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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients are reported with a chronic progressive illness characterized by dementia, ataxia and spasticity. There were no myoclonic jerks and both had normal electroencephalograms (EEG). Pathological findings in three brain biopsies were those of viral meningoencephalitis with perivenous demyelination. Serological data in both patients indicated the presence of measles virus infection. Intracytoplasmic structures resembling measles virus nucleocapsids were found in the brain biopsy of one patient. Immunofluorescent staining showed antibody in the temporal lobe biopsy of both patients. It is suggested that these patients are examples of a chronic form of measles meningoencephalitis hitherto undescribed.
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PMID:An unusual form of measles meningoencephalitis. A report of two cases. 9 70

Progressive multifocal leukoencephalopathy (PML) developed in a 64-year-old woman who had undergone hemodialysis treatment due to chronic renal failure (CRF) for 6 yr. Her initial symptom was ataxia, and computed tomographic (CT) scan and magnetic resonance imaging (MRI) suggested a demyelinating disease of the cerebellum. Her cell-mediated immunity was impaired. At autopsy, the cerebellar disease was confirmed as PML by ultrastructural and immunohistochemical studies. Moreover, the JC type of papova virus infection was verified by Southern blot analysis.
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PMID:Cerebellar form of progressive multifocal leukoencephalopathy in a patient with chronic renal failure. 131 17

We report on a 19 month old female who has been retarded developmentally after a severe varicella infection contracted from her mother 4 months after delivery. Her titer of varicella-zoster virus (VZV) IgG antibody has been remarkably high for 4 years after the infection. Natural killer activity and the specific cellular immunity to VZV, as tested by delayed type hypersensitivity skin test and lymphocyte-proliferation assay, were impaired. She had an improvement of ataxia and then progressed developmentally after administration of an anti-viral agent. Administration of oral corticosteroids and methylprednisolone transiently decreased titers of VZV antibody and, contrary to previous reports, returned natural killer activity to normal levels. We suggest that this case may be a persistent VZV infection similar to congenital cytomegalovirus infection due to selective defects in cellular immunity including NK cells. Whether this specific deficient cellular immunity is genetically determined or secondary to the viral infection is speculative. This is the first known report of such a case.
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PMID:Persistently high antibody titers and deficient specific cellular immunity to varicella-zoster virus in a retarded patient after varicella infection. 132 33

Maedi-visna virus infection in a flock of sheep in Scotland was associated with respiratory disease, neurological disease, mastitis and lameness. The major clinical signs were dyspnoea (particularly on exercise), progressive fore- and hindlimb ataxia and balance defects, mammary induration and multilimb lameness, occasionally with enlarged carpal joints. Pathological examinations revealed lesions in the lungs, central nervous system, mammary glands and joints which were consistent with those induced by maedi-visna virus. The was no clinical or pathological evidence of concurrent sheep pulmonary adenomatosis, and pulmonary bacterial infections, when they occurred, were superimposed on the lesions due to maedi-visna virus.
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PMID:Clinicopathological investigation of primary, uncomplicated maedi-visna virus infection. 838 91

The triad of ataxia, areflexia and ophthalmoplegia was first described as a variant of the Guillain-Barre syndrome in 1932 by Collier. In 1956, Miller Fisher reported three patients with ataxia, areflexia, and ophthalmoplegia as a separate entity. Since then, 223 cases of Miller Fisher syndrome have been published. The male/female ratio is 2:1 with a mean age of 43.6 years at the onset of the disease. A viral infection preceded the neurological symptoms in 71.8% of cases with an average symptom-free interval of 10 days. First symptoms were diplopia (38.6%) or ataxia (20.6%). An areflexia was present in 81.6% of cases. Cranial nerves other than the oculomotor nerves were involved in 127 cases (56.9%): cranial nerves 7 (45.7%), 9 and 10 (39.9%), and 12 (13%) were involved. In 53 cases a tetraparesis occurred. An elevated protein value was present in 134 patients (64.4%); cerebrospinal fluid findings were normal in 56 patients. Eighteen patients showed a mild pleocytosis. Besides the cerebellar type of ataxia, initial disturbances of consciousness (n = 8), supranuclear oculomotor signs (n = 22), and pathology findings in electroencephalography (n = 38), computed tomography (n = 8), and magnetic resonance imaging (n = 2) were reported as evidence for a central nervous system involvement in the Miller Fisher syndrome. The prognosis of Miller Fisher syndrome was good--recovery occurred after a mean time period of 10.1 weeks. Residual symptoms were present in 74 cases (33.2%), and a recidivism of the Miller Fisher syndrome was reported in seven patients. Eight patients died. Of six patients with autopsy findings, four exhibited central nervous system lesions.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The Miller Fisher syndrome. Review of the literature. 153 3

Eight vaccinated dogs suddenly developed progressive ataxia, paresis or paralysis of short duration. A histopathological examination revealed a non-suppurative meningoencephalitis suggestive of a viral infection, and immunohistochemical examination confirmed the presence of canine distemper virus antigen in five of the dogs. Distemper had not been suspected from the clinical examination of the dogs.
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PMID:Canine distemper infection associated with acute nervous signs in dogs. 159 46

Postmortem examination of 21 patients showed a vacuolar myelopathy resembling that associated with the acquired immunodeficiency syndrome. Underlying diseases included six cases of leukemia or lymphoma, five of carcinoma, three of systemic lupus erythematosus, two of chronic lung disease, and one each of cadaveric renal transplant, cirrhosis, diabetes, hemophagocytic syndrome, and viral encephalitis. Fourteen patients were on long-term steroid therapy and 10 of these also had immunosuppressive chemotherapy. No patient had the acquired immunodeficiency syndrome, although one received blood transfusions in 1978. Signs and symptoms consistent with myelopathy included paraparesis in seven patients, ataxia in one, and bilateral extensor plantar reflexes in one. Microscopic examination showed vacuolation in spinal cord white matter primarily located in posterior and lateral columns. Lipid-laden macrophages and axonal changes were proportional to the severity of the vacuolation, which was severe in five patients, moderate in 10, and mild in six. Eight patients had coexistent viral diseases elsewhere in the central nervous system, but viral-associated antigens or genomic material was not found in regions of vacuolated spinal cord white matter. Although the etiology of these myelopathies is unknown, their association with immune suppression and coexistent viral infection of the central nervous system suggests that an opportunistic viral infection may be important.
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PMID:Idiopathic myelopathies with white matter vacuolation in non-acquired immunodeficiency syndrome patients. 186 65

Molecular epidemiology may help fill the gap between epidemiological and biological models for cancerogenesis, allowing useful comparisons between series of cases carrying different biological characteristics. The defective regulation of immune responses is probably the common basis of cancer origin in genetically determined immune deficiency (GDID). Lymphomas are the most common neoplasms, showing an extremely high incidence in early age, frequent unusual location (extra-nodal) and histology, and rapid progression and spread with little response to therapy. A high incidence of lymphoma is also found in acquired (AIDS) or iatrogenic (transplant recipients) immune defective patients. The emergence of a malignant clone may be linked to unregulated polyclonal B- or T-cell proliferation and to disturbances in chromosomal rearrangements and in clonal selection, which are unique features of the immune cells regulation. It is useful to compare patterns of malignancies observed in GDIDs and in chromosomal breakage syndromes (CBS). In ataxia-teleangectasia (AT), selective errors at sites of special recombination involved in immune cell rearrangements may account for both immune deficiency and frequent types of malignant transformation. Different cytogenetic alterations and different types of malignancies are more common in Bloom syndrome, and in other GDIDs unrelated to chromosomal fragility, possibly due to different regulatory impairments. Viral infection (EBV, HPV, CMV) is likely to be a factor in any of the above steps. Therefore, individual exposure to viral (or other environmental) agents may be related to frequent location (skin, ano-genital areas, digestive tracts) of nonlymphatic cancers both in some GDIDs and in acquired or iatrogenic immune deficiencies. Cells that are homozygous for GDID are not malignant themselves, but are more likely to undergo new mutations to malignancy, due both to disregulation of the immune system and to environmental agents.
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PMID:Molecular epidemiology of cancer in immune deficiency. 203 52

Twenty of 89 consecutive patients with the acquired immunodeficiency syndrome (AIDS) in whom autopsies were performed over a 3 1/2-year period had a vacuolar myelopathy that was most severe in the lateral and posterior columns of the thoracic cord. Light and electron microscopy showed that vacuoles were surrounded by a thin myelin sheath and appeared to arise from swelling within myelin sheaths. Signs and symptoms referable to the spinal-cord lesions, including paraparesis, often accompanied by spasticity or ataxia (or both), were present in all five patients with marked pathological changes, in five of seven patients with moderate changes, and in two of eight patients with mild changes. Fourteen patients were demented. The clinical presentation was sufficiently distinctive to provide a guide for antemortem diagnosis. Possible causes of the vacuolar changes include uncharacterized viral infection or a metabolic derangement related to selective nutritional deficiency.
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PMID:Vacuolar myelopathy pathologically resembling subacute combined degeneration in patients with the acquired immunodeficiency syndrome. 397 73

A central nervous system disease of mink occurred in three unrelated fur farms in Oregon in September, 1981. Only kits four to five months old were affected. Clinical signs consisted of posterior ataxia progressing to complete posterior paralysis with loss of motor control and sensation. Complete or partial recovery occurred in approximately 1.5 months in most mink. Microscopic lesions consisted of severe nonsuppurative meningoencephalitis and meningomyelitis with vacuolation of the white matter of the brain and spinal cord. Canine distemper virus infection and other recognized causes were ruled out on the basis of clinical signs, history, lesions, or laboratory findings. Experimental inoculations of mink with brain and spinal cord specimens from affected mink failed to reproduce the disease.
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PMID:Nonsuppurative meningoencephalomyelitis of unknown etiology in mink. 398 55


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