Journal of the Japan Epilepsy Society
Online ISSN : 1347-5509
Print ISSN : 0912-0890
ISSN-L : 0912-0890
HHE Syndrome
with Particular Attention to Pathoplastic Aspects and Auras
Kousuke KanemotoAkira SengokuOsamu KanazawaItsuo Kawai
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1990 Volume 8 Issue 1 Pages 39-45

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Abstract

Out of 412 epileptic in-patients treated in 1986 through 1989, the thirty patients who had had a past history of HH syndrome satisfying the definition of Gastaut (1959) were selected for this study. The purpose of this paper is to examine pathoplastic effects of primary lesions produced by initial HH syndrome on secondary epileptogenic lesions manifested clinically as epileptic seizures. The subjects were divided into three groups: those who had only temporal foci (TLE-group), those who had extratemporal foci and distinct clinical symptoms of partial seizure (PE-group), and those who had electroclinical manifestations of generalized seizure (GE-group). The results were as follows:(1) The interval between the HH syndrome and the secondary epilepsy was much shorter in GE-group (1.7±4.0 years) than in TLE-group (7.7±6.2 years)(2) The proportion of right hemiplegia is much higher in PE-group (63%) and GE-group (67%) than in TLE-group (38%):(3) Compared with control-group (51 patients with simple partial seizure and TLE and without HHE syndrome), TLE-group in our study was characterized by a high frequency of autonomic aura (54% v. 33%) and low frequency of anxiety aura (0% v. 18%) and illusion of familiarity (9% v. 25%).
Considering earlier onsets of epilepsy in PE-group and GE-group than in TLE-group (Table 2), high proportion of right hemiplegia in the former groups supported Ounsted's theory tht left hemisphere is more vulnerable in the early course of cerebral maturation. We suggested further that peculiar constellations of auras in TLE-group could be a characteristic of medial temporal lobe epilepsy.

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