Document Detail


Neurofibromatosis type one and West syndrome: a relatively benign association.
MedLine Citation:
PMID:  8392464     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fifteen patients with neurofibromatosis type one (NF1) and West syndrome (WS) were studied. The evolution of WS was clearly different from that seen in the other neurocutaneous syndromes, especially tuberous sclerosis. Steroids were efficacious and spasms disappeared. There was no relapse after discontinuation of steroids and antiepileptic drugs were successfully stopped several years later. Mental outcome was also very good. The association of both conditions does not seem to be a coincidence. WS with NF1 seems remarkably benign and resembles idiopathic WS more than symptomatic WS.
Authors:
J Motte; C Billard; N Fejerman; Z Sfaello; H Arroyo; O Dulac
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Epilepsia     Volume:  34     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:    1993 Jul-Aug
Date Detail:
Created Date:  1993-08-17     Completed Date:  1993-08-17     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  723-6     Citation Subset:  IM    
Affiliation:
Unité de Neurologie Pédiatrique, American Memorial Hospital, CHU, Reims, France.
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MeSH Terms
Descriptor/Qualifier:
Adrenocorticotropic Hormone / therapeutic use
Anticonvulsants / therapeutic use
Comorbidity
Electroencephalography
Female
Follow-Up Studies
Humans
Infant
Male
Neurofibromatosis 1 / diagnosis,  drug therapy,  epidemiology*
Prognosis
Retrospective Studies
Spasms, Infantile / diagnosis,  drug therapy,  epidemiology*
Tomography, X-Ray Computed
Treatment Outcome
Chemical
Reg. No./Substance:
0/Anticonvulsants; 9002-60-2/Adrenocorticotropic Hormone

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


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