Abstract
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease of undetermined etiology and pathogenesis. Clinically, there are combinations of extrapyramidal, cerebellar, pyramidal and autonomic dysfunctions.
Diagnosis is sorted according to criteria into possible, probable and definite MSA. Our patient is a 43-year-old man who suffred from atypical neurological symptomatology and cognitive impairment of uncertain etiology.
There was no family history of neurodegenerative disease. This case ilustrates the relevance of detailed neuropsychological assessment and that D2 receptor assay with 123I-IBMZ SPECT may be helpful in patients with normal MRI and questionable MSA.
The presence of dementia is considered not to be a feature of MSA. Cognitive impairment was one of initial syndrome in this case and it adds to the great clinicopathological complexity of MSA.
Treatment is mainly supportive, as no curative therapy is known.