Document Detail

Relief from episodic weakness with pyridostigmine in paramyotonia congenita: a family study.
MedLine Citation:
PMID:  19768756     Owner:  NLM     Status:  MEDLINE    
Pyridostigmine relieved episodic weakness in a family with paramyotonia congenita resulting from the R1448C mutation in the sodium channel gene. The transmission was autosomal dominant and the patients had paradoxical myotonia and exercise-induced weakness. On electrophysiological studies there were myotonic potentials, and there was progressive reduction of compound muscle action potential (CMAP) amplitudes after short exercise associated with clinical weakness. Pyridostigmine in doses of 60 mg three times daily abolished the drop in the postexercise CMAP amplitude and reduced the amplitude decrement to slow rate repetitive stimulation, but there continued to be a drop in amplitude on exposure to cold. The decline of the CMAP amplitude on exposure to cold was controlled by treatment with phenytoin. The clinical and electrophysiological features are discussed in relation to therapy with pyridostigmine and phenytoin.
Satish V Khadilkar; R K Singh; K A Mansukhani; J A Urtizberea; D Sternberg
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Muscle & nerve     Volume:  41     ISSN:  1097-4598     ISO Abbreviation:  Muscle Nerve     Publication Date:  2010 Jan 
Date Detail:
Created Date:  2009-12-21     Completed Date:  2010-01-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  United States    
Other Details:
Languages:  eng     Pagination:  133-7     Citation Subset:  IM    
Department of Neurology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, and Honorary Neurologist, 110, New Wing, First Floor, Bombay Hospital, 12, New Marine Lines, Mumbai, India.
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MeSH Terms
Cholinesterase Inhibitors / therapeutic use
Follow-Up Studies
Muscle Weakness / drug therapy*,  etiology,  physiopathology
Myotonic Disorders / complications*,  drug therapy,  physiopathology
Pyridostigmine Bromide / therapeutic use*
Reg. No./Substance:
0/Cholinesterase Inhibitors; 101-26-8/Pyridostigmine Bromide

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

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