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Biomedical subjects

J B Bodensteiner

Publications and source records attributed to J B Bodensteiner.

At least 109 records · Page 6Linked to original sources
PubMed1983

Cerebral infarction related to neck position in an adolescent.

A 16-year-old boy developed a left occipital lobe infarction after falling asleep with his head extended and rotated to the left. He was found to have an anomalous cerebrovascular system, in which the entire blood supply to the left occipital cortex derived from the right vertebral artery. It has been shown that vertebral artery flow can be compromised by physiologic degrees of extension of the neck and rotation of the head to the opposite side. To our knowledge, this is the only reported case of cerebral cortical infarction due to temporary arterial obstruction of the vertebral artery during physiologic hyperextension of the neck and rotation of the head. All previous cases attributed to this mechanism have been brainstem, cerebellar, or cervical spinal cord involvement with thrombosis of the vessel in question. Anomalies of the cerebrobasilar vascular system may place these individuals in jeopardy during otherwise harmless physical activities.

Adolescent↗
PubMed1981

Asterixis associated with sodium valproate.

Intoxication with most anticonvulsants can produce asterixis. Asterixis rarely occurs with therapeutic serum anticonvulsant levels. We report two patients with asterixis who were taking valproic acid and had serum levels within the therapeutic range. Neither patient had clinical or laboratory evidence of hepatotoxicity. Only one other patient has been reported with valproate-associated asterixis in the absence of toxic serum drug levels or hepatotoxicity. Asterixis seems to be due to a central effect of the drug unrelated to hepatotoxicity or sedation.

Adult↗
PubMed1980

Progressive dystonia masking ataxia in ataxia-telangiectasia.

A 10-year-old boy with ataxia-telangiectasia had severe progressive dystonic posturing that masked the ataxia until treatment relieved the dystonia. A younger sister had mor classical neurologic manifestations of the disease. However, both children had telangiectasia, immunologic abnormalities, and other features of ataxia-telangiectasia. The pathologic changes that have been found in the basal ganglia at autopsy and the occurrence of choreoathetosis, oculomotor disturbances, and now dystonia indicate that the function of the basal ganglia in patients with ataxia-telangiectasia is abnormal. Children who have basal ganglial abnormalities should be studied for ataxia-telangiectasia.

Ataxia Telangiectasia↗
PubMed1980

Problems in family practice: Seizure disorders.

The current seizure classification recognizes two major categories, partial (focal) and generalized. Sublcasses of this system are determined by clinical and electroencephalographic manifestations of the seizures. Neonatal seizures are difficult to recognize and classify but require prompt and appropriate treatment for best results. Infantile spasms are important to recognize because of their grave prognosis and because they respond to steroid medications but not standard anticonvulsants. Febrile convulsions represent a continuing treatment controversy but have a good prognosis. The diagnosis of epilepsy is based on clinical history. Laboratory studies help classify the type of seizure and identify the etiology. The computerized tomography (CT) scan has simplified diagnostic evaluation. The extent of the evaluation must be adjusted to meet individual requirements. The choice of anticonvulsant is dependent on seizure type as well as the side effects and cost of the drug. Anticonvulsants have potential side effects which can be minimized by judicious dosage adjustments utilizing serum anticonvulsant levels when appropriate.

Adolescent↗
PubMed1979

Chronic ECHO type 5 virus meningoencephalitis in X-linked hypogammaglobulinemia: treatment with immune plasma.

A patient with X-linked hypogammaglobulinemia developed chronic meningoencephalitis. ECHO virus type 5 was repeatedly cultured from cerebrospinal fluid (CSF). Infusions of high-titer, specific plasma resulted in clinical improvement, but failed to eradicate the virus. After more intensive plasma infusions, the virus could not be cultured from the CSF. The patient died 8 months after institution of intensive therapy. The cause of death was unknown. Autopsy showed persistence of perivascular and meningeal inflammation. Specific anti-ECHO-virus-5 plasma was shown to be more effective in lowering CSF ECHO-virus titers than was plasma without anti-ECHO-virus antibody.

Adolescent↗
PubMed1978

Intracellular calcium accumulation in Duchenne dystrophy and other myopathies: a study of 567,000 muscle fibers in 114 biopsies.

Ultrastructural studies have shown plasmalemmal defects in a proportion of non-necrotic muscle fibers in Duchenne dystrophy, suggesting that intracellular calcium overloading may be an important mechanism of muscle fiber degeneration. To investigate this assumption, we studied the localization of calcium with the von Kóssa method, with alizarin red, and with glyoxalbis-(o-hydroxyanil) in serial, fresh-frozen sections of 114 biopsy specimens. The biopsy material included Duchenne dystrophy (24 cases), other dystrophies (27 cases), inflammatory myopathies (47 cases), and normal controls (11 cases). Counted in each specimen were every muscle fiber, the so-called large-dark fibers, and all calcium-positive fibers. Separate counts were made of the large-dark, necrotic, and other types of calcium-positive fibers. In Duchenne dystrophy, non-nectotic calcium-positive fibers occurred with a mean frequency of 4.83 percent. For all other groups, the corresponding value was 0.57 percent, with a range of 0.21 percent (normals) to 1.76 percent (scleroderma), p less than 0.001. Large-dark fibers were 12 times more common in Duchenne dystrophy than in all other cases. Forty-three percent of the fibers were calcium-positive in Duchenne dystrophy, whereas calcium-positive large-dark fibers were extremely rare in the other cases.

Biopsy↗
PubMedDate not supplied

Gastroparesis in neonatal myotonic dystrophy.

Myotonic dystrophy in the neonate is commonly accompanied by facial diplegia, generalized muscular hypotonia, talipes equinovarus, and muscular respiratory failure. The gastrointestinal manifestations of this disease include poor sucking, choking, regurgitation, aspiration, and swallowing difficulties. Gastroparesis can be a major contributor to the feeding difficulties experienced by these infants. We report on an infant with congenital myotonic dystrophy in whom a severe gastric motility problem was alleviated by metoclopramide therapy. This smooth muscle manifestation may be an important and potentially remediable source of morbidity in these infants.

Female↗
PubMedDate not supplied

Facioscapulohumeral muscular dystrophy: the choice of a biopsy site.

The utilization of so-called standard muscles for biopsy in patients with the clinical features of facioscapulohumeral muscular dystrophy (FSH) often results in nondiagnostic pathologic findings. Five patients are presented who had biopsies of the supraspinatus muscle in addition to one of the standard muscles. The supraspinatus muscle was diagnostic in all five cases. The findings suggest that the supraspinatus muscle should be considered as the biopsy site of first choice in those patients in whom the clinical picture suggests facioscapulohumeral-type muscular dystrophy.

Adolescent↗
PubMedDate not supplied

Benign extracerebral fluid collections: a cause of macrocrania in infancy.

In order to determine the frequency and natural history of benign extracerebral fluid collections, computed tomography reports from a period of 26 months at Oklahoma Children's Memorial Hospital were reviewed (total scans: 3,411). Bilateral frontal extracerebral fluid collections were found in 94 infants under 1 year of age. Eighty-two infants had computed tomography scans as part of the evaluation for macrocrania. Thirteen patients had the typical findings of benign extracerebral fluid collections but otherwise were completely asymptomatic. Longitudinal observation for up to 30 months failed to reveal any changes in neurologic status of these patients. Benign extracerebral fluid collections are a relatively common cause of macrocrania in infants. The presence of these fluid collections is not of immediate concern, providing that clinical evaluations fail to identify either neurologic or developmental abnormalities.

Cephalometry↗
PubMedDate not supplied

Macro cisterna magna: a marker for maldevelopment of the brain?

Enlargement of the cisterna magna occurs in as many as 0.4% of reported patients and generally has been believed to represent a normal variant. Differentiation from Dandy-Walker malformations and other cystic structures has been emphasized. We reviewed 1,260 consecutive computed tomography reports in patients younger than 21 years of age and examined all scans in which enlargement of the cisterna magna was considered an isolated finding. Fourteen patients were identified (incidence: 1%). The primary reasons for obtaining computed tomographic scans included various clinical conditions but excluded symptoms indicative of posterior fossa disease. Developmental or neurologic abnormalities were present in 62% of these patients. Macro cisterna magna should not be dismissed as a normal variant, although the neurologic findings may not be specifically localized to the posterior fossa. This finding may be a marker for abnormal brain function most likely due to subtle disturbances in brain development.

Brain↗
PubMedDate not supplied

Wide cavum septum pellucidum: a marker of disturbed brain development.

A wide cavum septum pellucidum defined as a separation of greater than 1 cm of the leaves occurs uncommonly. Nine children with wide cavum septum pellucidum were studied; 8 were abnormal. Observed abnormalities included cognitive impairment (8), seizures (4), hypoplasia of the corpus callosum (4), optic nerve hypoplasia (2), and growth failure (4). The incidence of intellectual dysfunction, the association with midline anomalies of the brain, and growth failure all suggest that wide cavum septum pellucidum may represent part of a spectrum of midline brain anomalies.

Adolescent↗
PubMedDate not supplied

Partial agenesis of the anterior corpus callosum: correlation between appearance, imaging, and neuropathology.

Two patients with partial (semilobar) holotelencephaly are reported with apparent partial agenesis of the corpora callosa. These patients are of interest because they appear to possess the posterior portion of the corpus callosum but not its anterior component. Because the corpus callosum develops in the commissural plate rostrally and is elaborated in a rostral-caudal gradient, this apparent lack of the rostral component is embryologically puzzling. The neuropathologic studies of 1 patient resolved the apparent discrepancy between magnetic resonance imaging and embryology of the corpus callosum. With these data, we addressed the embryologic factors that may explain this malformation.

Abnormalities, Multiple↗