ICNC2018 Abstracts & Symposia Proposals, ICNC 2014

Font Size: 
WEST SYNDROME IN CHILD WITH DOWN SYNDROME: DESCRIPTION OF A NUMBER OF CASES.
Carla Rojas, Jocelyn Gutiérrez, Scarlet Witting, Paula Ortega, Francisca Faure

Last modified: 2014-04-03

Abstract


Introduction: The prevalence of episodes among people with Down Syndrome (DS) is greater than in the general population (1-13%). A third of these cases presents West Syndrome(WS) epileptic encephalopathy, characterized by spams, regression/ retardation in the psychomotor development (PMD) and hypsarrhythmia. Typically these cases have a satisfactory outcome. Objetive: To analyze clinical characteristics, EEGs, outcome of patients with DS associated with WS. Method: A retrospective descriptive study of 7 patients with DS associated with WS between January 2002 and May 2011 in our departamento of Child Neuropsychiatry. Results: Out of 7 patients (4/7 women) the average first occurrence of spams is at 9 months of age. Delay diagnosis: average age 6.2 months. 7/7 present retardation PMD, 2/7 regression PMD. 7/7 Flexor spam. 6/7 received ACTH and valproic 1/7 valproic only. Post cure ACTH: 4/6 complete termination of spams with normal EEG (2/4). 2/6 partial remission with persistent spams hypsarrhythmia (1/2) and severely abnormal EEG non hypsarrhythmic (1/2). Outcome: Patients with good initial response to treatment, clinical and/or electrical (4/7)showed no recurrence or persistence of crisis at 6 months follow up. Conclusions: Nearly 40% were unfavorable, not concordant with the literature.


Keywords


Infantile spasms, Down Syndrome, West Syndrome, Hypsarrhytmia

Conference registration is required in order to view papers.