Biomedical subjects

D L Hurst

Publications and source records attributed to D L Hurst.

23 records · Page 2Linked to original sources

PubMedDate not supplied

Expanded therapeutic range of valproate.

Twenty-five pediatric patients with serum valproate levels above 100 micrograms/ml (therapeutic range: 50-100 micrograms/ml) are reviewed for nondose-related side effects and seizure control. The dose of valproate varied from 50-100 mg/kg/day. All patients had generalized or mixed seizure disorders which were difficult to control. Seizure frequency decreased by more than 50% in 14 patients. For those patients with improved control, valproate levels ranged from 111-196 micrograms/ml. Patients were carefully monitored for side effects; nondose-related side effects were not encountered. Random valproate levels were between 100-200 micrograms/ml. The clinical response to valproate can be augmented by increasing it to the maximum tolerated dose.

Adolescent↗

PubMedDate not supplied

Severe myoclonic epilepsy of infancy.

Severe myoclonic epilepsy of infancy (SMEI) is a recently identified seizure disorder with a uniformly poor prognosis. No successful therapy has been found for this disorder. Based on the treatment of 7 patients who qualified for the diagnosis in this report, SMEI has a better prognosis than recognized currently. All 7 patients were responding poorly to phenobarbital, phenytoin, or carbamazepine. Six of them responded to valproate. Two patients became seizure-free during valproate monotherapy. A succinimide was required as a second antiepileptic drug in 3 patients to achieve seizure control. Repeated episodes of "febrile status epilepticus" in 2 patients were controlled with lorazepam. Five of 7 patients are doing significantly better than the literature would suggest is possible. Seizure control can be achieved in SMEI with aggressive use of drugs which are beneficial for myoclonic seizures. Traditional drugs for tonic-clonic seizures are of little or no benefit in SMEI.

Adolescent↗

PubMedDate not supplied

Acute cerebellar swelling in varicella encephalitis.

A 4-year-old male developed encephalitis 2 weeks after the onset of varicella. During his evaluation neuroradiologic procedures documented cerebellar edema and demyelination. Cerebrospinal fluid titers confirmed varicella encephalitis. To our knowledge, this patient is the first reported with focal cerebellar edema, an expected finding, with a clinical varicella infection as documented by computed tomography and magnetic resonance imaging.

Brain Edema↗

PubMedDate not supplied

Variable phenotypes in a family kindred with adrenoleukodystrophy.

Adrenoleukodystrophy, an X-linked recessive disorder characterized by progressive demyelination of the central nervous system and adrenal insufficiency, usually manifests at 4-8 years of age. We report a 20-month-old male who presented with the sudden onset of status epilepticus and cortical blindness; initially, he had complete resolution of these findings, but experienced a relapse 3 months later. The initial computed tomographic scans depicted cerebral edema and possible "watershed infarcts:" however, over the next 2 weeks before discharge from the hospital, the cortical blindness and ataxia both resolved. During the next 2 months, he exhibited no symptoms: he had no seizures and his neurologic examinations were normal. Three months after the initial hospitalization, he developed what the mother believed was "a weakness on his right side." Magnetic resonance imaging confirmed severe white matter disease. Adrenoleukodystrophy was clinically suspected and an assay of plasma levels confirmed an elevation of C26 long-chain saturated fatty acid levels. After the patient's diagnosis of adrenoleukodystrophy was confirmed, long-chain fatty acid levels were obtained on his 5-year-old brother and his mother. This child had the earliest known onset of X-linked adrenoleukodystrophy.

Adrenoleukodystrophy↗

PubMedDate not supplied

Epidemiology of severe myoclonic epilepsy of infancy.

Severe myoclonic epilepsy of infancy (SMEI) is a newly recognized epileptic syndrome. It is characterized by multiple febrile seizures, often prolonged, subsequent development of uncontrollable mixed-myoclonic seizures, and, eventually, psychomotor retardation. Drugs for myoclonic epilepsy--valproate (VPA), the suximides, and the benzodiazepines--have been shown to be useful in SMEI. Among children with seizures in the National Institute of Neurological and Communicative Disorders and Stroke Collaborative Perinatal Project (NCPP), one individual with SMEI was identified. This finding from the NCPP suggests that the incidence of SMEI is approximately 1 in 40,000 children. Such an incidence is supported by observations at the Texas Tech University Health Sciences Center.

Brain↗