Astrocytic changes in white matter of ALS brain.
Explore the source record for details and available documents.
Amyotrophic Lateral Sclerosis↗
Biomedical subjects
I Feigin
Publications and source records attributed to I Feigin.
Supraophthalmic intracarotid infusion of BCNU for malignant glioma.
We treated five patients with 11 supraophthalmic infusions of BCNU at 200 mg/m2 every 2 months. All three patients with residual tumors showed marked CT response after one infusion. Two patients with bilateral tumors had no response on the contralateral side. All four evaluable cases showed evidence of BCNU neurotoxicity. CT findings superficially resembled tumor recurrence, but white matter changes, nonspecific gyral enhancement, and delayed calcification were more indicative of neurotoxicity. There were no procedure-related complications. One autopsy suggested that direct parenchymal damage might be responsible for delayed neurotoxicity. Supraophthalmic BCNU infusion, at this dosage, is too toxic for cerebral tissue.
Adult↗
Sarcoma arising in metastatic carcinoma in the brain. A second instance.
A second instance in which a carcinoma metastatic to the brain has induced the formation of a sarcoma in the associated cerebral blood vessels, is reported. This is analogous to the more common gliosarcoma, a tumor in which a primary glioma, most often anaplastic astrocytoma (glioblastoma multiforme), has induced the formation of a similar sarcoma, with both neoplastic tissues in the same tumor mass. The formation of the sarcoma is attributed to a neoplastic change in the markedly hyperplastic endothelial cells of the cerebral blood vessels that are very commonly found with anaplastic astrocytomas and are often found in relation to metastatic carcinoma. The progression of hyperplasia to neoplasia has long been considered of oncogenetic significance, and in this specific circumstance, appears to be due to some factor or substance which passes from the carcinoma cells to the cerebral vessels.
Astrocytoma↗
Cerebellocele and associated central nervous system anomalies in the Meckel syndrome.
Three cases with the Meckel syndrome were autopsied and found to have: arhinencephaly , polymicrogyria , aqueductal stenosis, heterotopia of glial tissue, hypoplasia or agenesis of the cerebellar vermis, cranium bifidum associated with large occipital ventriculocele and others. The anomalies at the level of posterior fossa in this condition are classified as those belonging to the Chiari type III group of anomalies. This unusual set of anomalies which forms pathogenetic link between the Dandy-Walker and Chiari-Arnold group of anomalies in the posterior fossa seems to be very frequent in the Meckel syndrome. The therapeutic emphasis is on genetic counseling in view of the recessive inheritance of the syndrome.
Abnormalities, Multiple↗
Neural defects in Say-Gerald (VATER) syndrome.
The Say-Gerald (VATER) syndrome consists of vertebral defects, anal atresia, tracheoesophageal fistula, radial dysplasia and renal defects. 2 children with Say-Gerald (VATER) syndrome were autopsied: the first child was found to have hydrocephalus, aqueductal stenosis and probable craniosynostosis, and the second child had hypoplasia of the nerve roots and anterior and posterior horns of the spinal cord, corresponding to the hypoplastic limb. It is suggested that the Say-Gerald syndrome is a multifocal developmental disorder in which central nervous system anomalies may be found. The children with Say-Gerald syndrome should have a complete neurological examination in order to rule out a potentially treatable central nervous system defect.
Abnormalities, Multiple↗
Intracranial lipomas, hydrocephalus and other CNS anomalies in oculoauriculo-vertebral dysplasia (Goldenhar-Gorlin syndrome).
13 cases of Goldenhar-Gorlin syndrome are presented in which numerous central nervous system anomalies have been found. These include occipital encephalocele, hydrocephalus, aqueductal stenosis, agenesis of corpus callosum, multiple congenital lipomas and many others. Pertinent literature has been reviewed. It is concluded that any part of the central nervous system can be involved in this condition and that careful evaluation is indicated in order to rule out a treatable intracranial anomaly.
Adolescent↗
Extensive advanced maturation of medulloblastoma to astrocytoma and ependymoma.
Four cases of medulloblastoma in children are reported in which a marked degree of differentiation towards neoplastic adult glioma cells was observed. The original immature and highly malignant tissue had largely disappeared in three and completely disappeared in the fourth. The mature tissues which replaced them included astrocytoma tissues in all four, but ependymoma tissues predominated in two. These cases illustrate a general principle applicable to embryonic tumors, defined as those originating in cells that are still immature. Such maturation and differentiation are presumably associated with a decrease in growth rate and an improved prognosis. A continuation of the process of maturation may account for the unexpectedly favorable course sometimes noted with gliomas in children. The processes of differentiation, dedifferentiation, and anaplasia are discussed, particularly the semantic implications of the terms, and it is suggested that the term "dedifferention" is unfortunate since it may have two different meanings. It may be used as a synonym of anaplasia, or it may imply a progression of change backward on the embryologic pathways along which primitive cells normally mature. The latter may not occur, but the assumption that it does leads to views concerning the glioma group as a whole and the phenomena described in this paper, which we hold to be untentable.
Astrocytoma↗
Encephalocele (cerebellocele) in the Goldenhar-Gorlin syndrome.
A patient with unilateral preauricular appendages and occipital mass is described who died on the fifth day of life. An autopsy demonstrated congenital megabladder and megaureters and dysplastic kidneys. The neuropathological examination revealed cranium bifidum in the occipital region, defective posterior arch of the C1 vertebral body, agenesis of vermis and a large cerebellocele. It is suggested that some patients with the Goldenhar-Gorlin syndrome may have prominent central nervous system involvement in spite of only relatively slight facial involvement.
Cerebellar Diseases↗
The visual system in myelin disorders. Vascular and other factors.
Explore the source record for details and available documents.
Brain Edema↗
Iniencephaly: a neuropathologic study.
Five cases of iniencephaly are reviewed. Numerous central nervous system malformations were found at all levels, including microencephaly, polymicrogyria, heterotopic glial tissue in the leptomeninges, atresia of the ventricular system, marked disorganization of the brain stem, vermian agenesis, large cerebellar cyst, and disorganization of the spinal cord tissue. The cerebellum was normal in one case. Numerous skeletal anomalies were found as well as marked retroflexion of the craniocervical junction. We concluded that cerebral anomalies, although severe, are not specific for iniencephaly. Cerebellar anomalies, on the other hand, were considered to share some morphologic features between Dandy-Walker and Arnold-Chiari, i.e., Chiari type II and Chiari type III, malformations.
Abnormalities, Multiple↗
The effect of saponification on the mucopolysaccharides of the ground substance of the human brain: the relation to focal edema and multiple sclerosis.
The acid mucopolysaccharides of brain tissues are disclosed by their metachromatic staining with toluidine blue following saponification with potassium hydroxide, presumably as a result of the liberation of acid groups previously esterified. Earlier histochemical studies had disclosed the presence of neutral mucopolysaccharides by staining with the periodic acid-Schiff technique, and such staining is intensified by prior saponification. Many biochemical studies have reported the presence of both acid and neutral mucopolysaccharides in brain tissues. Within the white matter following brain edema, the quantity of stained mucopolysaccharides is decreased in the plaques of multiple sclerosis and pontine myelinolysis, and in the lesions of diffuse sclerosis. All of these are characterized by myelin loss with relative preservation of axons. The known physiological effects of the mucopolysaccharides on the water content of normal tissues, and on the properties and diffusability of the increments of fluid that constitute edema, lead to the suggestion that edema may play a major role in the pathogenesis of the demyelinating diseases, including multiple sclerosis.
Brain Chemistry↗
The mucopolysaccharides of the ground substance of the human brain.
The concept of the presence of mucopolysaccharides forming an interstitial ground substance in central nervous tissues is revived. The biochemical reports of both neutral and acid mucopolysaccharides in both gray and white matter of the normal brain are reviewed. The earlier histochemical reports and the histochemical studies in this laboratory are appraised and are thought to demonstrate the presence of neutral mucopolysaccharides in both gray and white matter, more evident in the former. These mucopolysaccharides are thought somewhat greater in the arcuate white matter than in the deeper white matter and greater in the striae of white matter coursing through the corpus striatum than in the internal capsule. The presence of acid mucopolysaccharides in normal brain substance is not clearly demonstrated histochemically, though they have been noted in some oligodendrogliomas and in rare well differentiated astrocytomas. The possibility is suggested that acid mucopolysaccharides are present, as the biochemical studies indicate, but that their acid groups are normally blocked or otherwise rendered non-reactive, preventing their histochemical disclosure in normal tissues but serving as a source of those seen in pathologic conditions. The concept of a ground substance has important implications for the understanding of the distribution of excellular fluid in brain tissues in both the normal state and in edema.
Astrocytes↗
The influence of the ground substance on the extracellular water of normal and edematous human brain: focal edema and the demyelinating diseases, including multiple sclerosis.
The presence of a ground substance in brain provides a mechanism by which edema localized to one region of the white matter might occur without spreading diffusely into the adjacent tissues. The most common such localization is the sparing of the arcuate white matter when the deeper white matter is markedly edematous. This may be related to the higher concentration of mucopolysaccharides in the former. Petechial hemorrhages in the white matter may be surrounded by a zone free of edema, although the hemorrhagic zone itself is almost certainly edematous. This, and the presence of a central zone within some of the petechiae forming a ring hemorrhage may reflect the influence of the ground substance. Focal lesions of the dorsum of the corpus callosum and similar lesions of the basal surface of the pons, these probably due to traumatization by the contiguous falx or arteries, are characterized by myelin loss and axon preservation, a characteristic of edema; the surrounding tissues are not edematous. Severe hypertension is sometimes associated with the presence of clusters of focal perivenous demyelinating lesions in the white matter, the axons being preserved. These resemble the lesions of acute disseminated encephalomyelitis and may be due to edema; they are surrounded by nonedematous white matter. It is suggested that the same concept may apply to the focal demyelinating lesions of acute disseminated encephalomyelitis, multifocal leukoencephalopathy, central pontine myelinolysis and of multiple sclerosis, i.e. the "true" demyelinating diseases, just as has already been suggested for diffuse sclerosis.
Astrocytes↗
An axoplasmic component of the Schmidt-Lanterman deformation.
In three instances, peripheral nerves containing numerous Schmidt-Lanterman discontinuities of myelin revealed transverse, wedge-shaped, or arrowhead-shaped axoplasmic excrescences visible with axon stains. These were arranged with a frequency and length like that of the myelin clefts, but the axonal materials were located within the myelin masses bordering the clefts, not in the clefts themselves. These axoplasmic changes are not consistent with most assumptions concerning the physiologic functions of the clefts, and supports the concept that the clefts are artifactual, due possibly to mechanical factors acting an abnormal nerve fibers.
Adult↗
Subarachnoid dissemination of thoracic chordoma.
Chordoma at the T-9 level, originally manifested by lumbosacral radiculopathy, developed in a 50-year-old man. The patient underwent three operations and radiation therapy for this condition, but ten years later, thoracic myelopathy developed, followed by left facial weakness. On autopsy, extensive dissemination of chordoma was found along the base of the brain and in the leptomeninges of the spinal cord.
Arachnoid↗
Laminar scars in cerebral white matter: a perinatal injury due to edema.
Branched plate-like demyelinated lesions were present in the gyral and central white matter of two individuals, 3 and 54 years of age. The degenerated areas contained very few axons and were densely gliotic, and in the older case, contained connective tissue fibers and were continuous with a large parenchymal cyst. The lesions were covered on both sides by normally myelinated white matter, often representing only the subcortical arcuate white matter, occasionally being considerably broader. In some of the latter zones, there was a central area of less severe degeneration which, however, spared the arcuate zone which remained normal. It is suggested that these lesions represent the effects of edema induced by birth injury, under circumstances which permitted the continued formation and maturation of white matter after the edema had subsided. The edema is thought due for the most part to diffuse hypoxia and acidosis, but other mechanisms, such as infection, venous stasis and trauma, may have contributed to the pathogenesis of the edema, and to the lesions directly.
Birth Injuries↗
The role of edema in diffuse sclerosis and other leukoencephalopathies.
The pathologic changes associated with broad zones of demyelination of the cerebral white matter, the diffuse sclerosis group, are analyzed with respect to the contribution of cerebral edema to their pathogenesis. The characteristics of cerebral edema in general are reviewed, and the interpretation offered that only one type of cerebral edema, that characterized by an extracellular accumulation of fluid, occurs in man. In the diffuse sclerosis group, edema is most readily recognized by the tendency for the arcuate zone of the white matter to be spared when the deeper white matter is severely degenerated. Edema is demonstrated in metachromatic leukodystrophy, globoid cell leukodystrophy, adrenoleukodystrophy, disorders of amino acid metabolism, trauma, hypertensive disease, radiation effect, diffuse hypoxia with acidosis, and other conditions, i.e., both in disorders of myelin metabolism and in conditions in which normal myelin is injured by extrinsic influences. These groups cannot be consistently distinguished morphologically, in part because the injury by edema often obscures any primary morphologic change which may have been present. A familial tendency does not necessarily denote a metabolic abnormality specifically related to the chemistry of a myelin constituent, since the lesions might result from edema induced by some other type of familial process. Such familial processes might be metabolic, but are not specifically related to myelin formation or catabolism, as might be true of diseases like phenylketonuria or maple syrup urine disease. Edema may also occur in instances in which a familial disease induces diffuse hypoxia and acidosis, as exemplified by two instances of severe white matter degeneration in subacute necrotizing encephalomyelopathy; one of these is an additional instance of apparent "crib death" by this cause.
Adolescent↗