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Biomedical subjects

D L Hurst

Publications and source records attributed to D L Hurst.

At least 19 recordsLinked to original sources
PubMed1996

Methsuximide therapy of juvenile myoclonic epilepsy.

Currently valproic acid is considered to be the drug of first choice for juvenile myoclonic epilepsy (JME) resulting in a 70-90% control rate for all seizure types associated with JME. In those situations where valproic acid fails to control seizure activity, results in unacceptable side-effects, or is declined due to potential side-effects, an alternative effective monotherapy would be desirable. Five adolescent female patients were placed on methsuximide for JME. All five patients have been seizure free with the use of methsuximide and four out of five are now on methsuximide monotherapy with good success. C.L. has now had complete seizure control on methsuximide monotherapy, a total of 1200 mg a day, for 7 years with the exception of one seizure event occurring on an attempted discontinuation of methsuximide after being 5 years seizure free. Methsuximide monotherapy as demonstrated in these five patients is an effective treatment for JME.

Adolescent↗
PubMed1995

The use of felbamate to treat infantile spasms.

Based on the initial successful use of felbamate for infantile spasms in an infant with tuberous sclerosis, three additional infants with infantile spasms of different etiologies who had failed conventional therapies were treated with felbamate. Three of the four patients have shown complete resolution of infantile spasms. All responding patients did so within 1 week of starting felbamate. The one treatment failure had an initial reduction of seizure frequency and severity but has not maintained that response long term. Controlled studies are needed to firmly establish that felbamate is both safe and effective for the treatment of infantile spasms. As these cases document, felbamate is currently available for use in infantile spasms, and the frequent conversion of infantile spasms to Lennox-Gastaut syndrome, for which felbamate is approved, makes its use in infantile spasms logical.

Anticonvulsants↗
PubMed1993

Early severe infantile botulism.

Two neonates with a history of diarrhea, abrupt apnea, and suspected sepsis were proved to have infantile botulism. Initial symptoms in both infants suggested other diagnoses. Electrophysiologic studies were important in confirming the diagnosis. Early severe infantile botulism may be rare but should be considered in neonates who have hypotonia and respiratory arrest or a sepsis-like clinical picture.

Acute Disease↗
PubMed1993

The rapid change to monotherapy with valproic acid in children.

A review of hospitalized epilepsy patients at Texas Tech University identified 20 children during a 4-year period who had undergone a rapid change from other anticonvulsants to valproate monotherapy. All patients had difficult-to-treat or intractable epilepsy syndromes. In each case, sodium valproate was introduced over a 2- to 4-day period as all other anticonvulsants were withdrawn quickly and sequentially. No acute side effects were seen during hospitalizations, and only one patient reported gastrointestinal complaints. At time of discharge, 12 of the 20 patients were seizure free. Fifteen of the 20 patients were maintained on monotherapy following discharge. By the end of the study period, 12 of the 20 patients showed improvement with valproate, with nine patients experiencing complete seizure control. A rapid switch to valproate monotherapy can be done safely in an inpatient setting.

Adolescent↗
PubMed1991

Pulsed irrigation enhanced evacuation: new method for treating fecal impaction.

Fecal impaction is a common problem in patients with neurologic impairment. The pulsed irrigation enhanced evacuation (PIEE) procedure is a new method of clearing fecal impactions using pulses of small amounts of warm water to rehydrate stool and improve peristalsis. Thirty-seven PIEE procedures were performed on 28 patients with a variety of neurologic problems. The PIEE procedure was observed to be effective and safe. It should be a useful adjunct to a proper bowel management program.

Enema↗
PubMed1987

Unexpected seizures during hemodialysis. Effect of dialysate prescription.

To assess the effects of the dialysate prescription on the intradialytic neurological stability of children requiring chronic hemodialysis (HD), continuous EEG monitoring (CEM) was performed on five children before, during and after HD against: (1) low sodium acetate (LAc: Na 132 mEq/l, acetate 38 mEq/l); (2) high sodium acetate (HAc: Na 144 mEq/l, acetate 41 mEq/l), and (3) low sodium bicarbonate (LBi: Na 133 mEq/l, bicarbonate 35 mEq/l) dialysate. Three children, two with clinically well-controlled seizure disorders and one with no seizure history, exhibited subclinical seizures on LAc and HAc but improved neurological stability on LBi. Two children had essentially unchanged CEM studies on any HD regimen. Symptoms of disequilibrium were noted in four of the five children on LAc, two of the five on HAc and only one of the five on LBi. The data suggest that bicarbonate HD may enhance intradialytic neurological stability, particularly in children with known seizure disorders. Furthermore, CEM was found to be a useful tool for evaluating the neurological stability of children during HD.

Adolescent↗
PubMed1984

Bromide psychosis: a literary case.

Author Evelyn Waugh, noted for such works as Brideshead Revisited, published in 1957 an autobiographical account of his own bromide psychosis in the novel The Ordeal Of Gilbert Pinfold. Until now, however, the fact of his bromide poisoning had gone unrecognized both in biographies of Waugh and in analyses of the novel. Ordeal gives a detailed, accurate, day-by-day description of bromide psychosis. This unusual, witty, and fascinating piece of literature actually provides the most complete monograph on the subject. In this article we review the literature on bromide poisoning, discuss the events of the novel and of Waugh's life in light of his bromide psychosis, and suggest the relevance of the case to medicine.

Bromides↗
PubMed1983

Phenotypic variation in biotinidase deficiency.

Biotinidase deficiency is the usual biochemical defect in biotin-responsive late-onset multiple carboxylase deficiency. We reviewed the clinical features of six patients with the enzyme deficiency and compared them with features described in the literature in children with late-onset MCD. In all of the reported probands, MCD was diagnosed because they had metabolic ketoacidosis and organic aciduria in addition to various neurologic and cutaneous symptoms, such as seizures, ataxia, skin rash, and alopecia. Although in several of our patients biotinidase deficiency was also diagnosed because they manifested a similar spectrum of findings, others never had ketoacidosis or organic aciduria. Thus the initial features of biotinidase deficiency usually include neurologic or cutaneous symptoms, whereas organic aciduria and MCD are delayed, secondary manifestations of the disease. These findings suggest that biotinidase deficiency should be considered in any infant or child with any of these neurologic or cutaneous findings, with or without ketoacidosis or organic aciduria. If the diagnosis cannot be excluded, such individuals should be given a therapeutic trial of pharmacologic doses of biotin.

Amidohydrolases↗
PubMed1979

X-linked hypogonadism, gynecomastia, mental retardation, short stature, and obesity--a new syndrome.

Five male members in four generations of the same family had hypogonadism, gynecomastia, mental retardation, obesity, and short stature. The X-linked mode of inheritance, the distinctive facies, the normal size of the hands and feet, and the true gynecomastia are the main characteristics. Endocrine evaluation and histologic studies of the testes suggest partial hypogonadotropic hypogonadism. This disorder represents a new syndrome distinct from others previously described.

Adult↗
PubMed1979

Stimulus and procedural determinants of the forgetting of conditioned fear.

Two studies investigated factors believed to influence the retention of acquired fear in rats. The results show clearly that the lack of forgetting over 24-hr interval reported by previous investigators is due to a depression of performance on the immediate test (3-min retention interval) resulting from the procedure of not handling the rats during conditioning but then handling them on every trial during the retention test. In accord with the McAllister's interpretation of related results, the depression of performance after short retention intervals may be due to a lack of generalization from training to the testing situation. As the retention interval is lengthened, the amount of generalization increases and escape performance improves on a relative basis. The nature of the retention function may be severely distorted by selection of an inappropriate paradigm for its evaluation.

Animals↗
PubMedDate not supplied

The use of imipramine in minor motor seizures.

A study is presented of 15 pediatric patients treated with imipramine for uncontrolled seizures during the past two years. Of these patients, 53% had an initial reduction of seizures of greater than 80%. At one year, 26% were still seizure-free. Drop attacks were especially responsive to therapy; 100% (7/7) responded initially to imipramine therapy. Most patients studied had been receiving valproate and another major antiepileptic drug prior to imipramine therapy. This study confirms previous reports and suggests that the use of imipramine in combination with other antiepileptic drugs provides excellent results, particularly in patients with drop attacks.

Adolescent↗
PubMedDate not supplied

Carbamazepine-exacerbated epilepsy in children and adolescents.

Forty-nine children and adolescents whose seizures reportedly worsened while receiving carbamazepine (CBZ) were studied retrospectively. Twenty-six patients met criteria for excellent documentation of carbamazepine-exacerbated seizures. Four epileptic syndromes were particularly affected: childhood absence epilepsy; focal symptomatic, frontal lobe epilepsy; Lennox-Gastaut syndrome; and severe myoclonic epilepsy of infancy. Eight of the 26 patients developed new-onset absence seizures and three patients with established absence epilepsy experienced absence status. Other seizure types, including atonic, tonic-clonic, and myoclonic, developed in eight patients treated with CBZ, and new generalized spike-and-wave discharges were observed in electroencephalograms of nine patients. CBZ is a widely used, effective antiepileptic drug, particularly for partial or partial complex seizures; however, if uncontrolled, generalized seizures occur after CBZ is prescribed for children or adolescents with absence or mixed seizures, a trial of CBZ discontinuation is warranted. The data reported here do not permit calculation of the incidence of this phenomenon.

Adolescent↗