Life Long Learning in Neurology MULTIPLE-CHOICE QUESTIONS Prepared By Neurology Residents Team Medical City Hospitals-Baghdad iraqineurologist@yahoo.com iraqineurologist@yahoogroup.com © 2008 All rights reserved. Continuum Contents Internal medicine 1 Neurogenetics 9 Spinal Cord Disorder 22 Stroke prevention 30 Critical care neurology 41 Infectious disease 49 Muscle disease 61 Neuro-otology 70 Psychiatry for neurologist 79 Headache 88 Movement disorder 99 Dementia 107 Sleep disorder 116 Epilepsy 126 Multiple sclerosis 134 Autonomic Disorders 144 CONTENTS Continuum Contents 1 INTERNAL MEDICINE Feb. 2005 TYPE A QUESTIONS (ONE BEST ANSWER) 1. Most evidence suggests that the major efferent limb of the inflammatory reflex is carried in: A. The sympathetic chain B. The phrenic nerve C. The vagus nerve D. Postganglionic sympathetics arising in the stellate ganglia E. The long thoracic nerve Correct Answer: The correct answer is C. Evidence suggests that information leaving the central nervous system regarding regulation of inflammation is carried mainly in the vagus nerve. The sympathetic chain is the origin of postganglionic fibers for sympathetic innervation of visceral organs. The phrenic nerve carries motor axons for the diaphragm, and the postganglionic sympathetics are destined to provide sympathetic innervation to the heart, lungs, and neighboring structures. 2. Autopsy studies in patients with gluten ataxia have demonstrated within the cerebellum lymphocytic infiltration and loss of: A. Stellate cells B. Mossy fibers C. Granule cells D. Purkinje cells E. Basket cells Correct Answer: The correct answer is D. Hadjivassiliou and colleagues have reported the presence of Purkinje cell loss and lymphocytic infiltration in the cerebellum and posterior columns of the spinal cord in several individuals with gluten ataxia, suggesting the presence of an immune-mediated inflammatory process as the mechanism for the development of gluten ataxia. Hadjivassiliou M, Boscolo S, Davies-Jones GA, et al. The humoral response in the pathogenesis of gluten ataxia. Neurology 2002;58:1221-1226. 3. A 25-year-old primigravida is seen in urgent consultation shortly after experiencing a first generalized tonic- clonic seizure. She is 1 day postpartum after vaginal delivery of a full-term infant. Pregnancy was complicated by early preterm labor treated successfully with magnesium sulfate. Delivery was uncomplicated with stable vital signs and no sign of maternal infection. Examination shows a still somnolent woman who has bitten her tongue. Vital signs show a blood pressure of 150/90 mm Hg. She moves all limbs spontaneously with brisk deep tendon reflexes throughout; Babinski's sign is present bilaterally. She becomes oriented over the course of the examination and complains of a headache and blurred vision. Magnetic resonance imaging (MRI) imaging of the brain done later that day shows large areas of increased signal in the occipital lobes bilaterally on T2-weighted and fluid- attenuated inversion recovery (FLAIR) sequences. What is the most important causative factor in this patient's problem? A. Degree of hypertension B. Rapid rise of blood pressure C. Integrity of collateral circulation D. Postpartum hypercoagulability E. Amniotic fluid emboli Correct Answer: The correct answer is B. This patient has eclampsia with primary endothelial cell dysfunction. In these patients, who were not long-term hypertensives, "rapid rise of blood pressure over time rather than the absolute blood pressure carries the greatest risk" (see chapter 5, "Hyperperfusion Syndromes," p. 99).Collateral circulation is irrelevant. Postpartum hypercoagulability or amniotic fluid emboli is unlikely. 4. 4. Which of the following vasculitic syndromes is most likely to be associated with the presence of serum antineutrophil cytoplasm autoantibodies (ANCA)? A. Polyarteritis nodosa B. Giant cell arteritis C. Takayasu's arteritis D. Wegener granulomatosis E. Leukocytoclastic skin vasculitis Correct Answer: The correct answer is D. ANCA with a diffuse cytoplasmic staining pattern (cANCA) with specificity for proteinase 3 (PR3) are found in over 90% of patients with untreated Wegener granulomatosis. None of the other vasculitides listed above are commonly associated with ANCA. Microscopic polyangiitis shows 2 considerable overlap with Wegener granulomatosis and is frequently associated with antimyeloperoxidase ANCA or anti-PR3-ANCA. Churg-Strauss angiitis is also associated with ANCA. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004;117:39-50. 5. The following is a serum marker associated with arterial hypercoagulability: A. D-dimer B. High sensitivity C-reactive protein C. Factor V Leiden mutation D. Protein C deficiency E. Protein S deficiency Correct Answer: The correct answer is A. D-dimer is a marker for both arterial and venous thrombosis. The other choices are all markers of venous thrombosis only. 6. A 70-year-old man with a history of unstable angina and hypertension undergoes coronary artery bypass grafting and is transferred postoperatively to the surgical intensive care unit for further care. On the second postoperative day, he is noted by staff to be disoriented and poorly cooperative with his care. A neurological consultant is called. The patient is afebrile with stable vital signs. He appears distracted and is unable to state where he is or how long he has been there. He looks off to various corners of the room; he moves all four limbs normally. Deep tendon reflexes are symmetric, and plantar responses are flexor. He has been receiving tapering doses of morphine sulfate, along with other medications. The consultant diagnoses encephalopathy. Which of the following is the most likely cause of this condition? A. Microemboli B. Hypoxemia C. Drug withdrawal D. Focal cerebral infarction E. Infection Correct Answer: The correct answer is A. Showers of embolic material to the brain are the most likely cause of postoperative encephalopathy, on the same spectrum as those who suffer definitive strokes after surgery. There is no evidence for poor oxygenation or infection with stable vital signs; drug withdrawal is unlikely with careful tapering of doses. 7. The central nervous system (CNS) regulates inflammation via two mechanisms: a humoral system and a neural system. Stimulation of what nervous structure is known to result in an inhibition of inflammatory cytokine synthesis in all visceral organs? A. Cingulate gyrus B. Amygdala C. Pontine reticular formation D. Superior cervical ganglion E. Vagus nerve Correct Answer: The correct answer is E. Stimulation of the vagus nerve has been shown to inhibit inflammatory cytokine synthesis in all visceral organs, including those that belong to the reticuloendothelial system, via release of acetylcholine, which reacts with macrophage receptors. This direct inhibitory influence does not occur with stimulation of the other structures. 8. A 67-year-old insurance salesman presents to his physician with complaints of chronic cough, unintentional loss of 25 lbs over the past 3 months, andthe development of poor balance, numbness on the lateral aspect of the right leg and foot, and burning in his left foot over the past 2 weeks. Nerve conduction studies/electromyography demonstrate a predominantly sensory neuronopathy with superimposed mononeuritis multiplex polyneuropathy. Blood work includes a normal glycosylated hemoglobin level, an elevated anti-Hu antibody titer, and a negative human immunodeficiency virus serology. Right peroneal nerve biopsy yields pathological evidence of transmural inflammation of epineurial vessels. Which of the following is the most likely diagnosis? A. Wegener granulomatosis B. Polyarteritis nodosa C. Microscopic polyangiitis D. Paraneoplastic vasculitis E. Nonsystemic vasculitic neuropathy Correct Answer: The correct answer is D. Paraneoplastic vasculitis, most likely due tolung neoplasm, is the most likely diagnosis. None of the other options is associated with a positive anti-Hu antibody but could present with weight loss and neuropathy. 9. A 38-year-old male is referred to the neurologist with a 2-year history of gradually progressive deterioration in balance. His past medical history is significant for chronic diarrhea and difficulty maintaining his weight. His 3 examination is remarkable for moderate truncal ataxia, mild dysarthria, and mild intention tremor in the upper extremities. The antibody test most useful in establishing the diagnosis of gluten ataxia is: A. Antitissue transglutaminase B. Antigliadin C. Antiendomysial D. Antireticulin E. Antiglutenin Correct Answer: The correct answer is B. Hadjivassiliou and colleagues believe that the presence of immunoglobulin G antigliadin antibodies, even though somewhat nonspecific, is the best marker for gluten ataxia because it is the most sensitive indicator of gluten sensitivity, which may be present even in the absence of gluten enteropathy. Correct Answer: Antitissue transglutaminase, antiendomysial, and antireticulin antibodies are all more specific markers for gluten enteropathy but are less frequently positive in gluten ataxia. Antiglutenin antibodies are not typically measured in celiac disease. Hadjivassiliou M, Grünewald R, Sharrack B, et al. Gluten ataxia in perspective: epidemiology, genetic suspectibility and clinical characteristics. Brain 2003; 126:685-691. 10. A 65-year-old man with a history of hypertension, hyperlipidemia, and intractable angina undergoes coronary artery bypass grafting with cardiopulmonary bypass. His immediate postoperative recovery is unremarkable, and he begins outpatient cardiac rehabilitation. One month after surgery, his therapists note that he seems withdrawn at times and appears reluctant to participate fully in his treatment. He notes some difficulty falling asleep at night and tells his wife he feels as though, "I might die in my sleep." He takes a beta-blocker, nitrate, and statin drug but admits to sometimes missing a pill. Approximately 1 year after surgery, he has not resumed all of his normal activities and begins to complain of recurrent chest pain. His cardiologist performs an exercise tolerance test, and no ischemic changes are noted. Which of the following is the most likely explanation for his recurrent symptoms? A. Poor exercise tolerance B. Reocclusion of grafts C. Cognitive impairment D. Medication noncompliance E. Depression Correct Answer: The correct answer is E. No ischemic changes have been demonstrated on testing, and although we do not have any information on the patient's premorbid mood, "…depression at 3 months is strongly associated with the return of angina at 1 year and 3 years" (see chapter 2, "Neurological Aspects of Cardiac Surgery With Emphasis on Coronary Artery Bypass Grafting," page 51). 11. The hallmark of the encephalopathy of fulminant hepatic failure is: A. Parkinsonism due to manganese deposition in the globus pallidus B. Nystagmus and ophthalmoplegia C. Diabetes insipidus D. Korsakoff's psychosis E. Cerebral edema with increased intracranial pressure Correct Answer: The correct answer is E. The hallmark and defining characteristic of the encephalopathy that develops in fulminant hepatic failure is cerebral edema with consequent increased intracranial pressure, which can develop very rapidly and can compromise cerebral perfusion. Its pathogenesis is not fully understood. Correct Answer: Hyperintensity within the globus pallidus on T1-weighted MRI images is often seen in individuals with chronic hepatic failure and is probably due to manganese deposition. Nystagmus and ophthalmoplegia are classic features of Wernicke's encephalopathy, which occurs in individuals with thiamine deficiency, often in the setting of long-standing ethanol abuse. Diabetes insipidus is a consequence of posterior pituitary dysfunction. Korsakoff's psychosis, characterized by prominent impairment of short-term memory with confabulation, is another consequence of thiamine deficiency, typically in the context of chronic ethanol abuse. 12. A 70-year-old man with a history of coronary artery disease and bilateral carotid stenosis (last estimated at between 50% to 69% on both sides) is evaluated for coronary artery bypass grafting. Six months prior to this, he saw a neurologist for a transient episode of aphasia; at that time, MRI scanning of the brain, including diffusion- weighted imaging (DWI), showed small vessel disease. He undergoes conventional on-pump surgery with cross- clamping of the aorta; 1 day postoperatively, he has difficulty expressing himself and mild right pronator drift. A computed tomography (CT) scan of the brain fails to demonstrate any acute lesion. When he is extubated and placed in a chair, staff members note that he has difficulty swallowing and moving both arms fully. A neurological consultant is called and diagnoses a left frontal infarct while suspecting that a more diffuse problem may be 4 present. MRI scan of the brain with DWI and magnetic resonance angiography is obtained. What is the most likely finding on these studies? A. Small vessel disease B. Left carotid occlusion C. Bilateral watershed infarcts D. Bilateral middle cerebral artery infarcts E. Diffuse cerebral edema Correct Answer: The correct answer is C. As in Case 2-1, this patient likely suffered the consequence of multiple emboli. Although the focal findings suggest possibly a unilateral left middle cerebral artery infarct, a carotid occlusion would likely have produced a larger deficit. The patient also has findings (eg, dysphagia) suggestive of bilateral disease but with a level of consciousness and examination not likely to be as severe as bilateral middle cerebral artery infarcts. 13. Acute sensorineural hearing loss is associated with: A. Celiac disease B. Ulcerative colitis C. Whipple's disease D. Hepatic encephalopathy E. Crohn's disease Correct Answer: The correct answer is B. Both acute sensorineural hearing loss and chronic subclinical hearing loss have been reported in the setting of ulcerative colitis. The pathogenesis of the neuronal damage is presumed to be on an autoimmune basis. Summers RW, Harker L. Ulcerative colitis and sensorineural hearing loss: is there a relationship? J Clin Gastroenterol 1982;4:251-252. 14. 14. A 45-year-old woman with a history of metastatic breast cancer is begun on intravenous cyclosporine A therapy after undergoing bone marrow transplant. Two hours after her first infusion of cyclosporine, she complains of a headache; within an hour, she is noted to have a generalized tonic-clonic seizure. A neurological consultant is called later that day and finds visual loss in the right hemifield and left superior quadrant. MRI scanning of the brain shows confluent areas of enhanced signal intensity in the occipital lobes on T2-weighted and FLAIR imaging; DWI and gradient-echo imaging are normal, and no enhancing lesions are seen with gadolinium. The consultant suggests repeating the imaging study in a few weeks. What is most likely to be seen on the repeat study? A. Resolution of the lesions B. Bioccipital infarctions C. Intracerebral metastases D. Petechial hemorrhage E. Brain stem compression Correct Answer: The correct answer is A. The patient's findings on her initial MRI scan are consistent with vasogenic edema, as seen in cyclosporine A use (p. 102). In most cases, "diffusion-weighted imaging (DWI) has been used mostly to distinguish the cytotoxic edema of infarction from the vasogenic edema of HTE [hypertensive encephalopathy]" (p. 102). Although it is possible for this patient to have suffered permanent infarctions, the normal DWI finding on her initial imaging study argues against this. There is no suggestion of metastases on the gadolinium-enhanced images, nor of brain stem compression. Petechial hemorrhage is possible but not seen on gradient-echo imaging. 15. A 42-year-old man presents with a history of recurrent oral and genital ulcerations. He has had episodes of retinal vasculitis and is followed by a neuro-ophthalmologist. In addition, he has had three episodes of encephalopathy that brought him to evaluation in the emergency department. During two of these episodes, cerebrospinal examination showed lymphocytic pleocytosis with elevated protein. He also has had problems with pseudotumor cerebri in the past. Which of the following diseases does he most likely have? A. Sjögren syndrome B. Behçet's disease C. Rheumatoid arthritis D. Lymphomatoid granulomatosis E. CNS borreliosis Correct Answer: The correct answer is B. Behçet's disease is characterized by a triad of oral and genital ulcers and uveitis and may be associated with cutaneous, retinal, and CNS vasculitis. Encephalopathy may occur due to direct inflammation of the CNS. Pseudotumor may also occur from smoldering vasculitis involving the cerebral veins. Siva A, Altintas A, Saip S. Behçet's syndrome and the nervous system. Curr Opin Neurol 2004;17:347-357. 5 16. A 65-year-old man with a history of hypertension, diabetes mellitus, and depression undergoes coronary artery bypass grafting for intractable angina. He awakens 10 hours after surgery. Shortly thereafter, staff notes that he is not following commands and appears to stare off into space. Medications include morphine sulfate, intravenous beta-blockers, and heparin. He is intubated; vital signs are remarkable for a blood pressure of 110/60 mm Hg and a regular heart rate. A neurological consultant is called and finds the patient to be awake but attending only to the right. He moves the right side spontaneously and withdraws the left leg. Laboratory tests show a glucose of 150 mg/dL. What is the most likely mechanism for this patient's problem? A. Medication-induced delirium B. Hyperglycemia C. Prolonged hypotension D. Atheroembolic event E. Intracerebral hemorrhage Correct Answer: The correct answer is D. The patient has a focal ischemic event in a major arterial territory. He has mild hyperglycemia, although no history of a prior stroke is known. Although he is in a younger age group, the presence of diabetes and hypertension makes his risk of stroke higher than any other category in his age group (Table 2-5). 17. A 75-year-old man with a history of coronary artery disease, hypertension, and hyperlipidemia is brought to the emergency department 1 hour after an episode at home during which he was noted to mumble something, then shake on the right side, and then fall to the ground. Five days earlier he had undergone left carotid endarterectomy for a 90% stenosis of the left internal carotid artery. Surgery was uneventful, and he was discharged to home on the fourth postoperative day. When seen in the emergency department, he has difficulty naming objects and complains of a headache. Vital signs are significant for a blood pressure of 165/90 mm Hg. Mild right pronator drift is present. What is the most likely mechanism underlying this patient's neurological event? A. Defective cerebral autoregulation B. Intracerebral steal syndrome C. Focal ischemia D. Cytotoxic edema E. Thrombogenesis Correct Answer: The correct answer is A. There is good evidence for the postulated postendarterectomy hyperperfusion syndrome to be the result of chronic hypoperfusion in the setting of high-grade carotid stenosis, leading to a shift of the autoregulatory curve to the left and, hence, an intolerance of the restored perfusion pressures after revascularization. Focal ischemia and cytotoxic edema might occur as a result of embolization from the endarterectomy site or from acute thrombosis, but these are less likely than post-carotid endarterectomy hyperperfusion to cause headache and seizure. 18. An apparently healthy man has a high-sensitivity C-reactive protein level (hsCRP) that falls in the highest quartile for a large population of similar men. How does his relative risk for myocardial infarction compare to that of a similar man with an hsCRP level that falls within the lowest quartile? A. Equivalent B. One time greater C. Three times greater D. Five times greater E. Ten times greater Correct Answer: The correct answer is C. In the study by Ridker and colleagues, (1997) in which 22,000 apparently healthy men were followed for 10 years, the risk of myocardial infarction was 3 times greater in those men whose hsCRP fell within the top quartile compared to the risk of the men within the lowest quartile. Hadjivassiliou M, Boscolo S, Davies-Jones GA, et al. The humoral response in the pathogenesis of gluten ataxia. Neurology 2002;58:1221-1226. Hadjivassiliou M, Boscolo S, Davies-Jones GA, et al. The humoral response in the pathogenesis of gluten ataxia. Neurology 2002;58:1221-1226. Pai JK, Pischon T, Ma J, et al. Inflammatory markers and the risk of coronary heart disease in men and women. N Engl J Med 2004;351:2599-2610. Ridker PM, Cushman M, Stampfer MJ, et al. Inflammation, aspirin and the risk of cardiovascular disease in apparently healthy men. N Engl J Med 1997;336:973-974. 19. A 55-year-old man is seen in follow-up by his physician for hypertension. He has a history of chronic renal failure due to hypertensive disease. He is maintained on four medications for blood pressure, including clonidine, metoprolol, hydrochlorothiazide, and lisinopril. His blood pressure in the office is 195/100 mm Hg. Two days later, he is brought to the emergency department by ambulance after suffering a seizure. Blood pressure is 240/120 mm 6 Hg; papilledema is present. A CT scan of the brain done urgently shows poor differentiation of the gray-white junction. Damage to which of the following structures can be implicated in this patient's problem? A. Adventitia B. Astrocytic podocyte C. Basal lamina D. Endothelium E. Muscularis Correct Answer: The correct answer is D. The endothelium makes up the major functional component of the blood- brain barrier to protein tracers, suggesting that it is the major barrier to production of edema. 20. A distinguishing clinical characteristic of Whipple's disease is: A. Oculomasticatory myorhythmia B. Myoclonic ataxia C. Palatal myoclonus D. Internuclear ophthalmoplegia E. Myotonia paradoxica Correct Answer: The correct answer is A. Oculomasticatory myorhythmia consists of the combination of pendular convergence nystagmus and concurrent slow,rhythmic, synchronous contractions of the masticatory muscles and is accompanied by supranuclear vertical gaze paresis. Its presence is virtually pathognomonic for Whipple's disease, although it actually develops in only approximately 20% of patients with the disease. Correct Answer: Myoclonic ataxia (Ramsay Hunt syndrome) has been reported in the setting of celiac disease. Palatal myoclonus (now termed palatal tremor) in its secondary, or symptomatic, form is produced by pathological processes damaging the brain stem and may result in hypertrophy of the inferior olive. Internuclear ophthalmoplegia is produced by lesions involving the median longitudinal fasciculus and is often associated with multiple sclerosis. Myotonia paradoxica occurs in the setting of paramyotonia congenita and is characterized by myotonia that worsens with repeated activity. Louis ED, Lynch T, Kaufmann P, et al. Diagnostic guidelines in central nervous system Whipple's disease. Ann Neurol 1996;40:561-568. Schwartz MA, Selhorst JB, Ochs AL, et al. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's Disease. Ann Neurol 1986;20:677-683. 21. A 46-year-old male physician is quite worried about his personal risk of stroke despite the fact that he does not smoke or have hypertension or hyperlipidemia. His father had several strokes in his early 60s despite a lack of demonstrable risk factors including a negative evaluation for hypercoagulable states. He wonders if a blood test would help to determine his risk of stroke. Which of the following markers of inflammation is the most predictive of relative risk of stroke? A. Tumor necrosis factor-[alpha] B. High-sensitivity C-reactive protein C. Interleukin 6 D. Interleukin 10 E. Soluble CD40 ligand Correct Answer: The correct answer is B. High-sensitivity C-reactive protein is the most reliable and consistent predictor of arterial thrombosis and the only inflammatory biomarker clinically proven to add predictive information to both the Framingham Risk Score and to formal definitions of metabolic syndrome. Tumor necrosis factor-[alpha], interleukin 6, and soluble CD40 ligand are also markers of inflammation but have not been found to have the predictive value of high-sensitivity C-reactive protein. Interleukin 10 is an anti-inflammatory cytokine. 22. A 5-year-old girl with glomerulonephritis, renal failure, and hypertension who has had three generalized tonic- clonic seizures in a period of 3 hours is taken to the emergency department. On arrival, her blood pressure is 180/110 mm Hg, and treatment is begun with intravenous sodium nitroprusside. A neurological consultant is called 5 hours later. Blood pressure at that time is 140/90 mm Hg. She is mildly confused and complains of a headache. Flame hemorrhages are seen in the eyes. An adult patient with a similar presentation was admitted the day before; his blood pressure on arrival to the emergency department was noted to be 240/140 mm Hg. What is the most likely explanation for the greater vulnerability of the child than the adult for this problem? A. Autoregulation in children comprises a broader mean arterial pressure (MAP) range B. Endothelial cells in children are less developed C. Renal compensatory mechanisms are less available D. Childhood hypertension is less aggressively treated E. The range of autoregulation in children is shifted to lower MAP 7 Correct Answer: The correct answer is E. As a result of the developmentally lower upper threshold of autoregulation, autoregulatory breakthrough will occur at lower blood pressures in children. 23. Serum C-reactive protein is a biomarker of inflammation. What percentage of the population variance in C- reactive protein levels appears related to inherited factors? A. 10% B. 25% C. 50% D. 75% E. 90% Correct Answer: The correct answer is C. Approximately 50% of the variance in C-reactive protein levels occurs on a familial basis. This suggests that low-grade systemic inflammation may be determined in part by several common gene polymorphisms. 24. A 75-year-old woman with a history of hypertension develops abrupt onset of headache and confusion. On physical examination, blood pressure is 210/120 mm Hg. Pupils react normally, but she has obvious difficulty seeing in both hemifields. A CT scan of the brain does not demonstrate a hemorrhage. MRI of the brain with diffusion-weighted sequences does not demonstrate a stroke, and FLAIR imaging is suggested. What finding is most likely to be seen on FLAIR imaging? A. Cytotoxic edema B. Petechial hemorrhage C. Major branch occlusion D. Vasogenic edema E. Hydrocephalus Correct Answer: The correct answer is D. Gross hemorrhage and hydrocephalus have been ruled out by CT scan, and petechial hemorrhage should be seen on conventional MRI. Diffusion-weighted imaging has effectively ruled out a major branch occlusion. FLAIR imaging clearly demonstrates the vasogenic edema that is the primary imaging finding in hypertensive encephalopathy. 25. An association with migraine has been reported for: A. Crohn's disease B. Viral hepatitis C. Ulcerative colitis D. Celiac disease E. Whipple's disease Correct Answer: The correct answer is D. In a recent study, Gabrielli and colleagues (2003) discovered the presence of biopsy-confirmed celiac disease in 4.4% of 90 patients with migraine. A gluten-free diet resulted in improved control of migraine severity in all affected individuals. Correct Answer: Migraine has not been specifically associated with inflammatory bowel disease, hepatitis, or Whipple's disease. Gabrielli M, Cremonini F, Fiore G, et al. Association between migraine and Celiac disease: results from a preliminary case-control and therapeutic study. Am J Gastroenterol 2003;98:625-629. 26. A-25 year-old primigravida in her 30th week of pregnancy is seen in the emergency department 2 days after the onset of headache, confusion, and visual changes. Vital signs include a blood pressure of 160/100 mm Hg. SheC appears distracted. Tone is increased throughout. Which of the following is the best treatment for her hypertension? A. Sodium nitroprusside B. Magnesium sulfate C. Enalaprilat D. Furosemide E. Phentolamine Correct Answer: The correct answer is A. Lowering the blood pressure with a drug that has rapid onset and short duration of action is recommended. Magnesium sulfate is used to treat epileptic complications of eclampsia (not hypertension); enalaprilat is contraindicated in pregnancy while diuretics are generally not used since most patients are felt to be volume contracted. Phentolamine is used in hypertensive crises induced by monoamine oxidase inhibitors, as well as in pheochromocytoma. 27. A 70-year-old man with a history of hypertension undergoes coronary artery bypass grafting with conventional on-pump technique. His immediate recovery is unremarkable, and he is discharged to home on the fifth postoperative day. He begins outpatient cardiac rehabilitation and is able to increase his exercise tolerance gradually over the next 2 months. At 2 months postoperatively he resumes driving but has some difficulty responding quickly in the car. He has not yet resumed responsibility for balancing his checkbook. He is sent for [...]... in the hands and feet Her examination also shows percussion myotonia in the thenar eminence On genetic testing, the infant has evidence of 10 00 CTG repeats in the 3' untranslated region of the myotonic dystrophy protein kinase gene Which of the following best explains the finding that the infant has more severe disease than the mother? A X-linked dominant inheritance pattern B Anticipation C Germline... Ann Neurol 19 83 ;14 :54 3-5 53 Questions 32 and 33 refer to the following case vignette: A 28-year-old man has been experiencing prominent abdominal pain with waxing and waning diarrhea that has generally been nonbloody He has lost 50 lbs in the past year He has also been treated for renal calculi in the past 32 Colonoscopy with intestinal biopsy in this individual is likely to show: A Diffuse inflammation... effects, 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase inhibitors (statins) have anti-inflammatory actions that reduce atherosclerotic plaque inflammation and rupture Which of the following is one of the mechanisms by which statins may reduce inflammation? A Inhibition of tumor necrosis factor-[alpha] (TNF-[alpha]) secretion B Induction of arterial thrombosis C Down-regulation of interleukin 10 ... elaboration D Increase of interleukin 1 release E Increased T-cell activation Correct Answer: The correct answer is A Statins have been shown to cause in vitro inhibition of TNF-[alpha] secretion TNF-[alpha] is one of the most important substances in the acute inflammatory cascade, causing other mononuclear cells to release inflammatory cytokines The other choices reflect increased activation of the inflammatory... The American College of Rheumatology 19 90 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis) Arthritis Rheum 19 90;33 :10 9 4 -1 10 0 39 A 38-year-old man has experienced several months of intermittent fevers and an involuntary weight loss of 10 lbs He has had problems with swelling and pain in his knees, wrists, and elbows Antinuclear antibody and rheumatoid factor... symptoms, particularly in the face of a prior focal lesion The treatment is rehydration with normal saline and intravenous infusion of insulin It may be necessary to encourage increased water intake or administer hypotonic saline 35 A 45-year-old man with a history of alcoholism and cirrhosis is found down on the street and transported to the emergency department He appears to be acutely intoxicated... neurological involvement in 31. 5% of patients with neurological complications of their inflammatory bowel disease Myopathy accounted for 16 % of the cases, cerebrovascular complications 21% , and myelopathy 26% Seizures are an infrequent complication of inflammatory bowel disease Lossos A, River Y, Eliakim A, Steiner I Neurologic aspects of inflammatory bowel disease Neurology 19 95;45: 41 6-4 21 TYPE R QUESTIONS... 19 95;45: 41 6-4 21 TYPE R QUESTIONS (EXTENDED MATCHING) For each of the following patients with a fluid or electrolyte abnormality in questions 34 to 37, select the correct treatment A Free water restriction B 0.9 % normal saline with insulin C 10 % calcium gluconate D Intravenous potassium chloride (KCl) E 3% saline 34 A 55-year-old man with hypertension and non-insulin-dependent diabetes is admitted to the hospital... superficial submucosa B Periodic acid-Schiff 9 (PAS)-positive inclusions in macrophages in the lamina propria C Mucosal plaquelike lesions over the colonic surface D Normal-appearing colonic mucosa E Inflammation extending deeply into the colonic wall with noncaseating granulomas Correct Answer: The correct answer is E Crohn's disease is typically characterized by abdominal pain with diarrhea that is nonbloody... reduced, and Babinski sign is absent The sensory examination is normal Genetic testing reveals that the patient is homozygous for mutations in the ATM gene on chromosome 11 q Which of the following is the most appropriate counseling for this child's parents? A Their 15 -year-old son has a 25% chance of developing the disorder B The patient's future offspring have a 50% chance of developing the disorder . ataxia. Neurology 2002;58 :12 2 1- 1 226. 3. A 25-year-old primigravida is seen in urgent consultation shortly after experiencing a first generalized tonic- clonic seizure. She is 1 day postpartum. sensorineural hearing loss: is there a relationship? J Clin Gastroenterol 19 82;4:25 1- 2 52. 14 . 14 . A 45-year-old woman with a history of metastatic breast cancer is begun on intravenous cyclosporine. gluten ataxia. Neurology 2002;58 :12 2 1- 1 226. Hadjivassiliou M, Boscolo S, Davies-Jones GA, et al. The humoral response in the pathogenesis of gluten ataxia. Neurology 2002;58 :12 2 1- 1 226. Pai JK,