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 27 year-old patient developed a progressive neurological multisystem disorder. Initial signs were cerebellar ataxia and dementia, followed by rigidity and oculomotor dysfunction. Myoclonus was not present. MRI showed a marked atrophy of the spinal cord, the cerebellum, and mild (sub)cortical atrophy. CSF contained oligoclonal bands, but no anti-glutamic acid dehydrogenase antibodies. He died 33 months after onset of symptoms. Autopsy revealed widespread neuropathological alterations including perivascular lymphocytic cutting, neuronal cell loss, and micro/astrogliosis the distribution of which corresponded to the changes seen in MRI. The diagnosis of progressive encephalomyelitis with rigidity was pathohistologically confirmed. Brain samples were negative for neurotrophic viruses tested by polymerase chain reaction. A new variant of this rare disorder is described initially presenting with ataxia and dementia, but without myoclonus.
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PMID:A new variant of progressive encephalomyelitis with rigidity associated with cerebellar ataxia and dementia: correlation of MRI and histopathological changes. A case report. 917 49

An outbreak of avian encephalomyelitis (AE) in 7 broiler flocks, with signs of ataxia and paralysis is reported. The diagnosis was made by immunofluorescence, histopathology and virus isolation. The breeding flock had a temporary drop in egg production and reduced hatchability caused by late embryonic mortality. The breeding flock had not been vaccinated against AE. The problems were probably caused by vertical transmission of AE virus.
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PMID:[Ataxia and paralysis in meat chickens, as a result of an infection with avian encephalomyelitis virus in the breeding flock]. 964 55

To examine the role of tumor necrosis factor (TNF)-alpha in the pathogenesis of degenerative disorders of the central nervous system (CNS), transgenic mice were developed in which expression of murine TNF-alpha was targeted to astrocytes using a glial fibrillary acidic protein (GFAP)-TNF-alpha fusion gene. In two independent GFAP-TNFalpha transgenic lines (termed GT-8 or GT-2) adult (>4 months of age) animals developed a progressive ataxia (GT-8) or total paralysis affecting the lower body (GT-2). Symptomatic mice had prominent meningoencephalitis (GT-8) or encephalomyelitis (GT-2) in which large numbers of B cells and CD4+ and CD8+ T cells accumulated at predominantly perivascular sites. The majority of these lymphocytes displayed a memory cell phenotype (CD44high, CD62Llow, CD25-) and expressed an early activation marker (CD69). Parenchymal lesions contained mostly CD45+ high, MHC class II+, and Mac-1+ cells of the macrophage microglial lineage with lower numbers of neutrophils and few CD4+ and CD8+ T cells. Cerebral expression of the cellular adhesion molecules ICAM-1, VCAM-1, and MAdCAM as well as a number of alpha- and beta-chemokines was induced or upregulated and preceded the development of inflammation, suggesting an important signaling role for these molecules in the CNS leukocyte migration. Degenerative changes in the CNS of the GFAP-TNFalpha mice paralleled the development of the inflammatory lesions and included primary and secondary demyelination and neurodegeneration. Disease exacerbation with more extensive inflammatory lesions that contained activated cells of the macrophage/microglial lineage occurred in GFAP-TNFalpha mice with severe combined immune deficiency. Thus, persistent astrocyte expression of murine TNF-alpha in the CNS induces a late-onset chronic inflammatory encephalopathy in which macrophage/microglial cells but not lymphocytes play a central role in mediating injury.
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PMID:Late-onset chronic inflammatory encephalopathy in immune-competent and severe combined immune-deficient (SCID) mice with astrocyte-targeted expression of tumor necrosis factor. 973 27

The primary lesions of eastern equine encephalomyelitis (EEE) virus infection in the horse are limited to the brain and spinal cord. Intestinal lesions in addition to the changes in the central nervous system were found in a 6-month-old male Tennessee Walking Horse. One week prior to death, this colt was vaccinated for EEE virus, western equine encephalomyelitis virus, influenza virus, equine rhinopneumonitis virus, and tetanus. The clinical signs consisted of ataxia and rear-end weakness, with a body temperature of 102.8 F. Gross lesions consisted of yellowish discoloration, swelling, edema, and hemorrhage of the brain stem and dark discoloration of the gray matter of the spinal cord. Microscopic lesions in the small intestine were mainly in the muscular layer and consisted of multifocal areas of myonecrosis and lymphomonocytic infiltration with a few focal areas of mild fibrous connective tissue proliferation. Occasional focal mild perivascular lymphocytic infiltration was observed in the submucosa. Lesions in the brain and spinal cord consisted of widespread areas of perivascular lymphomonocytic cuffing, focal areas of necrosis, neutrophilic infiltration, hemorrhage, neuronal degeneration, and gliosis. Hepatic changes consisted of periportal lymphocytic infiltration and mild vacuolar degeneration of hepatocytes. EEE virus was isolated from the intestine and detected by DNA in situ hybridization.
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PMID:Intestinal lesions in a horse associated with eastern equine encephalomyelitis virus infection. 982 95

We report 10 children with the diagnosis of acute disseminated encephalomyelitis. Diagnosis was based on clinical and radiologic findings, and after acute encephalitis was excluded by negative culture and antibody results. The most common presenting symptom was ataxia, followed by optic neuropathy, cranial nerve palsy, convulsions, motor dysfunction, and loss of consciousness. Brain magnetic resonance imaging showing bilateral symmetrical hyper-intense lesions of the same age in brain stem, subcortical white matter, thalamus, basal ganglia, or cerebellum was the mainstay of the diagnosis. The presence of a preceding event (either an infection or vaccination) was present in 8 of 10 patients. Brain computed tomographic scans were abnormal in 3 of 10, and electroencephalogram was normal in all patients. High-dose corticosteroids were given to six patients, one received low-dose steroids, and the other three had symptomatic follow-up. Those who relapsed were mainly from the symptomatic follow-up group. Only one patient (the youngest) receiving high-dose methylprednisolone relapsed. Therefore, early high-dose steroid treatment seems to be the most effective treatment in acute disseminated encephalomyelitis and can prevent relapses.
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PMID:Acute disseminated encephalomyelitis in childhood: report of 10 cases. 1019 Feb 72

Two hundred and twelve patients with clinically evidenced encephalomyelitis disseminata (ED), hospitalized in a neurological hospital, were observed with regard to psychopathological characteristics and cognitive changes in conformity with ICD-10 diagnostic criteria. The basis of this investigation was a standardized psychiatric interview. The age of the patients averaged 47 years whereas the duration of the disease averaged 14.3 years. 83.5% of the patients had a disease history of more than 6 years. The medium range of EDSS scores was 5.95%, the BPRS 36.7%. In 5.2% of the patients the course of ED was primarily chronic-progressive while 48% suffered from the intermittent, incomplete-reversible form: 47.6% developed secondary chronic-progressive symptoms. 18 psychopathological symptoms could be identified, the main symptom was depressive mood (49%), followed by impairment of affective sensitivity (34.9%) and affective instability/incontinence (31.1%). The most prevalent diagnoses were dementia (23.1%), organic personality disorder (18.5%), mild cognitive impairment (9%), and depressive disorder (7.6%) Only 33.5% were psychopathologically unaffected. The duration of the disease in all demented patients exceeded 6 years. Patients with an organic personality disorder showed a marked increase in the later stages of their illness in contrast to patients suffering from depressive disorder. At the beginning of ED, a highly significant (p < 0.0001) impairment of vision was found in all psychiatric patients. Dementia patients and organic personality patients, on the other hand, showed an advanced degree of ataxia. Actually, there was a considerably lesser incidence of pareses in the non-psychopathological group whereas ataxia was significantly more prevalent in the three cognitively impaired ED-subgroups than in the control group. These findings set the stage for constructive discussions, taking due consideration of existing research results on ED with particular reference to the implications regarding future research as well as the clinical therapy of patients.
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PMID:Psychopathological changes and cognitive impairment in encephalomyelitis disseminata. 1036 56

An augmentation of experimental allergic encephalomyelitis (EAE) was observed when monoclonal antibody (mAb) to intercellular adhesion molecule 1 (ICAM-1) was administered after adoptive transfer. Clinical disease was more severe in the ICAM-1 specific mAb-treated EAE mice and included prominent ataxia compared to the PBS-treated controls or Theiler's murine encephalomyelitis virus (TMEV) infected mice treated with ICAM-1 specific mAb. Neuropathologic evaluation demonstrated a distinctly different distribution of lesions in the anti-ICAM-1-treated EAE mice which featured prominent demyelination and inflammation in the cerebellum, brainstem and cerebrum. These structures were minimally involved in the control mice and mAb treatment did not alter the neuropathology in TMEV-infected mice. These results indicate that anti-ICAM-1 can alter trafficking of lymphocytes and mononuclear cells in EAE but not TMEV-induced demyelinating disease.
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PMID:Contrasting effects of anti-adhesion molecule therapy in experimental allergic encephalomyelitis and Theiler's murine encephalomyelitis. 1040 64

Clinical and MRI appearances were reviewed in 25 cases of acute disseminated encephalomyelitis (ADEM) seen in a university hospital in South India. Specific viral infections and Semple antirabies vaccination together accounted for 56% of antecedent events. Clinical and MRI features were suggestive of diffuse/multifocal form in 15 patients. Of the 10 patients with clinically site restricted forms, two patients with dorsal myelitis and one patient with polyradiculitis had asymptomatic cerebral white matter lesions. MRI was essentially normal in all the four patients with acute ataxia following varicella infection. Of the 13 patients with multiple white matter lesions, lesions were asymmetrical in size and morphology in nine patients and symmetrical in four patients. Two of them had extensive destructive lesions and one patient had multiple discrete lesions. Lesions occurred at the corticomedullary junctions in seven patients. The distribution was subcortical and/or centrum semiovale in 10 patients. The regions affected include internal capsule(s), thalami, brainstem, cerebellar peduncles and cerebellum. No specific differences were observed in patients with ADEM following specific viral infections, Semple antirabies vaccination, and nonspecific upper respiratory tract infections. There was fairly good correlation between clinical and MRI findings in 81% of patients. Patients with incomplete recovery showed extensive and/or multifocal lesions.
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PMID:Acute disseminated encephalomyelitis: clinical and MRI study from South India. 1045 Jul 98

West Nile (WN) virus infection is a mosquito-borne flavivirosis endemic in Africa and Asia. Clinical disease is usually rare and mild and only in a few cases the infection causes encephalomyelitis in horses, fever and meningoencephalitis in man. We report here the clinical and pathological findings in an epidemic of the disease involving 14 horses from Tuscany, Italy. All cases were observed from August to October 1998. Affected horses showed ataxia, weakness paresis of the hindlimbs and, in 6 cases, there was paraparesis progressing to tetraplegia and recumbency within 2 to 9 days. Eight animals recovered without any important consequences. Serological investigations revealed positivity to WN virus in all the 14 horses and the agent was isolated from the cerebellum and spinal cord of an affected horse. Postmortem examination was carried out on 6 horses. The neuropathological pattern was that of a mild to moderate, nonsuppurative polioencephalomyelitis with constant involvement of the ventral horns of the thoracic and lumbar spinal cord, where focal gliosis and haemorrhage were also apparent in some cases. Differential diagnoses with other equine viral encephalomyelitides are discussed. Climatological and environmental characteristics of the geographic area in which the outbreaks occurred suggest the existence of suitable conditions for the development of the disease. This is the first report of WN virus equine encephalomyelitis in Italy.
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PMID:Clinical and neuropathological features of West Nile virus equine encephalomyelitis in Italy. 1066 82

Clinical abnormalities in multiple sclerosis (MS) have classically been considered to be caused by demyelination and/or axonal degeneration; the possibility of molecular changes in neurons, such as the deployment of abnormal repertoires of ion channels that would alter neuronal electrogenic properties, has not been considered. Sensory Neuron-Specific sodium channel SNS displays a depolarized voltage dependence, slower activation and inactivation kinetics, and more rapid recovery from inactivation than classical "fast" sodium channels. SNS is selectively expressed in spinal sensory and trigeminal ganglion neurons within the peripheral nervous system and is not expressed within the normal brain. Here we show that sodium channel SNS mRNA and protein, which are not present within the cerebellum of control mice, are expressed within cerebellar Purkinje cells in a mouse model of MS, chronic relapsing experimental allergic encephalomyelitis. We also demonstrate SNS mRNA and protein expression within Purkinje cells from tissue obtained postmortem from patients with MS, but not in control subjects with no neurological disease. These results demonstrate a change in sodium channel expression in neurons within the brain in an animal model of MS and in humans with MS and suggest that abnormal patterns of neuronal ion channel expression may contribute to clinical abnormalities such as ataxia in these disorders.
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PMID:Sensory neuron-specific sodium channel SNS is abnormally expressed in the brains of mice with experimental allergic encephalomyelitis and humans with multiple sclerosis. 1102 57


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