Neuropathology of early-infantile epileptic encephalopathy with suppression-bursts; comparison with those of early myoclonic encephalopathy and West syndrome
M. Itoh et al., Neuropathology of early-infantile epileptic encephalopathy with suppression-bursts; comparison with those of early myoclonic encephalopathy and West syndrome, BRAIN DEVEL, 23(7), 2001, pp. 721-726
For the critical lesions and pathomechanism of early-infantile epileptic en
cephalopathy (EIEE) with suppression-bursts, we investigated the brains of
EIEE, early myoclonic encephalopathy (EME), and West syndrome (WS) patients
using immunohistochemical technique and neuropathological examination. We
could compare with the results of these diseases.
The EIEE patients had the most severe lesions, which were in the putamen, t
halamus, hippocampus as well as the tegmentum of the brainstem. Among the s
yndromes, EIEE brains showed the most expanded lesions. Tyrosine hydroxylas
e-immunopositive cells and fibers were not demonstrated in EIEE, but were d
etected in WS. Reduced tyrosine hydroxylase immunoexpression in the EIEE br
ains was in the putamen, globus pallidus, and substantia nigra. Tryptophan
hydroxylase immunoreactivity was reduced in the three epileptic syndromes,
but especially in EIEE. Reduced expression of tyrosine hydroxylase and tryt
ophan hydroxylase may demonstrate dysfunction of the catecholaminergic and
serotonergic neurons. From this study, the lesions in EIEE were widespread,
including in the lower brainstem and cerebellum, compared with in EME and
WS. Dysfunction of the catecholaminergic and serotonergic systems could be
suggested. These characteristic changes may lead to the pathophysiology of
EIEE. (C) 2001 Elsevier Science B.V. All rights reserved.