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Biomedical subjects

D Soffer

Publications and source records attributed to D Soffer.

At least 109 records · Page 6Linked to original sources

Visual evoked potentials in experimental allergic encephalomyelitis.

We have compared the clinical signs, brain pathology and visually evoked responses (VEP) of guinea pigs with experimental allergic encephalomyelitis (EAE). Animals immunized with myelin basic protein had a milder disease, both from the clinical and histological points of views, compared to those immunized with crude white matter extract. However, VEP findings were quite similar in both groups. The VEP of the majority of animals from both groups showed changes before or at the same time that neurological signs appeared. Electrophysiological responses were usually characterized by abnormal wave shapes and prolonged latencies. Recovery of the VEP usually preceded the recovery from clinical signs. In contrast, the severity and incidence of brain tissue pathology was not correlated to either clinical signs or VEP changes. Possible explanations of the electrophysiological, clinical and histopathological changes and their time-course are discussed.

Animals↗

Intracranial meningiomas following low-dose irradiation to the head.

Earlier reports have suggested that low-dose ionizing irradiation might be involved in induction of intracranial meningiomas. One of the problems in evaluating irradiation-induced neoplasms is the belief that these tumors have no distinguishing features to indicate their etiology. In an attempt to identify such features in meningiomas following irradiation, a group of 42 post-irradiation meningiomas (PIM's) has been compared with a group of 84 non-PIM control meningiomas. These 42 PIM's included all the intracranial meningiomas diagnosed at the Hadassah University Hospital during the years 1952 to 1981 in individuals treated in childhood with low-dose x-ray therapy for tinea capitis. Although the individual PIM does not seem to differ from a "spontaneous" meningioma, this study indicates that PIM's as a group have distinct characteristics, namely, their location at the site of maximal irradiation, and features suggesting rapid growth and aggressive biological behavior. There was a significantly higher number of calvarial tumors (p less than 0.001), a high proportion of multiple meningiomas, a higher recurrence rate following apparent complete excision (p less than 0.02), and an increased number of histologically malignant meningiomas (p less than 0.01). The demonstration of features that distinguish PIM's from meningiomas of other etiology supports the suggestion that low-dose ionizing irradiation was involved in the pathogenesis of these tumors.

Female↗

Massive hemorrhagic necrosis of the spinal cord in metastatic cord compression.

A fusiform longitudinal hemorrhagic necrosis of the spinal cord is described in a patient with spinal cord compression caused by metastases from an adenocarcinoma of the prostate. The lesion extended from the D3 down to L2 level with maximal involvement of the D7-D8 segments. The shape, location, and distribution of the lesion and its prominent hemorrhagic component corresponded to Jellinger's category of cylindrical liquefaction necrosis, probably due to venous obstruction. The multiple thrombi found in epidural veins supported such a pathogenic mechanism, which although extremely rare has been reported previously in five cases.

Adenocarcinoma↗

Solitary dural plasmacytoma with conspicuous cytoplasmic inclusions.

A solitary intracranial plasmacytoma originated from the left parietal meninges of a 67-year-old woman; the patient had a four-month history of generalized seizures. Immunoelectrophoresis, performed nine months after surgery, revealed a monoclonal IgG band in the cerebrospinal fluid (CSF) but not in the blood. A peculiar feature of the tumor cells was the presence of abundant hyaline, eosinophilic cytoplasmic inclusions. Ultrastructurally, these inclusions consisted of electron-dense floccular material within distended cisterns of endoplasmic reticulum (ER). These ultrastructural features resemble "thesaurocytes" originally described by Paraskevas et al. in IgA myeloma. Tumors composed mainly of such inclusion-bearing cells are extremely rare. Only a fraction of the tumor cells stained positively for IgG with the immunoperoxidase technique; most inclusion-bearing cells did not stain. It is therefore unlikely that the electron-dense material is stored immunoglobulins.

Cytoplasm↗

Alpha-fetoprotein suppresses experimental allergic encephalomyelitis.

Experimental allergic encephalomyelitis (EAE), induced in guinea pigs by immunization with myelin basic protein (MBP) or whole CNS homogenate was successfully treated, as well as partially prevented, by daily administration of fetal alpha-fetoprotein (alpha FP). alpha FP which is produced in high quantities during pregnancy can inhibit both the cell-mediated immune response to MBP and the binding of MBP antibody to the antigen in vitro. It has a non-specific immunosuppressive effect on both the cellular and humoral responses. It is suggested that the ability of alpha FP to suppress an experimental autoimmune disease, as presented in this model of EAE, indicates that clinical remissions of human autoimmune disease during pregnancy may be attributed to the effect of this natural substance.

Animals↗

Cystic glioma of the brain stem with prolonged survival.

An unusual case of benign stem glioma with a 15-year survival is presented. In spite of 12 years of clinical quiescence, a focus of the tumor in the upper brain stem was found at necropsy. Morphologically, the tumor was a cystic pilocytic astrocytoma. The few previously reported similar cases in the same location were also associated with a favorable prognosis. It is suggested that, in analogy to the cystic cerebellar and cerebral astrocytomas, there is a group of cystic pilocytic astrocytomas of the brain stem with benign biological behavior.

Adolescent↗

Morphologic analysis of axo-glial membrane specializations in the demyelinated central nervous system.

Axo-glial membrane specializations, occurring in demyelinated spinal cord lesions in guinea pigs with chronic, relapsing experimental allergic encephalitis (EAE) and a case of multiple sclerosis (MS) between naked axons and scarring astrocytes, have been analyzed in transverse and longitudinal sections by transmission electron microscopy. The most common axo-glial specialization was of the desmosome-type and was plaque-like and punctate. It was not related to the region of subaxolemmal densification at the nodes of Ranvier. Synapse-like and gap junctions were also seen, but rarely, between naked axons and astrocytes in chronic EAE. In MS, only desmosome-like specializations were encountered between axons and glia and they were less extensive than in the guinea pig material. Surprisingly, similar chronically demyelinated lesions in the optic nerves of guinea pigs, failed to display axo-glial membrane specializations. It is speculated that their presence in spinal cord lesions is related to the proximity of gray matter and is of relevance to regeneration.

Animals↗

Central nervous system involvement in adult-onset Gaucher's disease.

Neuropathological findings are reported in an adult with Gaucher's disease. In this patient, Gaucher cells with characteristic storage material were found throughout the brain and also in the leptomeninges. They were confined to the perivascular space of small blood vessels and were commonly associated with intense perivascular, glio-mesodermal fibrillary reaction. Neuronal storage was not seen. Glucosylceramidase activities were severely deficient in both brain and liver and glucosylceramide was abnormally increased in the liver of this patient. The significance of the neuropathological changes is briefly discussed.

Age Factors↗

Rosenthal fibers formation in the central nervous system. Its relation to Alexander's disease.

Subpial and subependymal gliosis with abundant Rosenthal fiber (RF) formation are described in two patients. In one, the process appeared as a narrow band over the entire neuraxis and was not accompanied by neurologic manifestations. In the other, the changes were mostly restricted to the lower medulla and upper cervical cord and displayed invasive features. The glial proliferation with RF in the second patient was associated with an inflammatory component, dense collagen deposition, perivascular Schwannosis and demyelination. Involvement of some of the medullary nuclei and their tracts may explain the patient's vague bulbar symptomatology. The underlying cause which led to astrocytic proliferation with excessive RF in these two patients remains elusive. Possible factors contributing to this phenomenon are discussed in the context of Alexander's disease.

Aged↗

Cockayne syndrome: unusual neuropathological findings and review of the literature.

Two siblings with Cockayne syndrome (CS) are described and the literature on the subject is briefly reviewed. Of particular interest were the unusual neuropathological findings in 1 of the patients. These included microcephaly, white matter atrophy with patchy loss of myelinated fibers, calcifications of the basal ganglia, occasional ferrugination of cerebral and cerebellar neurons, and severe cerebellar degeneration. Findings not previously reported in CS were proliferation of extremely bizarre astrocytes, neurofibrillary tnagles, and pigmentation of the globus pallidus. We conclude that brain involvement in CS is a result of primary degeneration in the central nervous system rather than being secondary to angiopathy or normal pressure hydrocephalus, as previously suggested.

Adolescent↗

The anatomic basis of visual agnosia.

In a patient with associative visual agnosia without alexia, there was bilateral infarction in the distribution of the posterior cerebral arteries, with corticosubcortical lesions in both occipitotemporal regions, sparing the corpus callosum. Bilateral loss of visual-limbic connections may underlie associative visual agnosia, and bilateral lesions of the inferior longitudinal fasciculi may be the necessary and sufficient lesions for this syndrome. Alexia was absent in this case, perhaps because the corpus callosum was intact.

Agnosia↗

Localized cytomegalovirus encephalitis contiguous to metastatic nasopharyngeal carcinoma.

We report a case of focal infection of the brain with cytomegalovirus (CMV) in a 14-year-old boy with metastatic lymphoepithelioma (nasopharyngeal carcinoma). Cytomegalovirus-bearing subependymal glia were only seen in the fourth ventricle, in close proximity to tumor cells, these were associated with an intense inflammatory cell exudate. The latter was due to enterococcal meningitis. There was no evidence of systemic CMV infection, and the typical encephalitis with glial nodules seen in acquired forms of the disease was lacking. We postulate that the infection gained access to the brain either with the tumor cells, which happened to carry CMV genome, or with virus-carrying polymorphonuclear leukocytes migrating to the bacterial meningitis. It is also possible that the proliferating subependymal glia were unduly susceptible to CMV when the host defense mechanisms were compromised.

Carcinoma, Squamous Cell↗

Clinical features of the Guillain-Barré syndrome.

In a country-wide search for patients with Guillain-Barré syndrome (GBS) in Israel, 89 patients were found between 1969 and 1972 who met standardized diagnostic criteria. These cases, derived from a well defined population, were analyzed to provide a more accurate picture of the full range of clinical signs than is available from selected case series in the literature. Preceding illnesses, presenting symptoms, maximal neurological deficit, reflex changes, sensory deficit, cranial nerve, sphincter, respiratory, autonomic disturbances and spinal fluid changes were determined. Mortality was 5.6% which is lower than in many series. Alternatives to account for the apparent benignity of GBS in Israel were offered.

Adult↗

Disseminated Herpes simplex virus infection in ataxia-telangiectasia.

The clinical and pathological features are described in a child with ataxia-telangiectasia, complicated by fatal disseminated herpes simplex virus infection. Herpes simplex virus was isolated from the patient's blood, and the histopathological findings in the skin, liver and adrenals were consistent with herpes simplex virus infection. The patient had a combined immune deficiency state, as a part of the ataxia-telangiectasia syndrome. She had imparied cellular immune response to herpes simplex virus and developed no antibodies against the virus. To our knowledge, this is the first fatal case of disseminated herpes simplex virus infection in ataxiatelangiectasia.

Ataxia Telangiectasia↗

Epidemiology of Guillain-Barré syndrome.

From 1969 through 1972, a nationwide search for cases of Guillain-Barré syndrome (GBS) is Israel revealed 89 patients. The average annual age-adjusted incidence was 0.75 per 10(5) persons. Overall incidence of the syndrome was similar in Jewish groups of diverse ethnic backgrounds. Arabs had a lower overall incidence than Jews (0.46 per 10(5) persons), perhaps attributable to fewer Arabs at risk in older age groups. Peaks of incidence occurred among individuals over 60 and under 4 years of age when all cases were combined. No clear seasonal or geographic clustering of GBS was evident in Israel during the 4 years of this study. The incidence of GBS in the present study agrees with previous population-based estimates.

Adolescent↗

Aseptic meningitis: frequency among Israeli ethnic groups.

The relative frequency of aseptic meningoencephalitis (AME) was compared in populations of diverse origin, A countrywide search of Israel during 1969-1970 disclosed 1350 cases who fit strict diagnostic criteria. The average annual incidence was 21.6 per 100000 population. The total incidence was similar in Afro-Asian, Euro-American and Israeli Jewish groups but among Israeli Arabs, the incidence was apparently lower. Age-specific incidence showed a peak in infants under one year of age among Arabs and Afro-Asian Jews whereas Euro-Americans and Israeli Jews had a peak incidence at 5-9 years. Larger family size among Arabs and Afro-Asian Jews might account for the higher incidence in infants. Age-specific incidence may be a better index than total incidence of important differences in AME among various ethnic groups.

Adolescent↗