Age-Based Characteristics of West Syndrome in Patients with Mitochondrial Disease / 대한소아신경학회지
Journal of the Korean Child Neurology Society
;
(4): 197-204, 2018.
Artigo
em Inglês
| WPRIM
| ID: wpr-728821
ABSTRACT
PURPOSE:
West syndrome is a severe form of age-specific epilepsy that typically affects infants younger than 2 years of age with mitochondrial disease. We aimed to examine age-specific characteristics of the syndrome in these patients.METHODS:
We retrospectively analyzed 54 patients with West syndrome diagnosed with mitochondrial disease between March 2006 and March 2016. We compared treatment strategies and diagnostic and clinical variables between patients with early-onset ( < 6 months of age) and late-onset (≥6 months of age) seizures.RESULTS:
Seizure was the first symptom in 30 (90.9%) and 13 (65%) patients of the early-onset and late-onset groups, respectively (P=0.046). Delayed development was observed in 3 (9.1%) and 7 (35%) patients of the early-onset and late-onset groups, respectively (P=0.023). Lactate levels were normal in 17 patients (55%) of the early-onset group and 5 (25%) of the late-onset group (P=0.036), while initial brain magnetic resonance imaging (MRI) findings were normal in 23 (67.6%) and 8 (40%) patients of the early-onset and late-onset groups, respectively. Final MRI findings were abnormal in 32 patients (94.1%) of the early-onset group and 18 (90%) of the late-onset group (P=0.036). Although ketogenic diets reduced seizure frequency in both groups, the difference was not significant.CONCLUSION:
There is no significant difference in epilepsy-related variables when patients are divided based on a cut-off age of 6 months. However, differences in the first symptom at onset and MRI findings were observed. Although lactate levels were not of significant diagnostic value in the early-onset group, they may be in the late-onset group.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Convulsões
/
Espasmo
/
Espasmos Infantis
/
Acidose Láctica
/
Encéfalo
/
Imageamento por Ressonância Magnética
/
Estudos Retrospectivos
/
Ácido Láctico
/
Doenças Mitocondriais
/
Epilepsia
Tipo de estudo:
Estudo observacional
Limite:
Humanos
/
Lactente
/
Recém-Nascido
Idioma:
Inglês
Revista:
Journal of the Korean Child Neurology Society
Ano de publicação:
2018
Tipo de documento:
Artigo
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