370. The answer is b. (Fauci, 14/e, pp 2307-2311, 2377.) Cluster headaches are often referred to as "suicide headaches" because of the severity of the symptoms. These recurring headaches are accompanied by facial flushing, nasal stuffiness, tearing, and a partial Horner syndrome (there is no anhidrosis). They are more common in men (the usual age is 20-50) than women and are exacerbated by alcohol use. Migraine headaches do not have this timing or duration. Tension headaches are bilateral, nonthrobbing, and symmetric. They are usually located in the frontal or occipital areas of the skull and are thought to be related to muscle contraction. They are often described as being viselike. The headache of sinusitis is not abrupt in onset or cessation and patients often have tenderness with percussion of the sinuses. Trigeminal neuralgia (tic douloureux) is a paroxysmal severe facial pain over the distribution of the trigeminal nerve. Women are affected more than men, and patients are usually over the age of 40. The pain of trigeminal neuralgia can be triggered by simply touching the skin near the nostril.
371. The answer is a. (Fauci, 14/e, pp 1120, 2409-2414, 2366, 2382, 2387-2388.) The patient most likely has multiple sclerosis, a demyelinat-ing disease characterized by visual impairment, an afferent pupillary defect (Marcus Gunn pupil) diplopia, nystagmus, limb weakness, spasticity, hyperreflexia, extensor plantar reflexes, vertigo, ataxia, dysarthria, scanning speech, emotional lability, and bladder dysfunction. Patients with optic neuritis are at risk for developing blindness. Friedreich's ataxia is an autosomal recessive disease in which young patients present with pes cavus foot deformity, spasticity, areflexia, ataxia, and cardiomyopathy. Patients with acute transverse myelitis initially present with back pain followed by weakness and loss of sensation below the level of the pain. Often, there may be bladder and bowel incontinence. Transverse myelitis may be seen after vaccination or infections. Brown-Sequard syndrome (cord hemisection) is characterized by contralateral loss of pain and temperature and ipsilateral spasticity, weakness, hyperreflexia, extensor plantar reflex, and loss of pro-
prioception (vibration and position sense). Patients with syringomyelia have bilateral paralysis, muscle atrophy, and fasciculations along with pain and temperature sensory loss in a shawl-like or capelike distribution.
372. The answer is d. (Fauci, 14/e, pp 2325-2346.) There are three types of stroke: subarachnoid hemorrhage, cerebral infarction, and intra-cerebral hemorrhage. This patient presents after complaining of a severe headache. She has neck stiffness and no focal deficit on neurologic exam. The loss of consciousness requires bihemispheral dysfunction, and this along with the abrupt history is most consistent with a subarachnoid hemorrhage (SAH). Common causes of SAH include ruptured aneurysm (i.e., berry) and arteriovenous malformation (AVM). Intracerebral hemorrhage (ICH) rarely produces coma (must be significantly large to do so) and patients do not complain of headache (does not involve the meninges). Patients with ICH have focal deficits that appear abruptly and slowly progress over hours. An embolic stroke can involve any carotid artery but must be bilateral to cause loss of consciousness. Patients often have a history of atrial fibrillation or cardiac problems.
373. The answer is d. (Fauci, 14/e, pp 1007, 2368-2371.) Amyotrophic lateral sclerosis (ALS) is a degenerative disease that is the result of lower (anterior horn cells) and upper (corticospinal tracts) motor neuron loss. Patients present with asymmetric muscle weakness, atrophy, fasciculations, spasticity, hyperactive reflexes, and extensor plantar reflexes. Patients may complain of dysphagia and difficulty holding the head up. Pott's disease is tuberculosis of the thoracic vertebral bodies. Todd's paralysis is a transient paralysis following a seizure. Werdnig-Hoffmann disease is "floppy baby" disease; infants present with fasciculations. Poliomyelitis is a lower motor neuron disease.
374. The answer is c. (Seidel, 14/e, pp 285-286.) Internuclear ophthalmoplegia (INO) is caused by a lesion in the medial longitudinal fasciculus (MLF) and may be due to glioma in children, multiple sclerosis in young adults, or vascular infarction in the geriatric age group. INO commonly causes paresis of adduction of the ipsilateral eye (patients cannot look medially), horizontal nystagmus in the contralateral abducting eye, vertical nystagmus with upward gaze, and intact convergence.
375. The answer is b. (Fauci, 14/e, pp 167, 1989-1990.) Patients with pseudotumor cerebri (benign intracranial hypertension) present with headache and papilledema. They are often obese women in their childbear-ing years. Other possible causes include hypervitaminosis A and the use of oral contraceptives or antibiotics (tetracycline). Lumbar puncture will reveal an elevated opening pressure. Treatment includes weight reduction and repeated lumbar punctures to reduce intracranial pressure. A complication of pseudotumor cerebri is blindness; patients with visual changes may require emergency optic nerve sheath decompression. Pituitary adenomas are benign tumors that may cause a bitemporal hemianopsia and endocrine disturbances, such as hyperprolactinemia (galactorrhea), acromegaly or gigantism, and Cushing's disease. A ruptured berry aneurysm causes a subarachnoid hemorrhage (SAH). Patients present with the acute onset of severe headache, photophobia, and neck stiffness. Adults commonly have supratentorial primary brain tumors (astrocytoma including glioblas-toma multiforme is the most common), while children have infratento-rial primary brain tumors (medulloblastoma is the most common). Overall, metastatic brain tumors are more common than primary brain tumors. The most common metastatic brain tumors come from the Lung, Breast, Skin, Kidney, GI tract (mnemonic: Lots of Bad Stuff Kills Glia). The headache of tumor is often continuous; exacerbated by coughing, sneezing, movement, and Valsalva maneuver; and worse in the morning.
376. The answer is c. (Seidel, 4/e, p 775.) The corneal reflex is normal when touching the cornea (trigeminal nerve provides sensation) causes bilateral eye closure (facial nerve provides motor). This reflex will not occur on the side of a facial nerve paralysis.
377. The answer is c. (Sapira, pp 479-480.) The test for the oculocephalic or "doll's eyes" reflex is performed by rapidly rotating the head from side to side. If the brainstem is intact in a comatose patient, the eyes will move con-jugately in the direction opposite to the head rotation. If the brainstem is not intact, the eyes will move disconjugately or not at all. The oculovestibular or caloric reflex is performed by introducing ice water into the external auditory canal. The comatose patient with an intact brainstem will respond with deviation of the eyes to the side of the irrigation. If the brainstem is not intact, the reflex will be absent or the eyes will move disconjugately.
378. The answer is a. (Fauci, 14/e, pp 2455,2504,2506-2507.) The triad of nystagmus and paralysis of eye muscles, ataxia, and confusion is associated with Wernicke syndrome. Korsakoff syndrome consists of confabulation, confusion, and recent memory loss. These disorders are often found in thi-amine (B1)-deficient malnourished alcoholics and are secondary to lesions in the mamillary bodies. Niacin deficiency (pellagra or vitamin B3 deficiency) causes the triad of D's (Dementia, Dermatitis, and Diarrhea). Kluver-Bucy syndrome is due to lesions in the amygdala; patients present with hypersex-uality, compulsive attention to detail, docile behavior, and an inability to recognize objects visually (agnosia). Delirium tremens is seen 48-96 h following abstinence from alcohol; patients present with insomnia, confusion, tremors, delusions, visual hallucinations, and hyperactivity of the autonomic nervous system (i.e., sweating, tachycardia, fever, and dilated pupils).
379. The answer is e. (Fauci, 14/e, pp 2300-2301, 2463.) Acute inflammatory polyneuropathy or Guillain-Barre syndrome is a progressive, symmetrical, autoimmune demyelinating disorder that affects distal areas first (legs) and marches proximally to involve the arms, trunk, and intercostal, neck, and cranial muscles. Patients often have an antecedent viral infection (respiratory or gastrointestinal) or a history of a recent immunization. Patients are areflexic and have sensory and motor deficits with cranial nerve involvement. Poliomyelitis is a viral meningoencephalitis that destroys the anterior horn cells and causes an asymmetric flaccid weakness with fasciculations and hyporeflexia (lower motor neuron). Charcot-Marie-Tooth disease (CMT) is an inherited, slowly progressive peripheral sensory-motor neuropathy causing distal muscle atrophy ("inverted champagne bottle legs" or "stork legs") and sensory loss. Patients with CMT typically have pes cavus or hammer toe foot deformities.
380. The answer is a. (Seidel, 4/e, pp 777-778.) The tongue will deviate to the left with a left hypoglossal nerve palsy. The nerve is purely motor.
381. The answer is e. (Fauci, 14/e, pp 2467-2468.) The patient most likely has carpal tunnel syndrome (CTS), which is compression of the median nerve by the transverse volar ligament of the wrist. Patients complain of pain and paresthesias of the hand and weakness and atrophy of the thenar muscles. The Tinel sign (tapping the median nerve at the wrist) and Phalen sign (forced wrist flexion) intensify the symptoms. Risk factors for CTS include pregnancy, diabetes mellitus, hypothyroidism, rheumatoid arthritis, amyloid infiltration as seen in patients with multiple myeloma, acromegaly, and repetitive trauma. Ulnar nerve paralysis causes a "claw hand" deformity. Radial nerve palsy causes wristdrop. Erb-Duchenne palsy (C5-C6) causes weakness of the shoulder and elbow and results in the "waiter's tip" position (arm dangles at the side with palm in a backward position with fingers flexed). Klumpke-Dejerine palsy (C8-T1) is a triad of claw hand deformity, absent triceps reflex, and Horner syndrome. Patients with cervical radicu-lopathy (C6 or C7 root) complain of neck pain that radiates to the arm (radicular pain), dermatomal sensory loss, and decreased reflexes.
382. The answer is e. (Seidel, 4/e, pp 788, 900.) Clonus is rapidly alternating involuntary contraction and relaxation of skeletal muscle. Deep tendon reflex (DTR) response is graded on a scale from 0 to 4+:
0 = No response 1+ = Sluggish or diminished response 2+ = Active or expected response
3+ = More brisk than expected and slightly hyperactive response 4+ = Intermittent or transient clonus; hyperactive and brisk response
383. The answer is c. (Seidel, 4/e, p 780.) The Romberg test is performed by having the patient stand with feet together, head erect, and eyes open. The patient is then examined for steadiness and then asked to close his or her eyes. A positive test occurs when the patient displays increased unsteadiness with the eyes closed but not with the eyes open. A positive Romberg test may be seen in diseases that affect the dorsal columns, such as tabes dorsalis and vitamin B
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