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Biomedical subjects

J B Bodensteiner

Publications and source records attributed to J B Bodensteiner.

At least 73 records · Page 4Linked to original sources
PubMed1994

Cerebral dysgenesis. An overview.

A significant portion of patients with neurodevelopmental abnormalities (mental retardation, learning disabilities, and so forth) have no definable cause for these problems. Mounting evidence suggests a substantial number of these idiopathic conditions have subtle abnormalities of brain development (cerebral dysgenesis) as the inherent pathophysiologic event. In this article the authors summarize normal and abnormal brain development, the diagnostic approach to idiopathic neurodevelopmental anomalies, and the new molecular genetic insights into the underlying causes of brain malformations.

Brain↗
PubMed1993

Hypertrophic cardiomyopathy during corticotropin therapy for infantile spasms. A clinical and echocardiographic study.

OBJECTIVE: To evaluate the incidence and clinical features of abnormal cardiac hypertrophy during treatment of infantile spasms with corticotropin. DESIGN: A prospective, echocardiographic study. PARTICIPANTS: Eighteen children treated for infantile spasms with high-dose corticotropin at a large tertiary children's hospital. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Abnormal cardiac hypertrophy was seen in 13 (72%) of 18 patients. Five of 18 patients (group 1) developed hypertrophic cardiomyopathy with asymmetric septal hypertrophy; none developed significant left ventricular outflow obstruction. Concentric left ventricular hypertrophy was seen in eight patients (group 2), whereas no changes were observed in five (group 3). CONCLUSION: Abnormal ventricular hypertrophy occurs in the majority of patients treated with corticotropin for infantile spasms. In our study, many of these patients developed hypertrophic cardiomyopathy with dramatic asymmetric septal hypertrophy; however, none developed significant obstruction to left ventricular outflow. All changes were reversible, and did not require premature discontinuation of therapy.

Adrenocorticotropic Hormone↗
PubMed1992

Clinical features of vascular thrombosis following varicella.

OBJECTIVE: To define the clinical characteristics, neuroimaging features, and outcome of five patients with post-primary varicella zoster virus infection hemiparesis and to offer a hypothesis to explain the predilection for the involvement of the cerebral vasculature in this condition. DESIGN: Patient series. SETTING: Five patients were treated during a 14-month period in a private pediatric neurology practice in a medium-size southwestern city. INTERVENTIONS: Steroids (two patients) and antiplatelet drugs (two patients). No observed effects of therapy. RESULTS: The onset of the hemiparesis occurred several weeks (mean, 5.4 weeks) following an episode of the chickenpox. Magnetic resonance imaging was more sensitive than computed tomography or angiography in demonstrating the area of involvement deep in the cerebral hemispheres. The prognosis was good regardless of the therapy administered, as all patients recovered completely or nearly completely. CONCLUSIONS: Primary varicella zoster virus infection with delayed-onset hemiparesis typically occurs approximately 6 weeks after primary varicella zoster virus infection. Magnetic resonance imaging is the most sensitive neuroimaging tool in these children. The prognosis is good, with recovery of function and no recurrences in our patients. The innervation of the carotid artery and the characteristics of the varicella zoster virus itself together provide the local and systemic factors that may trigger the vasculopathy responsible for this syndrome.

Chickenpox↗
PubMed1992

Evaluation of the child with idiopathic mental retardation.

A review of the advances in diagnostic techniques for evaluation of children with idiopathic mental retardation is presented. The current status of the use of clinical genetics, cytogenetics, molecular genetics, and neuroimaging in evaluating children with mental retardation is emphasized. Special attention is given to the evaluation of children with mental retardation and "autism" or "cerebral palsy."

Abnormalities, Multiple↗
PubMed1992

The cavum septi pellucidi in term and preterm newborn infants.

We reviewed cranial sonographic studies done on 108 normal newborn infants to determine the prevalence and variability in size of the cavum septi pellucidi (CSP). Infants were classified according to gestational age by 2-week intervals. At 24 weeks, only four normal scans were identified. Between 26 and 34 weeks, ten consecutive normal scans were used since all infants had a CSP. At 36, 38, and 40 weeks, all normal scans were counted in order to obtain a prevalence estimate. A CSP was seen in all normal infants below 36 weeks of gestational age. At 36, 38, and 40 weeks, only 69%, 54%, and 36%, respectively, had CSPs. There was no significant change in the width, depth, or area of the CSP with age, birth weight, or biparietal diameter. A CSP greater than 0.95 cm in width or greater than 0.81 cm in depth is outside the normal range and may represent anomalous development of the midline structures of the brain.

Child↗
PubMed1991

Clinical and morphometric analysis of the hypoplastic corpus callosum.

A total of 307 children were evaluated over a 3-year period in our neurogenetics clinic. Review of their medical records demonstrated 26 patients with diagnoses of anomalies of the corpus callosum. Morphometric analysis was performed on those 23 patients qualitatively assessed as having a hypoplastic (small, but morphologically intact) corpus callosum. Morphometric data were compared with clinical correlates for each patient. From these data, we conclude that the hypoplastic corpus callosum is not a normal variant of development but rather an indicator of a more fundamental abnormality of cerebral development.

Agenesis of Corpus Callosum↗
PubMed1991

An adult-onset myopathy characterized by a double ring appearance of muscle fibers.

We report a 33-yr-old man with an unusual neuromuscular disorder characterized by progressive generalized weakness of 3 yr duration whose muscle biopsy showed a double ring appearance in most muscle fibers. This double ring appearance was due to a peripheral outer sarcoplasmic mass and an inner ring of annular myofibrils surrounding a core of normal longitudinally oriented myofibrils. Nerve conduction studies were normal. Electromyography showed fibrillations, positive waves, and increased brief duration, low amplitude, polyphasic potentials.

Adult↗
PubMed1991

Age-related changes in the relative growth of the posterior fossa.

We have established a normative data set for the relative size of the structures of the midline posterior fossa from birth to 90 years old. Data were obtained from morphometric analysis of midsagittal magnetic resonance imaging scans of the brain utilizing a simple image analysis system. There are several significant changes in the size of these structures with an increase in chronologic age. The relative size of the cisterna magna decreases with age. Anterior cerebellar vermal lobules (I through V) appear to grow more rapidly than the rest of the cerebellum. Other, less significant, trends include a decrease in the overall size of the cerebellum, superior posterior vermal lobules (VI and VII) and inferior posterior lobule (VIII) with an increase in age. It is, therefore, necessary to use age-standardized normative data when making morphometric correlations with clinical disorders.

Adolescent↗
PubMed1991

HLA patterns in children with parainflammatory leukoencephalomyelitis.

We have evaluated the human leukocyte antigen (HLA) phenotype of six children with parainflammatory leukoencephalomyelitis (PIL). Patients with PIL demonstrate an increased prevalence of the HLA-A1, -A28, -B44, -DR6, and -DR7 antigens. These HLA associations are different from those reported in other inflammatory demyelinating diseases, including multiple sclerosis, optic neuritis, Guillain-Barré syndrome, and chronic relapsing inflammatory polyneuropathy. The HLA constitution of the patient appears to be one important host factor in determining the nature of the immune response to an encephalitogenic challenge.

Child↗
PubMed1990

Extensive wormian bones in a patient with the Hallermann-Streiff syndrome.

A case is reported of a 5-month-old girl with Hallermann-Streiff syndrome who was evaluated for possible premature closure of the cranial sutures. Skull radiographs revealed numerous Wormian bones along sutures in the parietal skull bilaterally. Hallermann-Streiff syndrome is added to the list of diseases in which extensive Wormian bones can appear.

Bone Development↗
PubMed1990

Quantitative morphometric analysis of brain growth using magnetic resonance imaging.

Using a simple image analysis system, we have established normative data obtained from magnetic resonance imaging (MRI) scans for the relative size and growth of the major structural domains of the brain. Midsagittal scans of the head were analyzed in 95 subjects aged 0 to 20 years. Only scans with no demonstrable structural abnormalities of the brain were utilized. Age-related changes in the relative growth of specific structures were calculated by dividing patients into 5-year age increments. No age-related changes were found in the size of the posterior fossa, supratentorial intracranial space, or cisterna magna relative to the total intracranial vault space. A significant (P less than .01) increase in the size of the corpus callosum relative to that of the supratentorial structures and relative to the total intracranial vault space was noted to occur between the 6- to 10-year-old and 11- to 15-year-old age groups. This may reflect an increase in myelination of the corpus callosum that occurs during this period. A small reduction in the ratio of the posterior fossa to the supratentorial space was found between the 0- to 5-year-old and the 6- to 10-year-old age groups, but the biological significance of this difference is uncertain. It appears from these data that quantitative analysis of MRI scans can be used to draw objective conclusions about the relative sizes and growth of the major brain structures. The development of normative data for these structures and the simplicity of the methodology should have many clinical applications in the assessment of aberrant brain development.

Adolescent↗
PubMed1990

Hypoplastic corpus callosum in ocular albinism: indication of a global disturbance of neuronal migration.

Ocular albinism is distinguished from the more common oculocutaneous albinism by the presence of normal pigmentation of skin and hair in the former condition. Recent studies of ocular albinism have shown that the hypopigmentation of the optic fundus is associated with a number of anomalies of neuronal wiring involving the visual system. We present a patient with ocular albinism who also has a hypoplastic corpus callosum as determined by analysis of midsagittal magnetic resonance imaging scans. Previous studies of the hypoplastic corpus callosum indicate that this anomaly is a defect in neuronal migration as well. The finding of a hypoplastic corpus callosum in a patient with ocular albinism suggests a more generalized defect in neuronal migration not limited to the visual system.

Abnormalities, Multiple↗