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Biomedical subjects

J B Bodensteiner

Publications and source records attributed to J B Bodensteiner.

At least 91 records · Page 5Linked to original sources
PubMed1990

Infantile spasms.

Infantile spasms are a seizure disorder in young infants with diverse etiologies, suggesting that they arise from any disturbance of central nervous system function during susceptible periods of development. The prognosis for normal intellectual and neurologic development parallels that of the underlying etiology. Early and appropriate treatment with ACTH may lead to seizure control in a majority of patients. The treating physician must anticipate the side effects of this modality.

Anticonvulsants↗
PubMed1990

The floppy infant: recent advances in the understanding of disorders affecting the neuromuscular junction.

The clinician is often asked to evaluate the floppy infant. Numerous conditions that cause hypotonia in infancy are briefly outlined in this article. These conditions may affect the brain, spinal cord, or motor unit. Several disorders of neuromuscular transmission, including four distinct and recently described congenital myasthenic syndromes and infant botulism, are discussed thereafter.

Brain↗
PubMed1989

Problems in estimating the prevalence of epilepsy in children: the yield from different sources of information.

A prevalence study of childhood epilepsy in central Oklahoma allowed a comparison of different sources of case-finding. The percentage of all epilepsy cases identified was lowest (9%) from physicians' office records. Higher rates were obtained from review of emergency room records (40%), hospital inpatient records (45%), electroencephalography (EEG) requisitions (68%) and outpatient clinic records (62%). Nearly one-third of the 1159 epilepsy cases were identified from only one source. EEG requisitions provided most of these cases. Although the percentage of all abstracts from one source that identified cases was lowest for EEG requisitions (43%), these abstracts were prepared with much less effort and time than were abstracts from hospital or clinic records, which had higher levels of efficiency (hospital = 52%, clinic = 73%). Hospital inpatient records identified a disproportionate number of children with newborn seizures, simple partial epilepsy, and partial seizures secondarily generalised, and children with multiple developmental handicaps. Fully 10% of all cases had an episode of status epilepticus and, as might be expected, emergency room and hospital inpatient records preferentially identified them. We conclude that case-finding for epidemiological studies of epilepsy in children in some communities is best done through review of EEG requisitions, supplemented by information from outpatient records.

Adolescent↗
PubMed1989

Reversible treatment-related leukoencephalopathy.

We present two children with acute lymphocytic leukemia who developed leukoencephalopathy following administration of a combination of intravenous ara = C and methotrexate during the consolidation phase of chemotherapy. Cranial magnetic resonance imaging showed widespread, abnormally high signal intensity in the deep white matter in both patients, though one patient had normal cranial computed tomographic scan. Treatment was modified, symptoms resolved in 1 to 2 weeks, and the white-matter changes resolved over 6 to 12 months. Intravenous cytarabine and methotrexate appear to act synergistically to enhance the potential for central nervous system toxicity. Awareness of this potentially serious complication of chemotherapy can facilitate timely recognition of leukoencephalopathy with the use of magnetic resonance imaging.

Child↗
PubMed1988

Gastritis with valproate therapy.

We have identified ten children who developed gastritis after prolonged anticonvulsant therapy that included either valproic acid or divalproex sodium. Presenting symptoms were primarily feeding difficulties, including anorexia and refusal to eat. Vomiting was present in two thirds of the patients, with diarrhea, weight loss, and abdominal pain occurring less frequently. Occult blood in stool samples was a late development. All patients responded to therapy with H2-receptor antagonists, oral antacids, or both, with prolonged treatment often necessary to prevent relapse. Although gastrointestinal tract side effects are common with the initiation of valproate sodium therapy, feeding difficulties after long-term treatment are less common. Gastritis should be suspected in children receiving valproate therapy when feeding difficulties arise, particularly if the symptoms are persistent or recurrent.

Adolescent↗
PubMed1988

Infantile botulism.

We present the first two known cases of infantile botulism in Oklahoma. The first case was due to type B toxin; the second was due to type A toxin. Both cases demonstrate most of the classic features of what now appears to be the most common form of botulism. Infantile botulism is an underrecognized but reversible cause of hypotonia. In most cases, the prognosis is excellent with institution of appropriate supportive care. The recognition of cranial nerve palsies or a history of constipation should raise the suspicion of infantile botulism. Aminoglycoside antibiotics and other agents that may precipitate or exacerbate neuromuscular blockade should be used with extreme caution in hypotonic infants until the cause of the hypotonia is clearly identified.

Botulinum Toxins↗
PubMed1988

Anterior cervical epidural abscess with pneumococcus in an infant.

Spinal epidural abscess is a rare infection in childhood. We report the first documented case of pneumococcal epidural abscess in an infant and review the literature regarding this entity. In children, the signs and symptoms of spinal epidural abscess may not be as helpful as those in older patients. Furthermore, the offending organism may not be the usual Staphylococcus seen in adults. Infants may recover neurologic function even after prolonged cord compression; however, a high index of suspicion is needed to make the diagnosis in a timely fashion.

Abscess↗
PubMed1988

Colpocephaly: frequency and associated findings.

Colpocephaly is an abnormal enlargement of the occipital horn of the lateral ventricle, also described as persistence of the fetal configuration of the lateral ventricles. Since it was first described, colpocephaly has been found in association with a number of abnormalities of the brain. In order to determine the frequency of this ventricular configuration at our hospital, we reviewed 3,411 computed tomographic (CT) scans done over a 26-month period. Fourteen cases were identified in which the CT scan had the appearance of colpocephaly. These CT scans were performed during the evaluation of a number of different clinical problems. Agenesis of the corpus callosum was the most frequently associated malformation. The types of malformations commonly associated with colpocephaly suggest that colpocephaly is merely a marker of disordered brain formation and that insults occurring anytime between one and four months of gestation may result in this anomaly.

Abnormalities, Multiple↗
PubMed1988

Parainflammatory leukoencephalomyelitis: clinical and magnetic resonance imaging findings.

Parainflammatory leukoencephalomyelitis is a broad term used to include the spectrum of disorders that affect the central nervous system following infection, immunization, or other noxious stimuli. There is a wide range of clinical and pathologic severity, ranging from acute cerebellar ataxia to acute hemorrhagic leukoencephalopathy. With the improved survival of these patients, magnetic resonance imaging provides a window to the pathologic process, which can aid in the long-term management of these patients. Although lesions of the brainstem and spinal cord correlate well to clinical symptoms, multiple cortical lesions may be present without specific localizing signs. The distribution of magnetic resonance lesions is different from that commonly seen in multiple sclerosis. In some cases, prolonged immunosuppression may be required to prevent recrudescence of the inflammatory response.

Cerebellum↗
PubMed1987

Chronic sorrow in families of disabled children.

Among professionals who work with disabled children and their families, the need has arisen for better articulation of the concept of chronic sorrow, a bereavement process parents experience in response to life with a disabled child. This article presents information and concepts about the nature of chronic sorrow that permit clarification and understanding of the meaning of what is otherwise often confusing parent behavior. Implications for the professional in assisting the parent's movement through the bereavement process will be discussed.

Adaptation, Psychological↗
PubMed1986

Proximal duplications of chromosome 15: clinical dilemmas.

The apparently rare cytogenetic abnormality of partial trisomy 15 was diagnosed by the authors in a patient presenting with developmental retardation, macrocephaly with ventricular enlargement and prominent subarachnoid spaces, hypotonia, low-set ears, hyperextensible wrists and hands, high arched palate, tapering fingers, right esotropia, and bilateral metatarsus adductus. Clinical findings in this case are similar to previously reported cases of proximal duplications of chromosome 15 and bear some similarity to the Prader-Willi syndrome. However, our patient did not have the severe hypotonia, early failure to thrive, or genital abnormalities seen in classical Prader-Willi syndrome. This case supports the theory that a variety of cytogenetic aberrations in proximal 15q can cause a "Prader-Willi-like" syndrome. Increased clinical suspicion is needed when patients are seen with hypotonia, retarded development and mild dysmorphism if the variety of phenotypes are to be delineated.

Abnormalities, Multiple↗
PubMed1985

Control of myofibrillar ATPase activity and force in myodystrophic muscle.

Myofibrillar ATPase activity was measured as a function of the free calcium concentration in skeletal muscles of control and myodystrophic mice. In addition, the force developed in skinned extensor digitorum longus (EDL) fibers of control and myodystrophic mice was measured as a function of the free calcium concentration, and a histomorphometric study was performed on soleus and EDL muscles of control and myodystrophic mice. The results showed that the myofibrillar ATPase activity and the force-generating mechanisms of control and myodystrophic muscles were controlled to the same relative degree by equivalent concentrations of calcium ions. Upon maximal activation of the ATPase activities, we measured 18% less activity in myodystrophic muscles than in control muscles. Maximal activation of the force-generating capacity in skinned fibers showed there was no significant difference in force produced in the control compared to myodystrophic fibers. The histomorphometric study revealed no alteration in the relative distribution of different fiber types in myodystrophic compared to control muscles. However, the histomorphometry did reveal a larger slow (type 1) relative cellular area compared to total cross-sectional area in myodystrophic muscle than in controls. We propose that the lower ATPase activity but equal force-generating capacity of myodystrophic muscles compared to control muscles is due to myodystrophic muscles being composed of a greater fraction of myofibrils from slow (type 1) fibers than control muscles.

Adenosine Triphosphatases↗