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Biomedical subjects

M A Fishman

Publications and source records attributed to M A Fishman.

At least 37 records · Page 2Linked to original sources

Brain biopsy in herpes simplex encephalitis.

Routine brain biopsy is often recommended in all patients suspected of having herpes simplex encephalitis (HSE). This approach is based upon the lack of ability to make the diagnosis of HSE on clinical grounds, the need to exclude other diseases for which there is specific therapy, and to stop empiric therapies. Acyclovir is a relatively safe, effective antiviral agent with little toxicity as currently used to treat HSE. Thus this is not a problem. Careful review of alternate diagnoses established at brain biopsy reveals few for which biopsy is the only and best way of making the diagnosis. Thus empiric therapy with acyclovir should be considered in some children in whom all appropriate diagnostic techniques fail to establish specifically treatable diseases. Early detection of HSV antigen in CSF may provide a non-invasive means of early diagnosis in the future.

Acyclovir↗

Update on bacterial meningitis.

Recently, advances in identifying the etiologic agent, improving antibiotic therapy, and understanding the pathogenesis of complications of bacterial meningitis have been made. The acute and long-term sequelae and their courses have been documented. Acridine orange staining of the cerebrospinal fluid may identify bacteria in children with partially treated meningitis when gram-staining is not helpful. Monoclonal antibodies for meningococcus group B antigen have been developed and may prove useful for testing cerebrospinal fluid. Several newer cephalosporins have been shown to have excellent in vitro activity against the bacteria commonly associated with meningitis. They are indicated in the treatment of infants between 4 and 8 weeks of age, children in septic shock, children with liver disease, and children with infection with gram-negative enteric agents or bacteria resistant to ampicillin and chloramphenicol. Vasculitis and cerebral infarction may result in some of the complications, such as seizures and hemiparesis, noted in children, and their consequences can be documented by various neuroimaging procedures. The prognosis for ataxia is good, while that for sensorineural deafness is poor. The majority of children will have neither intellectual deficits nor difficulty with academic achievement. An effective vaccine against Haemophilus influenzae type b has been developed and is recommended for children between 18 and 60 months of age.

Adolescent↗

Basal ganglia calcifications in a case of biotinidase deficiency.

Biotinidase deficiency leads to a biotin-deficient state, with cardinal symptoms of ataxia, alopecia, and skin rash presenting in infancy. Previous reports of head CTs in patients with biotinidase deficiency did not note basal ganglia calcifications. We report the first case of biotinidase deficiency with basal ganglia calcifications. There were no symptoms referable to basal ganglia dysfunction.

Amidohydrolases↗

Encephalocraniocutaneous lipomatosis.

Encephalocraniocutaneous lipomatosis is a distinct clinical syndrome characterized by unilateral cerebral malformations and ipsilateral scalp, face, and eye lesions. The cutaneous lesions are confined to the head. The central nervous system lesions consist in part of cerebral hemiatrophy, porencephaly, and defective opercularization of the insula. The intracranial lesions and clinical symptomatology are progressive, and appear, in part, to have a vascular pathogenesis. This suggests that a primary defect in the formation of tissues derived from embryonic mesenchyme may be responsible for the syndrome and that tissues derived from ectoderm may be secondarily affected.

Adolescent↗

Megalencephaly due to impaired cerebral venous return in a Sturge-Weber variant syndrome.

An infant with a Sturge-Weber variant syndrome developed progressive megalencephaly and eventual hydrocephalus, which required shunting. Cerebral angiography revealed absence of the deep cerebral venous system and the development of abnormal drainage channels via the periorbital veins. It is postulated that the abnormal enlargement of the brain was due to the impaired venous return. Resistance of the brain to continued expansion may have caused an increase in hydrostatic pressure and the development of hydrocephalus.

Angiomatosis↗

Acute effect of glycerol on net cerebrospinal fluid production in dogs.

The effect of glycerol administration on cerebrospinal fluid (CSF) formation in dogs was studied by means of a ventriculocisternal perfusion technique. Net CSF production rate decreased after oral administration of glycerol (3 gm/kg) from a baseline level of 42.33 +/- 6.68 microliter/min (mean +/- standard error) to a trough of 10.33 +/- 4.88 microliter/min at 90 minutes after administration (p less than 0.025). Serum osmolality concomitantly increased from a baseline value of 296 +/- 2.83 to 309 +/- 4.7 mOsm/kg H2O at 90 minutes. The mean percentage change in CSF production inversely correlated to the mean percentage change in serum osmolality, r = -0.85. Thus, glycerol administration decreases net CSF formation, and this effect may be related in part to the rise in serum osmolality.

Animals↗

Progressive ventriculomegaly following minor intracranial hemorrhage in premature infants.

Progressive hydrocephalus following subependymal-intraventricular hemorrhages (SEH-IVH) in premature infants has been noted after moderate to severe degrees of hemorrhage. The ventricular dilatation often has been noted to resolve spontaneously, or not continue to progress after four weeks of age. 11 premature infants with moderate to marked degrees of ventricular enlargement following minor degrees of SEH-IVH have been identified over an 18-month period, and in six of these infants the dilatation continued to progress after one month of age. This sequence of events has not been well documented, and it emphasizes the need to closely follow all infants who have sustained any degree of intracranial hemorrhage. The authors recommend routine real-time ultrasound brain-scans at one month of age for all infants with SEH-IVH, and continued close observation with serial head-measurements of those with enlarged ventricular systems at that time.

Cerebral Hemorrhage↗

Studies with antisera against peripheral nervous system myelin and myelin basic proteins. I. Effects of antiserum upon living cultures of nervous tissue.

We studied the effects of antiserum against rat peripheral nervous system (PNS) myelin, rat or chicken central nervous system myelin basic protein (BP), or rabbit P2 protein from PNS myelin on myelinated cultures containing only rat dorsal root ganglion neurons and Schwann cells. While anti-PNS myelin serum consistently produced segmental PNS demyelination, anti-BP serum and anti-P2 serum did not. The culture results suggest that the myelin PNS proteins P1 (identical to basic protein from central nervous system myelin) and P2 are not exposed on the extracellular surfaces of myelin-related Schwann cells in tissue culture.

Animals↗

Studies with antisera against peripheral nervous system myelin and myelin basic proteins. II. Immunohistochemical studies in cultures of rat dorsal root ganglion neurons and Schwann cells.

Antiserum against rat peripheral nervous system (PNS) myelin contained immunoglobulins which bound preferentially to the extracellular surfaces of myelin-related Schwann cells in intact cultures of dorsal root ganglion (DRG) neurons and Schwann cells, while antiserum against basic protein (BP) from central nervous system myelin or the PNS basic protein P2 did not. We demonstrate the presence of PNS myelin proteins P1 (identical to BP) and P2 by immunoperoxidase techniques in DRG cultures that had been treated to disrupt cellular membranes. These observations suggest that P1 and P2 are not exposed on the extracellular surfaces of myelin-related Schwann cells in culture. The results also support the hypothesis concerning the possible mechanisms by which anti-PNS myelin serum demyelinates DRG cultures, while anti-BP serum and anti-P2 serum do not.

Animals↗