A 65-year-old man is brought to the office due to a 2-year history of progressive cognitive decline. The patient has difficulty finding the right words and recognizing faces, and he has stopped driving because he gets lost on the streets. Over the past 6 months, he has had frequent falls, dizziness on standing, and urinary incontinence. The patient increasingly requires his wife's help to perform activities of daily living.
His seated blood pressure is 120/80 mm Hg and standing blood pressure is 100/70 mm Hg. Pulse increases from 90/min when sitting to 100/min when standing. Mini-mental state examination score is 16/30. The patient's face appears masked, but the rest of the cranial nerves are intact. He has bilaterally symmetrical cogwheel rigidity and bradykinesia in the arms but no tremor. His gait is slow and wide-based. The remainder of the patient's neurologic examination shows no abnormalities.
Basic blood work and spinal fluid analysis results are unremarkable. An MRI of the brain reveals diffuse cerebral and pontocerebellar atrophy that is out of proportion to the patient's stated age. A routine electroencephalogram shows diffuse slowing.
Which of the following is the most likely diagnosis?

Question

A 65-year-old man is brought to the office due to a 2-year history of progressive cognitive decline.  The patient has difficulty finding the right words and recognizing faces, and he has stopped driving because he gets lost on the streets.  Over the past 6 months, he has had frequent falls, dizziness on standing, and urinary incontinence.  The patient increasingly requires his wife's help to perform activities of daily living.
His seated blood pressure is 120/80 mm Hg and standing blood pressure is 100/70 mm Hg.  Pulse increases from 90/min when sitting to 100/min when standing.  Mini-mental state examination score is 16/30.  The patient's face appears masked, but the rest of the cranial nerves are intact.  He has bilaterally symmetrical cogwheel rigidity and bradykinesia in the arms but no tremor.  His gait is slow and wide-based.  The remainder of the patient's neurologic examination shows no abnormalities.
Basic blood work and spinal fluid analysis results are unremarkable.  An MRI of the brain reveals diffuse cerebral and pontocerebellar atrophy that is out of proportion to the patient's stated age.  A routine electroencephalogram shows diffuse slowing.
Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_9875_3c0f_853d_fd6c9ae3b0fd_MD0002_00 This patient likely has multiple system atrophy, a type of Parkinson-plus syndrome. Parkinson-plus syndromes make up approximately 10%-15% of cases of parkinsonism and can generally be distinguished from idiopathic Parkinson disease by the rapid onset of symptoms, symmetric neurologic findings (eg, bradykinesia, rigidity), absent tremor, early autonomic dysfunction (eg, urinary incontinence, orthostatic hypotension), and poor response to dopamine agonists (Choice C). The 3 most common Parkinson-plus syndromes include multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration, and each has a unique set of features. The diagnosis of multiple system atrophy is clinical, incorporating a combination of core findings that include akinetic-rigid parkinsonism, autonomic failure, and cerebellar ataxia (eg, wide-based, unsteady gait). Brain MRI may reveal nonspecific atrophy of the putamen, middle cerebellar peduncle, or pons but is mainly useful in excluding alternate conditions (eg, infarction, tumor). (Choice A) Dementia with Lewy bodies may present with cognitive impairment, parkinsonism, and autonomic dysfunction. However, other core features (eg, visual hallucinations, fluctuating cognition/alertness) are not evident in this patient. (Choice B) Frontotemporal dementia typically presents with changes in personality/behavior (eg, emotional blunting, social disinhibition) and language abnormalities (eg, aphasia). Neuroimaging often reveals frontal and temporal lobe atrophy. (Choice E) Sporadic spongiform encephalopathy (Creutzfeldt-Jakob disease) is an infectious prion-mediated neurodegenerative disorder characterized by rapidly progressive (<1 year) dementia and myoclonus. Educational objective: Parkinson-plus syndromes (eg, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration) can generally be distinguished from idiopathic Parkinson disease by the rapid onset of symptoms, symmetric neurologic findings, absence of tremor, early autonomic dysfunction, and poor response to levodopa. The hallmark features of multiple system atrophy include akinetic-rigid parkinsonism, autonomic failure, and cerebellar ataxia. __________ References: Multiple system atrophy (http://www.ncbi.nlm.nih.gov/pubmed/20739256) Second consensus statement on the diagnosis of multiple system atrophy. (http://www.ncbi.nlm.nih.gov/pubmed/18725592)

A 65-Year-Old Man Is Brought to the Office Due to a 2-Year