Clinical Manifestations and Surgical Outcome of Medically Refractory Epilepsy in Childhood / 대한소아신경학회지

PURPOSE: The cognitive and psychosocial morbidity of medically refractory epilepsy is considerable. Epilepsy surgery can play a important role in these patients. We investigated the clinical manifestations and the surgical outcome of our patients with medically refractory epilepsy. METHODS: We studied 27 patients under 15 years old who had epilepsy surgery at Samsung Medical Center between March 1995 and December 2001 retrospectively. RESULTS: The median age at first unprovoked seizure was 6 years, the median age at surgery was 11.02 years and duration of follow-up was 14 months to 7 years 2 months. Complex partial seizure was the most common type of seizures. Interictal and ictal discharges were lateralized in 66.7% and 81.5%, respectively. MRI showed abnormal findings in 23 patients and the cortical dysplasia was the most common pathologic finding. As per surgical outcomes, 77.8% of the patients became seizure free. Among patients with temporal lobe epilepsy(TLE), seizure free rate was 92.3% whereas patients with extratemporal lobe epilepsy(ETLE) showed 64.3% seizure free rate. All patients with TLE with focal lesions became seizure free. Patients with focal lesions which were concordant with findings of EEG, SPECT, and PET showed excellent surgical outcomes. However, patients with no focal lesion in MRI and discordant preoperative examinations showed poor outcomes. CONCLUSION: The results of epilepsy surgery in our patients were quite satisfactory. Good surgical outcome can be expected when the decision of surgery is made based on a good correlation among clinical, neuroradiological, and other preoperative examinations.

The Clinical Features of Acute Disseminated Encephalomyelitis in Children / 대한소아신경학회지

PURPOSE: Acute disseminated encephalomyelitis(ADEM) is an immunologically mediated demyelinating disease of the central nervous system. We investigated the clinical features and outcomes of children with ADEM presenting with different neurologic symptoms and clinical courses. METHODS: Fifteen cases(male 10 and female 5) of ADEM who were diagnosed in the department of pediatrics, Samsung Medical Center from July 1997 to April 2001 were reviewed. RESULTS: The ages at initial presentations were from 1 year 5 months to 14 year 8 months. The presence of preceding events was present in all patients. The most common presenting symptom was seizure(60%), followed by altered consciousness(40%), ataxia(33 %), cranial nerve palsy, headache, quadriparesis, visual disturbance, and tremor. Brain magnetic resonance imaging showed relatively symmetrical, multifocal hyperintense lesions on T2-weighted image. One out of 4 brain CT showed an abnormal finding and remaining 3 cases showed abnormal findings in MRI only. Two patients had normal MRI findings at the initial phase of the illness but later revealed MRI findings compatible with ADEM. Thirteen patients were managed on high dose methylprednisolone therapy and/or intravenous immunoglobulin. After treatments, all patients revealed rapid clinical recovery. Relapse occurred in four patients(26%) 3 weeks to 1 year 6 months after developing their first illness. Among them, two patients were diagnosed as multiple sclerosis later. CONCLUSION: We experienced 15 children with the initial diagnosis of ADEM. Even though immunosuppressive drugs were effective to eliminate their presenting symptoms in most patients, there was 26% of relapse rate with 2 patients with later diagnosis of multiple sclerosis during long-term follow-up suggesting heterogeneity existed with regard to etiology and clinical courses of ADEM.