| Co-Investigator(Kenkyū-buntansha) |
IMAI Kazuhide Oita Medical University, Department of Pediatrics, Associate, 医学部, 助手 (50295177)
MAEDA Tomoki Oita Medical University, Department of Pediatrics, Associate, 医学部, 助手 (80264349)
KOJO Masanobu Oita Medical University, Department of Pediatrics, Associate Professor, 医学部, 助教授 (10215262)
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| Research Abstract |
There is evidence of effectiveness of lesionectomy, lobectomy and collosotomy in patients with refractory seizures. Reports of long-term follow-up concerning patients with West syndrome who were submitted to surgeries are still limited. Thus, the aims of this study are as follows: 1) to detect a patient for the surgical indication, 2) to characterize the neuroradiological findings and their neuropathological findings, 3) to analyze the outcome of infantile spasms and psychomotor developments in patients with surgically treated West syndrome and followed up for a relevant period of time.
Patients: We performed a long-term follow-up of 7 patients with surgically treated West syndrome. 6 patients presented with early onset of seizures within the ages of 3 days - 1 month. 5 patients presented focal motor seizures, at first, and localized cortical dysplasia. An other patient with symptomatic West syndrome presented diffuse cortical dysplasia, lissencephaly. An other one patient presented wit
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h cryptogenic West syndrome, and with normal CT finding before ACTH therapy. Surgery, lesionectomy, MST, functional hemispherectomy and total callosotomy were performed for the patients between the ages of 9 months and 8 years. Two patients with cryptogenic West syndrome and diffuse lissencephaly were treated with total callosotomy. Three patients were treated with repeated ACTH therapy before surgery.
Results and Discussion: The West syndrome disappeared in all 7 patients. One patient became seizure-free even after the cessation of medical treatment. The cognitive outcomes were less favorable in three patients with delayed surgery after extensive/repeated ACTH and AEDs therapy, and a patient with lissencephaly. One patient, who had a localized cortical dysplasia and an early lesionectomy on age 9 months, without ACTH therapy, showed an almost normal poychomotor development. The neurological outcome might be remarkable in the patient with an early surgical intervention, without extensive ACTH/AEDs therapy, and functional and an atomical integrity of the "healthy contralateral" hemisphere.
The neuropathological findings in the resected cortex consisted of columnar architecture, abnormal polarity, clustering and abnormal size-variations of the neuronal cells, mainly in the 3rd-4th layers of cerebral cortex, which suggested that the migration disturbance on the early embryonal phase might be essential for the pathogenesis of the West syndrome. Less
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