A 30-year-old man complains of unilateral headaches. He was diagnosed with migraine headaches at age 24. The headaches did not respond to triptan therapy at that time, but after 6 weeks the headaches resolved. He has had three or four spells of severe headaches since then. Currently his headaches have been present for the past 2 weeks. The headaches start with a stabbing pain just below the right eye. Usually the affected eye feels “irritated” (reddened with increased lacrimation). He saw an optometrist during one of the episodes and a miotic pupil was noted. Each pain lasts from 60 to 90 minutes, but he may have several discrete episodes each day. The neurological examination, including cranial nerve examination, is now normal.
What is your best approach to treatment at this time?
The history is classic for cluster headache, an often debilitating periodic pain syndrome. The typical cluster lasts for weeks and then remits. Like classic migraines, cluster headaches are unilateral and can be associated with autonomic symptoms (including Horner syndrome) on the symptomatic side. The following chart helps you to distinguish cluster headache from migraine:
Treatment of cluster headache involves two principles: (1) aborting the cluster and (2) relieving the headache when it occurs. Prednisone is usually given to abort the cluster; 40 to 60 mg per day is given for weeks and then tapered over a month or two. Propranolol and tricyclic antidepressants (which are given for migraine prevention) are much less helpful in the patient with cluster headache. Verapamil and carbamazepine are sometimes used if prednisone is ineffective. It is harder to relieve the individual headache in cluster disorder because each episode of pain is of shorter duration than in migraine. Triptans or high-flow oxygen (7-10 L per minute via face mask) may be effective. The pain in cluster headache is very severe, and suicides have occurred when the patient enters another stereotypical cluster. Proper diagnosis and treatment are therefore crucial. Neuroimaging studies are not indicated unless atypical features or focal neurological findings are present. Neuropsychiatric testing is expensive and would not be indicated in this patient with classic cluster headache.
A 47-year-old dentist consults you because of tremor, which is interfering with his work. The tremor has come on gradually over the past several years and seems more prominent after the ingestion of caffeine; he notices that, in the evening after work, an alcoholic beverage will decrease the tremor. No one in his family has a similar tremor. He is otherwise healthy and takes no medications. On examination his vital signs are normal. Except for the tremor, his neurological examination is normal; in particular there is no focal weakness, rigidity, or bradykinesia. When he holds out his arms and extends his fingers, you detect a rapid fine tremor of both hands; the tremor goes away when he rests his arms at his side.
What is the best next step in the management of this patient?
This patient’s action tremor (ie, brought out by sustained motor activity) and otherwise normal neurological examination are diagnostic of essential tremor. Fifty percent of patients will have a positive family history (benign familial tremor). The tremor is termed “benign” to separate it from Parkinson disease and other progressive neurological diseases and because it does not affect other areas of function; however, about 15% of patients (especially those in professions that require fine motor control) will be functionally impaired. An identical rapid fine action tremor can be seen in normal persons after strenuous motor activity or with anxiety. Hyperthyroidism, caffeine overuse, alcohol withdrawal, and use of sympathomimetic drugs (such as cocaine and amphetamines) can cause an identical tremor and can exacerbate the tremor in familial cases.
Neurological imaging is normal in patients with essential tremor. The EMG is nonspecific. This patient has no features (eg, weakness, fasciculations) to suggest motor neuron disease. Patients with essential tremor are managed with medications, especially beta-blockers, to decrease the severity of the tremor. Most neurologists feel that nonselective beta-blockers (blocking both beta-1 and beta-2 receptors) are most effective. They can be used on an “as needed” basis (ie, before performance of fine tasks) if the patient is not troubled by the tremor at other times. Primidone is also effective but is associated with more side effects, especially at higher doses.
A 35-year-old previously healthy woman suddenly develops a severe headache while lifting weights. A minute later she has transient loss of consciousness. She awakes with vomiting and a continued headache. She describes the headache as “the worst headache of my life.” She appears uncomfortable and vomits during your physical examination. Blood pressure is 140/85, pulse rate is 100/minute, respirations are 18/minute, and temperature is 36.8°C (98.2°F). There is neck stiffness. Physical examination, including careful cranial nerve and deep tendon reflex testing, is otherwise normal.
Which of the following is the best next step in evaluation?
An excruciating headache with syncope requires evaluation for subarachnoid hemorrhage (SAH). This occurs with rupture of an intracranial aneurysm, usually located at an arterial bifurcation in the anterior cerebral circulation. Rupture may occur spontaneously or at times of exercise. About 2% of persons have these aneurysms, and about one-fifth of these have multiple aneurysms. Fortunately only a small percentage of these persons ever experience rupture, which may be fatal. The headache that precedes or accompanies SAH is severe and often described as a “thunderclap” headache, meaning that it reaches its maximum intensity in seconds. Migraine may also cause severe headache, but usually reaches maximum intensity in 5 to 30 minutes. Syncope occurs in about one-half of patients with SAH and is thought to be due to accompanying cerebral artery spasm. Blood in the cerebrospinal fluid tends to irritate the meninges and may cause neck stiffness. Suspected subarachnoid hemorrhage mandates CT scanning as the initial test. In about 90% of patients, there will be enough blood to be visualized on a noncontrast CT scan. A contrast CT scan sometimes obscures the diagnosis because, in an enhanced scan, normal arteries may be mistaken for subarachnoid blood. If the CT scan is normal, a lumbar puncture will establish the diagnosis by demonstrating blood in the cerebrospinal fluid (CSF). As opposed to CSF blood from a traumatic lumbar puncture, the CSF blood does not clear with continued collection of fluid. Cerebral angiography is usually done to assess the need for surgery and to detect other aneurysms, but it is usually delayed because angiography may precipitate spasm, especially if performed right after the acute rupture. Holter monitor might be helpful in unexplained syncope but would not address the severe headache. Electroencephalography is sometimes used to diagnose seizures in a patient with unwitnessed and unexplained syncope, but would not be appropriate until subarachnoid hemorrhage has been excluded.
A 58-year-old man complains of the sudden onset of syncope. It occurs without warning and with no sweating, dizziness, or light-headedness. He believes episodes tend to occur when he turns his head too quickly or sometimes when he is shaving. Physical examination is unremarkable. He has no carotid bruits, and cardiac examination is normal.
Which of the following is the best way to make a definitive diagnosis in this patient?
When syncope occurs in an older patient as a result of head turning, wearing a tight shirt collar, or shaving over the neck area, carotid sinus hypersensitivity should be considered. It usually occurs in men above the age of 50. Baroreceptors of the carotid sinus are activated and pass impulses throughthe glossopharyngeal nerve to the medulla. Some consider the process to be quite rare. Gentle massage of one carotid sinus at a time may show a period of asystole or hypotension. This should be performed in a controlled setting with monitoring and atropine available. Most cases of carotid sinus hypersensitivity are not associated with significant carotid stenosis; if a carotid bruit is heard on physical examination, however, a duplex study should precede carotid massage. More expensive studies, such as Holter monitoring or electrophysiologic study, would be unnecessary if carotid sinus massage demonstrates the diagnosis.
An 82-year-old woman is evaluated for progressive dementia. She is on no medications; the family has not noticed urinary incontinence or seizure activity. Her MMSE score is 21 out of 30; she has no focal weakness or reflex asymmetry on physical examination. MR scan shows a 2.4-cm partly calcified, densely enhancing mass near the falx (shown below). There is no surrounding edema or mass effect.
What is the best approach to this patient’s management?
This patient has an asymptomatic meningioma, the commonest CNS tumor. The radiographic picture of a densely enhancing tumor near the surface of the brain is essentially diagnostic, and biopsy is not necessary. An occasional patient will have bony overgrowth of the skull as a result of the hypervascular tumor; such a patient may notice a change in the contour of the skull. Almost all meningiomas are benign and grow slowly. Many are discovered incidentally during CNS imaging for other problems. While large or symptomatic meningiomas are usually treated with surgical resection, this patient’s tumor should be followed at 3 to 6 month intervals with serial CT scans. Radiation therapy is unnecessary. Ventriculoperitoneal shunting would be indicated only if neuroimaging studies showed hydrocephalus. Phenytoin is used if seizures occur; seizures are less common in meningioma than in glial tumors that arise within the brain parenchyma. This patient’s tumor would not account for her intellectual decline (bilateral cortical disease is necessary to affect higher intellectual function), and craniotomy with resection in the very elderly often causes more problems that it treats.
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