Expanding concept of clinical conditions and symptoms in multiple system atrophy.

摘要: Multiple system atrophy (MSA) is an adult-onset, progressive neurodegenerative disorder. MSA patients show various phenotypes during the course of their illness including parkinsonism, cerebellar ataxia, autonomic failure, and pyramidal signs. classified into parkinsonian (MSA-P) or (MSA-C) variant depending on clinical motor phenotype at presentation. MSA-P MSA-C are predominant in Western countries Japan, respectively. The mean age onset 55 to 60 years. Prognosis ranges from 6 10 years, but some cases survive for more than 15 Early severe failure a poor prognostic factor. sometimes present with isolated symptoms/signs, median duration concomitant appearance symptoms was approximately 2 years our previous study. As presence combination essential current diagnostic criteria, early diagnosis difficult when symptoms/signs. We experienced who died before presentation symptoms/signs (i.e., premotor MSA). Detection nature consistent identification dysfunctional anatomical sites may increase probability MSA. Dementia another problem Although dementia had been thought be rare MSA, frontal lobe dysfunction observed frequently illness. Magnetic resonance imaging can cerebral longstanding cases. More recently, presenting frontotemporal preceding manifestations have reported. Novel criteria based expanding concept conditions will needed development disease-modifying therapies better management.