Abe M. Chutorian

Anterior ischemic optic neuropathy in children: Case reports and review of the literature

Anterior ischemic optic neuropathy, infarction of the optic nerve head owing to inadequate perfusion through the posterior ciliary arteries, is a common cause of visual loss in adults but is rarely reported in children, in part because the diagnosis is overlooked. We report two cases of young children undergoing chronic peritoneal dialysis, who suffered bilateral visual loss from anterior ischemic optic neuropathy. Predisposing local anatomic and multiple systemic factors included a small optic nerve head with little cupping, possible intraocular hypertension, and systemic hypotension, hypovolemia, and anemia. The literature on anterior ischemic optic neuropathy is reviewed.

Topiramate for the Treatment of Infantile Spasms

Topiramate is a new antiepileptic drug with a broad spectrum of efficacy. Reports on the use of topiramate for treatment of infantile spasms are limited. We prospectively followed 15 children with recently diagnosed infantile spasms treated with topiramate for efficacy and tolerability. Twelve patients had symptomatic infantile spasms, and two patients had cryptogenic infantile spasms. Topiramate was started at a dose of 3 mg/kg/day and titrated up to a dose of 27 mg/kg/day in 2 to 3 weeks. The primary efficacy measure was comparison of the seizure rate during the 2-week baseline with the median seizure rate during the first 2 months of treatment with topiramate. We also compared baseline electroencephalograms (EEGs) with post-treatment EEGs. The median seizure rate reduction during the first 2 months of treatment was 41% (P = .002). Three patients became spasm free (20%), five had > 50% reduction, and three had at least 25% reduction. Four patients did not respond. Three of 15 patients had clearing of hypsarrhythmia. Topiramate was generally well tolerated, with irritability being the most common side effect. Topiramate was efficacious and well tolerated; one patient discontinued the medication because of adverse effects. (J Child Neurol 2006;21:17—19).

Benign intracranial hypertension and Bell's palsy.

Benign intracranial hypertension is associated with a variety of underlying disorders, but the pathophysiology of the process is poorly understood. We have encountered three children with benign in...

Artificial insemination by donors: the need for genetic screening: late-infantile GM2-gangliosidosis resulting from this technique.

ARTIFICIAL insemination by donor has become a widely accepted method to induce pregnancy for infertile couples and to decrease the risk of inherited diseases. With increasing use of this method, ho...

Nitrazepam for the treatment oflennox-gastaut syndrome

Lennox-Gastaut syndrome is a severe childhood epileptic syndrome with encephalopathy and multiple seizure types, which are often intractable to treatment. Most of these children will ultimately become mentally retarded and dependent on others for their daily care. Antiepileptic drugs are the mainstay of treatment, however, no particular drug is entirely effective. Apart from the use of antiepileptic drugs, nonpharmacologic treatments are also considered (i.e., callosotomy, ketogenic diet, and vagus nerve stimulation), which have proven to be partially effective. We prospectively studied 14 children (11 months-8 years of age) with medication-resistant Lennox-Gastaut syndrome, being treated with nitrazepam (open-label compassionate protocol). We compared the 1-month baseline seizure frequency with the median seizure rate reduction during the first 12 months of treatment with nitrazepam. The median seizure rate reduction during the first 12 months of treatment with nitrazepam was 41% (P = 0.001), with more than 50% seizure reduction in 60% of patients. Two patients became seizure free, five patients demonstrated at least 50% reduction in seizure rates, six patients had at least 25% seizure rate reduction, and one patient did not respond. No patient had any serious adverse effects. Side effects included sedation in six children (40%) and drooling in nine patients (60%).

Toxic Pediatric Parkinsonism: Report of a Child With Metabolic Studies and Response to Treatment

We successfully treated a child with parkinsonism secondary to cytosine arabinoside with levodopa/carbidopa and attempted to elucidate the pathogenesis of the disorder by sequential analysis of cerebrospinal fluid biogenic amines, sequential magnetic resonance imaging, and fluorodopa positron emission tomography, which revealed both permanent and reversible biochemical and structural alterations. The parkinsonian disorder resolved in 9 months. Metabolic changes and structural abnormalities on imaging studies were chronologically correlated with the clinical course. (J Child Neurol 2003;18:812—815).