Age-Based Characteristics of West Syndrome in Patients with Mitochondrial Disease / 대한소아신경학회지
Journal of the Korean Child Neurology Society
; (4): 197-204, 2018.
Article
en En
| WPRIM
| ID: wpr-728821
Biblioteca responsable:
WPRO
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.Palabras clave
Texto completo:
1
Base de datos:
WPRIM
Asunto principal:
Convulsiones
/
Espasmo
/
Espasmos Infantiles
/
Acidosis Láctica
/
Encéfalo
/
Imagen por Resonancia Magnética
/
Estudios Retrospectivos
/
Ácido Láctico
/
Enfermedades Mitocondriales
/
Epilepsia
Tipo de estudio:
Observational_studies
Límite:
Humans
/
Infant
/
Newborn
Idioma:
En
Revista:
Journal of the Korean Child Neurology Society
Año:
2018
Tipo del documento:
Article