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Neurofibromatosis Type One and West Syndrome: A Relatively Benign Association
Author(s) -
Motte J.,
Billard C.,
Fejerman N.,
Sfaello Z.,
Arroyo H.,
Dulac O.
Publication year - 1993
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1157.1993.tb00452.x
Subject(s) - tuberous sclerosis , discontinuation , neurofibromatosis , medicine , epilepsy , pediatrics , dermatology , surgery , psychiatry , pathology
Summary: 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 succesfully 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.