HYALINE BODY MYOPATHY

被引：30
|
作者：
BAROHN, RJ
BRUMBACK, RA
MENDELL, JR
机构：
[1] UNIV OKLAHOMA,HLTH SCI CTR,DEPT PATHOL NEUROPATHOL,OKLAHOMA CITY,OK
[2] OHIO STATE UNIV,COLL MED,DEPT NEUROL,COLUMBUS,OH 43210
来源：
NEUROMUSCULAR DISORDERS | 1994年 / 4卷 / 03期
关键词：
HYALINE BODY; MYOPATHY; CONGENITAL LYSIS OF MYOFIBRILS; TYPE 1 MUSCLE FIBERS;
D O I：
10.1016/0960-8966(94)90027-2
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
Muscle biopsy from two unrelated patients, a male aged 40 and a female aged 3, with relatively non-progressive limb weakness since infancy, revealed numerous subsarcolemmal glassy, hyaline appearing bodies present in 20-30% of the fibres. Type 1 fibre predominance was present, and the hyaline bodies were exclusive to type 1 fibres. The hyaline bodies were negative with oxidative enzyme and periodic acid-Schiff stains. Electron microscopy showed the hyaline bodies to contain amorphous granular material of unknown composition. No membrane separated the hyaline bodies from the surrounding sarcoplasm. Hyaline body myopathy most likely represents a distinct congenital myopathy because of its childhoot-onset, non-progressive course, and distinct morphological features.
引用
收藏
页码：257 / 262
页数：6
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