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

Other Titles
Ictal SPECT-guided Epilepsy Surgery in a Patient with Forme Fruste Tuberous Sclerosis
Authors
Jun-Mo HwangEun-Ik SonIl-Man KimChang-Young Lee
Department
Dept. of Neurosurgery (신경외과학)
Issue Date
2004
Citation
대한신경외과학회지, Vol.36(6) : 490-492, 2004
ISSN
1225-8245
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.
URI
http://kumel.medlib.dsmc.or.kr/handle/2015.oak/39238
Appears in Collections:
1. Journal Papers (연구논문) > 1. School of Medicine (의과대학) > Dept. of Neurosurgery (신경외과학)
Keimyung Author(s)
손은익; 김엘; 이창영
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