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Astrocytosis and caudate atrophy
25%
8/32
Glial cytoplasmic inclusions
19%
6/32
Round intracellular tau protein aggregates
Beta-amyloid plaques
22%
7/32
Periventricular white matter plaques
6%
2/32
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This patient suffered from multiple system atrophy (MSA), a degenerative neurologic disorder that presents with symptoms of Parkinsonism (slow and stiff/rigid movement) unresponsive to levodopa, cerebellar ataxia, orthostatic hypotension, incontinence, impotence, and other autonomic symptoms. Definitive diagnosis may only be made on autopsy evaluation, with CNS tissue showing numerous glial cytoplasmic inclusions. Multiple system atrophy is a relatively rare disease that occurs more commonly in men and typically first presents in the 6th or 7th decade of life. MSA is a progressive disease with increasing disability and death often occurring within 10 years of symptom onset. There is no definitive diagnostic test; however, clinical diagnostic criteria have been developed. Still, it is often difficult to differentiate Parkinson's disease from MSA; the non-response to levodopa therapy is often a key determinant in separating these two diseases. No known therapy is present and supportive and hospice care are recommended. Wenning et al. review 203 cases of MSA as proven by pathologic examination. They were able to correlate pathologic changes on autopsy with specific symptom patterns. The amount of cell loss in the putamen and substantia nigra was associated with increased degrees of akinesia and rigidity. Cell loss at any site was not related to the degree of tremor. The amount of olivopontocerebellar atrophy correlated with degree of ataxia experienced. Wenning and Colisimo discuss diagnostic criteria for MSA. A rapid progression of the disease and poor or absent response to levodopa therapy should increase suspicion for atypical parkinsonian disorders such as MSA. Other diseases in addition to MSA that are classified as atypical parkinsonian disorders include progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and dementia with Lewy bodies (DLB). Illustration A summarizes the symptoms and presentation of MSA. Illustration B depicts the abundant glial cytoplasmic inclusions seen in MSA. Incorrect Answers: Answer 1: Astrocytosis and caudate atrophy are expected in Huntington's disease. Answer 3: Round intracellular tau protein aggregates are seen in Pick's disease. Answer 4: Beta-amyloid plaques are characteristic of Alzheimer's disease. Answer 5: Periventricular white matter plaques are characteristic of multiple sclerosis.
3.7
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