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)

Viljuisk encephalomyelitis (VE) is a severe neurologic disease characterized by slow progressive dementia', oligobradykinesia, low spastic paraparesis and speech disturbances. It develops in persons of 20-50 years old. VE occurs in a small region of middle Viljui, but for last years the focus has considerably expanded. Etiology of VE is still obscure. 194 families with VE patients were examined. The data obtained contradict the hypothesis of simple recessive inheritance of VE. The value of the heredity coefficient, calculated on the basis of the Falconer - Edwards model, is 22-29% for relatives of the first relation degree. It suggests the existence of individual hereditary determined susceptibility to VE. 14 secondary cases were observed in affected families among adopted relatives (adopted children, husbands and wives of patients) with a rate exceeding random possible frequency. These observations have been evaluated as an evidence of horizontal transmission of the disease from patients with chronic forms to healthy persons. Obligatory condition for the transmission consists in a long-term contact (as a rule, more than one year). According to all known characteristics VE should be refferred to slow infections.
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PMID:[Correlation of heredity and environmental factors in the etiology of Vilyui encephalomyelitis. I. Patient frequency in families]. 47 88

An elderly man presented with signs and symptoms indicating a rapidly progressive central and peripheral nervous system disease, which led to death within 3 months. The pathologic picture was that of a "paraneoplastic" encephalomyelitis and neuritis, but no cancer could be found. Supratentorial predilection for the limbic structures correlated well with an observed limbic dementia. Arguments favoring a direct toxic or metabolic effect of cancer as a cause for this syndrome was less convincing in view of this report.
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PMID:"Encephalomyeloneuritis" in the absence of cancer. 117 94

Viliuisk encephalomyelitis (VE), a progressive neurological disorder with a fatal outcome usually in several months to 6 yrs after disease onset, is seen only among the Iakut people of Siberia. The acute meningoencephalitic phase of the disease is followed by progressive dementia, rigidity and spastic tetraparesis. The disease is characterized by multiple micronecrotic foci with marked inflammatory reactions and gliosis in the grey matter. The acute febrile onset, with cerebrospinal fluid (CSF) pleocytosis and increased protein in the CSF, the epidemiology and the inflammatory neuropathology suggest the disease is infectious. Studies on household spread indicate an incubation time of up to several years. Viliuisk encephalomyelitis was restricted to an ethically distinct group of Iakut people of the Middle Viliui region, but in recent decades, with migration from this region, it has been spreading into previously unaffected Iakut populations. The occurrence of multiple VE cases in households and introduction of the disease by migrants into new populations indicate horizontal transmission in a setting of long intimate contact.
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PMID:Viliuisk encephalomyelitis in the Iakut people of Siberia. 139 13

The presence of tumor necrosis factor alpha (TNF alpha)/cachectin was investigated in 180 paired cerebrospinal fluid (CSF) and serum samples from patients with neurological diseases, and in five paired CSF and serum samples of Macaca cynomolgus monkeys with acute monophasic experimental autoimmune encephalomyelitis (AMEAE). TNF alpha was never detected in human CSF, even when an extensive demyelination was documented (active multiple sclerosis, acquired immunodeficiency syndrome (AIDS) dementia complex). Only one Macaca with AMEAE had detectable levels of TNF alpha in CSF but not in serum, suggesting an intrathecal synthesis of this cytokine in AMEAE.
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PMID:Tumor necrosis factor alpha (TNF alpha) and neurological diseases. Failure in detecting TNF alpha in the cerebrospinal fluid from patients with multiple sclerosis, AIDS dementia complex, and brain tumours. 272 41

Human T-lymphotropic virus type I (HTLV-I), the etiological agent of adult T-cell leukemia/lymphoma, also appears to be the cause of tropical spastic paraparesis, a chronic myelopathy reported in several different regions of the world. The prevalence of antibodies to HTLV-I in patients with chronic neurodegenerative disorders other than tropical spastic paraparesis and in patients with some muscle inflammatory disorders has been investigated. IgG antibodies to HTLV-I were measured in the sera and/or cerebrospinal fluid from 82 Guamanian patients with amyotrophic lateral sclerosis and parkinsonism-dementia, 164 Guamanian normal controls, 10 patients with kuru from the Eastern Highlands of Papua New Guinea, 4 patients with Viliuisk encephalomyelitis from the Iakut region of eastern Siberia, 45 Italian patients with multiple sclerosis, and 56 patients with polymyositis (49 from the United States and 7 from Jamaica). As determined by enzyme-linked immunosorbent assay, Western immunoblot, and gelatin particle agglutination techniques, serological evidence of HTLV-I infection was found in 1 patient with amyotrophic lateral sclerosis and 1 control subject from Guam, and in 1 patient from the United States and all 7 Jamaican patients with polymyositis. Except for the high seropositivity rate among the group of Jamaican patients with polymyositis, our data indicate that HTLV-I is an unlikely causative agent in the spectrum of the neurological diseases examined. The seropositivity of the 7 Jamaican patients with polymyositis requires further study.
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PMID:Seroprevalence of antibodies to HTLV-I in patients with chronic neurological disorders other than tropical spastic paraparesis. 289 13

Borna disease represents a unique model of a virus-induced immunological disease of the brain. Naturally occurring in horses and sheep, the mechanisms of pathogenesis have been studied in experimental animals, namely in the rat. Many investigations have revealed that the infection of the natural hosts principally follows the same pathogenic pathways as observed in rats, leading to a severe encephalomyelitis. This affliction of the central nervous system results in severe neurological disorders that again, are fully comparable in laboratory animals to those in the natural and the different experimental hosts. In addition, alterations have been reported which are also based on the infection of the brain and do not result in the classical encephalitic clinical picture but rather in alterations of behavior. However, to all of our knowledge, the various clinical pictures of Borna disease are not caused by the infecting virus itself but rather by the hosts immune response towards it, i.e. by a virus-induced cell-mediated immunopathological reaction. The importance of virus-specific CD4+ T cells as exemplified by a cultured T cell line and of CD8+ T cells as shown by immunomodulatory substances and specific antibody treatment in vivo for the pathogenesis of acute Borna disease will be elucidated here. In addition, evidence will be provided that virus-specific CD8+ T cells are also responsible for the dramatic brain atrophy in the chronic phase of the disease in rats. Therefore, Borna disease not only lends itself exquisitely well to the study of the pathogenesis of an immunopathological disease of the brain but also represents one of the few models for immune-mediated tissue destruction that eventually leads to brain atrophy and clinically to dementia.
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PMID:Pathogenesis of Borna disease. 821 98

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

Viliuisk encephalomyelitis (VE) is an unique neurological disease occurring in the Iakut (Sakha) people of Siberia. Evolution of the disease follows one of three broad clinical forms: subacute, slowly progressive or chronic. Death occurs within 3 to 6 months in subacute cases and within 6 years in the slowly progressive cases. Chronic cases lack a subacute phase but show a slowly progressive dementia associated with bradykinesia, dysarthria and spastic paraparesis that stabilizes late in the disease process. In subacute and slowly progressive cases, focal necrotizing encephalomyelitis is seen at necropsy. Chronic cases show multifocal areas of lysis with a gliotic margin, predominantly within grey matter, lacking associated chronic inflammatory changes seen in the other forms of the disease. Epidemiological studies are consistent with a disease of low-grade communicability, but laboratory studies have so far failed to reveal an infectious organism. The spectrum of neuropathological changes are reviewed in this examination of 11 cases. Although the aetiology of VE remains obscure, further studies are warranted since it may represent a novel disease process.
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PMID:Viliuisk encephalomyelitis--review of the spectrum of pathological changes. 922 30

We report a 76-year-old man who developed blurred vision and dementia. He was apparently well until April 4, 1990 (70-year-old at that time) when he had a sudden onset of bilateral loss of vision. Corrected vision was 0.1 (right) and 0.09 (left). He was admitted to the ophthalmology service of our hospital on April 9, 1990, and neurological consultation was asked on April 11. Neurologic examination revealed alert and oriented man without dementia. Higher cerebral functions were intact. He had bilateral large visual field defects with loss of vision; he was only able to count the digit number with his right eye and to recognize hand movement with his left eye. Otherwise neurologic examination was unremarkable. General physical examination was also unremarkable; he had no hypertension. Cranial CT scan was normal on April 11; lumber spinal fluid contained 1 cell/microliter, 63 mg/dl of sugar, and 97 mg/dl of protein; myelin basic protein was detected, however, oligoclonal bands were absent. He was treated with methylprednisolone pulse therapy and oral steroid, however, no improvement was noted in his vision. He started to show gaze paresis to left, ideomotor apraxia, agnosia of the body, and dementia. Cranial CT scan on June 11 revealed a low density area in the deep left parietal white matter facing the trigonal area of the lateral ventricle. He was discharged on July 2, 1990. Hasegawa dementia scale was 2/32.5 upon discharge. In the subsequent course, he showed improvement in his mental capacity and Hasegawa dementia scale was 22.5/32.5 in 1991, however, no improvement was noted in his vision. In 1994, he started to show mental decline in that he became disoriented, and showed delusional ideation of self persecution and depersonalization with occasional confusional state. He also showed unsteady gait. Cranial MRI on February 13, 1996 revealed a T2-high signal intensity lesion on each side of the parietal deep white matter more on the left and another T2-high signal intensity lesion in the left pons as well as in the right thalamus. He complained of right hypochondrial pain and was admitted to another hospital on April 22, 1996. He was markedly confused and demented. He continued to show bilateral loss of vision, but no motor palsy was noted. Cranial CT scan on April 23, 1996 revealed diffuse cortical atrophy and ventricular dilatation in addition to the low density areas in both parietal deep white matter. He developed jaundice in the middle of May. Abdominal CT scan revealed multiple low-to iso-density areas in the liver and marked iso-to high-density swelling of the right kidney. The patient expired on June 9th, 1996. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had had a carcinomatous limbic encephalitis with optic neuropathy and a choleduct carcinoma. Other opinions entertained included acute disseminated encephalomyelitis with optic neuritis, and granulomatous angiitis of the central nervous system. Some participants thought the primary site of the carcinoma was the right kidney with metastasis to the liver. Post mortem examination revealed a mixed type carcinoma in the right kidney with liver metastases. Neuropathologic examination revealed an incomplete softening in the optic chiasm and the left optic nerve, and in the left parieto-occipital areas. (The right hemisphere was frozen for future biochemical assay.) One of the adjacent cortical arteries had an organized thrombus. Other arteries and arterioles also showed sclerotic changes. Some of the leptomeningeal arteries were positive for Congored staining as well as for beta-amyloid immunostaining. Many senile plaques were seen diffusely in the cerebral cortex and neurofibrillary tangles were seen in the CA1 area and the parahippocampal gylus. No cellular infiltrations or demyelinated foci were seen. The neuropathologic features were consistent with circulatory disturbance based on the amyloid angiopa
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PMID:[A 76-year-old man with loss of vision and dementia]. 928 74

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


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