, Goldblum JR. Neuropathology, 1st ed. Philadelphia, PA: Elsevier; 2005.

38. d, 39. d, 40. e

The histopathology shown in Figure 12.6 is consistent with CJD. The five human prion diseases are kuru, CJD, variant CJD (“mad cow disease”), Gerstmann-Straüssler-Scheinker syndrome, and fatal familial insomnia. These diseases share the neuropathologic features of neuronal loss, glial cell proliferation, absent inflammatory response, and vacuolization of the neuropil, which produces the characteristic spongiform appearance.

Sporadic CJD occurs at a rate of approximately 1/1,000,000 population per year. CJD is a rapidly progressive dementia associated with variable extrapyramidal/pyramidal tract signs, myoclonus, and ataxia, and death typically ensues within 1 year. Sporadic cases account for 85% to 95%, whereas 5% to 15% are familial, with an autosomal dominant pattern of inheritance. The pathology occurs when the normal prion protein (PrP), which is primarily an α-helical structure, converts into an abnormal form containing a higher percentage of β-pleated sheets. The abnormal form is insoluble, polymerizes and accumulates intracellularly, and is resistant to proteolysis. The prion protein gene (PRNP) coding for PrP is located on chromosome 20p. The other chromosomes listed all relate to Alzheimer’s disease. Polymorphism at codon 129 of the PRNP is believed to determine susceptibility for CJD, and homozygosity for either methionine or valine at codon 129 has a strong correlation with the various forms of CJD, including sporadic, iatrogenic, variant (“mad cow disease”) and familial forms. Neuroimaging findings in CJD include cortical ribbon sign, pulvinar sign, and increased T2 signal in the neocortex, thalamus, caudate, and putamen (see Chapter 15). Increased T2 signal in the globus pallidus is not a typical finding in CJD.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Lewis V, Hill AF, Klug GM, et al. Australian sporadic CJD analysis supports endogenous determinants of molecular-clinical profiles. Neurology. 2005; 65:113–118.

 Parchi P, Giese A, Capellari S, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol. 1999; 46:224–233.

 Prayson RA, Goldblum JR. Neuropathology, 1st ed. Philadelphia, PA: Elsevier; 2005.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

41. e, 42. b, 43. b

Figure 12.7 reveals neurofibrillary tangles (NFTs) associated with Alzheimer’s disease (AD). NFTs are intraneuronal collections of paired helical filaments made up of hyperphosphorylated tau protein. Negri bodies are seen in rabies, pick bodies are seen in Pick’s disease, and Bunina bodies are seen in amyotrophic lateral sclerosis.

Low level of education, repeated head trauma, and family history of dementia have been associated with increased risk of developing AD. The major risk factor for AD is aging. Gender has also been associated with risk, with female gender conferring a greater risk than male gender.

AD reveals hypometabolism of the posterior temporal and parietal regions on FDG-PET scan (discussed in question 7 and shown in Figure 12.1). To review, FDG-PET imaging of DLB reveals occipital hypometabolism greater than temporoparietal hypometabolism. Progressive supranuclear palsy is associated with global metabolic reduction, including anterior cingulate, basal ganglia (especially caudate and putamen), thalamus, and upper brain stem. FTD shows frontotemporal hypometabolism.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Dickson DW. Neuropathology of non-Alzheimer degenerative disorders. Int J Clin Exp Pathol. 2010; 3(1):1–23.

 Prayson RA, Goldblum JR. Neuropathology, 1st ed. Philadelphia, PA: Elsevier; 2005.

 Ryans NS, Fox NC. Imaging biomarkers in Alzheimer’s disease. Ann NY Acad Sci. 2009; 1180:20–27.

44. d, 45. c, 46. b

This patient demonstrates the classic triad of normal pressure hydrocephalus (NPH): cognitive dysfunction, gait impairment (often termed “magnetic gait”), and urinary incontinence. A brain MRI would be the best diagnostic test to start with and should reveal ventriculomegaly, which is out of proportion to cortical atrophy. It should be done prior to lumbar puncture to rule out intracranial masses, atrophy (with resultant ex-vacuo ventriculomegaly), and other pathology. In NPH, there may also be transependymal edema seen on MRI. A large-volume lumbar puncture (30 to 50 cc) with subsequent improvement in symptoms predicts a better chance of improvement from a shunting procedure. Therefore, the first choice of treatment should be a lumbar puncture, possibly followed by shunt procedure (depending on positive response to lumbar puncture). Patients with dementia for more than 2 years are less likely to improve with shunting.

 Shprecher D, Schwalb J, Kurlan R. Normal pressure hydrocephalus: Diagnosis and treatment. Curr Neurol Neurosci Rep. 2008; 8(5):371–376.

 Sudarsky L, Simon S. Gait disorder in late-life hydrocephalus. Arch Neurol. 1987; 44:263–267.

47. d

This histologic slide reveals granulovacuolar degeneration, associated with Alzheimer’s disease. Granulovacuolar degeneration results from the formation of abnormal neuronal intracytoplasmic granule-containing vacuoles. Lewy bodies are seen in DLB and are characterized by cytoplasmic inclusions with anti-ubiquitin and anti-α-synuclein immunohistochemistry. Pick bodies occur in Pick’s disease, and are characterized by silver staining, showing spherical aggregations of tau protein in neurons. Bunina bodies are seen in amyotrophic lateral sclerosis, and Negri bodies are seen in rabies.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Dickson DW. Neuropathology of non-Alzheimer degenerative disorders. Int J Clin Exp Pathol. 2010; 3(1):1–23.

 Prayson RA, Goldblum JR. Neuropathology, 1st ed. Philadelphia, PA: Elsevier; 2005.

48. b

Agnosia is loss of ability to recognize stimuli while the specific sense to detect the stimuli is not impaired. Prosopagnosia is the inability to recognize faces. Ability to recognize people using other cues is often preserved. Face recognition is thought to be a function of the right hemisphere, but prosopagnosia most commonly occurs with bilateral lesions of the temporo-occipital regions (bilateral fusiform gyri), as occurs with bilateral PCA infarction. It can also be seen as part of more diffuse processes that preferentially affect the temporal lobes, such as Alzheimer’s disease. Associated features may include achromatopsia (a disorder of color perception) and visual field deficits.

Topographagnosia, a defect in spatial orientation, is marked by inability to navigate in familiar places, read maps, draw floor maps of familiar places, and perform similar functions. It localizes to the nondominant posterior parahippocampal region, infracalcarine cortex, or nondominant parietal lobe.

Asomatognosia is marked by an indifferent inability to recognize one’s own body part. It most often localizes to the contralateral (usually nondominant) superior parietal lobule, the supramarginal gyrus, and/or its connections. In somatoparaphrenia, a form of asomatognosia, the patient denies ownership of a limb(s) and claims the limb is missing, or has been stolen.

Misoplegia is severe hatred of a limb, a rare form of agnosia seen in hemiparetic or hemiplegic patients following stroke. The patient may attempt to cut off the limb or damage it otherwise.

 Campbell W. Dejong’s The Neurological Examination, 6th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2009.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

49. c

The case describes Balint’s syndrome (also known as Balint-Holmes syndrome), which localizes to the bilateral parieto-occipital region. This syndrome consists of a triad of optic ataxia (a deficit of reaching for objects under visual guidance), oculomotor apraxia (gaze apraxia, inability to voluntarily move the eyes to a new point of visual fixation despite normal extraocular muscle function), and simultanagnosia (inability to visually perceive more than one object at a time). This syndrome has been described with a variety of pathologies, including neurodegenerative disorders, progressive multifocal leukoencephalopathy, bilateral watershed infarcts, and malignancy.

In general, though there are exceptions, lesions of the dorsal visual pathways that pass through the parieto-occipital regions can be thought of as leading to an abnormality in detecting “where”: where an object is in space, how to reach that object while looking at it. Lesions of the ventral, temporo-occipital pathways lead to an abnormality of detecting “what”: what an object is.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

50. c

Anton’s syndrome, also known as Anton-Babinski syndrome or visual anosognosia, manifests as cortical blindness with denial of visual loss and confabulation. This syndrome results from bilateral lesions of the medial occipital lobes (primary visual and visual association cortices). A delusional disorder is not suspected. In psychogenic blindness, confabulation does not typically occur and optokinetic nystagmus is typically preserved. A language disorder is not depicted here; receptive aphasia is discussed in question 53. Balint’s syndrome is discussed in question 49.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

51. d

Charles Bonnet syndrome, a form of release hallucinations, is a condition marked by vivid hallucinations that occur in people with severe visual impairment due to a variety of reasons, most commonly ophthalmologic. Patients acknowledge that these images are hallucinations. This may be mistaken for a delusional disorder or psychosis, but the patient’s insight into the fact that these are hallucinations helps rule this out. Anton’s syndrome is discussed in question 50. Balint’s syndrome is discussed in question 49.

 Cammaroto S, D’Aleo G, Smorto C, et al. Charles Bonnet syndrome. Func Neurol. 2008; 23(3):123–127.

52. c

This patient exhibits the syndrome of alexia without agraphia. Alexia is a loss of reading comprehension despite normal visual acuity. Ability to read individual letters of a word is often retained. Writing and language comprehension are normal in alexia without agraphia. It is a disconnection syndrome, due to lesions in the dominant (usually left) PCA territory, commonly involving the medial and inferior occipitotemporal region and splenium of the corpus callosum. The patient has a contralateral (usually right) homonymous hemianopia. Although the ipsilateral visual field is intact, words that are seen cannot be effectively read, as the lesion in the splenium of the corpus callosum prevents them from being transmitted to Broca’s area. This is not a disturbance of language per se, unlike Wernicke’s aphasia (discussed in question 53), and is not a neglect syndrome. Given the history, examination, and MRI findings, a psychogenic disorder is highly unlikely. Cortical blindness, or Anton’s syndrome, is associated with vision loss (see question 50).

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

53. e

Pure word deafness, or verbal auditory agnosia, is marked by impaired auditory comprehension of language, though hearing per se (of tones and other nonverbal sounds) is intact; audiogram is normal in these patients. There is normal comprehension of written language, distinguishing it from Wernicke’s (sensory) aphasia; Wernicke’s aphasia is characterized by inability to comprehend, read, or repeat, with fluent, nonsensical speech. The lesion causing pure word deafness is most often in the bilateral middle portion of the superior temporal gyri, sparing Wernicke’s area, but disrupting its connections with the primary auditory cortex (Heschl’s gyrus) and temporal lobe association cortices. Cases have been reported with unilateral dominant temporal lobe lesions. This may be associated with amusia, or agnosia for music.

In nonverbal auditory agnosia, there is agnosia to sounds, such as the sounds animals make or environmental sounds. This most often occurs with bilateral anterior temporal lesions, though nondominant temporal lobe lesions can lead to this as well.

Anomia is inability to name objects with otherwise relative preservation of language expression and comprehension. Patients are able to recognize objects but cannot name them. Anomia usually occurs in association with other features of Broca’s (expressive) aphasia (see question 54), though it may occur in isolation, particularly during recovery of a Broca’s (expressive) aphasia. Anomia may occur with a variety of lesions, including dominant hemisphere posteroinferior frontal gyrus and temporal lesions. It has also been reported to occur in angular gyrus syndrome, due to lesions of the dominant angular gyrus, in association with Gertsmann’s syndrome (discussed in question 70) and constructional difficulties.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

54. a

Aphemia, or pure word mutism, also referred to as verbal apraxia, is marked by an inability to speak fluently, impaired repetition, and intact auditory comprehension. A pure Broca’s (expressive) aphasia is characterized by inability to speak, write, name, or repeat, but intact comprehension. In aphemia, there is retained ability to write and comprehend written language. The lesion is in the dominant frontal operculum, anterior and superior to Broca’s area (posteroinferior frontal gyrus). Receptive aphasia is discussed in question 53; conduction aphasia is discussed in question 58; transcortical motor aphasia is discussed in question 57; and alexia without agraphia is discussed in question 52.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

55. a

Foix-Chavany-Marie syndrome, also known as anterior opercular syndrome, is characterized by severe dysarthria, bilateral voluntary paralysis of the lower cranial nerves with preserved involuntary and emotional innervation. This syndrome is associated with bilateral anterior opercular lesions, frequently in the setting of multiple infarcts.

 Mao CC, Coull BM, Golper LAC, et al. Anterior operculum syndrome. Neurology. 1989; 39:1169–1172.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

56. d

Transcortical sensory aphasia can be thought of as a Wernicke’s (receptive) aphasia, but with intact repetition. Transcortical sensory aphasia may be seen in dominant hemisphere MCA-PCA territory watershed infarction, thalamic lesions (thalamic aphasia), and in neurodegenerative disorders such as Alzheimer’s disease. Transcortical aphasias are also seen in the stages of recovery from other aphasia syndromes. Broca’s (expressive) aphasia is discussed in question 54; Wernicke’s (receptive) aphasia is discussed in question 53; conduction aphasia is discussed in question 58; and aphemia is discussed in question 54.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

57. e

This man exhibits a transcortical motor aphasia, which can be thought of as a Broca’s (expressive) aphasia, but with intact repetition. This aphasic syndrome is seen with a variety of cortical and subcortical dominant hemisphere lesions in the frontal lobe, but is most commonly seen in two general settings. The first occurs in the setting of watershed infarcts in the dominant hemisphere ACA-MCA watershed territory, sparing connections between Wernicke’s and Broca’s area, but impairing speech output due to disruption of connections between Broca’s area and the supplementary motor area. The supplementary motor area is located in the medial aspect of the superior frontal gyrus and can be thought of as the pacemaker for speech output. Second, transcortical motor aphasia is seen in the recovery phases of a Broca’s (expressive) aphasia.

 Goetz CG. Textbook of Clinical Neurology, 3rd ed. Philadelphia, PA: Saunders Elsevier; 2007.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

58. c

The internal arcuate fasciculus connects Wernicke’s area in the superior temporal gyrus to Broca’s area in the inferior frontal gyrus. Lesions in this fasciculus lead to conduction aphasia, in which repetition is impaired but other aspects of language are intact. Broca’s (expressive) aphasia is discussed in question 54 and Wernicke’s (receptive) aphasia is discussed in question 53. Aphasia resulting from thalamic lesions is often a transcortical sensory aphasia (discussed in question 56).

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

59. a

Amelodia or affective motor aprosodia localizes to the nondominant posteroinferior frontal gyrus, the nondominant hemisphere’s analogue to Broca’s area. Similarly, the inability to perceive and understand the emotional content of others’ speech, sensory or receptive aprosodia, localizes to the nondominant posterosuperior temporal gyrus, the nondominant hemisphere’s analogue to Wernicke’s area.

 Mesulam M. Principles of Behavioral and Cognitive Neurology, 2nd ed. New York: Oxford University Press; 2000.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

60. c, 61. c

This man suffers from pseudobulbar affect, also known as involuntary emotional expression disorder. The pathophysiology of pseudobulbar affect is complex, but it most often occurs in patients with bilateral lesions that disconnect the corticobulbar tracts from the brain stem cranial nerve nuclei. It is commonly seen in patients with diffuse subcortical dysfunction, as occurs with amyotrophic lateral sclerosis, multiple sclerosis, and following traumatic brain injury, but has also been reported in patients with focal mass lesions or acute infarctions. A dextromethorphan-quinidine combination has been shown to reduce and sometimes even eliminate pseudobulbar affect in patients with a variety of neurologic disorders.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

 Brooks BR, Thisted RA, Appel SH, et al. Treatment of pseudobulbar affect in ALs with dextromethorphan/quinidine: A randomized trial. Neurology. 2004; 63(8):1364–1370.

 Panitch HS, Thisted RA, Smith RA, et al. Randomized, controlled trial of dextromethorphan/quinidine for pseudobulbar affect in multiple sclerosis. Ann Neurol. 2006; 59(5):780–787.

62. b

In this patient, an infarct in the superior division of the left middle cerebral artery resulted in a Broca’s aphasia combined with right arm weakness. His left Wernicke’s area is intact, but because of disconnection between Wernicke’s area, left premotor cortex, and right premotor cortex, left-hand apraxia occurred. This is a rare disconnection syndrome.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

63. a

Apraxia is characterized by an impaired ability to execute a previously known movement. This woman exhibits ideomotor apraxia, which is suggested by use of a body part as an object during pantomime. Patients with ideomotor apraxia understand the movement that they are supposed to execute and achieve the general, overall movement, but exhibit abnormal postures and spatial errors. Ideomotor apraxia is seen with lesions in the dominant parietal cortex, in or around the area of the superior marginal and angular gyrus.

The dominant feature of conduction apraxia is impairment in imitation of movements. The localization of conduction apraxia is not well defined.

Ideational apraxia is characterized by impairment in the sequence of motions needed to carry out a specific movement. When asked to pantomime pouring a glass of water and drinking from it, patients with ideational apraxia will, for example, drink from the cup before pouring water into it. Ideational apraxia is seen in patients with bifrontal or biparietal dysfunction, as occurs in neurodegenerative disorders.

Disassociation apraxia is characterized by inability to execute a movement on command, but with normal ability to imitate. It has most commonly been reported to occur in the left hand in left hemispheric language-dominant patients who have left MCA territory lesions.

Conceptual apraxia is characterized by misconception of the function of objects in the environment. For example, a patient with conceptual apraxia may use a fork to eat soup or may pretend to use a screwdriver when asked to pantomime hammering a nail into a wall. Conceptual apraxia is seen with diffuse neurodegenerative processes, as well as with lesions in the nondominant hemisphere.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Goetz CG. Textbook of Clinical Neurology, 3rd ed. Philadelphia, PA: Saunders Elsevier; 2007.

64. c

Dressing apraxia localizes to the right parietal lobe. It often occurs in the setting of a neglect syndrome.

 Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology, 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

65. e, 66. b, 67. a

The prefrontal cortices (the areas of the frontal lobes anterior to the primary motor cortex) have extensive connections with various cortical and subcortical areas including the limbic system, basal ganglia, thalamus, and brain stem. The prefrontal cortex can be functionally divided into orbitofrontal, dorsolateral, and dorsomedial areas. Lesions of the frontal lobes, or disruptions in frontal-subcortical pathways, lead to a variety of syndromes that have extensive overlap, but can be broadly categorized on the basis of the most prominent features occurring with lesions to the different prefrontal regions.

The orbitofrontal cortex is located on the ventral aspect of the frontal lobes. It is involved in judgment, inhibition of socially inappropriate behaviors, as well as emotional and visceral functions. Lesions to this area, which commonly include trauma and olfactory groove or sphenoid wing meningiomas, lead to changes in personality, social disinhibition, facetiousness, inappropriate jocularity (witzelsucht), echopraxia, and utilization behavior (mimicking of use of objects in the environment), as depicted in question 65.

The dorsomedial prefrontal cortex is involved in motor initiation, goal-directed behavior, and motivation. Lesions to this area and bilateral lesions to the anterior cingulate, which commonly result from ACA infarcts and tumors, lead to apathy, indifference, loss of initiative, amotivation, and abulia, or a reduction (or in severe cases abolition) of movement and communication due, in part, to involvement of the supplementary motor area, as depicted in question 66. In extensive bilateral lesions to the dorsomedial prefrontal cortex, the most severe form of this nonparalytic akinesia, akinetic mutism, results. Because the paracentral lobule (the mesial projection of the somatosensory cortex) is involved in voluntary urinary continence, lesions to this area are often associated with urinary incontinence.

The dorsolateral prefrontal cortex is involved in the planning of motor activity and behavior, executive functioning, judgment, and problem solving. Lesions to this area lead to impaired judgment, impaired ability to plan, multitask, and problem solve, and anhedonia, or a lack of interest in previously pleasurable activities, as depicted in question 67.

Associated physical examination findings with frontal lobe pathology include frontal release signs, including grasp, suck, palmomental, snouting, and rooting reflexes.

 Goetz CG. Textbook of Clinical Neurology, 3rd ed. Philadelphia, PA: Saunders Elsevier; 2007.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

68. e

The Grooved Pegboard Test is a test of finger dexterity. The patient is timed as he/she places pegs into small grooved holes in a board. The grooves are oriented in different directions, requiring the patient to rotate the peg in their fingers, which increases the demands for distal dexterity. Right and left hands are performed separately, and the patient’s time is compared to normative data.

The Trail-Making Test part A times the patient as he/she connects numbers on a page, and is a test of simple speed of processing, visual search, and attention. The Trail-Making Test part B requires the patient to connect consecutive numbers and letters; in addition to the demands of Trails A, it requires set shifting (shifting between numbers and letters) and working memory (maintaining the correct sequence).

The Wisconsin Card Sorting Test requires the patient to arrange cards on the basis of a specific concept. It is a test of frontal lobe function, and assesses visual conceptualization and set shifting.

The Random Cancellation Test, a measure of visual attention and processing speed, assesses the ability to visually scan and identify specific targets in a large array of similar items.

In the Clock-Drawing Test, a test of visuospatial function but also auditory comprehension, attention, and executive function, the patient is asked to draw a clock (including the numbers) with the hands set at a specific time.

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Goetz CG. Textbook of Clinical Neurology, 3rd ed. Philadelphia, PA: Saunders Elsevier; 2007.

69. d

The case describes Capgras’ syndrome, which is characterized by the delusional belief that a person, often a member of the patient’s immediate family, is an identical-looking imposter.

Other delusional misidentification disorders include Fregoli’s syndrome (in which the patient believes that the same person exists in several disguises), intermetamorphosis (the belief that individuals have swapped identities with each other while maintaining the same external appearance), reduplicative paramnesia (which overlaps significantly with Capgras’ syndrome, but there is a delusion that there are identical places/objects rather than just people), and Cotard’s delusion (a person’s belief that they are dead or dying). Pseudocyesis is not classified under the delusional misidentification disorders, but is a delusion that a person is pregnant when they are in fact not; the patient may manifest the signs and symptoms of pregnancy. It is more common in females, but has been reported in males.

Delusional misidentification disorders and other types of delusions can be seen in neurodegenerative disorders such as Alzheimer’s dementia and Lewy body dementia, as well as in patients with structural brain lesions such as traumatic brain injury and cerebral infarction. Lesions are usually bifrontal or right hemispheric. Delusional misidentification disorders are also seen in primary psychiatric disorders such as schizophrenia, affective disorders, and delusional disorder.

 Devinsky O. Delusional misidentifications and duplications. Right brain lesions, left brain delusions. Neurology. 2009; 72:80–87.

 Forstl H, Almeida OP, Owen AM, et al. Psychiatric, neurological and medical aspects of misidentification syndromes: A review of 260 cases. Psychol Med. 1991; 21:905–910.

 Sadock BJ, Sadock VA, Ruiz P, eds. Kaplan and Sadock’s Comprehensive Textbook of Psychiatry, 9th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2009.

70. b

This man exhibits the features of Gerstmann’s syndrome, which is characterized by the tetrad of finger agnosia (inability to identify fingers bilaterally), right-left confusion, dyscalculia (inability to carry out calculations), and dysgraphia (inability to write). It localizes to the dominant inferior parietal lobule, particularly the dominant angular gyrus. Common causes include infarction of the inferior division of the MCA, in which case there may be associated contralateral visual field deficits.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

71. a

An infarct in the right MCA territory would be the best explanation for this patient’s physical examination findings. Lesions to the primary somatosensory cortex lead to cortical sensory loss, manifesting as loss of two-point discrimination, agraphesthesia (inability to perceive letters or numbers drawn on the hand), astereognosis (inability to recognize shapes manipulated in the hand), and extinction to bilateral simultaneous stimulation. These findings localize the lesion to the cortex, as opposed to brain stem or subcortical areas.

 Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology, 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

72. d

This boy exhibits anosognosia, or a lack of awareness of an acquired neurologic deficit, and hemispatial neglect syndrome, consistent with a lesion in the nondominant hemisphere involving the primary somatosensory cortex (area SI). A lesion in the thalamus can also lead to a neglect syndrome.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

73. b

The frontal eye fields are located in the middle frontal gyrus, and each frontal eye field is responsible for conjugate eye movement to the contralateral side. A lesion to the right frontal eye fields causes unopposed activity of the left frontal eye fields, leading to eye deviation to the right. A left hemiparesis with conjugate eye deviation to the right suggests a lesion in the right MCA territory. Focal motor seizures that involve the frontal eye fields lead to contralateral hemibody convulsions with conjugate gaze deviation to the side of the seizure manifestations (contralateral to the seizure focus). The parapontine reticular formation leads to ipsilateral conjugate gaze, and a lesion in the right pons would lead to a left hemiparesis and left gaze deviation due to unopposed action of the left parapontine reticular formation. A lesion in the left pons would lead to right hemiparesis with gaze deviation to the right. Gaze deviation with pontine lesions is not overcome by oculocephalic maneuver.

 Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology, 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

74. c

Damage to the posterolateral thalamus gives rise to Dejerine-Roussy syndrome, or thalamic pain syndrome, which is characterized by initial contralateral hemianesthesia followed weeks later by pain, hyperesthesia, and allodynia. A similar delayed central pain syndrome can occur with a lesion to the medial lemniscus, dorsal columns, or with lesions to the parietal operculum (the latter is also termed pseudothalamic syndrome). The spinothalamic tract projects to the ventral posterolateral nucleus of the thalamus, which in turn projects to the secondary somatosensory cortex (area SII). A lesion to the primary somatosensory cortex (area SI) would lead to contralateral loss of sensation to touch, joint position sense, and vibration, but would spare pain and temperature sensation, and would not cause a delayed pain syndrome.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

75. d

Declarative, or explicit memory, involves memory for facts or experiences. Declarative memory includes semantic knowledge, or knowledge for facts and objectives, and episodic knowledge, or knowledge of events. Nondeclarative, or implicit, memory involves memory of skills and other acquired behaviors. Lesions to the bilateral medial temporal lobes leads to loss of predominantly declarative (explicit) memory, leading to an anterograde amnesia, with a retrograde amnesia involving a specific period prior to injury, but usually with preservation of more remotely formed memories. On the other hand, there is not a specific lesion that would lead to loss of nondeclarative memory in general.

 Blumenfeld H. Neuroanatomy through Clinical Cases, 1st ed. Sunderland, MA: Sinauer Associates; 2002.

76. d

This man exhibits features of Klüver-Bucy syndrome, which is seen with bilateral medial temporal lobe lesions, involving the amygdala. Clinical features may include hyperorality, visual agnosia, hypersexuality, blunted emotional affect (docility), hypokinesia, and hypermetamorphosis (over-attention to minute stimuli in the environment). It is seen following herpes simplex encephalitis, with neurodegenerative disorders such as FTD, following anoxic-ischemic injury to the temporal lobes, and after bilateral temporal lobectomy (a historical procedure) (see question 21 for further discussion of Klüver-Bucy syndrome).

 Bradley WG, Daroff RB, Fenichel GM, et al. Neurology in Clinical Practice, 5th ed. Philadelphia, PA: Elsevier; 2008.

 Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology, 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

77. e

The vignette most likely describes corticobasal ganglionic degeneration (CBGD), in which alien limb syndrome occurs. The phenomenon of alien limb is marked by movement of a limb, sometimes seemingly purposefully, but not under voluntary control. Alien limb syndrome also occurs with lesions to the contralateral ACA territory, involving the corpus callosum or supplementary motor area. Progressive supranuclear palsy, Huntington’s disease, and CBGD are discussed further in Chapter 6. DLB is discussed in questions 10 and 11, and Pick’s disease in question 12.

 Ropper AH, Samuels MA. Adams and Victor’s Principles of Neurology, 9th ed. New York: McGraw-Hill; 2009.

 Rowland LP. Merritt’s Neurology, 11th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2005.

78. e

Consciousness is maintained by the function of a variety of structures, including the reticular activating system in the brain stem, the thalamus (particularly the intralaminar nuclei), and the frontal lobes, particularly the medial aspects. In order to significantly affect consciousness, these areas must be involved. Lesions to the occipital cortices would not be expected to affect consciousness per se, but would rather cause various visual disturbances including visual field deficits and cortical blindness.

 Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology, 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

79. a

This patient has a living will that clearly states his wishes, and has appointed a durable power of attorney for health care to make decisions on his behalf. The wishes of the patient, as communicated by his power of attorney, should be upheld. The history suggests that the patient had full mental capacity at the time of drafting of the living will, and in such cases, it should be upheld even if he later loses capacity to make decisions for himself. Even though the son is next of kin, legal appointment of a durable power of attorney supersedes the word of the next of kin. Although continued communication with the son is important, in order for him to better accept his father’s wishes and the management plans for him, his father’s wishes will be enforced regardless of the son’s ultimate acceptance. Involvement of the courts is not necessary because there is evidence that the patient has appointed his best friend as power of attorney and because a living will is available. Advanced directives, including living wills and durable power of attorney for health care, allow patients to exercise autonomy in their medical care even when they are unable to communicate at a specific time of medical decision making.

 Jonsen A, Siegler M, Winslade W. Clinical Ethics: A Practical Approach to Ethical Decisions in Clinical Medicine, 6th ed. New York: McGraw-Hill; 2006.