Abstract
Both West syndrome (WS) and Lennox–Gastaut syndrome (LGS) are associated with various
developmental disorders and it has been discussed whether the cerebral cortex or subcortical
structures are important in the pathogenesis of both epileptic syndromes. Here we
briefly review the literature on the neuropathological findings in WS and LGS, and
present our data on immunohistochemical analysis of the brainstem and limbic lesions
in autopsy cases of lissencephaly and sequels of hypoxic ischemic encephalopathy (HIE)
caused by perinatal asphyxia manifested as both WS and LGS (WS/LGS). Nowadays, the
neuroradiological examinations and surgical pathology in WS cases demonstrate dysplastic
cerebral lesions more frequently than previously expected. On the other hand, we have
delineated the common brainstem lesions such as small size of the tegmentum and spongy
state and/or gliosis in the central tegmental tract in a number of WS autopsy cases
of various etiologies. Recently, we reported the reduced expression of tyrosine hydroxylase,
methionine enkephalin and parvalbumin in the brainstem in autopsy cases of lissencephaly
and sequels of HIE manifested as WS/LGS, regardless of the cerebral changes. In the
same subjects, we examined the expression of glutamate transporters and calcium-binding
proteins in the limbic system by immunohistochemistry. These represent markers of
glutamate neurotoxicity and the GABAergic inhibitory neuron system, respectively.
The altered expressions of glial glutamate transporters and calcium-binding proteins
in the limbic system seemed to reflect temporal lobe sclerosis, irrespective of the
past history of WS, and there were no differences in the limbic involvement between
the cases manifested as WS/LGS and disease controls of sequels of HIE not manifested
as WS/LGS. It is more likely that the brainstem lesions contribute to the pathogenesis
of WS and/or LGS more than the heterogeneous limbic lesions in these cases.
Keywords
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Article info
Publication history
Accepted:
July 12,
2001
Received in revised form:
July 9,
2001
Received:
May 30,
2001
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
