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Ictal SPECT-guided Epilepsy Surgery in a Patient with Forme Fruste Tuberous Sclerosis

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Affiliated Author(s)
손은익김엘이창영
Alternative Author(s)
Son, Eun IkKim, ElLee, Chang Young
Journal Title
대한신경외과학회지
ISSN
1225-8245
Issued Date
2004
Abstract
Tuberous sclerosis is an autosomal dominant disease characterised by hamartomas (tubers) in many organ systems and the four major intracranial manifestations including cortical tubers, white matter abnormalities, subependymal nodules and subependymal giant cell astrocytoma. But there is immense variability in the clinical presentation of tuberous sclerosis and many incomplete forms (formes frustes) exist. Almost all patients with tuberous sclerosis have seizures and mental retardation. The authors experienced a 7-year-old boy with medically intractable epilepsy without any skin lesion or mental retardation. In terms of surgical standpoint for determination of extent of resection, corticectomy on the overriding cortex of right premotor and lesionectomy of periventricular calcified lesion were performed according to ictal single photon emission computed tomography(SPECT), which showed hyperperfusion in the subcortical and calcified area. Histopathologic findings showed a few cytologically abnormal neurons with extensive gliosis, containing many Rosenthal fibers, reactive astrocytes and dense calcification, composing of abundant calcospherites which suggested forme fruste tuberous sclerosis. During the follow-up period of eighteen months, seizure was free after surgery.
Alternative Title
Ictal SPECT-guided Epilepsy Surgery in a Patient with Forme Fruste Tuberous Sclerosis
Department
Dept. of Neurosurgery (신경외과학)
Publisher
School of Medicine
Citation
Jun-Mo Hwang et al. (2004). Ictal SPECT-guided Epilepsy Surgery in a Patient with Forme Fruste Tuberous Sclerosis. 대한신경외과학회지, 36(6), 490–492.
Type
Article
ISSN
1225-8245
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/39238
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Neurosurgery (신경외과학)
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