Astrocytic changes in white matter of ALS brain.
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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↗
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↗
Mixed mesenchymal tumors: meningioma and nerve sheath tumor.
Two spinal tumors are reported which consisted of an admixture of meningiomatous and nerve sheath tumor tissues. The former was represented by whorled tissues in both cases, syncytial areas in one and psammoma bodies in the other. The latter was represented by cells with parallel oriented, elongated nuclei and intercellular connective tissue fibers, which contained neurons and myelinated axons. Each of the tumors contained anaplastic areas of increased cellularity and atypism, in addition to the well differentiated areas. On tumor was at cervical levels in a 55 year old man; he has survived for 7 years post-operatively, but with continued pain and motor difficulties. The other was at lumber levels in a 68 year old man; died 6 weeks after operation. No autopsy was obtained. There were no stigmata of von Recklinghausen's disease in either patient. It is suggested that these tumors should not be viewed as teratoid, i.e. of mixed mesenchymal and neurectodermal character. If Schwann cells are considered mesenchymal, as is suggested in other studies, the tumors would represent mixed mesenchymal tumors, like those frequently observed elsewhere, e.g. the angiomyolipomas.
Aged↗
Case reports. Histologic evidence of radiation necrosis following radiotherapy of primary cerebral tumor.
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Adult↗
Further observations on subacute necrotizing encephalomyelopathy in adults.
The clinical and neuropathlogical aspects of four cases of subacute necrotizing encephalomyelopathy (SNEM) in adults, are described. In one, the disease was precipitated by a surgical procedure, and caused death; this resembled cases previously reported. In the other three, the disease was recognized as an incidental pathological finding, without apparent morbidity. In these, the pathologic lesions were few in number, but were characteristic in location and nature, most specifically in the preservation of neurons within the devasted tissues. The disease in adults is thought due to a less severe metabolic defect than that which causes death in infants, and may be more common. When extrinsic stress is superimposed, the disease may become severe and cause death as in the first case and those previously reported. This might be prevented by the prophylactic use of those therapeutic agents which have had a favorable effect in infants, particularly very high doses of thiamine. The prevelence of the disease in adults, including the incomplete cases included in this report, suggests that such prophylactic measures may be warranted when stress is anticipated, as with surgery.
Age Factors↗
Maturation and anaplasia in neuronal tumors of the peripheral nervous system; with observations on the glial-like tissues in the ganglioneuroblastoma.
Six cases of peripheral ganglioneuroblastoma are described and analyzed; in general these contained neuroblasts, mature ganglion cells and cells of intermediate degrees of maturity. Portions of the tumor resembled the glial tissues of the central nervous system, but this is spurious, no glial cells or fibers being present. Such tissues contained cells of intermediate maturity, immature axons which characteristically stain poorly with silver stains for axons, and a paucity of connective tissue fibers. These tumors exhibit a tendency for continued maturation with decreased malignancy, and for anaplastic change with increased malignancy. These opposing tendencies make it difficult to predict the outcome with complete certainty, except possibly in those cases in which maturation is complete. Schwann cells are present in those tissues which have matured to form neurons with axonal processes. The Schwann cells are probably reactive, not neoplastic. The presence of Schwann cells in such mature tissues is most readily explained on the assumption that Schwann cells are mesenchymal in character, and have been formed by differentiation of multipotential primitive reticular cells in response to the formation of axons by the maturing neurons.
Adult↗
Anaplastic neuronal tumors of brain.
A multicentric neuronal tumor of brain with unique morphologic features is described. It is compared with four other markedly anaplastic brain tumors containing neoplastic neurons. Demonstration of axon processes, which must be carefully distinguished from other similarly stained structures, is essential for the recognition of such tumors. Recent experience suggests that anaplastic neuronal tumors are more frequent than is generally realized. It is suggested that axon stains should be more widely employed in the investigation of atypical or highly pleomorphic gliomas.
Adult↗
Edema of the cortical gray matter of the human cerebrum.
Five cases of brain tumor are reported in which an extracellular edema of the cerebral cortex was recognized in the form of lakes of eosinophilic, PAS positive material, presumably serum proteins, permeating among the neuronal and glial cells and processes. In three, the astrocytes and their processes were stained in replicate paraffin sections by a gold sublimate technique. The astrocytic changes were variable. Most often, the astrocytes changed from protoplasmic to fibrillary, with long deeply stained processes, the cells sometimes becoming large and distorted. Many became enlarged and smudgy, with short stubby processes. Only a few showed the fragmentation and disintegration of processes. Only a few showed the fragmentation and disintegration of processes which are regularly observed in edematous white matter. The neuronal processes remained essentially normal. In comparison with edema of white matter, edema of the cortex is rare, involves only small portions of tissue, and differs in its effects on astrocytes. The edema in both cortex and white matter is extracellular. We have not been able to recognize a specific intracellular form of edema, grossly or with light microscopic techniques.
Aged↗
Congenital ophthalmoplegia in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome). A clinicopathologic study and review of the literature.
Two patients with Goldenhar-Gorlin syndrome showed paralysis of one or more extraocular eye movements on neurologic examination. At autopsy, a third patient showed unilateral agenesis of trochlear and abducens nerves and corresponding brain stem nuclei. Congenital ophthalmoplegia is not infrequent in Goldenhar-Gorlin syndrome and may be due to hypoplasia or agenesis, or both, of extraocular muscles, extraocular nerves, and brain stem nuclei.
Abducens Nerve↗
Clinical and pathological study of 24 cases of gliosarcoma.
The authors review the clinical and pathological features of 24 patients with gliosarcoma. The study revealed the following findings. Gliosarcoma occurs more frequently than is indicated in the literature, and in our series was present in 8% of all cases of glioblastoma multiforme. The presenting features are not significantly different from those of glioblastoma multiforme. The lesion often presents itself at surgery as a firm, well circumscribed mass within the temporal lobe, and at surgery it is commonly mistaken for a meningioma. There is an increased likelihood of metastasis compared to that of glioblastoma. The prognosis is no worse than that of glioblastoma, in spite of the addition of sarcomatous elements.
Adult↗
Congenital facial neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).
Four patients with clinical features of Goldenhar-Gorlin syndrome who showed facial paralysis on clinical examination are presented. The fourth case died following surgery for cleft lip. Autopsy revealed hypoplasia of the right facial nerve in its intracranial segment, with small right facial nucleus in the brain stem. Nosological aspects of the Goldenhar-Gorlin syndrome are discussed. Peripheral facial paralysis, as a part of this syndrome, is reviewed in the light of clinical and pathological findings and in its relationship to cardiac anomalies. It is suggested that Goldenhar-Gorlin syndrome is a part of a so-called cardiofacial syndrome.
Abnormalities, Multiple↗
The occurrence and characteristics of non-myelinated neuromas within central nervous tissue.
Three cases are described in which neuromas composed of non-myelinated axons were present within central nervous tissues in areas of tissue destruction, together with neuromas of peripheral myelinated axons. The non-myelinated neuromas were larger than the myelinated, but contained very much fewer Schwann cells and less connective tissue fibers. It is suggested that they took origin from heterotopic non-myelinated peripheral nerves, just as the myelinated neuromas are thought to take origin from heterotopic myelinated peripheral nerves. The non-myelinated neuromas are very much less common than the myelinated neuromas, and the inference may be drawn that their nerves of origin may be very much less common, a malformational rarity. Because of their rarity, and the very limited proliferation of Schwann cells which follow their injury, these non-myelinated perivascular nerves are not likely to provide the Schwann cells which produce the regenerated peripheral myelin about some denuded but perserved central axons in myltiple sclerosis. These may take origin from multipotential primitive reticular cells within the central nervous tissues, as is consistent with the thesis perviously offered that Schwann cells are mesenchymal in character. It may also be inferred that any neurogenic control of cerebral circulation would be limited to an effect on the larger, extracerebral vessels in the subarachnoid space.
Aged↗
Unilateral arhinencephaly in goldenhar-gorlin syndrome.
The post-mortem examination of the brain of a 2 1/2-year-old girl with clinical featutes of oculo-auriculo-vertebral dysplasia and hemifacial microsomia (Goldenhar-Gorlin syndrom) revealed a unilateral absence of the olfactory foramina of the lamina cribrosa of the ethmoid bone and ipsilateral absence of olfactory bulb and tract. Other cerebral abnormalities were also present. In this report, unilateral arhinencephaly in this disorder has been documented for the first time and an attempt has been made to correlate various nosological, clinical and pathological aspects of the case with previously reported instances of arhinencephaly. It is concluded that unilateral arhinencephaly occurs in a variety of cranial and facial abnormalities and is not specific for any particular syndrome.
Abnormalities, Multiple↗
Congenital trigeminal neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).
A 2 1/2 year old child with clinical features of Goldenhar-Gorlin syndrome showed diminished pinprick sensation over the right half of the face. After surgery for the cleft lip, the child died. Neuropathological investigations showed agenesis of the right trigeminal nerve and hypoplasia of the right trigeminal brain-stem nuclei. Nosological aspects of the Goldenhar-Gorlin syndrome and previously reported cases of congenital trigeminal anaesthesia in this disorder are discussed. It is suggested that the hypoplasia of the trigeminal nerve is responsible for the diminished facial sensation seen in some patients with this craniofacial syndrome.
Abnormalities, Multiple↗