Answers

The answer is a. (Swaiman, pp 442-444.) Children with Tay-Sachs disease die prematurely and exhibit mental retardation, seizures, and blindness. This is a ganglioside storage disease that occurs more commonly in Ashkenazi Jews than in the general population. The early-onset form will produce macrocephaly and a cherry red spot in the fundus. 192. The answer is a. (Swaiman, p 452.) The disease responsible for the accumulation of glucosylceramide is Gaucher's disease. Gaucher's disease is...

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A 61-year-old man, who smokes five packs of cigarettes per day and has hypertension, had an abdominal aortic aneurysm repair 8 h ago. The surgery went very well, and there were no reported perioperative complications. Now the patient is unable to move his legs and states that they are numb. On examination, he has a flaccid paresis of both lower extremities and has impaired pinprick sensation to a T9 level bilaterally. Joint proprioception is normal. 430. The most likely diagnosis in this case...

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For each clinical scenario, choose the CSF pattern most likely to be found. 38. A 26-year-old woman with a 7-year history of epilepsy develops a generalized convulsion while shopping. She is taken to an ER, but no one accompanying her is aware of the previous history of epilepsy. Because she has a protracted postictal period, numerous investigations are performed over the course of the next hour. CT scan is completely normal, but her arterial blood gases reveal a mild acidosis. (SELECT 1...

Questions

DIRECTIONS Each item below contains a question or incomplete statement followed by suggested responses. Select the one best response to each question. A 65-year-old right-handed woman began having neurological problems about 1 week ago. She began experiencing nausea, vomiting, and numbness in the left hand and left foot. Today she had a generalized convulsion, and since then she has had a throbbing headache that is worse when she bends forward. On examination, the only deficits she has are loss...

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A 57-year-old woman with a history of diabetes mellitus and hyperthy-roidism presents to the emergency room with a history of 2 days of vertical and horizontal diplopia. There is moderate orbital pain. On examination, her left eye is deviated downward and outward. It can be passively moved medially and upward. The pupils both react normally. 479. This woman most likely has 480. The etiology of her diplopia is most likely 481. A 33-year-old operating room nurse accidentally has blood splashed in...

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A 32-year-old man living along the coast of Massachusetts presents with an acutely evolving left facial weakness. Although he has no facial pain or numbness, he does complain of diffuse headache. He has no history of diabetes mellitus or other systemic illnesses, but does report newly appearing joint pains and a transient rash on his right leg that cleared spontaneously more than 1 month prior to the appearance of the facial weakness. On examination, he has mild neck stiffness and pain on hip...

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A 19-year-old woman with complaints of headaches and visual blurring has prominent bulging of both optic nerve heads with obscuration of all margins of both optic discs. Her physician is reluctant to pursue neurologic studies because the patient is 8 months pregnant and had similar complaints during the last month of another pregnancy. Her physical and neurologic examination are otherwise unrevealing. 368. If neuroimaging studies were to be performed on this woman, they probably would reveal...