Of 1300 epileptic patients 76 (5.8%) were found to have juvenile myoclonic epilepsy (JME). These 76 patients were examined at the epilepsy outpatient clinic of Bakirköy State Hospital for Neurological and Psychiatric Diseases between 1991 and 1996 and data obtained were analysed retrospectively. Clinically typical absence seizures were reported in 40.8%, myoclonic jerks in 100%, and generalized tonic-clonic seizures in 82.9% of the patients. Neurological and mental examination was normal for all patients with the exception of three cases; two with essential tremor and one with minimal dysarthria. Precipitating factors were noted in 85.5% of cases. Abnormal EEG was recorded in 73 (6.1%) patients. Abnormalities mainly consisted of generalized discharges of spike/polyspike and slow-wave (86.6%) and generalized paroxysmal theta or delta (9.2%). Fifteen (19.7%) had focal abnormalities and 20 (26.4%) had photoconvulsive discharges. Of the 76 patients, 40 (52.6%) were not diagnosed at the initial interview; definite diagnosis was delayed by a mean of 5.9 years. As a result of misdiagnosis at the initial interview 40 patients had been administered AED except for valproate. After reassessment of clinical and EEG findings, the medication was changed to valproate therapy. As a result, 65 of our JME patients (85.5%) were seizure free after a one-year follow-up period.

Rasmussen's syndrome is characterized by intractable seizures and progressive neuropsychiatric deterioration secondary to unilateral cortical inflammation and tissue destruction. Diagnosis of Rasmussen's syndrome in the early phase depends mainly on the clinical features. Neuroimaging and histopathologic examinations may not be specific during this period. We report a case of Rasmussen's syndrome followed by serial MRI and magnetic resonance spectroscopy (MRS) studies over a 3- to 16-month period. A healthy 6-year-old boy presented with focal motor seizures. An MRI study demonstrated prominent enlargement and T2 hyperintensity of the left mesial temporal lobe and perisylvian region. This early finding evolved to volume loss and later progressive atrophy of the ipsilateral hemisphere when epilepsia partialis continua occurred. Being aware of those early MRI features in a patent with increasing frequency of focal motor seizures should suggest Rasmussen's syndrome. In addition, we found prominently increased myoinositol concentration in atrophic cortex which might reflect increased gliosis in the late period of the disease.

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We studied the efficacy of captopril, an angiotensin‐converting enzyme inhibitor in treating persistent moderate or severe proteinuria in children with various glomerular diseases other than minimal‐change nephrotic syndrome. Captopril was administered for 3 months to 15 normotensive and nonazotemic or mildly azotemic patients (12 boys, 3 girls) in whom corticosteroid and cytotoxic treatment had failed to induce remission. Urinary protein excretion decreased from 2873.14±1937.50 (mean ± s.e.m.) to 1684.71 ± 1463.13 mg/day (P < 0.05). The reduction in proteinuria was not related to a significant fall in systemic blood pressure or a change in renal function. Serum albumin did not rise and side effects due to captopril were not observed. We concluded that, in the short term, captopril can be used safely and effectively for decreasing the proteinuria of nephrotic children unresponsive to conventional therapy.