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Biomedical subjects

J B Bodensteiner

Publications and source records attributed to J B Bodensteiner.

At least 55 records · Page 3Linked to original sources
PubMed1995

Clobazam for intractable pediatric epilepsy.

We report our experience with add-on clobazam therapy over a 5-year period in 63 children with refractory epilepsy. The mean duration of epilepsy was 6.7 years. Children were followed for 15 to 64 months. Of 63 children, 57 were developmentally delayed, and 54 had a symptomatic/cryptogenic epilepsy. Forty-one percent became either seizure free or had a greater than 90% reduction in seizure frequency. Seizure frequency was reduced 50% to 90% in another 24%. The average daily dose of clobazam was 0.8 mg/kg. Thirty-five percent had the medication withdrawn for persistent or unacceptable side effects or the development of tolerance (seven patients). Side effects included severe aggressive outbursts, hyperactivity, insomnia, and depression with suicidal ideation. Clobazam is a useful add-on medication for 65% of children with epilepsy. Clinical utility may be limited by behavioral side effects in some patients.

Adolescent↗
PubMed1995

Opsoclonus myoclonus syndrome secondary to Epstein-Barr virus infection.

We report the first two children to have the "opsoclonus myoclonus syndrome" after a serologically confirmed acute Epstein-Barr virus infection. Although opsoclonus was absent in one patient, both patients had all other features typical of this syndrome. Patients without opsoclonus have been described as having an isolated postinfectious myoclonus syndrome but probably lie within the spectrum of opsoclonus myoclonus syndrome. The prognosis for the opsoclonus myoclonus syndrome in children with Epstein-Barr virus infection and without evidence for a neuroblastoma appears to be better than in those patients where a cause cannot be identified.

Antibodies, Viral↗
PubMed1995

Sudden unexpected death in patients with Dandy-Walker malformation.

An uncommon but well-recognized occurrence in patients with Dandy-Walker malformation is sudden unexpected death. The mechanism of demise has not been established. We report three patients with Dandy-Walker malformation that experienced sudden unexpected death without uncal or tonsillar herniation, the mechanism usually proposed for demise in such situations. Our findings suggest the possibility of vascular compromise as the cause of the sudden unexpected death in these patients. Early and effective relief of the pressure in the posterior fossa may prevent the occurrence of this catastrophic complication.

Brain↗
PubMed1995

Differential involvement of the brain in neonatal asphyxia: a pathogenic explanation.

Multiple cystic lesions in brain parenchyma supplied by the anterior cerebral circulation is a recognized pattern of cerebral injury associated with hypoxic-ischemic encephalopathy in the term infant. This report presents a series of seven infants (gestational age, 39.3 +/- 2.8 weeks; range, 36 to 44 weeks) who developed multicystic encephalomalacia in the distribution of the anterior cerebral circulation after severe neonatal asphyxia. Cerebral imaging and pathologic studies demonstrate relative preservation of the cerebellum, brain stem, and cerebral structures supplied by the vertebrobasilar circulation. Compared to the vertebrobasilar vasculature, the anterior cerebral vessels in the term infant have dense sympathetic innervation. Asphyxia, a potent sympathetic stimulator, may induce vasoconstriction in the anterior circulation and differentially accentuate the effects of hypoxia/ischemia on cerebral tissue.

Asphyxia Neonatorum↗
PubMed1994

Esthesioneuroblastoma presenting as an orbital mass in a young child.

A 3-year-old boy presented with uniocular proptosis and ophthalmoplegia. Investigation revealed a mass involving the right orbit with extension into the left orbit and paranasal sinuses, and intracranial extension involving both frontal lobes. Biopsy of an enlarged cervical node and the intranasal mass revealed esthesioneuroblastoma. This tumor has been reported rarely in a child this age, and only 12 case reports document patients under 10 years of age. The presentation as an orbital mass is previously unreported and must now be considered in the differential diagnosis of proptosis in childhood. The usual clinical, radiological, and pathological features of olfactory esthesioneuroblastoma are reviewed.

Child, Preschool↗
PubMed1994

Congenital myopathies.

Several dozen congenital myopathies are defined by clinical and morphological criteria. The application of the current generation of scientific techniques including immunohistochemistry and molecular genetics has resulted in the expansion of our knowledge and understanding of the well-established conditions including central core myopathy and centronuclear/myotubular myopathy and allowed greater understanding of the interrelationships of some of the less common or less well-established conditions. In the near future molecular genetics may allow the identification of the specific gene defect in many of these diseases. This article reviews the major congenital myopathies and presents some of the information gained by application of new technology to these conditions.

Humans↗
PubMed1994

Acute and delayed cerebral infarction after wasp sting anaphylaxis.

A 52-year-old man developed a severe anaphylactic reaction after a wasp sting. Slurred speech and left hemiparesis were noted a few hours later. Three-and-one-half weeks later, he became acutely obtunded and quadriparetic. Angiographic studies demonstrated complete and near-complete occlusions of the right and left internal carotid arteries, respectively. A mechanism is suggested for delayed ischemic stroke after wasp sting anaphylaxis that involves cerebrovascular sympathetic innervation.

Acute Disease↗
PubMed1994

Effective utilization of home-video recordings for the evaluation of paroxysmal events in pediatrics.

In young children, differentiating seizures from nonepileptic "spells" by history alone can be difficult, even for the specialist. Nonepileptic spells may be psychogenic (rare in the young child) or physiologic in origin. Physiologic spells misidentified as epileptic may be treated with antiepileptic medication where none is needed. Recording spells on video provides a potentially valuable supplement to the clinical history in arriving at a correct diagnosis. In practice, however, most home-video recordings prove difficult to interpret. Simple instructions to parents on the manner in which spells should be recorded will greatly increase the usefulness of home-video recordings. A systemic analysis of precipitating factors and the evolution and resolution of the spell will very often exclude seizures. The widespread availability of home-video recordings provides the pediatrician with an opportunity to visually examine a child's spells, often avoiding unnecessary investigations and referrals and allowing the physician to more confidently offer reassurance to the parents.

Anticonvulsants↗
PubMed1994

Cerebral white-matter changes suggesting leukodystrophy in ataxia telangiectasia.

Ataxia telangiectasia is an autosomal recessive disorder characterized by progressive cerebellar ataxia, recurrent sinopulmonary infections, oculocutaneous telangiectasia, selective immunoglobulin deficiency, and defective cellular immunity. We report a 4-year-old girl with ataxia telangiectasia whose initial magnetic resonance imaging (MRI) scan at 17 months of age showed leukoencephalopathy compatible with a leukodystrophy, a neuroimaging feature of ataxia telangiectasia that has not been described. Ataxia telangiectasia was not suspected until the child developed more typical clinical features. Diffuse white-matter high signal intensity on T2-weighted MRI scans may occur in the early stages of ataxia telangiectasia. This disease should be considered in the differential diagnosis of any child with a history and MRI findings suggestive of one of the leukodystrophies. The nonneurologic manifestations of ataxia telangiectasia may be of help diagnostically in this clinical setting.

Ataxia Telangiectasia↗
PubMed1994

The treatment of infantile spasms by child neurologists.

Infantile spasms is a refractory seizure disorder for which a number of different treatment regimens are available. No information is available on which treatment regimens are most widely used and which would be of practical importance in designing clinical trials to determine efficacy of new treatments. We proceeded to gather data on the most commonly employed methods of treating infantile spasms. A survey was mailed in 1991 to all junior, active, and emeritus members of the Child Neurology Society asking details about the treatment of infantile spasms. Telephone follow-up on a random sample of nonresponders was made. The total response rate was 58.3%. Most respondents who treat infantile spasms use corticotropin (ACTH) as their drug of first choice (88%). The most frequently used dosage was 40 IU per day, and the most frequent duration of treatment was 1 to 2 months. The most frequently reported side effects of ACTH or oral corticosteroid treatment were cushingism, behavior changes or irritability, hypertension, topical infections, and systemic infections. Of those not using ACTH, valproic acid was the next most commonly employed agent, followed by oral corticosteroids. The results were similar for all respondents regardless of age, sex, type of practice, number of cases of infantile spasms seen, location (United States or abroad) or whether the survey was completed by mail or telephone. These data suggest that there is a relative uniformity among child neurologists in the management of infantile spasms despite the publication of many alternative treatment strategies.

Adrenal Cortex Hormones↗