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A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS

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Multiple system atrophy: prognostic indicators of survival.

Juan J Figueroa¹, Wolfgang Singer, Ajay Parsaik

¹Department of Neurology, Medical College of Wisconsin, Milwaukee, WI.

Movement Disorders : Official Journal of the Movement Disorder Society

|June 10, 2014

Summary

Early severe autonomic failure in multiple system atrophy (MSA) significantly predicts shorter survival. This finding highlights the importance of monitoring autonomic function for prognosis in MSA patients.

Keywords:

MSA autonomic failure mortality survival

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Area of Science:

Neurology
Autonomic Neuroscience

Background:

Multiple system atrophy (MSA) presents with neurological and autonomic symptoms that can indicate prognosis.
Quantifying early autonomic dysfunction's impact on mortality in MSA is crucial but lacks standardized methods.

Purpose of the Study:

To retrospectively analyze early clinical features of MSA patients to identify predictors of mortality.
To investigate the prognostic value of quantified autonomic failure using the composite autonomic severity score (COMPASS) and thermoregulatory sweat test (TST).

Main Methods:

Retrospective review of 49 autopsy-confirmed MSA cases.
Assessment of early neurological and autonomic symptoms, including COMPASS and TST.
Cox proportional hazard analysis to identify mortality predictors.

Main Results:

Median survival in MSA was 8.6 years.
Early generalized autonomic failure (COMPASS ≥ 6) and early bladder catheterization were associated with significantly shorter survival.
Older age at onset, early bladder catheterization, and early generalized autonomic failure were independent predictors of shorter survival.

Conclusions:

Early development of severe generalized autonomic failure is a significant risk factor for reduced survival in MSA, more than tripling the risk.
While other factors like gender or specific symptoms were not predictive, autonomic assessment is vital for MSA prognosis.