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Tibial muscular dystrophy in a Belgian family.

Authors :
Van den Bergh PY
Bouquiaux O
Verellen C
Marchand S
Richard I
Hackman P
Udd B
Source :
Annals of neurology [Ann Neurol] 2003 Aug; Vol. 54 (2), pp. 248-51.
Publication Year :
2003

Abstract

We report a Belgian family with autosomal dominant, late-onset, distal myopathy with selective foot extensor muscle involvement of the lower legs. Linkage to the tibial muscular dystrophy (TMD) locus 2q31 was not evident at first because of incomplete disease penetrance in a 50-year-old asymptomatic family member. An abnormal tibialis anterior muscle biopsy established her subclinical status and linkage of the family to the TMD locus. Mutation analysis showed a disease-specific, heterozygous point mutation in the last exon, Mex6, of the titin gene. This is the third mutation found in TMD and the second European family with TMD outside the Finnish population, suggesting that titinopathies may occur in various populations.

Details

Language :
English
ISSN :
0364-5134
Volume :
54
Issue :
2
Database :
MEDLINE
Journal :
Annals of neurology
Publication Type :
Academic Journal
Accession number :
12891679
Full Text :
https://doi.org/10.1002/ana.10647