96. The answer is c. (Ropper, p 166.) Classic migraine, but not common migraine, is preceded by an aura of neurological dysfunction. The aura is most often visual in nature, consisting of bright flashing lights, scintillating scotomas, or field cuts. Both kinds of migraine are most often characterized by a hemicranial throbbing headache associated with nausea, vomiting, photophobia, and phonophobia (aversion to sound). Familial patterns are not unusual with either classic or common migraine, although with classic migraine the probability that another family member will have a similar problem approaches 80%.
97. The answer is c. (Ropper, pp 298-299.) As with classic migraine, with basilar migraine, women are more susceptible than men, disturbances of vision are common, the aura usually resolves within 10-to-30 minutes, and the headache invariably follows, rather than precedes, the neurological deficits; however, the character and severity of neurological deficits associated with basilar migraine are distinct. The visual change may evolve to complete blindness. Irritability may develop into psychosis. Rather than a mild hemiparesis, the patient may have a transient quadriplegia. Stupor, syncope, and even coma may appear and persist for hours.
98. The answer is e. (Ropper, pp 182-183.) This woman probably has trigeminal neuralgia (tic douloureux). The treatment options for this facial pain disorder include carbamazepine. Although carbamazepine is a potent antiepileptic medication, other antiepileptic medications, such as pheno-barbital and divalproex sodium, are usually ineffective in blunting the pain. Phenytoin and gabapentin are other antiepileptics useful in the management of trigeminal neuralgia. Analgesics and anti-inflammatory drugs, such as indomethacin, are notably ineffective in managing this disorder.
99. The answer is a. (Ropper, pp 883, 884.) Multiple sclerosis is often associated with trigeminal neuralgia, which is then termed symptomatic trigeminal neuralgia because it occurs as a symptom of another illness. This is caused by a plaque within the brainstem. Other causes of symptomatic trigeminal neuralgia include basilar artery aneurysms, acoustic schwannomas, and posterior fossa meningiomas, all of which may cause injury to the fifth cranial nerve by compression. The Tolosa-Hunt syndrome is a presumably inflammatory disorder that produces ophthalmoplegia associated with headache and loss of sensation over the forehead. Pupillary function is usually spared, and the site of pathology is believed to be in the superior orbital fissure or the cavernous sinus. It is usually not associated with trigeminal neuralgia.
100. The answer is e. (Ropper, pp 185-186.) Unlike patients with trigeminal neuralgia, who describe paroxysmal, lancinating pains, patients with atypical facial pain usually feel a constant, deep pain. Although atypical facial pain is often bilateral, it may be unilateral and fairly limited in its distribution. The cheek, nose, or zygomatic regions are often affected by this idiopathic pain syndrome. The pain is often sensitive to antidepressant medication, a characteristic that has led some to suggest that the syndrome is invariably caused by depression. Progressive loss of sensation in the distribution of the fifth cranial nerve (CN) should prompt a careful search for an underlying malignancy invading the nerve either intracranially or in the face.
101. The answer is a. (Ropper, p 172.) This patient has common migraine. Of the agents listed, only sumatriptan is generally considered of use to abort a headache. The triptans are a group of medications that act as agonists at serotonergic receptors (specifically, the 5HT-1 receptors), and they have been found to be very effective at stopping migraine headaches. Vera-pamil and amitriptyline hydrochloride may be used as prophylactic (preventative) therapy. Phenobarbital is an anticonvulsant and is not typically used to treat migraine. Nitroglycerine can actually precipitate headaches in susceptible individuals. Nausea is a frequent accompaniment of migraine. Metoclopramide hydrochloride may be effective in relieving the nausea, but it also reduces gastric stasis, which can retard absorption of oral medications. Certain antiemetics, such as prochlorperazine, may relieve nausea and also provide relief from the headache itself. Additional agents that might be of benefit in abortive therapy include ibuprofen, aspirin, acetaminophen, isometheptene, or ergotamine.
102. The answer is d. (Ropper, pp 166-174.) Several medications are effective as prophylactic agents in the treatment of migraine. These include amitriptyline hydrochloride, propranolol, verapamil, and valproate. Most experts recommend initiating prophylactic therapy only when headaches occur at least one to two times per month. Metoclopramide hydrochloride, sumatriptan, and ergotamine tartrate are appropriately used to treat an acute attack of migraine and should not be prescribed on a daily basis. Daily use of these medications can establish a rebound syndrome that results in a chronic daily headache. OCPs may be associated with either an increase or decrease in the frequency of migraines, but are not generally used as a treatment for migraine. Some experts recommend not prescribing OCPs for patients with migraine for fear of increasing the risk of a stroke, although OCPs are probably safe to use in most patients with common migraine.
103. The answer is a. (Ropper, pp 176-177.) The history is typical for a tension-type headache. These headaches are often associated with neck muscle spasm leading to reduced neck range of motion and paracervical tenderness. Papilledema and neuroimaging abnormalities would be associated with headaches caused by an intracranial mass. The EEG is generally normal in patients with headaches, unless there is underlying damaged brain.
104. The answer is a. (Ropper, pp 166-174.) Classic migraine is usually familial, involves a unilateral, throbbing head pain, and diminishes in frequency with age. The blind spot, or scotoma, that may develop as part of the aura of a classic migraine attack will involve the same visual field in both eyes. This defect usually changes over the course of minutes. It typically enlarges and may intrude on the central vision. The margin of the blind spot is often scintillating or dazzling. If this margin has a pattern like the battlement of a castle, it is called a fortification spectrum, or teichopsia. Homonymous hemianoptic defects of the sort that develop during the aura of a classic migraine indicate an irritative lesion that is affecting one part of the occipital cortex in one hemisphere of the brain. The changes in the scotoma over the course of minutes indicate that the irritative phenomenon sets off a cascade of events in the visual cortex that temporarily disturbs vision in a progressively larger area. Other focal neurological phenomena may precede classic migraine; the most common are tingling of the face or hand, mild confusion, transient hemiparesis, and ataxia. Fatigue, irritability, and easy distractibility often develop before a migraine. Affected persons usually also have hypersensitivity to light and noise during an attack.
105. The answer is d. (Ropper, pp 182-183.) Trigeminal neuralgia may develop in the context of multiple sclerosis—an association suggested by this woman’s other neurological problems. The development of trigeminal neuralgia (tic douloureux) indicates that demyelination has probably extended to the brainstem and may be involving trigeminal nerve connections. A more detailed history would probably reveal that the patient has had pain in the eye that now has disturbed vision. This is expected with the optic neuritis, which is typically associated with multiple sclerosis. Other symptoms commonly reported at this age by patients with previously undiagnosed multiple sclerosis include bed wetting, changes in speech, and gait instability.
106. The answer is b. (Ropper, pp 174-176.) The term cluster headache refers to the tendency of these headaches to cluster in time. They may be distinctly seasonal, but the triggering event is unknown. The pain of cluster headache is usually described as originating in the eye and spreading over the temporal area as the headache evolves. In contrast to migraine, men are more often affected than women, and extreme irritability may accompany the headache. The pain usually abates in less than 1 hour. Affected persons routinely have autonomic phenomena associated with the headache that include unilateral nasal congestion, tearing from one eye, conjunctival injection, and pupillary constriction. The autonomic phenomena are on the same side of the face as the pain. These phenomena are similar to those elicited by the local action of histamine and gave rise to the now largely abandoned term Horton histamine headaches.
107. The answer is f. (Ropper, pp 178-179.) The greatest risk from temporal arteritis is loss of vision in association with the headache. The erythrocyte sedimentation rate is usually dramatically elevated, and the abolition of symptoms with corticosteroid therapy is equally dramatic. Temporal arteritis is largely nonexistent in persons less than 50 years of age and rare in those less than 60. Many patients exhibit persistent fevers and progressive weight loss. The temporal arteries are likely to be pulseless or at least thickened. Biopsy of the artery often reveals a giant cell arteritis.
108. The answer is j. (Ropper, pp 183-184.) The rash preceding the facial pain was probably caused by herpes zoster, a virus that erupts in the severely ill elderly and in immunosuppressed persons. The virus is manifested earlier in life as chickenpox and remains dormant for decades in most people. Tricyclic drugs, such as imipramine hydrochloride, are often more useful than analgesics in suppressing the pain associated with this postviral syndrome.
109. The answer is b. (Ropper, pp 601-605.) Pseudotumor cerebri, or idiopathic intracranial hypertension, is a condition of unknown cause that results in increased intracranial pressure, predominantly affecting obese women in their childbearing years. Symptoms include headaches, transient visual obscurations, progressive visual loss, pulsatile tinnitus, diplopia, and shoulder and arm pain. Neurological examination shows papilledema or optic atrophy if the syndrome has been long standing, and occasionally sixth nerve palsies may be present. Neuroimaging must be performed to exclude mass lesion or venous sinus obstruction, which can also lead to a similar syndrome of intracranial hypertension. Spinal fluid examination should be normal except for an elevated opening pressure. Additional causes of intracranial hypertension include systemic lupus erythematosus, renal disease, hypoparathyroidism, radical neck dissection, vitamin A intoxication, and steroid withdrawal. Treatment options include lumbar puncture, diuretics, ventriculoperitoneal shunting, and optic nerve sheath fenestration.
110. The answer is d. (Ropper, pp 180-181.) Thunderclap headache refers to the syndrome of the sudden onset of a very severe headache with no apparent structural cause. When a patient presents with “the worst headache of my life,” the initial concern should always be for a subarachnoid hemorrhage, particularly in the presence of meningismus, focal deficits, or a change in the level of consciousness. CT scanning is indicated to exclude hemorrhage, but because CT may be negative in up to 5% to 10% of cases of subarachnoid hemorrhage, lumbar puncture is necessary if CT is negative to exclude small amounts of blood. Some reports have suggested that even in the absence of blood on a lumbar puncture, an underlying aneurysm may still be the cause of acute, severe headache, because sudden changes in the wall of the aneurysm may provoke severe pain. These reports would suggest that angiography should be performed in all such patients to exclude aneurysm. It remains unknown, however, whether these cases represent coincidental occurrence of thunderclap headache and an incidental, asymptomatic aneurysm. Several series have shown that many patients with thunderclap headache tend to go on to develop more typical migraine, raising the possibility that the thunderclap headache is simply the initial presentation of their migraine.
111. The answer is h. (Ropper, pp 606-607.) Headaches that occur on standing indicate the presence of intracranial hypotension. Most often, this is the result of recent lumbar puncture, either for diagnostic purposes or after spinal anesthesia. The hole in the dura created by the spinal tap presumably allows fluid to continue leaking out, and this creates a condition of decreased pressure within the spinal canal, which causes traction on the pain-sensitive meninges of the brain. Other causes of intracranial hypotension include continued leak of cerebrospinal fluid (CSF) from the subarachnoid space after head trauma, neurosurgery, or even pneumonectomy (thoracoarachnoid fistula); occult pituitary tumor; a leak from a dural tear in the spinal root sleeves; traumatic nerve root avulsion; or systemic illness such as dehydration, diabetic coma, uremia, or meningoencephalitis. With leakage of CSF into nasal passages, the patient may complain of rhinorrhea. In some cases, no cause is apparent even after a thorough evaluation.