A 55-year-old man comes to the office because of numerous falls for the past few weeks. Yesterday, he felt so dizzy that he fell on the ground and hurt his knees. He has also noticed dry mouth, dry skin, and erectile dysfunction over this period. His medical history is significant for the recent onset of resting tremors. He was diagnosed with diabetes 6 months ago, which is controlled with diet. Blood pressure is 121/84 mm Hg supine, and 92/61 mm Hg standing. Physical examination reveals rigidity and bradykinesia. What is the most likely diagnosis of this patient?

Question

A 55-year-old man comes to the office because of numerous falls for the past few weeks.  Yesterday, he felt so dizzy that he fell on the ground and hurt his knees.  He has also noticed dry mouth, dry skin, and erectile dysfunction over this period.  His medical history is significant for the recent onset of resting tremors.  He was diagnosed with diabetes 6 months ago, which is controlled with diet.  Blood pressure is 121/84 mm Hg supine, and 92/61 mm Hg standing.  Physical examination reveals rigidity and bradykinesia.  What is the most likely diagnosis of this patient?

Quizplus · Accepted Answer

The clinical presentation of this patient is suggestive of multiple system atrophy (Shy-Drager syndrome), which is a degenerative disease characterized by the following: ParkinsonismAutonomic dysfunction (postural hypotension, abnormal sweating, disturbance of bowel or bladder control, abnormal salivation or lacrimation, impotence, gastroparesis, etc)Widespread neurological signs (cerebellar, pyramidal or lower motor neuron) Parkinsonism Autonomic dysfunction (postural hypotension, abnormal sweating, disturbance of bowel or bladder control, abnormal salivation or lacrimation, impotence, gastroparesis, etc) Widespread neurological signs (cerebellar, pyramidal or lower motor neuron) Always consider Shy-Drager syndrome when a patient with Parkinsonism experiences orthostatic hypotension, impotence, incontinence, or other autonomic symptoms. The accompanying bulbar dysfunction and laryngeal stridor may be fatal. Anti-Parkinsonism drugs are generally ineffective, and treatment is aimed at intravascular volume expansion with fludrocortisone, salt supplementation, alpha-adrenergic agonists, and application of constrictive garments to the lower body. (Choice A) Idiopathic orthostatic hypotension is seen secondary to the degeneration of postganglionic sympathetic neurons; however, the pathology is confined to the autonomic nervous system, and there are no signs of Parkinsonism or cerebellar involvement. (Choice B) Horner syndrome is a regional dysautonomia characterized by anhydrosis, miosis, and ptosis. (Choice C) Riley-Day syndrome or familial dysautonomia is an autosomal-recessive disease seen predominantly in children of Ashkenazi Jewish ancestry. It presents at birth (unlike in this patient) with feeding problems and low muscle tone; it is characterized by gross dysfunction of the autonomic nervous system (eg, no tears) with severe orthostatic hypotension (swings from severe hypertension to postural hypotension). (Choice D) Diabetic neuropathy can explain the autonomic symptoms in this patient, but autonomic neuropathy is a late complication of diabetes mellitus, and would not be expected in a patient with diet-controlled diabetes for the past 6 months. Educational objective: Always consider multiple system atrophy when a patient with Parkinsonism experiences orthostatic hypotension, impotence, incontinence, or other autonomic symptoms.

A 55-Year-Old Man Comes to the Office Because of Numerous