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 Volume 71, Number 9, August 26, 2008
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NEUROLOGY 2008;71:670-676
© 2008 American Academy of Neurology

Second consensus statement on the diagnosis of multiple system atrophy

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 and M. Vidailhet, MD, PhD

AFFILIATIONS From the Departments of Neurology, University of Michigan (S.G.), Ann Arbor, MI; Medical University of Innsbruck (G.K.W., W.P., K.S.), Austria; Mayo Clinic Rochester (P.A.L., P.S), Minnesota; and University Hospital Bonn (T.K.), Germany; Hammersmith Hospital and Department of Clinical Neuroscience (D.B.), Imperial College London, UK; Neurovascular Medicine (Pickering) Unit Faculty of Medicine (C.J.M.), Imperial College London, UK; Department of Pathology and Laboratory Medicine (J.Q.T.), University of Pennsylvania, Philadelphia, PA; National Hospital Queen Square (N.W.W., C.F., A.L., N.Q., T.R.), London, UK; Department of Neurological Sciences (C.C.), University of Rome La Sapienza, Italy; INSERM U289 Hôpital de la Salpêtrière (A.D.), Paris, France; New York University School of Medicine (H.K.), New York, NY; Vanderbilt University Clinical Research Center (D.R.), Nashville, TN; and Federation of Neurology and INSERM U679 (M.V.), Pierre Marie Curie Paris-6 University, France.

Address correspondence and reprint requests to Dr. Sid Gilman, Department of Neurology, University of Michigan, 300 N. Ingalls St., 3D15, Ann Arbor, MI 48109-5489 sgilman{at}umich.edu

Background: A consensus conference on multiple system atrophy (MSA) in 1998 established criteria for diagnosis that have been accepted widely. Since then, clinical, laboratory, neuropathologic, and imaging studies have advanced the field, requiring a fresh evaluation of diagnostic criteria. We held a second consensus conference in 2007 and present the results here.

Methods: Experts in the clinical, neuropathologic, and imaging aspects of MSA were invited to participate in a 2-day consensus conference. Participants were divided into five groups, consisting of specialists in the parkinsonian, cerebellar, autonomic, neuropathologic, and imaging aspects of the disorder. Each group independently wrote diagnostic criteria for its area of expertise in advance of the meeting. These criteria were discussed and reconciled during the meeting using consensus methodology.

Results: The new criteria retain the diagnostic categories of MSA with predominant parkinsonism and MSA with predominant cerebellar ataxia to designate the predominant motor features and also retain the designations of definite, probable, and possible MSA. Definite MSA requires neuropathologic demonstration of CNS {alpha}-synuclein–positive glial cytoplasmic inclusions with neurodegenerative changes in striatonigral or olivopontocerebellar structures. Probable MSA requires a sporadic, progressive adult-onset disorder including rigorously defined 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 that may be a clinical or a neuroimaging abnormality.

Conclusions: These new criteria have simplified the previous criteria, have incorporated current knowledge, and are expected to enhance future assessments of the disease.

Abbreviations: [11C]HED = [11C]hydroxyephedrine; AAN = American Academy of Neurology; AAS = American Autonomic Society; DSM-IV = Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition; DWI = diffusion-weighted imaging; ED = erectile dysfunction; FDG = [18F]fluorodeoxyglucose; FXTAS = fragile X–associated tremor/ataxia syndrome; MCP = middle cerebellar peduncle; MIBG = [123I]metaiodobenzylguanidine; MR = magnetic resonance; MSA = multiple system atrophy; MSA-C = MSA with predominant cerebellar ataxia; MSA-P = MSA with predominant parkinsonism; OH = orthostatic hypotension; PD = Parkinson disease; SCA = spinocerebellar ataxia; SMC = Safety Monitoring Committee; RV = residual volume; UPDRS = Unified Parkinson's Disease Rating Scale.

Author's affiliations are listed at the end of the article.

Supported in part by grants from the NIH (National Institute of Neurological Disorders and Stroke grant 1 R13 NS055459), Novartis Pharmaceuticals, and Chelsea Therapeutics.

Disclosure: Author disclosures are provided at the end of the article.

Received January 4, 2008. Accepted in final form May 23, 2008.






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