Document Detail


Reflex seizures in Rett syndrome.
MedLine Citation:
PMID:  22258043     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Reflex seizures are a rare phenomenon among epileptic patients, in which an epileptic discharge is triggered by various kinds of stimuli (visual, auditory, tactile or gustatory). Epilepsy is common in Rett syndrome patients (up to 70%), but to the authors' knowledge, no pressure or eating-triggered seizures have yet been reported in Rett children. We describe three epileptic Rett patients with reflex seizures, triggered by food intake or proprioception. One patient with congenital Rett Sd. developed infantile epileptic spasms at around seven months and two patients with classic Rett Sd. presented with generalised tonic-clonic seizures at around five years. Reflex seizures appeared when the patients were teenagers. The congenital-Rett patient presented eating-triggered seizures at the beginning of almost every meal, demonstrated by EEG recording. Both classic Rett patients showed self-provoked pressure -triggered attacks, influenced by stress or excitement. Non-triggered seizures were controlled with carbamazepine or valproate, but reflex seizures did not respond to antiepileptic drugs. Risperidone partially improved self-provoked seizures. When reflex seizures are suspected, reproducing the trigger during EEG recording is fundamental; however, self-provoked seizures depend largely on the patient's will. Optimal therapy (though not always possible) consists of avoiding the trigger. Stress modifiers such as risperidone may help control self-provoked seizures.
Authors:
Ana Roche Martínez; M Itziar Alonso Colmenero; Andreia Gomes Pereira; Francesc X Sanmartí Vilaplana; Judith Armstrong Morón; Mercé Pineda Marfa
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Epileptic disorders : international epilepsy journal with videotape     Volume:  13     ISSN:  1294-9361     ISO Abbreviation:  Epileptic Disord     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2012-01-19     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100891853     Medline TA:  Epileptic Disord     Country:  France    
Other Details:
Languages:  eng     Pagination:  389-93     Citation Subset:  IM    
Affiliation:
Department of Child Neurology.
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