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Biomedical subjects

J J Gilbert

Publications and source records attributed to J J Gilbert.

At least 73 records · Page 4Linked to original sources
PubMed1983

Paraparesis following intrathecal chemotherapy.

We studied a case of flaccid paraplegia that immediately followed intrathecal injection of cytosine arabinoside diluted in 1.5% bacteriostatic water (1.5% benzyl alcohol in H2O). The complication was reversed by rinsing the CSF space with saline. Autopsy showed fibrosis and remyelination of lumbosacral nerve roots. In acute and chronic animal experiments, we showed that benzyl alcohol in commercially used concentrations can have a local anesthetic and an irreversible toxic effect on nerve fibers. This provides a sufficient explanation for many cases of paraparesis following intrathecal chemotherapy.

Animals↗
PubMed1983

Cerebral edema associated with meningiomas.

The cerebral edema, as judged by computed tomographic scan, associated with supratentorial meningiomas was assessed in 55 cases. No relationship to the occurrence or the degree of edema could be established with respect to meningioma location, histological type, tumor vascularity, cellularity, number of mitotic figures, necrosis, calcification, or cortical invasion. The larger the meningioma, the more likely the presence of and the severity of cerebral edema. The edema is a significant factor in the occurrence of clinical signs and symptoms. A biopsy of cerebral cortex and white matter underlying a transitional meningioma in a patient with associated cerebral edema demonstrated perivascular astrocytic end-feet swelling in the cortex and considerable extracellular fluid in the white matter. The ultrastructural appearance is similar to that seen with primary and metastatic brain tumors and with experimental vasogenic cerebral edema.

Brain Edema↗
PubMed1982

A variant form of metachromatic leukodystrophy without arylsulfatase deficiency.

The clinical, pathological, and biochemical findings in a young woman with a new variant of metachromatic leukodystrophy (MLD) are reported. The patient showed slow early development and deteriorated further during her first two decades. Nerve conductions were slow, and a sural nerve biopsy showed features of a sulfatide lipidosis. Urinary sulfatide excretion was comparable to that of patients with classic MLD, yet in vitro activity of arylsulfatase A and B and cerebroside sulfatase activity were normal. Skin fibroblasts cultured in medium supplemented with 3H-labeled sulfatide showed accumulation of labeled sulfatide in large amounts, implying a defect in sulfatide hydrolysis in vivo in spite of intact enzyme activity in vitro.

Adult↗
PubMed1982

Passive transfer studies in Guillain-Barré polyneuropathy.

Serum and lymphocytes from patients with acute Guillain-Barré polyneuropathy were injected into rat sciatic nerves. Serum from 13 of 17 patients produced perivenular demyelination, associated with lymphocytic infiltration. The pattern of demyelination differed from that caused by experimental allergic neuritis serum. The level of serum demyelinating activity as greatest early in the disease and then decreased. The demyelinating factor was heat-labile but not complement-dependent. Circulating lymphocytes did not cause demyelination in eight patients.

Adolescent↗
PubMed1982

Selective spinal cordectomy: clinicopathological correlation.

The operation of selective spinal cordectomy is rarely performed. The cases of 10 paraplegic patients who underwent this procedure are presented. Their clinical indications were varied, including posttraumatic syringomyelia, uncontrollable leg spasticity, posttraumatic spontaneous neurogenic leg pain, and repair of a myelomeningocele gibbus. The cordectomy specimen was available for pathological examination in nine cases. Specimens removed from or near the site of spinal cord trauma showed extensive gliosis, leptomeningeal fibrosis, and schwannosis. This latter feature possibly represents an attempt at spinal cord repair by the dorsal root ganglion cells. Four specimens contained a syrinx with features including a thick gliotic wall and enlarged Virchow-Robin spaces, features that have been implicated in the pathogenesis of posttraumatic syringomyelia. Clinical results in the patients with syringomyelia and uncontrollable leg spasticity have been excellent. Cordectomy did not provide permanent relief in the patients with neurogenic leg pain. The authors conclude that selective spinal cordectomy is a valuable procedure for specific indications.

Adult↗
PubMed1982

Cavum vergae without cavum septi pellucidi.

The cavum septi pellucidi and cavum vergae are midline developmental cavities in the brain. They usually communicate and obliterate from posterior to anterior, the posterior cavum vergae obliterating first and then usually the anterior cavum septi pellucidi. A cavum vergae without a cavum septi pellucidi would thus be unexpected; however, such a case was seen at the time of autopsy, which suggests that the embryologic events in this region may not always occur in the usual sequence.

Aged↗
PubMed1981

Multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a new case with light and electron microscopic studies.

An 18-year-old girl died following a slowly progressive neurodegenerative disease of nine years duration. At 9 years of age, she developed intellectual deterioration associated with speech difficulty, pseudobulbar palsy and ataxia. The progression included spastic quadriplegia, anarthria, severe dysphagia, ophthalmoplegia, and pes cavus. There was no family history. The brain was uniformly small and the substantia nigra was not pigmented. Neuronal loss and gliosis involving globus pallidus, subthalamic nucleus, thalamic nuclei, brain stem, cerebellum, and spinal cord gave the picture of multisystem atrophy. Intranuclear hyaline inclusions were observed in numerous neurons of the central and peripheral nervous system. These were auto-fluorescent and were made up of intermingled straight filaments (8-9 nm in diameter). Only two previously reported cases showing these same inclusions are known. They are reviewed, compared, and discussed in relation to primary neuronal degenerations.

Brain Diseases↗
PubMed1981

The AB-variant of metachromatic leukodystrophy (postulated activator protein deficiency). Light and electron microscopic findings in a sural nerve biopsy.

THe histopathological findings in a sural nerve biopsy of a new distinct variant of metachromatic leukodystrophy (MLD) are compared to those of classical MLD. The clinical and histological features are typical of a sulfatide lipidosis, yet in vitro activities of arylsulfatases A and B and cerebroside sulfatase are normal. Intact skin fibroblasts, when cultured in a medium supplemented with labelled sulfatide, show impaired in vivo sulfatide hydrolysis. A deficiency of the requisite activator protein is postulated.

Adult↗
PubMed1981

Neurenteric cysts of the spinal cord mimicking multiple sclerosis.

A 54-year-old man died following a 20-year course of intermittent neurologic symptoms. He carried a diagnosis of multiple sclerosis, with most severe involvement thought to be in the spinal cord. At autopsy, two neurenteric (enterogenous) cysts were found to indent the anterior spinal cord -- at the T4 and T11 cord levels. Partial diplomyelia was present in the lumbo-sacral cord. These findings represent a unique combination of congenital abnormalities. Their embryogenesis and pathogenesis are discussed.

Diagnosis, Differential↗
PubMed1981

Cerebellar astrocytoma with benign histology and malignant clinical course. Case report.

Juvenile cerebellar astrocytoma characteristically has a very benign course and good prognosis. A case is reported of juvenile cerebellar astrocytoma with massive craniospinal leptomeningeal spread prior to surgical intervention. The patient died 8 months after the onset of symptoms and only 5 weeks after presentation to the hospital. At postmortem examination, it was found that tumor encased the brain and spinal cord. The histology was benign. The literature on cerebellar astrocytoma is reviewed.

Astrocytoma↗
PubMed1980

Passive transfer of demyelination by experimental allergic neuritis serum.

Serum from rabbits with experimental allergic neuritis (EAN) when injected into rat sciatic nerves produced rapidly evolving demyelination followed by remyelination. Myelinating and non-meylinating Schwann cells as well as myelin itself were damaged by 15 min after injection. Myelin degradation was well advanced prior to involvement by macrophages at 12 h. The demyelinating factor was myelin-specific and complement-dependent. The evidence suggests that the EAN antigen may reside in Schwann cell membranes as well as in myelin.

Animals↗
PubMed1979

Nerve and muscle biopsy: electrophysiology and morphology in polyneuropathy.

We correlated the results of biopsy of a muscle nerve, a sensory nerve, and tibialis anterior muscle with electrophysiologic studies in 13 patients with sensorimotor polyneuropathy and 6 patients with normal findings. There were significant correlations between teased fiber changes and conduction abnormalities in both muscle nerves and sensory nerves. The density of large myelinated fibers in the lateral fascicle of the deep peroneal (LFDP) nerve correlated significantly with both the motor unit estimate and compound action potential amplitude of the extensor digitorum brevis (EDB) muscle. Other characteristics of the EDB muscle compound action potential related poorly to teased fiber abnormalities. There was good correlation of needle electrode study of the EDB muscle with teased fiber analysis of the LFDP nerve and with the morphology of the tibialis anterior muscle in 75 percent of the cases, and only minor discrepancies in the remainder. These results emphasize the close relationship between certain structural and electrophysiologic changes in subacute and chronic polyneuropathy.

Adult↗
PubMed1978

The muscular pain-fasciculation syndrome.

Five cases of a chronic neuromuscular syndrome consisted of muscular aching and sometimes burning pain, fasciculations, cramps, fatigue, and occasional paresthesia. The disorder affected the legs and, less commonly, the girdle, trunk, and arm muscles. The symptoms were enhanced by physical activity and were usually improved by rest. Neither muscular wasting nor weakness was found, although the condition was present for an average of 4.7 years and, in one patient, as long as 10 years. Electrophysiologic studies showed motor abnormalities indicative of axonal degeneration and muscle fiber denervation, most marked in the legs. Light microscopy of skeletal muscle and spinal cord in one case disclosed evidence of mild denervation atrophy in muscle, but no loss of anterior horn cells. The findings are compatible with a benign polyneuropathy.

Adult↗
PubMed1978

Adult onset nemaline myopathy.

A 47-year-old man had symptoms of nemaline myopathy for approximately 1 year. There were marked elevations of creatine kinase, a feature not previously described. Examination of the nervous system at autopsy failed to reveal any abnormalities.

Age Factors↗
PubMed1977

Polymorphism and reproductive mode in the rotifer, Asplanchna sieboldi: relationship between meiotic oogenesis and shape of body-wall outgrowths.

Sexuality and polymorphism are closely coupled in the rotifer Asplanchna sieboldi. In a graded response to dietary tocopherol, embryos develop body-wall outgrowths of various sizes and shapes. Also in a graded response to this compound, some of the affected females produce eggs undergoing meiotic instead of mitotic oogenesis. The haploid eggs of such mictic females develop parthenogenetically into males instead of females. The incidence of mictic females among animals with different shapes was studied among cohorts from mothers subjected to different inducing conditions. The hypothesis that external tocopherol concentrations absolutely fix the probability of meiotic oogenesis was rejected. The other extreme hypothesis, that the probability of meiotic oogenesis is fixed by morphotype, was rejected for animals at the low end of the morphotypic scale but accepted for the more strongly-affected individuals. The probability of meiotic oogenesis is thus constant for the higher morphotypes. The ascertainment of morphotype frequencies in natural or laboratory populations may suffice for estimating the incidence of sexual forms; furthermore studies of factors affecting the body-wall-outgrowth response may also bear directly upon regulation of sexual reproduction in this species.

Animals↗