Document Detail

Progressive extrinsic ophthalmoplegia with peripheral neuropathy and storage of muscle glycogen.
MedLine Citation:
PMID:  92534     Owner:  NLM     Status:  MEDLINE    
A case of progressive extrinsic ophthalmoplegia associated with peripheral neuropathy and hypertriglyceridemia type IV is described. Motor and sensory conduction velocities of the spinal nerves were severely decreased, while the EMG of the facial muscles was more suggested of a myopathic disorder. Electron microscopic study of biopsies of biceps and peroneus brevis muscles disclosed many ragged red fibers, mainly type I, which contained typical abnormal mitochondria. Other fibers, all type II, contained increased amounts of glycogen between myofibrils or beneath the sarcolemmal membrane, but the mitochondria were normal. These fibers were more abundant in the peroneus brevis than in the biceps muscle. Nerve biopsy revealed marked loss of myelinated fibers, but neither mitochondrial changes nor glycogen storages were evident in Schwann's cells. Biochemical investigations confirmed the increased amount of glycogen in both muscle biopsies and revealed a decrease of guanilcyclase. Phosphorylase, phosphorylase b kinase, adenilcyclase, and carnitine concentrations were all normal. The pathogenesis of this syndrome is discussed and the relationship between mitochondrial abnormalities and glycogen accumulation in muscle tissue are considered.
M Moggio; G Valli; C Cerri; G Scarlato; G Pellegrini
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of neurology     Volume:  221     ISSN:  0340-5354     ISO Abbreviation:  J. Neurol.     Publication Date:  1979 Jul 
Date Detail:
Created Date:  1980-02-26     Completed Date:  1980-02-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0423161     Medline TA:  J Neurol     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  25-37     Citation Subset:  IM    
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MeSH Terms
Glycogen / metabolism
Mitochondria, Muscle / ultrastructure
Muscles / metabolism,  ultrastructure
Neural Conduction
Ophthalmoplegia / metabolism,  pathology*
Peripheral Nervous System Diseases / metabolism,  pathology*
Reg. No./Substance:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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