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Biomedical subjects

E M Kaye

Publications and source records attributed to E M Kaye.

At least 37 records · Page 2Linked to original sources
PubMed1991

Modified encephaloduroarteriosynangiosis as a surgical treatment of childhood moyamoya disease: report of five cases.

Moyamoya disease is an idiopathic disorder characterized by progressive occlusion of the internal carotid and anterior and middle cerebral arteries, with formation of an extensive abnormal collateral circulation at the base of the brain. Many neurosurgical procedures have been designed to bypass these occluded vessels. The results of one of these procedures, modified encephaloduroarteriosynangiosis was reviewed in five children followed for 3 1/2 to 19 1/2 years. Modified encephaloduroarteriosynangiosis performed unilaterally in one and bilaterally in four of the children, appeared to halt neurologic deterioration, despite angiographic progression, in four of the five children.

Brain Ischemia↗
PubMed1989

Transient neonatal hyperglycinemia.

Two patients with neonatal seizures and subsequent normal neurological development were found to have nonketotic hyperglycinemia. In both patients, hyperglycinemia resolved at 6 weeks of age. After cerebrospinal fluid glycine levels were normalized, the seizures stopped completely in one child and were markedly improved in the other. The possible mechanisms for the hyperglycinemia are discussed.

Amino Acid Metabolism, Inborn Errors↗
PubMed1988

Nervous system involvement in Fabry's disease: clinicopathological and biochemical correlation.

A detailed neuropathological and biochemical study was performed to evaluate the accumulation of ceramide trihexoside within the central and peripheral nervous systems of 2 patients with Fabry's disease. Luxol fast blue-staining lipid was noted in the leptomeninges and in the choroidal stroma; biochemical studies showed increased quantities of ceramide trihexoside in the cerebrospinal fluid. The permeable blood-brain barrier regions of the central and peripheral nervous systems contained increased quantities of ceramide trihexoside. Some central nervous system nuclei associated with autonomic function were also noted to store lipid. Other areas of increased ceramide trihexoside accumulation included specific cortical and brainstem structures. Possible sources for this selective accumulation of ceramide trihexoside within the central and peripheral nervous system include transport of glycolipid from the systemic vascular network and retrograde transsynaptic glycolipid transport.

Brain↗
PubMed1987

Myelopathy in neonatal and infantile lupus erythematosus.

Two children with lupus erythematosus and myelopathy were studied. The first child developed the neonatal lupus erythematosus syndrome associated with transplacentally acquired anti-Ro/SSA antibodies. The cutaneous manifestations of neonatal lupus erythematosus disappeared but a residual myelopathy was confirmed at 16 months of age. The second child developed cutaneous lupus erythematosus at 3 months of age associated with a total deficiency of the Clr component of complement. A myelopathy and mesangial glomerulonephritis developed at 2 years of age which required treatment with corticosteroids. These two children with CNS lupus erythematosus, one associated with transplacentally acquired antibodies and the other associated with a complement deficiency, may suggest an immune-mediated mechanism for the pathogenesis of myelopathy in childhood lupus erythematosus.

Antibodies, Antinuclear↗
PubMed1986

Type 2 and type 3 Gaucher disease: a morphological and biochemical study.

Glucocerebroside levels were measured in the brains of patients with neuronopathic forms (types 2 and 3) of Gaucher disease and compared to those obtained from control brain. Nine separate brain regions (frontal, temporal, occipital, and cerebellar cortices; thalamus; corpus striatum; pons; medulla; and dentate nuclei) were analyzed. In all the Gaucher brains studied, the greatest glucocerebroside accumulation occurred within the occipital cortex, with lesser amounts in the temporal and frontal areas. The cerebellar cortex, corpus striatum, and thalamus in Gaucher brains had mildly increased levels of glucocerebroside, especially when the values were expressed as a percentage of total non-hydroxy fatty-acid cerebroside. Brainstem structures (pons and medulla) and dentate nuclei did not have increased glucocerebroside levels when compared to levels from similar control areas. However, when glucocerebroside concentration was expressed as a percentage of total non-hydroxy fatty-acid cerebroside, the type 2 Gaucher brainstem structures did show a slight increase in glucocerebroside levels over control levels. Neuropathological studies demonstrated the presence of Gaucher cells, gliosis, and microglial nodules within the type 2 brains. The neuropathological findings correlated with the glucocerebroside accumulation in the type 2 brains. Despite the similar pattern of glucocerebroside accumulation in the type 3 brain, no neuropathological abnormalities were seen. Thus, this study demonstrated that within several brain regions, both neuronopathic forms of Gaucher disease have elevated glucocerebroside levels, and that in the type 2 brains, the glucocerebroside accumulation correlated positively with the neuropathological findings.

Brain↗
PubMed1986

Transient post-traumatic cortical blindness: brief v prolonged syndromes in childhood.

Six children who sustained head trauma of varying degrees experienced self-limited cortical blindness--complete bilateral visual loss associated with normal pupillary responses to light. In five cases associated with relatively minor head trauma, full visual function returned within 24 hours. In the other case, associated with parietal and occipital contusions, the period of complete blindness was prolonged, lasting 1 week, followed by a striking recovery over an additional week. A bilateral inferior altitudinal visual field deficit has persisted over 6 years, with emergence of a complicated migraine syndrome. Sequential computed tomographic (CT) scans over a 4-year period demonstrate bioccipital and right parietal injury, correlating clinical and anatomic findings. This case is the first description of prolonged transient post-traumatic cortical blindness in a child with CT correlation. It underscores the clinical, radiographic, and pathophysiologic differences between syndromes of brief and prolonged transient post-traumatic cortical blindness in childhood.

Adolescent↗
PubMed1984

Separation and quantitation of perbenzoylated glucocerebroside and galactocerebroside by high-performance liquid chromatography.

Perbenzoylated gluco- and galactocerebrosides were separated and quantitated with a modification of existing HPLC methodology. A linear gradient of 0.9-18.4% dioxane in hexane was used to separate the derivatives on a pellicular silica column heated to 65 degrees C. The elevated temperature was the major modification that permitted the separation. This system was compared to a previously reported silica column/isopropanol in hexane system.

Animals↗
PubMed1983

Biochemical studies in a patient with subacute neuropathic Gaucher disease without visceral glucosylceramide storage.

Autopsy samples were obtained from a 12.5-year-old girl who died with a neurologic disorder consisting of myoclonus, myoclonic epilepsy, spasticity, strabismus, and mild mental retardation but no hepatosplenomegaly. Studies in leukocytes, cultured skin fibroblasts, brain, liver, and spleen of this patient revealed glucosylceramide beta-glucosidase (EC 3.2.1.45, glucocerebrosidase) activity about 10% of controls, and well in the range found in samples from Gaucher disease patients. Extraction of the lipids from liver and spleen with chloroform-methanol (2:1) did not show accumulation of glucosylceramide or other lipid. Examination of the lipids in brain by high performance liquid chromatography revealed the presence of glucosylceramide, which is not found in brain samples from controls. Pathologic examination of the liver and spleen revealed no evidence of Gaucher disease. The brain showed many degenerative lesions and loss of neurons. There was no complementation of glucocerebrosidase activity when the cells from this patient were hybridized with cells from patients with Type 1 or Type 2 Gaucher disease. The reason for the lack of glucosylceramide storage in the liver and spleen has not been determined.

Autopsy↗
PubMed1981

Neonatal herpes simplex meningoencephalitis associated with fetal monitor scalp electrodes.

Two full-term unrelated infants developed herpetic lesions at the site of scalp fetal monitor electrodes several days after uncomplicated labor and vaginal delivery. The mothers had been asymptomatic during pregnancy. In addition to the scalp vesicles, one infant had unilateral herpetic conjunctivitis; the other infant had vesicles on the face without ocular involvement. Both babies had cerebrospinal fluid (CSF) pleocytosis and elevated protein. Herpes virus was cultured from the vesicles and CSF. Computed tomography showed low density areas in the frontotemporal regions. On follow-up, seizures persisted and neurologic development was impaired. These patients illustrate the potential risk of infection when internal fetal monitoring is used in low risk pregnancies.

Brain↗
PubMedDate not supplied

Infantile G(M1) gangliosidosis: complete morphology and histochemistry of two autopsy cases, with particular reference to delayed central nervous system myelination.

Inborn metabolic errors causing lysosomal storage, such as beta-galactosidase deficiency (G(M1) gangliosidosis [G(M1)]), have well-recognized effects on cellular function and morphology. In some classically "neuronal" storage diseases, including G(M1), neuroradiologic observations of infants have suggested a delay in myelination on the basis of persistently "immature" signal intensities monitored over time. We sought to evaluate in a semiquantitative fashion the pattern and degree of myelination in two infantile G(M1) patients, one boy and one girl, autopsied at 15 months of age. We assigned myelination degrees for defined sites on an ordinal scale of 0 to 4, and compared them to published population-based values for autopsied infants. In both patients, earlier-myelinating structures were comparable in development to that expected for postconceptional age, whereas later-myelinating structures were delayed. These data correlate well with the neuroradiologic diagnosis of myelination delay in these infants and suggest that the metabolic defect has a primary influence on myelin development, in addition to effects related to neuronal storage. Furthermore, our analysis by light and electron microscopy and lectin histochemistry of both CNS and systemic tissues, several of which had not been described, add to the understanding of the stored material in different cell types.

Autopsy↗