Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A brief description is given of neurofibrillary changes of the paired helical filament type in a variety of chronic neurological diseases. These include subacute sclerosing panencephalitis, lead encephalopathy, tuberous sclerosis, Down syndrome, Hallervorden-Spatz disease, and lipofuscinosis. In these conditions, with the exception of Hallervorden-Spatz disease neurofibrillary changes were previously recognized but paired helical filaments were identified only in some cases. Moreover, in the present series, the age of patients at death was often younger than in previously recorded cases.
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PMID:Alzheimer neurofibrillary tangles in diseases other than senile and presenile dementia. 15

Diseases of the central nervous system (CNS) occurring during treatment of acute lymphoblastic leukemia (ALL) may be of leukemic or nonleukemic origin. Well known examples for CNS disease of nonleukemic origin are somnolence following prophylactic CNS irradiation, methotrexate-induced encephalopathy and acute infections caused by bacteria, viruses and toxoplasma gondii. Less known is the fact that also subacute CNS infections may occur in patients undergoing cytostatic therapy. Progressive multifocal leukoencephalopathy and subacute sclerosing panencephalitis (SSPE) are examples of this category of disease. Up to now 11 well documented cases of SSPE were reported occurring during treatment of ALL. Main clinical features were disorders of behaviour, consciousness and speach, seizures, paresis and inappropriate secretion of ADH. Several authors were able to demonstrate a deficiency of cellular immunity in patients with SSPE. In some cases this deficiency was consistent with reduced reactivity of T-lymphocytes against measles antigen only. The presence of inhibiting factors may be responsible for this phenomenon. Other authors found a normal or increased function of cellular immunity in SSPE; In hamsters occurrence of SSPE is induced by the simultaneous injection of hamster-adapted SSPE virus and antihamster lymphocyte serum. We, therefore, conclude that also in humans SSPE appearing during treatment of ALL is due to immunosuppression.
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PMID:[Non-leukemic disease of the central nervous system in children with acute lymphoblastic leukemia. III. Subacute sclerosing panencephalitis (author's transl)]. 36 90

A girl developed subacute sclerosing panencephalitis (SSPE). Eight years earlier she had had measles infection contracted shortly after cytotoxic treatment and radiotherapy for a spinal neuroblastoma. The case illustrates that typical SSPE, like immunosuppressive measles encephalopathy, can arise after drug-induced immunosuppression, and supports the view that these diseases probably represent opposite ends of a spectrum induced by measles virus infection in an individual with some form of immunological deficiency.
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PMID:Subacute sclerosing panencephalitis after drug-induced immunosuppression. 50 19

A child with acute lymphoblastic leukaemia, being treated in the UKALL II Trial, had while in remission an attack of measles and made a normal recovery. Four months later she developed an acute encephalopathy and died within two weeks. The brain showed mild inflammatory features and widespread inclusion bodies in neurones and glial cells. Immunofluorescence proved an infection with measles virus. Similar cases have been called SSPE; reasons are given for preferring the term "measles inclusion-body encephalitis".
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PMID:Measles inclusion-body encephalitis in a child with treated acute lymphoblastic leukaemia. 78 1

Cultures of a mouse cell line (PAM) were treated with 71 multiple sclerosis (MS) and 45 non-MS samples. Of the cultures treated with MS material, 80 percent (58) showed a reduction in cell yield (compared to untreated controls) of at least 20 percent by the third passage after inoculation. The MS samples were from 40 MS cases, and a total of 36 cases yielded at least one positive sample. The agent responsible for the decrease was not limited to nervous tissue, but was also found in serum, cerebrospinal fluid, spleen, kidney, and lymph node of MS patients. Positive samples were present at every stage of the disease. None of the non-MS samples yielded cell counts significantly different from untreated controls. The non-MS category included 12 samples from healthy individuals, 13 assorted non-central nervous system disease samples, and the following central nervous system disease samples: six subacute sclerosing panencephalitis, three Huntington's chorea, two Parkinsonism, six amyotrophic lateral sclerosis, one stroke, one encephalopathy, and one epilepsy. Brain homogenates from mice inoculated with MS tissues elicited the decrease, whereas brain homogenates from mice inoculated with non-MS samples did not.
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PMID:Multiple sclerosis-induced reduction in the yield of a mouse cell line. 112 Jun 12

Two cases of N-acetylaspartic aciduria in siblings are described and compared to the 18 cases already reported. The disease should be considered in childhood when a syndrome of severe encephalopathy with macrocephaly, blindness caused by optic atrophy and diffuse leucodystrophy on CT scan occurs. Urinary organic acids gas chromatography confirms the diagnosis. It is probably inherited as an autosomal recessive trait. Aspartoacylase activity deficiency has been reported and this assay could possibly be used for prenatal diagnosis. Pathogenesis is not clearly understood but N-acetylaspartic acid (NAA) seems to be essential for central nervous system myelination. Clinical and anatomic features of N-acetylaspartic aciduria are very similar to Van Bogaert-Bertrand disease (cerebral spongy degeneration or Canavan disease) but heterogeneity of this disease cannot excluded.
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PMID:[N-acetylaspartic aciduria. Clinical, biological and physiopathological study]. 192 28

This case report is about an eleven year old boy with new developed symptoms of a cellular immundeficiency and a positive HIV-serology 33 months after a CNS-leukemia relapse. After 18 weeks a progredient neurological symptomatology is beginning with motor, cognitive and behavioral disturbances and a brain atrophy in the CT-scan. These cerebral manifestations are explainable as an encephalopathy both through HIV and after CNS-leukemia. A SSPE has been excluded. CT, EEG, Evoked Potentials do not show differential diagnostic pathognomonic findings regarding both diseases. The CSF findings hint at a persistent virus infection compatibel with the postulated slow virus pathogenesis of the AIDS-Encephalopathy. We conclude, that in this case an etiological diagnoses is only possible through histological brain examination and through demonstration of HIV or HIV-antigen in brain tissue respectively. AZT, which is reported to be effective against the cerebral AIDS-manifestations could not be applicated because of the existing pancytopenia.
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PMID:[AIDS encephalopathy in childhood or the late sequela of central nervous system leukemia?]. 316 63

The neurodegenerative diseases of infancy and childhood include disorders in which there is progressive loss of neurological function due to structural abnormalities of the central nervous system. Well over six hundred disorders, many of which are rarely seen, can be included in this category. Yet, the conditions represent collectively over one-fourth of all admissions to pediatric neurology services. Five-year samples of admission characteristics of 1218 patients from two medical centers over twenty-two years permit an estimate of the frequency of the neurodegenerative diseases. The six most-encountered diagnoses, in declining order, were: subacute sclerosing panencephalitis; neuronal ceroid lipofuscinosis; tuberous sclerosis with degeneration; West disease, or idiopathic degenerative encephalopathy associated with infantile spasms; Werdnig-Hoffmann disease, and hereditary spastic paraplegia. A classification is offered grouping the neurodegenerative disorders into five major categories: polioencephalopathies, leukoencephalopathies, corencephalopathies, spinocerebellopathies, and diffuse encephalopathies. Disorders in each subgroup may be either genetic or nongenetic. Neurodegenerative diseases have multiple causes, including metabolic, viral, immunopathic, environmental, and epileptogenic. The cause of many remains unknown.
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PMID:Neurodegenerative diseases of infancy and childhood. 630 58

Generalized and focal periodic EEG patterns were studied in 62 patients. Generalized periodic suppression bursts and generalized periodic slow-wave complexes (GPSC) occurred in patients under anesthesia or drug intoxication, and with anoxic/metabolic encephalopathies. When these conditions were excluded, GPSC indicated the diagnosis of subacute sclerosing panencephalitis or other encephalitides. Suppression bursts in comatose patients after cardiorespiratory arrest indicate an unfavorable outcome. Generalized periodic triphasic waves occurred only in patients with metabolic-anoxic encephalopathies. Generalized repetitive sharp transients were observed in patients with anoxic encephalopathy. Periodic lateralized epileptiform discharges (26 cases) were associated with clinical seizures in 24 cases and were noted almost exclusively in destructive hemispherical lesions. Periodicity represents a profound disruption of normal electrophysiological rhythms and indicates either structural or functional involvement of cortical and subcortical structures.
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PMID:Clinical significance of periodic EEG patterns. 676 64

Subacute sclerosing panencephalitis (SSPE) developed in a patient in whom natural measles infection was anteceded by immunization with measles immune serum globulin (ISG). This observation prompted experimental studies of the role of antibody in viral persistence. When Balb/c mice were infected with the hamster neurotropic measles virus, acute encephalopathy was fatal in 80% of the animals. When measles antibody was administered 3 days after virus inoculation, the acute disease was abolished and subacute encephalitis had a 30% mortality. The subacute disease was characterized by the presence of neuronal viral antigen, meningitis, and encephalitis. Induction of viral persistence was therefore a consequence of antibody transfer during viral infection. Caution is advised in human prophylaxis with immune globulin.
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PMID:Subacute sclerosing panencephalitis after passive immunization and natural measles infection: role of antibody in persistence of measles virus. 719 61


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