Document Detail

Electromyography in congenital nemaline myopathy.
MedLine Citation:
PMID:  2674705     Owner:  NLM     Status:  MEDLINE    
To clarify the discrepancies between earlier reports of electromyography (EMG) in congenital nemaline myopathy (CNM), conventional electromyography was done on 13 patients with CNM, and results were compared with those of 18 earlier EMG examinations of the same patients. Fiber density was measured in 10 patients with a computerized method and neuromuscular jitter in 3 with single-fiber EMG. With age, the EMG abnormality progressed, and "neuropathic" EMG features developed in distal muscles. In 9 of 10 patients fiber density was higher than normal. In two of three patients jitter was abnormal. Motor (13 of 13 patients) and sensory (3 of 3 patients) nerve conduction velocities were normal. Our results seem to explain the conflicting reports of EMG in CNM. We conclude that active degeneration and regeneration of muscle fibers takes place in CNM and suggest that the "neuropathic" motor unit potentials seen in our patients may be secondary to myopathic disease activity.
C Wallgren-Pettersson; K Sainio; T Salmi
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Muscle & nerve     Volume:  12     ISSN:  0148-639X     ISO Abbreviation:  Muscle Nerve     Publication Date:  1989 Jul 
Date Detail:
Created Date:  1989-10-23     Completed Date:  1989-10-23     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  587-93     Citation Subset:  IM    
Children's Hospital, University of Helsinki, Finland.
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MeSH Terms
Muscular Diseases / congenital,  physiopathology*

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