2008;71:670-676 | S. Gilman, MD, FRCP G.K. Wenning, MD, PhD P.A. Low, MD, FRACP, FRCP(Hon) D.J. Brooks, MD, DSc, FRCP, FMedSci C.J. Mathias, MBBS, DPhil, DSc, FRCP, FMedSci J.Q. Trojanowski, MD, PhD N.W. Wood, MB, ChB, PhD, FRCP C. Colosimo, MD A. Dürr, MD, PhD C.J. Fowler, FRCP H. Kaufmann, MD T. Klockgether, MD, PhD A. Lees, MD, FRCP W. Poewe, MD N. Quinn, MD, FRCP T. Revesz, MD D. Robertson, MD P. Sandroni, MD, PhD K. Seppi, MD M. Vidaillhet, MD, PhD
The second consensus statement on the diagnosis of multiple system atrophy (MSA) was developed following a 2007 conference of experts in clinical, neuropathologic, and imaging aspects of MSA. The new criteria retain the diagnostic categories of MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar ataxia (MSA-C), and the designations of definite, probable, and possible MSA. Definite MSA requires neuropathologic demonstration of CNS α-synuclein-positive glial cytoplasmic inclusions with neurodegenerative changes in striatonigral or olivopontocerebellar structures. Probable MSA requires a sporadic, progressive adult-onset disorder including autonomic failure and poorly levodopa-responsive parkinsonism or cerebellar ataxia. Possible MSA requires a sporadic, progressive adult-onset disease including parkinsonism or cerebellar ataxia and at least one feature suggesting autonomic dysfunction plus one other feature. The new criteria simplify the previous ones, incorporate current knowledge, and are expected to enhance future assessments of the disease.The second consensus statement on the diagnosis of multiple system atrophy (MSA) was developed following a 2007 conference of experts in clinical, neuropathologic, and imaging aspects of MSA. The new criteria retain the diagnostic categories of MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar ataxia (MSA-C), and the designations of definite, probable, and possible MSA. Definite MSA requires neuropathologic demonstration of CNS α-synuclein-positive glial cytoplasmic inclusions with neurodegenerative changes in striatonigral or olivopontocerebellar structures. Probable MSA requires a sporadic, progressive adult-onset disorder including autonomic failure and poorly levodopa-responsive parkinsonism or cerebellar ataxia. Possible MSA requires a sporadic, progressive adult-onset disease including parkinsonism or cerebellar ataxia and at least one feature suggesting autonomic dysfunction plus one other feature. The new criteria simplify the previous ones, incorporate current knowledge, and are expected to enhance future assessments of the disease.