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Biomedical subjects

R J Baumann

Publications and source records attributed to R J Baumann.

7 recordsLinked to original sources

Extending neurologic services to rural children.

The University of Kentucky provides neurologic services to rural children by a traveling clinic. In 1978, 438 children (including 231 new patients) made 646 clinic visits. The primary diagnoses were appropriate for a neurology clinic; epilepsy was the most common (74 of 231) among new patients. A community survey of school-age children found the clinic serving 45 percent of "active epileptics." Clinic patients had a higher seizure frequency and came from a more disadvantaged background that nonclinic patients. These data show that an urban-based traveling clinic can identify and care for rural children with neurologic disorders.

Child

Juvenile amaurotic idiocy (neuronal ceroid lipofuscinosis) and lymphocyte fingerprint profiles.

Lymphocytes from 3 children with a form of juvenile amaurotic idiocy (characterized by retinal blindness, progressive dementia, and extrapyramidal motor disturbance) were studied by electron microscopy. Numerous fingerprint profiles (FP) were found in vacuolated lymphocytes from all 3 patients and an asymptomatic younger sibling of 1 patient who subsequently became symptomatic. We propose that the combination of this clinical picture and vacuolated lymphocytes with FP is sufficiently distinctive for clinical and research purposes until the biochemical defect is discovered. Wider utilization of ultrastructural study of lymphocytes should increase the number of children diagnosed and allow detection of asymptomatic patients.

Adolescent

Lactic acidosis associated with cerebellar vermal atrophy and cardiomyopathy.

The association of fluctuating neurological signs and congestive cardiomyopathy with chronic lactic acidosis is described in a 5 1/2 year-old-boy who ultimately succumbed to congestive heart failure. The autopsy findings included severe atrophy of the anterior cerebellar vermis and a hypertrophied heart with left sided endocardial fibroelastosis. Skeletal and cardial muscle calcification was prominent and probably due to the effect of intracellular metabolic alterations associated with lactic acidosis. A review of the literature shows that the combination of cardiomyopathy, isolated atrophy of cerebellar vermis and muscle fiber calcification have not been reported in association with idiopathic lactic acidosis previously.

Acidosis

Epilepsy in rural Kentucky: prevalence in a population of school age children.

Seizure prevalence among school age children residing in a rural western Kentucky area (Hardin County) was determined utilizing a method designed to minimize false positives and to allow estimation of false negatives. The observed prevalence of epilepsy is 5.7/1,000 and of febrile seizures 17/1,000. Because of the high rate of false negative responses detected by random sample interview, the true rate of febrile seizures is believed to be closer to a projected rate of 31/1,000.

Age Factors

An estimate of the true prevalence of epilepsy in a rural Appalachian population.

Relationship between reported and true prevalence of seizures in communities are often confounded by such factors as: availability of appropriate diagnostic and therapeutic resources, sophistication of medical records and disease reporting systems, a uniform definition of cases the social implications of the disease in that area. Through applications of an epidemiologic survey, monitored to take these factors into account, we have developed data which are considered an accurate estimate of the true prevalence of seizures in the school age population of a relatively isolated mountain community of 19 000. Analysis suggested a 50% greater prevalence of seizures than expected from other studies of similar age groups. Although exact comparison of these data with data from other studies is hampered by variation in definitions, there is a strong suggestion that a high rate of seizures does exist in this community. Some possible explanations are examined and plans for further investigation are described. The methodology employed in the survey is presented as a potential model for consideration.

Appalachian Region