PubMed Health⌕ Search

Biomedical subjects

M W Levinsohn

Publications and source records attributed to M W Levinsohn.

13 recordsLinked to original sources

Infantile X-linked ataxia and deafness: a new clinicopathologic entity?

We describe an X-linked disorder of the CNS, characterized by onset, in infancy, of hypotonia, ataxia, sensorineural deafness, developmental delay, esotropias, and optic atrophy, and by a progressive course leading to death in childhood. Pathologically, neuron loss and gliosis of the dentate nucleus and inferior olive are conspicuous; involvement of the cerebellar cortex is less prominent. In the proband, the red nucleus, dorsal motor nucleus of the vagus, and central auditory pathways were severely affected. The mother of the proband, now 33, has self-limited episodes of ataxia, and cerebellar atrophy for which no other cause is apparent. The unique heredity, pathology, and clinical picture distinguish this entity from previously described inherited or metabolic ataxias.

Adult↗

Neurologic sequelae of cardiac catheterization.

Neurologic complications are a known and at times tragic consequence of cardiac catheterization. During a four-year period, 1,362 procedures were performed at our institution on children less than 15 years of age. Eighteen children, without prior nervous system disease, developed neurologic sequelae within 24 hours of cardiac catheterization (1.3%). Three presented with seizures only, ten with neurologic deficits, and five with both focal seizures and stroke. Computerized tomography scans (16 patients) demonstrated areas of nonhemorrhagic infarction in all but one. The 18 patients were studied retrospectively for possible factors contributing to the neurologic complications. They were matched to controls by age, cardiac lesion, and catheterization technique. Data examined included catheterization duration and approach, contrast agent, precatheterization urine specific gravity, pre- and postcatheterization hematocrit, blood loss, and intracatheterization events. Catheter and catheter sheath clots were noted in both patient groups. The neurologic complication group had 7/18 intracatheterization neurologic events versus only one in the controls and the change in hematocrit was greater in the complication group (t = 2.89, p less than 0.01). Due to this study's results and to prevent possible thromboembolic events, a heparinization protocol was instituted and patients were prospectively observed for neurologic complications. No neurologic complications have occurred over three years since the institution of this protocol.

Cardiac Catheterization↗

Intelligence and hypoxemia in children with congenital heart disease: fact or artifact?

Previous studies have reported lower intelligence for cyanotic than for acyanotic children with congenital heart disorders, a finding attributed to the degree of hypoxemia present. Several important variables have not been examined consistently, however, including coexisting neurologic or genetic disorders, definitive surgery, degree of sickness, age at testing sex and social class. The present study examined the relation of these variables to obtained intelligence measures for 82 consecutively admitted children, excluding children with abnormal neurologic examinations and those having received definitive surgery. Consistent with earlier reports, intelligence quotients for the acyanotic children (112.81 +/- 14.52 mean +/- SD) were significantly higher (t = 2.60; p = 0.006) than for the cyanotic group (103.50 +/- 15.81). Although sex, race and social class were not significantly different between the 28 cyanotic and the 54 acyanotic children, the cyanotic children were significantly sicker (x2 = 9.12; p = 0.005) and younger (t = 4.10; p = 0.001). However, when young and old children and the degree of sickness within cyanotic and acyanotic groups were compared, no significant differences were found. These findings demonstrate that intelligence differences between cyanotic and acyanotic children persist when the effect of neurologic abnormalities and definitive surgery is removed and remain despite the severity of sickness or child's age at testing.

Adolescent↗

Intracranial volume pressure response in infants and children: preliminary report of a predictive marker in metabolic coma.

Intracranial volume-pressure response was assessed in 6 children suffering from metabolic coma and ranging in age 2 months-13 years. No untoward pressure or infectious complications occurred. The relationship between baseline mean intracranial pressure (MICP) and volume-pressure response assessment (VPRA) in these patients seems to be exponential rather than linear. By itself, the test is no better than baseline MICP at identifying patients at greatest risk of developing significantly increased intracranial pressure (ICP). When used in conjunction with baseline MICP, this method of VPRA identifies a population with an 80% risk of developing serious ICP elevations within a 4 h time period. Patients with best overall prognosis had significantly lower mean VPRA values than those with poorest overall prognosis.

Adolescent↗

Agenesis of the corpus callosum: a study of the frequency of associated malformations.

Review of 11 cases of agenesis of the corpus callosum studied at our institution revealed a high incidence of associated anomalies. None patients had associated malformations of the central nervous system, 6 involving the pyramidal system. Eight cases were associated with malformations in the rest of the body. Review of completely examined cases from the literature yielded 47 examples of associated malformations. These were varied and without consistent pattern, with the possible exception of facial abnormalities.

Adolescent↗

Facilitation of exchange transfusions with Scribner shunts in Reye's syndrome.

Scribner shunts were placed in nine patients, who had clinical and laboratory evidence of Reye's syndrome and ranged in age from four to fifteen years, to facilitate repeated, rapid exchanges of large volumes of blood. A method of inserting the Scribner shunt is described. Over a period of 30 to 90 minutes, 3 to 8 units of blood were easily exchanged without hemodynamic or mechanical difficulties. There were no infections; all shunts were removed from survivors after four to eight days without permanent sequelae.

Adolescent↗

Reye's syndrome and cerebellar intracytoplasmic inclusion bodies.

A patient with Reye syndrome had intracytoplasmic inclusion bodies in Purkinje cells. Electron microscopical examination demonstrated the inclusions to contain internal particles, but these were not identifiable as known virions. Results of viral studies were normal.

Basal Ganglia↗