Document Detail

Neuropharmacology of progressive myoclonus epilepsy: response to 5-hydroxy-L-tryptophan.
MedLine Citation:
PMID:  7543407     Owner:  NLM     Status:  MEDLINE    
Low concentrations of the serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) in cerebrospinal fluid (CSF) of patients with progressive myoclonus epilepsy (PME) suggest hypofunctional serotonergic neurotransmission. To study this hypothesis, we enrolled 6 patients with PME [Unverricht-Lündborg disease (U-L), mitochondrial encephalomyopathy, or Lafora disease] in a controlled, double-blinded, dose-ranging, cross-over add-on pilot clinical trial of 5-hydroxy-L-tryptophan (L-5-HTP) plus carbidopa after 2 other patients had received open-label L-5-HTP for compassionate use. Prestudy CSF 5-HIAA concentrations were low (< 20 ng/ml) in 6 patients regardless of the etiology of PME. One patient with U-L disease showed clinical improvement and a fivefold increase in CSF 5-HIAA, and 1 with Lafora disease showed a twofold increase in CSF 5-HIAA without improvement. A patient with Lafora disease reported enough improvement in myoclonus-evoked convulsions to continue chronic use of the drug. One patient with mitochondrial encephalomyopathy developed status epilepticus during treatment with L-5-HTP. As a group, patients had no statistically significant changes in myoclonus evaluation scale scores, subjective and objective measures of ataxia, seizure frequency, antiepileptic drug (AED) levels, or routine blood tests. These data suggest a serotonergic abnormality regardless of the underlying etiology of PME, but one that seldom responds to acute treatment with L-5-HTP.
M R Pranzatelli; E Tate; Y Huang; R H Haas; J Bodensteiner; S Ashwal; D Franz
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Publication Detail:
Type:  Clinical Trial; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Epilepsia     Volume:  36     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:  1995 Aug 
Date Detail:
Created Date:  1995-09-14     Completed Date:  1995-09-14     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  783-91     Citation Subset:  IM    
Department of Neurology, George Washington University, Washington, D.C., USA.
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MeSH Terms
5-Hydroxytryptophan / therapeutic use*
Carbidopa / therapeutic use
Cross-Over Studies
Double-Blind Method
Drug Therapy, Combination
Epilepsies, Myoclonic / cerebrospinal fluid,  drug therapy*,  metabolism
Hydroxyindoleacetic Acid / cerebrospinal fluid
Mitochondrial Encephalomyopathies / cerebrospinal fluid,  drug therapy,  metabolism
Pilot Projects
Serotonin / metabolism
Grant Support
Reg. No./Substance:
38821-49-7/Carbidopa; 50-67-9/Serotonin; 54-16-0/Hydroxyindoleacetic Acid; 56-69-9/5-Hydroxytryptophan

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