Second consensus statement on the diagnosis of multiple system atrophy

S. Gilman, G. K. Wenning, Phillip Anson Low, D. J. Brooks, C. J. Mathias, J. Q. Trojanowski, N. W. Wood, C. Colosimo, A. Dürr, C. J. Fowler, H. Kaufmann, T. Klockgether, A. Lees, W. Poewe, N. Quinn, T. Revesz, D. Robertson, P. Sandroni, K. Seppi, M. Vidailhet

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Abstract

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 α-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.

Original languageEnglish (US)
Pages (from-to)670-676
Number of pages7
JournalNeurology
Volume71
Issue number9
DOIs
StatePublished - Aug 26 2008

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Multiple System Atrophy
Cerebellar Ataxia
Parkinsonian Disorders
Synucleins
Inclusion Bodies
Levodopa
Neuroimaging
Neuroglia

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Gilman, S., Wenning, G. K., Low, P. A., Brooks, D. J., Mathias, C. J., Trojanowski, J. Q., ... Vidailhet, M. (2008). Second consensus statement on the diagnosis of multiple system atrophy. Neurology, 71(9), 670-676. https://doi.org/10.1212/01.wnl.0000324625.00404.15

Second consensus statement on the diagnosis of multiple system atrophy. / Gilman, S.; Wenning, G. K.; Low, Phillip Anson; Brooks, D. J.; Mathias, C. J.; Trojanowski, J. Q.; Wood, N. W.; Colosimo, C.; Dürr, A.; Fowler, C. J.; Kaufmann, H.; Klockgether, T.; Lees, A.; Poewe, W.; Quinn, N.; Revesz, T.; Robertson, D.; Sandroni, P.; Seppi, K.; Vidailhet, M.

In: Neurology, Vol. 71, No. 9, 26.08.2008, p. 670-676.

Research output: Contribution to journalArticle

Gilman, S, Wenning, GK, Low, PA, Brooks, DJ, Mathias, CJ, Trojanowski, JQ, Wood, NW, Colosimo, C, Dürr, A, Fowler, CJ, Kaufmann, H, Klockgether, T, Lees, A, Poewe, W, Quinn, N, Revesz, T, Robertson, D, Sandroni, P, Seppi, K & Vidailhet, M 2008, 'Second consensus statement on the diagnosis of multiple system atrophy', Neurology, vol. 71, no. 9, pp. 670-676. https://doi.org/10.1212/01.wnl.0000324625.00404.15
Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008 Aug 26;71(9):670-676. https://doi.org/10.1212/01.wnl.0000324625.00404.15
Gilman, S. ; Wenning, G. K. ; Low, Phillip Anson ; Brooks, D. J. ; Mathias, C. J. ; Trojanowski, J. Q. ; Wood, N. W. ; Colosimo, C. ; Dürr, A. ; Fowler, C. J. ; Kaufmann, H. ; Klockgether, T. ; Lees, A. ; Poewe, W. ; Quinn, N. ; Revesz, T. ; Robertson, D. ; Sandroni, P. ; Seppi, K. ; Vidailhet, M. / Second consensus statement on the diagnosis of multiple system atrophy. In: Neurology. 2008 ; Vol. 71, No. 9. pp. 670-676.
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T1 - Second consensus statement on the diagnosis of multiple system atrophy

AU - Gilman, S.

AU - Wenning, G. K.

AU - Low, Phillip Anson

AU - Brooks, D. J.

AU - Mathias, C. J.

AU - Trojanowski, J. Q.

AU - Wood, N. W.

AU - Colosimo, C.

AU - Dürr, A.

AU - Fowler, C. J.

AU - Kaufmann, H.

AU - Klockgether, T.

AU - Lees, A.

AU - Poewe, W.

AU - Quinn, N.

AU - Revesz, T.

AU - Robertson, D.

AU - Sandroni, P.

AU - Seppi, K.

AU - Vidailhet, M.

PY - 2008/8/26

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N2 - 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 α-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.

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