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Consensus statement on the diagnosis of multiple system atrophy

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TLDR
The clinical features of the disease are described, which include four domains: autonomic failure/urinary dysfunction, parkinsonism and cerebellar ataxia, and corticospinal dysfunction, and criteria are set to define the relative importance of these features.
Abstract
We report the results of a consensus conference on the diagnosis of multiple system atrophy (MSA). We describe the clinical features of the disease, which include four domains: autonomic failure/urinary dysfunction, parkinsonism and cerebellar ataxia, and corticospinal dysfunction. We set criteria to define the relative importance of these features. The diagnosis of possible MSA requires one criterion plus two features from separate domains. The diagnosis of probable MSA requires the criterion for autonomic failure/urinary dysfunction plus poor levodopa responsive parkinsonism or cerebellar ataxia. The diagnosis of definite MSA requires pathological confirmation.

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Journal ArticleDOI

Delayed emergence of a parkinsonian disorder or dementia in 81% of older men initially diagnosed with idiopathic rapid eye movement sleep behavior disorder: a 16-year update on a previously reported series

TL;DR: The vast majority of men > or =50 years old initially diagnosed with iRBD in this study eventually developed a parkinsonian disorder/dementia, often after a prolonged interval from onset of iR BD, with the mean interval being 14 years while the range extended to 29 years.
Journal ArticleDOI

Multiple system atrophy

TL;DR: Up-to-date knowledge on the clinical diagnosis and molecular pathology of MSA is summarized and the role of additional investigations that may support a clinical diagnosis are reviewed.
Journal ArticleDOI

Multiple system atrophy: an update

TL;DR: Advances in the early recognition of this disease have resulted in revised diagnostic criteria, including, for the first time, neuroimaging indices, and novel therapeutic options targeting disease modification have been investigated in clinical trials.
References
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Journal ArticleDOI

Consensus statement on the definition of orthostatic hypotension, pure autonomic failure and multiple system atrophy.

TL;DR: A Consensus Connce was convened on 16 November 1995 in Phoenix, Arizona, USA with the specific aim of generating a consensus on three specific items: the definition of orthostatic hypotension, pure autonomic failure (Bradbury Eggleston syndrome), and multiple system atrophy.
Journal ArticleDOI

Clinical features and natural history of multiple system atrophy. An analysis of 100 cases.

TL;DR: The clinical features and natural history of 100 patients diagnosed as probable multiple system atrophy (MSA) are described, and in all 14 cases who underwent post-mortem examination of the brain, the diagnosis was confirmed pathologically, providing some validation of the clinical diagnostic criteria used.
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