The distal dystrophy of Welander is a rare form on late onset muscular dystrophy.


Synonym: myopathia distalis tarda heredetaria:


Inheritance: usually autosomal domiant


Genetic locus: uncertain (possibly chromosome 14)


Clinical features:

(1) The onset is between 40 and 60 years of age.

(2) The earliest sign is a progressive weakness of the intrinsic hand muscles.

(3) Weakness of the tibialis anterior muscle may occur later, with or without pseudohypertrophy of the calf.


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