Gelastic seizures in clusters in a case of West syndrome after perinatal hypothalamic hemorrhage.
We describe a 4-month-old Japanese infant with West syndrome with gelastic seizures in clusters. Smile developed around 3 months and gradually increased in frequency and intensity. Positron emission tomography showed hypoperfusion in bilateral hypothalamus. Interictal electroencephalogram (EEG) showed hypsarrythmia. Simultaneous video/EEG monitoring was performed. At first, a smile-like episode developed every several seconds, gradually increased to an abrupt flexion of the neck and extremities, and gradually decreased to a smile-like episode at the end. Ictal EEG revealed desynchronization. ACTH was effective. Smiles are common emotional responses in infancy. However, EEG and neuroimaging should be considered in a case of perinatal asphyxia and delayed development.
Kito
M
M
Department of Pediatrics, Minami Seikyo Hospital, Nagoya, Japan.
Yabuta
K
K
Kato
S
S
Osawa
M
M
Okuda
H
H
D001238:Asphyxia Neonatorum / Q000150:complications; D004569:Electroencephalography; D005260:Female; D006470:Hemorrhage / Q000150:complications*; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications*; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D013036:Spasms, Infantile / Q000209:etiology* / Q000503:physiopathology; D014055:Tomography, Emission-Computed