MYOTONIC SYNDROME (A Report of Myotonica Congenita and Myotonica Dystrophia each in 5 Families)
- VernacularTitle:肌强直综合征
- Author:
Huiguang HOU
- Publication Type:Journal Article
- From:
Academic Journal of Second Military Medical University
1982;0(02):-
- CountryChina
- Language:Chinese
-
Abstract:
This paper presents 14 cases of rnyotonia congenita in 5 families and 21 cases of myotonica dystrophia in another 5 famlies.The former consists of 10 males,4 females, age at 8-32; the latter consists of 17 males, 4 females, age at 10-28. The symptoms are classical. With myotonia congenita, there are difficulty in relaxation following strenous muscle contraction, percussion myotonia and elec-tromyographic myotonic response; while in myotonica dystrophia, there are multisystemic involvement including myotonia and muscular atrophy simultaneously, accompanied by frontal baldness, myopia, cataracts, testicular atrophy, bundle-branch heart block, hypofunction of thyroid gland and adrenal gland, etc. Electromyography also shown myotonic response.The muscle biopsy of two cases of myotonica dystrophia shown non-specific muscle degenration and atrophy.But the nerve endings and motor plates are intact on special staining. The inherited pattern,important diagnostic points and the values of muscle biopsy are discussed.
