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Clinical variability of congenital myopathy with type 1 fiber atrophy: a long-term observation of three cases
- Source :
- Acta paediatrica Japonica : Overseas edition. 36(2)
- Publication Year :
- 1994
-
Abstract
- The variable clinical courses of three cases of congenital fiber type disproportion (CFTD) over a period of 10 years are presented. All showed improvement in early childhood, but subsequently, varying degrees of deterioration were noted: specifically, marked deterioration in case 2 and decreased muscle strength in case 3. Maximal motor function levels were attained differently among the cases. Histological findings included type 1 fiber hypotrophy and increased internal nuclei in common in all cases. Fine structural changes, such as patchy areas of myofibrillar degeneration, were noted in cases 1 and 2 (second biopsy), and cytoplasmic bodies were seen in case 2 (second biopsy). Myotubes were noted in case 3. The degree of cyto-architectural changes did not correlate with clinical severity. The heterogeneity of CFTD is also discussed.
- Subjects :
- Adult
Male
Adolescent
Muscular Diseases
Child, Preschool
Muscles
Humans
Child
Subjects
Details
- ISSN :
- 03745600
- Volume :
- 36
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Acta paediatrica Japonica : Overseas edition
- Accession number :
- edsair.pmid..........494cd66ce6bedb3dcf67cfea4095650b