Neuropathological analysis on pathogenesis in infantile spasms

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Previously, we reported the common functional brainstem lesions in the autopsy cases of lissencephaly (LIS) and perinatal hypoxic ischemic encephalopathy (HIE) having both infantile spasms (IS) and Lennox-Gastaut Isyndrome (LGS). To investigate the pathogenesis of IS and LGS, we immunohistochemically examined the expression of neiuotransmitters, neuropeptides, calcium-binding proteins and/or glutamate transporters in the brainstem and limbic system in child autopsy cases. In cases of LIS and HIE suffering from both epileptic syndromes, there were no IS/LGS-specific changes in the expressions of either calcium-binding proteins or glutamate transporters in the brainstem and limbic system. The brainstem showed reduced expressions of neurotransmitters in both the cases of hereditary dentatorubral-pallidoluysian atrophy suffering from another refractory progressive myoclonic epilepsy and the cases of subtle cerebral dysgenesis having IS but not LGS, although the changes were not related to the severity of epileptic seizures. In addition, the amygdaloid lesions in the severely handicapped were clarified to have relationships with the etiology but not either the severity of epileptic seizures or the hippocampal lesions. It is suggested that the brainstem lesions in IS and/or LGS can be involved in the disease progression more than the limbic lesions.