Multiplex Families With Multiple System Atrophy

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Multiplex families with multiple system atrophy.

BACKGROUND Multiple system atrophy (MSA) has been considered a sporadic disease, without patterns of inheritance. OBJECTIVE To describe the clinical features of 4 multiplex families with MSA, including clinical genetic aspects. DESIGN Clinical and genetic study. SETTING Four departments of neurology in Japan. Patients Eight patients in 4 families with parkinsonism, cerebellar ataxia, and ...

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Multiple system atrophy.

Multiple system atrophy (MSA) is an adult-onset sporadic progressive neurodegenerative disorder of unknown etiology. It is clinically characterized by the variable combination of autonomic failure, parkinsonism, cerebellar ataxia, and pyramidal signs. The present review summarizes up-to-date knowledge on the clinical diagnosis and molecular pathology of MSA. We also review the role of additiona...

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Multiple system atrophy.

Multiple system atrophy is a neurological disorder that has gone unrecognized for too long due to its involvement across multiple regions of the central nervous system. This disorder is finally being unveiled through increased reporting in the scientific literature. Further research will enhance our understanding of this disease and lead to more effective treatment regimens as well as an improv...

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Multiple system atrophy.

Although the precise definition of multiple system atrophy has been difficult, a recent consensus in diagnostic criteria for multiple system atrophy has been achieved. This should lead to progress in defining the underlying pathophysiology of the neuroendocrine, autonomic and motor deficits characteristic of multiple system atrophy. Hopefully, these developments will lead to effective treatment.

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Survival of patients with multiple system atrophy.

We investigated the survival of patients with multiple system atrophy (MSA) in a follow-up study of 59 patients admitted to Nagoya University Hospital between 1976 and 1991. They consisted of 24 patients with olivopontocerebellar atrophy (OPCA), 25 with Shy-Drager syndrome (SDS) and 10 with striatonigral degeneration (SND). The mean age at onset was 54 years, the 3-year survival rate from onset...

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ژورنال

عنوان ژورنال: Archives of Neurology

سال: 2007

ISSN: 0003-9942

DOI: 10.1001/archneur.64.4.545