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March 23, 2009

Natural history and clinical features of the flail arm and flail leg ALS variants

L. C. Wijesekera, MRCP, S. Mathers, FRACP, P. Talman, PhD, FRACP, C. Galtrey, PhD, MRCP, M. H. Parkinson, MRCP, J. Ganesalingam, MRCP, E. Willey, BA, M. A. Ampong, C. M. Ellis, PhD, FRCP, C. E. Shaw, MD, FRCP, A. Al-Chalabi, PhD, FRCP, and P. N. Leigh, FMedSci, FAANAuthors Info & Affiliations

March 24, 2009 issue

72 (12) 1087-1094

https://doi.org/10.1212/01.wnl.0000345041.83406.a2

Abstract

Objective: We sought to define the significance of brachial amyotrophic diplegia (flail arm syndrome [FA]) and the pseudopolyneuritic variant (flail leg syndrome [FL]) of amyotrophic lateral sclerosis (ALS; motor neuron disease).

Methods: We analyzed survival in clinic cohorts in London, UK (1,188 cases), and Melbourne, Australia (432 cases). Survival from disease onset was analyzed using the Kaplan- Meier method and Cox proportional hazards model.

Results: In the London cohort, the FA syndrome represented 11% and the FL syndrome 6% of the sample. Median survival was 35 months for limb onset and 27 months for bulbar onset ALS, whereas this was 61 months for FA syndrome (p < 0.001) and 69 months for FL syndrome (p < 0.001). Five-year survival in this cohort was 8.8% for bulbar onset, 20% for limb onset, 52% for FA syndrome, and 64% for FL syndrome. The ratio of men to women was 4:1 in the FA group compared to 2:1 in other limb onset cases. Excluding lower motor neuron FA and FL cases, progressive muscular atrophy comprised 4% of the sample and had a prognosis similar to typical limb onset ALS. In the Melbourne cohort, median survival for limb onset ALS was 31 months, bulbar onset 27 months, FA syndrome 66 months (p < 0.001), and FL syndrome 71 months (p = 0.001).

Conclusions: The flail arm (FA) and flail leg (FL) syndromes had significantly better survival than typical amyotrophic lateral sclerosis (ALS) or progressive muscular atrophy cases that were not classified as FA or FL. Our findings underline the clinical and prognostic importance of the FA and FL variants of ALS.

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Information & Authors

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Published In

Neurology^®

Volume 72 • Number 12 • March 24, 2009

Pages: 1087-1094

PubMed: 19307543

Copyright

© 2009.

Publication History

Published online: March 23, 2009

Published in print: March 24, 2009

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Authors

Affiliations & Disclosures

L. C. Wijesekera, MRCP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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S. Mathers, FRACP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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P. Talman, PhD, FRACP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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C. Galtrey, PhD, MRCP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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M. H. Parkinson, MRCP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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J. Ganesalingam, MRCP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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E. Willey, BA

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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M. A. Ampong

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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C. M. Ellis, PhD, FRCP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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C. E. Shaw, MD, FRCP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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A. Al-Chalabi, PhD, FRCP

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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P. N. Leigh, FMedSci, FAAN

From MRC Center for Neurodegeneration Research (C.W., C.G., M.H.P., J.G., E.W., M.A.A., C.M.E., C.E.S., A.A.-C., P.N.L.), Kings College London, Institute of Psychiatry, Department of Clinical Neuroscience, London, UK; and Department of Neurology (S.M., P.T.), Calvary Health Care, Bethlehem, Melbourne, Australia.

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Notes

Address correspondence and reprint requests to Prof Leigh, MRC Center for Neurodegeneration Research, Kings College London, Institute of Psychiatry, PO 41, Department of Clinical Neuroscience, London SE5 8AF, UK [email protected]

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