M. Kito et al., Gelastic seizures in clusters in a case of West syndrome after perinatal hypothalamic hemorrhage, J PERIN MED, 29(4), 2001, pp. 357-359
We describe a 4-month-old Japanese infant with West syndrome with gelastic
seizures in clusters. Smile developed around 3 months and gradually increas
ed in frequency and intensity. Positron emission tomography showed hypoperf
usion in bilateral hypothalamus. Interictal electroencephalogram (EEG) show
ed hypsarrythmia. Simultaneous video/EEG monitoring was performed. At first
, a smile-like episode developed every several seconds, gradually increased
to an abrupt flexion or the neck and extremities, and gradually decreased
to a smile-like episode at the end. Ictal EEG revealed desynchronization. A
CTH was effective. Smiles are common emotional responses in infancy. Howeve
r, EEG and neuroimaging should be considered in a case of perinatal asphyxi
a and delayed development.