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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis

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Second consensus statement on the diagnosis of multiple system atrophy.

S Gilman¹, G K Wenning, P A Low

¹Department of Neurology, University of Michigan, 300 N. Ingalls St., 3D15, Ann Arbor, MI 48109-5489, USA. sgilman@umich.edu

|August 30, 2008

Summary

New diagnostic criteria for multiple system atrophy (MSA) were established in 2007. These updated guidelines simplify diagnosis and incorporate current knowledge for improved patient assessment.

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Area of Science:

Neurology
Neuroscience
Pathology

Background:

Established 1998 consensus criteria for Multiple System Atrophy (MSA) diagnosis.
Advances in clinical, laboratory, neuropathologic, and imaging studies necessitated revised criteria.
A second consensus conference was held in 2007 to update diagnostic guidelines.

Framework:

Retained diagnostic categories: MSA with predominant parkinsonism and MSA with predominant cerebellar ataxia.
Maintained designations: definite, probable, and possible MSA.
Defined neuropathologic hallmarks for definite MSA (CNS alpha-synuclein-positive glial cytoplasmic inclusions).

Implementation:

Probable MSA requires sporadic, progressive adult-onset disorder with autonomic failure and levodopa-unresponsive parkinsonism or ataxia.
Possible MSA includes parkinsonism or ataxia plus autonomic dysfunction features and a clinical/neuroimaging abnormality.
Consensus methodology was used to reconcile expert-written criteria from specialized groups.

Implications:

The revised criteria simplify previous guidelines.
Incorporates the latest scientific knowledge on MSA.
Expected to enhance future disease assessments and research.