Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014070 (encephalomyelitis)
 13,017 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of an encephalomyelitis in a child with acute lymphoblastic leukemia is reported. The patient was a 5-year-old boy who developed seizures, progressive confusion, and coma after radiation and intrathecal methotrexate therapy. Computed tomography (CT) of the brain showed bilateral hypodensities in the posterior parietal and temporal regions. At autopsy, perivascular inflammation, microglial nodules without intranuclear viral inclusions, and bilateral necrosis of the temporoparietal and hippocampal regions were seen in the brain and spinal cord. Paraneoplastic encephalomyelitis is generally recognized in adult patients with underlying malignancy but, to our knowledge, has not been reported in children with leukemia. This report should alert the clinicians to an entity that must be included in the differential diagnosis of leukemic children with progressive neurologic disorder.
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PMID:Encephalomyelitis associated with leukemia in a child. 347 99

Mice bearing a methylcholanthrene-induced tumour were tested for their cell mediated reactivity to the experimental allergic encephalomyelitis (EAE) peptide of human myelin basic protein (MBP) in the leucocyte adherence inhibition (LAI) test. Tested over a range of peptide concentrations, peritoneal cells (PC) from tumour-bearing mice exhibited optimal adherence inhibition at 640 ng/ml; PC from normal and parasite-infected mice were unreactive. The EAE peptide also stimulated PC from tumour-bearing mice in the E-rosette augmentation (ERA) test and in the macrophage migration inhibition (MMI) test. MMI appeared to be the most sensitive assay, in that significant reaction at peptide concentrations well below those giving significant LAI and ERA. LAI reactivity to the peptide was detected 5 days after tumour transplantation, and continued to be detectable even with very large tumours. In vitro assays were confirmed by demonstration of EAE peptide recognition in vivo, in tumour-bearing and tumour-excised mice, using the delayed-type hypersensitivity reaction. The present experiments demonstrate an antigenic determinant in murine tumours, similar to the well-characterized EAE peptide of human MBP, and establish an animal model for study and characterization of common tumour-associated antigens.
Br J Cancer 1982 May
PMID:Cellular immunity to encephalitogenic peptide in tumour-bearing mice. 617 29

The anti-cancer drug Aclacinomycin A (ACM) was able to inhibit the humoral immune response of mice against sheep red blood cells. This could be demonstrated in the formation of antibody secreting cells (PFC) and serum antibody titers, when ACM was administered either together with the antigen or three days after antigen application. Cellular immunity was not affected by the drug. In two murine Graft-vs-Host (GvH) disease models leading to two different B cell dependent auto-immune diseases (immune complex glomerulonephritis and immune hemolytic anemia) a protective effect of ACM was observed when it was administered at the time of the graft. The application of ACM in the induction phase mitigated the development of glomerulonephritis and prevented animals from dying due to hemolytic anemia. Only a slight therapeutical effect was observed when ACM was given after the appearance of clinical symptoms. In a T cell induced auto-immune disease (experimental allergic encephalomyelitis (EAE], ACM had no discernible effect on the course of the disease. It seems that the therapeutic effects of ACM on GvH-diseases are mediated via suppression of B-lymphocytes.
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PMID:The influence of Aclacinomycin A on the immune response and on experimental immune disorders. 633 88

Paraneoplastic cerebellar degeneration accompanying gynecological or breast malignancies is frequently associated with an autoantibody response, termed "type I" or "anti-Yo" directed against cytoplasmic antigens of cerebellar Purkinje cells. The role of this antibody response in the pathogenesis of paraneoplastic cerebellar degeneration is unknown; however, it is also not known whether anti-Purkinje cell antibodies from the systemic circulation bind to target Purkinje cell antigens under the conditions of brain inflammation and blood-brain barrier disruption, which are frequently present at the onset of cerebellar symptoms. Inbred Lewis rats received intraperitoneal injections of type I or normal IgG in the setting of blood-brain barrier disruption induced by adoptive transfer of experimental allergic encephalomyelitis (EAE) and were killed after 24, 48, and 96 h. Brains of these animals were studied histologically for evidence of EAE and immunohistochemically for binding of human or endogenous rat IgG to target neurons. Rat IgG was detected around vessels and in Purkinje cells of all animals studied. Human IgG was detected around vessels of all animals. In animals examined 96h after receiving type I human IgG, human IgG was identified within processes of Purkinje cells and within occasional Purkinje cell bodies. Uptake of type I IgG by other cell types was not observed, and neuronal uptake of IgG was not seen in brains of animals receiving normal human IgG. Our data demonstrate that circulating type I IgG is internalized by cerebellar Purkinje cells in the setting of blood-brain barrier disruption and suggest a mechanism by which an antibody response directed against cytoplasmic antigens of Purkinje cells may reach target antigens at the onset of paraneoplastic cerebellar degeneration.
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PMID:Uptake of systemically administered human anticerebellar antibody by rat Purkinje cells following blood-brain barrier disruption. 761 Jul 65

Paraneoplastic cerebellar degeneration (PCD) is a rare complication of systemic cancer. PCD may present as a "pure", severe pan-cerebellar syndrome of subacute progression or be only one clinical feature in the setting of extensive CNS disease. The most characteristic form of "pure" PCD is associated with the presence of an anti-Purkinje cell antibody (AB), called anti-Yo, in patients with breast or ovarian cancer. The primary tumor is very often unknown when the cerebellar signs occur, and extensive investigations, including laparotomy or prolonged follow-up may be required to demonstrate its presence. More rarely, others AB than anti-Yo are discovered during PCD. Almost 50% of patients with "pure" PCD do not have circulating anti-neuronal AB. In the cases, the primary cancer is more often known and the clinical course of the cerebellar syndrome may be slower. Cerebellar degeneration may also occur during paraneoplastic encephalomyelitis. In this setting, the cerebellar signs which may be isolated at the onset, become associated with other signs of neuraxis involvement (limbic encephalitis, brainstem encephalitis, myelitis and particularly, subacute sensory neuronopathy) during the course of the disease. When a paraneoplastic encephalomyelitis is associated with a small cell lung cancer, an antineuronal AB called anti-Hu is frequently found. Finally PCD may be associated with the opsoclonus-myoclonus syndrome with the Lambert-Eaton syndrome.
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PMID:[Paraneoplastic cerebellar degeneration]. 786 50

Small cell lung cancer (SCLC) is known to express the HuD protein, the neuronal antigen homologous to Drosophila Elav and Sxl genes involved in neuronal and sex development. HuD is the target of an immune response including high titered antibodies causing paraneoplastic encephalomyelitis and sensory neuropathy. Because the p53 recessive oncogene is mutated and anti-p53 antibodies frequently occur in cancer patients, we wondered if the development of anti-HuD antibodies signaled the presence of HuD mutations in lung cancer. The HuD gene was mapped to chromosome region 1p using a human-mouse hybrid cell panel. We confirmed that 26 of 46 cancer (43 lung cancer and 3 mesothelioma) cell lines expressed HuD mRNA and that this expression, as well as protein expression by Western blot, correlated strongly with the SCLC neuroendocrine phenotype. Southern blot and single-strand conformation polymorphism analyses showed that HuD was not mutated in 78 lung cancers, including patients with the severe paraneoplastic syndrome. Northern blot analysis showed that lung cancer cell lines expressed two major mRNAs (4.3 and 4.0 kilobases) of HuD. We found the three previously described alternative spliced mRNA forms (HuDpro, HuD, and HuDmex). In addition, we also found HuD mRNA had an alternative splicing form in its 5'-coding region. This alternative splice introduced 87 base pairs of sequence and a termination codon resulting in a predicted small, truncated protein (11 amino acids) reminiscent of the male-specific truncated protein in the Sex-lethal (Sxl) gene of Drosophila. However, mRNAs encoding both full-length and truncated proteins were expressed in all SCLCs. These results show that the HuD gene is not mutated in lung cancer, including tumors from patients producing anti-HuD antibodies, but HuD expression is an independent marker or determinant of the neuroendocrine differentiation seen in SCLC.
Cancer Res 1994 Sep 15
PMID:Molecular analysis of the HuD gene encoding a paraneoplastic encephalomyelitis antigen in human lung cancer cell lines. 806 66

Solitary focal demyelination (SFD) in the brain is an uncommon and poorly understood disorder of uncertain etiology that may represent an intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis. In a few reported cases of SFD, the patient was briefly noted to have a nonneurological malignancy. We studied two patients who had solitary focal lesions in the brain. Utilizing magnetic resonance imaging and tissue biopsy, we found the characteristics of the brain lesions in these two patients to be those of SFD. In our combined experience over the past 10 years, we have encountered no similar brain lesions at our medical center. We found it remarkable that both of these patients also had malignancy outside of the nervous system. One had a seminoma, and the other a lymphoma. We conclude that some cases of SFD in the brain may occur as a paraneoplastic disorder associated with nonneurological malignancies.
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PMID:Solitary focal demyelination in the brain as a paraneoplastic disorder. 853 48

A neurologic paraneoplastic syndrome may be the first sign of an occult and treatable cancer. Some syndromes are associated with autoantibodies against neuronal antigens. Patients with cerebellar degeneration and ovarian or breast cancer have antibodies against 34 and 62 kilodalton (kDa) proteins in Purkinje cell cytoplasm: anti-Yo antibodies. Patients with encephalomyelitis or sensory neuronopathy and small cell lung cancer have antibodies against 35-40 kDa neuronal nuclear proteins: anti-Hu antibodies. Patients with opsoclonus-myoclonus and breast cancer have antibodies against 55 and 80 kDa neuronal nuclear proteins: anti-Ri antibodies. Patients with Lambert-Eaton myasthenic syndrome and small cell lung cancer have antibodies against voltage-gated calcium channels (anti-VGCC) in motor nerve terminals. The presence of anti-neuronal antibodies strongly indicates that a neurological syndrome is paraneoplastic, and often identify the site of an occult neoplasm. However, the absence of detectable antibodies does not rule out the presence of an underlying tumour.
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PMID:[Neurologic paraneoplastic syndromes and anti-neuronal antibodies]. 863 63

Paraneoplastic limbic encephalitis (LE) is considered a particular manifestation of paraneoplastic encephalomyelitis (PEM), a remote effect of cancer almost always associated with anti-neuronal antibodies (anti-Hu; also called ANNA 1) and small cell lung carcinoma (SCLC). In order to define the frequency of anti-Hu antibodies in LE with SCLC and to analyse possible clinical differences between patients with and without anti-Hu antibodies, the charts of 16 patients with LE and SCLC were reviewed. Eight patients (50%) had anti-Hu antibodies (anti-Hu+) whereas eight patients (50%) had no detectable anti-neuronal antibodies (anti-Hu-). The clinical and laboratory features of LE and time to diagnosis of SCLC were similar in the anti-Hu+ and anti-Hu- groups. Involvement of other areas of the nervous system compatible with the diagnosis of PEM was observed in seven (87.5%) patients of the anti-Hu+ group but in only one (12.5%) of the anti-Hu- group (P = 0.012). Five patients, including four of the anti-Hu- group, had a partial improvement of the LE after treatment of the SCLC. Another anti-Hu- patient improved spontaneously. Six patients of the anti-Hu+ group died from the neurological disorder, whereas in the anti-Hu- group the cause of death was progression of the SCLC in the three patients who died. The results of this study indicate that the absence of anti-Hu antibodies does not rule out the presence of an underlying SCLC in patients with a clinical diagnosis of LE. Patients with LE and SCLC who are without anti-Hu antibodies are less likely to develop PEM and seem to improve more often after treatment of the cancer than those who present anti-Hu antibodies.
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PMID:Limbic encephalitis and small cell lung cancer. Clinical and immunological features. 921 77

We report the case of a 62-year-old man affected by anti-Hu-associated paraneoplastic encephalomyelitis. The underlying tumor was a neuroendocrine cancer of the rectum expressing Hu antigen. The neurologic presentation was limited to moderate sensitive neuropathy associated with two complex partial seizures (dreamy state) without any further signs of limbic encephalopathy. A paraneoplastic etiology should be considered in patients with moderate symptomatology. Paraneoplastic encephalomyelitis with anti-Hu antibodies is not always associated with small-cell lung cancer.
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PMID:[Paraneoplastic encephalomyeloneuritis with anti-Hu antibodies and cancer of the rectum]. 929 26


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