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

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.

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 pnigel.leigh{at}iop.kcl.ac.uk

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.

Abbreviations: ALS = amyotrophic lateral sclerosis; CI = confidence interval; DTR = deep tendon reflex; FA = flail arm syndrome; FL = flail leg syndrome; LL = lower limbs; LMN = lower motor neuron; MND = motor neuron disease; NIV = noninvasive ventilation; PMA = progressive muscular atrophy; UL = upper limbs; UMN = upper motor neuron.

Supplemental data at www.neurology.org

*A.A.-C. and P.N.L. are joint senior authors who contributed equally.

Disclosure: The authors report no disclosures.

Received May 20, 2008. Accepted in final form January 8, 2009.

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