Many times over, emergency departments are visited by patients with refractory severe headaches. Within this melee of patients traveling through the emergency department are some with a potentially life-threatening condition, uncommon in frequency but devastating if not recognized. The emergency department physician and neurologist are commonly held responsible for their triage.1,2 The dilemma of improper “playing it safe” with a series of tests throwing up false-positives or running the risk of litigation due to incomplete investigations has become a major area of contention.2,3 Not all patients require neuroimaging studies or cerebrospinal fluid (CSF) examination; in fact, most do not. However, a split-second onset of persistent severe (“terrible”) headache typically indicates aneurysmal subarachnoid hemorrhage (see Chapter 13), and evaluation is urgently needed. In addition, this chapter considers other neurologic or nonneurologic disorders responsible for acute headache syndromes.
Minute analysis of onset and character of the presenting headache is a skill, and most of the time the diagnosis is reached after exposing characteristic features. These warning signs are shown in Table 6.1. Most disorders are acute neurologic conditions; in addition, acute severe headache may indicate equally serious disorders, such as acute sphenoid sinusitis, a first manifestation of malignant hypertension, or acute-angle glaucoma (Table 6.2). All of these disorders need different therapeutic approaches but should be considered by the neurologist.
A thunderclap headache should receive all attention. This headache refers to a split-second, extremely intense, totally unexpected headache that has not been experienced before and the patient feels as if struck by lightning (thunderclap) or as if the top of the head was blown off (like a volcano). When a loud handclap or finger snap is demonstrated, to indicate the sudden onset, patients will recognize that. Headache of this character may be short in maximal intensity but may persist for hours or be brief.1
Subarachnoid hemorrhage remains the main diagnostic consideration, and the diagnosis is established by computed tomography (CT) in 98 of 100 cases if seen within 12 hours of onset. The vast majority will be due to aneurysmal rupture.
Unfortunately, clinical signs, such as nuchal rigidity (rarely in the first hours), retinal or subhyaloid hemorrhage (predominantly patients in a very poor condition from a subarachnoid hemorrhage), or cranial nerve deficits (third nerve or sixth nerve palsy), are uncommon leads or absent in a ruptured aneurysm. Other infrequent conditions have been associated with thunderclap-like headaches, all very serious (Table 6.3).
Thunderclap headache may be without any objective abnormalities on neuroimaging (CT and all other magnetic resonance [MR] modalities) and CSF examination but this benign form is rather uncommon. The term for this clinical entity was coined by Day and Raskin4 but is also known as crash or blitz migraine.5 Some patients may go on to develop common migraine but not invariably so, and the link with established types of migraine is uncertain. Onset associated with exertion or orgasm is relatively common in thunderclap headaches.6 In a few patients, diffuse segmental vasospasm has been found by MR angiography (MRA) and angiogram.7 The phenomenon is not explained well, but an increased sympathetic tone has been proposed.7 Nifedipine may be helpful in some cases.8 Recurrences do occur in 10%–15% of cases, mostly within the first 6 months, but outcome is very good.9,10,11,12
Table 6.1. Warning Signs in Acute Headache
Table 6.2. Acute Severe Headache Syndromes from Nonneurologic Causes
Table 6.3. Symptomatic Thunderclap Headache Other than Subarachnoid Hemorrhage
Table 6.4. “Benign” Acute Headache Syndromes
Status migrainous, refractory trigeminal neuralgia, and cluster headache are other causes for acute severe headache.13 Severe-intensity, pulsating unilateral headache is aggravated by normal physical activity and associated with nausea and vomiting. Photophobia and sonophobia are common features in all of these disorders, but other differences of these more or less benign headaches are apparent (Table 6.4).
Refractory trigeminal neuralgia is characterized by episodic electrical sharp jabs of facial pain triggered by facial touch, chewing, talking, tooth brushing, and is commonly refractory to medication. Doses have been so high that intolerance has become a limiting factor.
Refractory cluster headache is fairly certain when patients present with excruciating retroorbital forehead, jaw, or cheek pain following the first division of the trigeminal nerve, with lacrimation, nasal congestion, ptosis, and eyelid swelling. Attacks last approximately 1 hour and are commonly accompanied by restlessness and rocking motions.13
Line of Action
The critical steps in patients with a new thunderclap headache are shown in Figure 6.1.
Subtle subarachnoid hemorrhage can be very difficult to detect (Fig. 6.2). If the CT scan is negative, CSF would still be able to document xanthochromia up to 2 weeks.14,15,16 However, CSF examination should be deferred until 4 hours have passed, to allow detection of xanthochromia from hemolysed erythrocytes freeing up oxyhemoglobin. CSF examination should include cell count, protein, and CSF pressure before sampling, as well as assessment of xanthochromia (see Color Fig. 6.3 in separate color insert).17,18 Spectrophotometry would be a valuable method to prove xanthochromia that is due to bilirubin or oxyhemoglobin, but the technique is not used in the United States. Conditions other than aneurysmal subarachnoid hemorrhage have only been recognized after MRI and thus undermine the generally held tenet that CSF and CT should be sufficient to exclude underlying causes. No series of MRIs have been published on patients with thunderclap headaches; thus, categorical recommendations to proceed with MRI or MRA in all cases are not solidly based on data. MRI has documented thunderclap headache associated with hypertensive encephalopathy, pituitary apoplexia, signs of intracranial hypotension and meningeal enhancement, and retroclival hematoma.19,20,21,22 MRA has documented carotid or vertebral artery dissection23 but should include the entire region from the origin of the arch to the circle of Willis to scrutinize for arterial dissection. Magnetic resonance venography may discover cerebral venous thrombosis. However, in retrospect, each of these disorders had other clinical or laboratory clues suggesting the diagnosis.
Figure 6.1 Critical steps in the evaluation of acute headache. CT, computed tomography; CSF, cerebrospinal fluid; MRI/A, magnetic resonance imaging/ angiography; MRV, magnetic resonance venography.
Figure 6.2 Serial computed tomographic scans of missed “warning leak.” Very subtle hemorrhage in the interhemispheric fissure in a patient with thunderclap headache initially interpreted as normal (a and b). Follow-up 2 months later (c and d) documents more dramatic presentation of ruptured anterior communicating artery aneurysm after recurrent severe headache.
Box 6.1. The Triptans
It is postulated that triptans inhibit the peripheral trigeminal nerve terminals and central transmission within the medulla sensory nuclei. They have an additional potent vasoconstrictor effect on cerebral meningeal and dural arteries. These effects are mediated through binding and activation of specific 5-hydroxytryptaminc receptors and thus are classified as serotonin agonists.
Major adverse effects involve facial flushing, tingling, and chest discomfort, which may be due to constriction of coronary arteries. Various triptans are available with different pharmacologic profiles, but comparison studies are scarce. Improvement of headache at 2 hours is approximately 60% with 100 mg of sumatriptan, with approximately 30% pain-free at 2 hours.25,26
In uncertain cases or in patients with ambiguous results, it is appropriate to proceed with a four-vessel cerebral angiogram. A cerebral angiogram may also seem appropriate in patients with a personal or family history of subarachnoid hemorrhage and disorders associated with cerebral aneurysms (e.g., polycystic kidney disease).14 In all other patients, a cerebral angiogram is probably not recommended if neurologic examination, CT, and CSF are normal. There are very few data on patients with a thunderclap headache, a normal CT and CSF, and uncontrovertible evidence of a recent rupture during visual inspection of the aneurysm by the neurosurgeon. Presence of an unruptured aneurysm can be coincidental, and the link between its discovery in patients with thunderclap headache may be a result of aggressive pursuit in evaluation of signs.24
Many patients with a migraine attack respond well to 900 mg of aspirin, 1000 mg of acetaminophen, or high doses of nonsteroidal antiinflammatory agents. However, triptans (Box 6.1) and droperidol have been used,27 including in the emergency department. Contraindications include familial hemiplegic migraine, basilar migraine, ischemic stroke, ischemic heart disease, Prinzmetal's angina, uncontrolled hypertension, combination with monoamine oxidase inhibitors or ergot compounds, and pregnancy.25,26 Abortive treatments for patients with “therapy-resistant, persistent” headache lasting for hours are shown in Table 6.5.28,29,30,31,32,33,34,35,36 This designation of severity is very difficult to define, and the boundaries with rebound headache and analgesic-induced headache are not always that clear. The success of each of these pharmaceutical approaches in this more severe state comes more from anecdotal clinical experience than clinical trials.
Table 6.5. Abortive Therapies in Unrelenting Head Pain
A randomized trial of intravenous magnesium sulfate and metoclopramide in migraine showed a less favorable response than with metoclopramide alone.37 The effect of dexamethasone may be much less than originally claimed.
Antiepileptic drugs, antispasticity drugs, and tricyclic antidepressants, often in combination, may not be successful in refractory trigeminal neuralgia. In these patients, a preliminary study has shown that fosphenytoin loading (15–20 mg/kg) was rapidly successful.38 Lamotrigine or topiramate may take weeks to exert a maximal effect. Other options are surgical, including ganglionic opioid analgesia, stereotactic radiosurgery, microvascular decompression, or percutaneous balloon compression.28,39,40,41
The response of cluster headache to subcutaneous sumatriptan or oxygen by nasal cannula is excellent, and refractory cases are quite uncommon.42 The trigeminal nerve, at least its peripheral position, may not be of major importance in generating cluster headache, because cluster headaches have remained after section of the nerve, and response to sumatriptan remained.43 Intranasal lidocaine or corticosteroids are good abortive second-line therapies.44 The options for severe cluster headache are shown in Table 6.5.
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