A female case of West syndrome with remission of spasms following multiple cerebral hemorrhages.
Multiregional wide-distribution hemorrhages of the left hemisphere occurred at 1 month of age in a girl with congenital factor V deficiency. At the age of 4 months, symmetrical spasms appeared in clusters and electroencephalography showed diffuse background attenuation in the left side and hypsarrhythmia only in the right. Brain CT scan showed that the left hemisphere including Rolandic area was completely infarcted. She was diagnosed with West syndrome and spasms were not controlled by anti-epileptic drugs. Following multiple intracerebral and subarachnoid hemorrhaging involving the right hemisphere at approximately 2 years of age, spasms and hemi-hypsarrhythmia abruptly disappeared, and complete remission of spasms persisted for 2 years. Taken together, the right-hemispheric cortex seemed to be primarily responsible for generation of symmetric spasms and hemi-hypsarrhythmia on electroencephalography. This case study failed to support the hypothesis that ictal discharges needs to be propagated from one to the other side though the corpus callosum in order to generate symmetric spasms. Rather, symmetric spasms can be explained by activation of subcortical structures such as the brain stem, ipsilateral spreading of electrographic discharges from the residual hemisphere, or intra-hemispheric propagation of ictal discharges.
Department of Pediatrics, Tohoku University School of Medicine, Aoba-ku, Sendai, Japan.
Naomi
N
Hino-Fukuyo
Kazuhiro
K
Haginoya
Mitsugu
M
Uematsu
Shigeru
S
Tsuchiya
D002543:Cerebral Hemorrhage / Q000503:physiopathology*; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D008875:Middle Aged; D013035:Spasm / Q000503:physiopathology*; D013036:Spasms, Infantile / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed