A Clinical Study of Tuberous Sclerosis with Epilepsies

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Other Title
  • てんかんを合併した結節性硬化症の臨床的考察

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Eleven children with tuberous sclerosis were studied with regard to epileptic seizures, EEG findings, mentality and computerized tomography (CT). Epileptic seizures were classified according to the international classification. The measurements of mentality were performed with Tsumori-Inage test and WISC-R. An EMI scanner was used in this study. According to the sites of the intracranial calcifications on CT, the patients were divided into two groups. Group I consisted of five cases, whose calcifications were found on subependymal regions only and group II, six cases, on cortical and/or subcortical regions in addition to subependymal regions. The follow-up period was five months to thirteen years.<BR>The results were summerized as follows.<BR>1. West syndrome occurred in five cases out of eleven, two in group I and three in group II.<BR>2. Although seven patients became seizure-free, four had West syndrome, Lennox syndrome or generalized tonic clonic seizure and these of four cases belonged to group II.<BR>3. Group II patients had earlier onset of epileptic seizures and severer mental retardation. Five cases in group II had seizures under 1 year of age, compared with two cases in group I. The mean IQ (DQ) was 84.6 in group I and 68.7 in group II.<BR>4. In one patient, an 8-year-old boy, a brain tumor was found on CT, which showed high density tumor mass near the septum pellucidum and asymmetrical dilatation of the lateral ventricles. Histological diagnosis was subependymal giant-cell astrocytoma.

Journal

  • NO TO HATATSU

    NO TO HATATSU 15 (5), 396-401, 1983

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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