Monday, April 25, 2011

Flail arm and flail leg variants of ALS

Neurology 2009; 72: 1087-1094

These are phenotypic variants of ALS that have been described a century or more ago that have unique characteristics including

1.  In flail arm, proximal wasting and weakness, in flail leg, distal weakness or wasting
2.  LMN variant with no clonus, hypertonia or UMN signs, or involvement of other extremity (leg +  arm) or bulbar involvement
3.  4:1 male predominance in FA, 1:1 gender equality in FL in London; 10:1 and 5:1 respectively in Melbourne series
4.  Relatively longer prognosis than other forms of ALS with lifespan of around 6 years

Synonyms FA:  Vulpian-Bernhardt syndrome, hanging arm syndrome, neurogenic man in a barrel syndrome, or amyotrophic brachial diplegia
Synonyms FL: Marie -Patrikios variant, pseudopolyneuritic variant of ALS, peroneal form of ALS

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