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J B Bodensteiner

Publications and source records attributed to J B Bodensteiner.

113 records · Page 7Linked to original sources
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Serial MRI and CT findings in infantile Krabbe disease.

Krabbe disease (globoid cell leukodystrophy) is an autosomal recessive childhood disorder characterized by severe motor and mental deterioration. The disease has been divided into 3 main types and further subdivided into several subtypes based on age of onset and symptoms. Initial clinical findings and magnetic resonance imaging (MRI) with several follow-up scans are presented to identify the order and extent of white matter involvement and developing brain atrophy in a child with the floppy infant variant of Krabbe disease. When the patient's clinical condition proceeded to stage 2, MRI disclosed severe involvement of the deep white matter around the atria and posterior limbs of the internal capsules. At the same time there was progression of normal myelination around the frontal horns. At age 32 months, the patient's clinical condition proceeded to stage 3; she did not interact with her environment. MRI revealed a significant decrease of white matter volume, generalized atrophy, and abnormal high signal in all white matter areas except the anterior limbs of the internal capsules. At the same time the volume of the central gray nuclei was decreased and also demonstrated abnormal high signal. Despite its sensitivity, MRI could not differentiate the findings of this variant of Krabbe disease from the classic form; therefore, subclassifications of Krabbe disease should be made on clinical grounds because they cannot be distinguished by biochemical or radiologic (MRI) criteria.

Brain↗
PubMedDate not supplied

Computed tomography and magnetic resonance imaging in late-onset globoid cell leukodystrophy (Krabbe disease).

A five-year-old white male presented with a history of progressive loss of vision that was subsequently followed by progressive corticospinal dysfunction. Evaluation revealed the presence of leukodystrophy which was confirmed by a deficiency of the enzyme, galactosylceramide beta-galactosidase. We present the clinical, computed tomographic, and magnetic resonance imaging features of this late-onset form of globoid cell leukodystrophy.

Atrophy↗
PubMedDate not supplied

Interobserver variability in the ILAE classification of seizures in childhood.

Observer variability in interpreting medical tests and in making diagnoses influences both clinical practice and research. Uniform classification of epileptic seizures is especially difficult. Although the ILAE classification scheme for seizures has been available for many years, the reliability of this system has not been previously assessed. Verbatim descriptions of seizure manifestations were transcribed from medical records as part of a large, population-based prevalence study of childhood epilepsy conducted in two countries in central Oklahoma. One senior neurologist and three neurology residents reviewed these descriptions independently and classified them by seizure type based on the ILAE system. Unweighted and weighted kappa statistics were used to assess the level of agreement between the study neurologist and each resident. The overall agreement between observer pairs in classifying seizure types based on all available descriptions was relatively poor (kappa = 0.24-0.38). Some improvement was evident when unclassified seizures were excluded, and comparisons were restricted to those based on descriptions with some degree of detail (kappa = 0.34-0.51). When specific types of seizures were classified, agreement was fair to excellent for most types (kappa = 0.45-0.90), with the exceptions of atypical absence (kappa = 0.11-0.28), partial seizures with secondary generalization (kappa = 0.26-0.40), and generalized motor seizures (kappa = 0.29-0.32). Sources of observer variability in addition to the classification scheme are considered. Use of specific criteria for the categorization of symptoms might improve the reliability of seizure classification.

Adolescent↗
PubMedDate not supplied

Prevalence of the epilepsies in children and adolescents.

The prevalence of epilepsy in children and adolescents from birth through age 19 years was determined for residents of two counties in central Oklahoma. Cases, identified from hospitals, clinics, private physicians' offices, and EEG laboratory and emergency room records, numbered 1,159, yielding a prevalence rate of 4.71 per 1,000. The prevalence was highest in children aged 1-4 years. Overall, males had a slightly higher prevalence rate than females (M:F = 1.1). However, the male/female ratio varied by age, with the group aged less than 1 year having the highest ratio (M:F = 1.5), and by type of epilepsy, with males having higher rates of simple partial epilepsy (M:F = 1.8) and infantile spasms (M:F = 1.5). The prevalence of epilepsy was higher in blacks than in whites. Differences in prevalence by race were confined primarily to generalized epilepsies (B:W = 1.8). The most common types of epilepsy were tonic, clonic, and tonic-clonic (1.14 per 1,000), complex partial (0.39 per 1,000), and partial seizures secondarily generalized (0.33 per 1,000). Approximately 70% of cases were considered idiopathic. Among the presumed causes were perinatal factors (7%), trauma (4%), central nervous system (CNS) infection (3%), and congenital/developmental factors (3). Sixty-five percent of cases had at least one additional medical problem. The most common types of comorbidity were motor handicap (13%) and developmental delay (24%).

Adolescent↗