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Cited by 275

The DOI matches its Crossref record; 20 of the 20 references it cites were found in a registry or an index.

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

Open access
Wijesekera, L. C., Mathers, S., Talman, P., Galtrey, C., Parkinson, M. H., Ganesalingam, J., Willey, E., Ampong, M. A., Ellis, C. M., Shaw, C. E., Al-Chalabi, A., Leigh, P. N.·2009·Neurology
DOI 10.1212/01.wnl.0000345041.83406.a2
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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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Metadata

Authors
Wijesekera, L. C., Mathers, S., Talman, P., Galtrey, C., Parkinson, M. H., Ganesalingam, J., Willey, E., Ampong, M. A., Ellis, C. M., Shaw, C. E., Al-Chalabi, A., & Leigh, P. N.
Venue
Neurology
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2009-03-24
Volume
72
Issue
12
Pages
1087-1094
ISSN
  • 0028-3878 (ISSN-L)
  • 1526-632X
DOI
10.1212/01.wnl.0000345041.83406.a2
OpenAlex
W2021977705
Cited by
275
References
20
FWCI
10.50
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Natural history and clinical features of the flail arm and flail leg ALS variants. https://app.cochat.ai/papers/10.1212/01.wnl.0000345041.83406.a2
The paper
Wijesekera, L. C., Mathers, S., Talman, P., Galtrey, C., Parkinson, M. H., Ganesalingam, J., Willey, E., Ampong, M. A., Ellis, C. M., Shaw, C. E., Al-Chalabi, A., & Leigh, P. N. (2009). Natural history and clinical features of the flail arm and flail leg ALS variants. Neurology. https://doi.org/10.1212/01.wnl.0000345041.83406.a2

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.