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Tibial muscular dystrophy in a Belgian family.
- 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.
- Subjects :
- Aged
Belgium
Connectin
Creatine Kinase blood
DNA genetics
DNA Mutational Analysis
Electromyography
Exons genetics
Family
Female
Gait Disorders, Neurologic physiopathology
Humans
Leg pathology
Male
Middle Aged
Muscle Proteins genetics
Muscle, Skeletal pathology
Muscle, Skeletal physiopathology
Muscular Dystrophies physiopathology
Pedigree
Point Mutation genetics
Protein Kinases genetics
Tomography, X-Ray Computed
Leg physiopathology
Muscular Dystrophies genetics
Subjects
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