Abstracts

CLINICAL AND ELECTROENCEPHALOGRAPHIC EVOLUTION IN WEST SYNDROME: COMPARATIVE FOLLOW-UP AND OUTCOME BETWEEN CRIPTOGENIC AND SYMPTOMATIC PATIENTS

Abstract number : 1.171;
Submission category : 4. Clinical Epilepsy
Year : 2007
Submission ID : 7297
Source : www.aesnet.org
Presentation date : 11/30/2007 12:00:00 AM
Published date : Nov 29, 2007, 06:00 AM

Authors :
G. L. Maia1, E. Garzon1, M. Spinosa1, M. Rahal1, C. Marx1, H. Carrete Jr.1, M. Gomes1, A. Martins1, A. Sakamoto1

Rationale: West syndrome (WS) usually consists of a triad formed by the association of epileptic spasms, neuropsychomotor delay and hypsarrhythmia, even though one of these features may be absent. Developmental delay and regression may either precede or follow the beginning of the epileptic spasms. This type of seizure may rarely continue throughout childhood. Most patients (50-70%), however, develop other seizure types and 18-50% evolves to Lennox-Gastaut Syndrome (LGS). WS is classified in terms of etiology as symptomatic or cryptogenic. The symptomatic group comprises 80% of WS cases. Few studies have compared the electrographic evolution in WS according to this classification. The aim of this study was to compare the clinical and electrographic outcome between these two groups. Methods: Retrospective study of patients diagnosed with WS being followed-up between 2002 and 2007 at the Child Epilepsy Ambulatory of the Neurology were evaluated giving emphasis to clinical and electrographic evolution .All patient had video-EEG or prolonged EEG for diagnosis and evolution during treatment for WS. The patients had ACTH, Vigabatrin, Valproic Acid, Topiramate or Nitrazepan for treatment of WS. The EEG were done once a week until WS be controlled. The long term follow-up was done with clinical history, prolonged EEG or video-EEG.Results: Our sample consisted of 10 patients with mean follow-up time of 38.3 moths (14 to 49), 8 male. The mean age at admission was 11.4 months. All patients presented with some degree of neuropsychomotor delay. In Group I, cryptogenic cases (3 patients) two had language delay, one had motor delay and one showed global impairment of neuropsychomotor delay. Out of seven patients with symptomatic WS (Group II), one showed only language delay, the remaining patients had motor deficits and global neuropsychomotor delay. Regarding the EEG evolution, the group I showed focal, multifocal and generalized epileptiform discharges (1 patient each) and the group II showed focal (4 patients) and multifocal (1 patient) epileptiform abnormalities. One of the 2 remaining cases showed non epileptform abnormalities and one normal EEG. Only 1 patient evolved to LGS (group I), 2 patients are seizure free (1 group I and 1 Group II), 6 patients evolved to focal epilepsy (group II) and 1 has both, focal and generalized seizures types (group I). Conclusions: LGS was not the most frequent evolution from WS in our study. The majority of these cases evolved with focal epilepsy and 2 patients are seizure free. Although the types of epileptic syndrome developed after WS obviously depend of etiology, it is known that the early diagnosis and treatment of WS, probably can modify the evolution. We believe that the high incidence of LGS described at the literature is dependent of etiologic bases and the time spend for WS control. Maybe the precocious treatment and diagnosis of WS and weekly EEG performed during the treatment of WS had contributed to this evolution.
Clinical Epilepsy