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Query: UMLS:C0085584 (encephalopathy)
18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The subacute spongiform encephalopathies include scrapie of sheep, transmissible mink encephalopathy, and kuru and Creutzfeldt-Jakob disease of man. These diseases are caused by filterable infectious agents with unique physical properties. The usual sources of infection in nature are not completely known. Epidemiological evidence suggests that the agents may enter the body through breaks in the skin and mucous membranes. Experimental studies of scrapie after subcutaneous inoculation demonstrated early replication of the agent in lymphoid tissues and later appearance in other organs; as the amount of agent in the central nervous system (CNS) increased, it decreased in or disappeared from lymphoid tissues. In preliminary studies of kuru and Creutzfeldt-Jakob disease, the infectious agents were regularly recovered from the brains of clinically-ill patients and experimental animals but only occasionally from organs outside the CNS. It remains to be seen if early events in the pathogenesis of the two human diseases, before the appearance of clinical signs, are similar to those in scrapie.
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PMID:Pathogenesis of subacute spongiform encephalopathies. 12 25

The researches of DIENER and co-workers and those of the SEMANCIK'S group, have recently established that some plant diseases, such as potato spindle tuber, citrus exocortis disease and chrysanthemum stunt, are caused by a new class of pathogens, named viroids. These are the smallest known agents (they are smaller than viruses) having a molecular weight of ca 10(5) daltons, and composed of a highly structured RNA, rich in guanine-cytosine base pairs without a capsid. Little is known about the origin, replication model and pathogenic mechanism of viroids and until now only speculations are possible on these subjects. Some properties of the unknown agents of slow virus diseases (scrapie, Kuru, Creutzfeldt-Jakob disease and mink transmissible encephalopathy), suggest that these alterations in the central nervous system are caused by a sort of animal viroid.
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PMID:[Discovery of viroids and possible relationship with human and veterinary medicine]. 13 7

Two human disease, kuru and Creutzfeldt-Jakob disease, and two animal diseases, scrapie and mink encephalopathy, comprise the group designated the subacute spongiform encephalopathies. Studies on these four classic conditions have generated a new philosophy, new concepts, and new technology that provide a basis for the study of chronic diseases and latent infections of man and animals. These aspects are discussed more broadly and in variable detail in the references listed on the following page.
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PMID:Classic slow virus diseases. 14 98

The effects of rapid intracarotid injection of 20 to 100 mg of sodium amobarbital were studied in three patients with bilateral myoclonic status epilepticus due to Jakob-Creutzfeldt disease, sequelae to anoxic encephalopathy, and hepatic coma, respectively. In each instance, the drug produced prompt abatement of clonic jerks contralaterally and attenuation of electroencephalographic epileptiform discharges ipsilaterally. These results suggest that the cerebral cortex actively participates in the elaboration of certain types of bilateral myoclonus in human beings.
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PMID:Electrographic and clinical effects of intracarotid sodium amobarbital on bilateral myoclonic status epilepticus. 32 84

Experimental Creutzfeldt-Jakob disease was serially transmitted from guinea pigs to Syrian hamsters with 100% incidence, morbidity, and mortality. All animals developed a subacute spongiform virus encephalopathy with neuronal destruction and concomitant astrocytic changes. In the first passage three different clinical syndromes were recorded, each with widely variant incubation times; these results suggested there may be different strains of the Creutzfeldt-Jakob agent, some of which may be partially separated when the agent is passaged from one species to another. Accumulations of neurofilaments in neuronal perikarya and processes and increased lipofuscin were suggestive of changes seen in senility and aging.
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PMID:Interspecies transmission of Creutzfeldt-Jakob disease to Syrian hamsters with reference to clinical syndromes and strains of agent. 35 55

Recent studies suggest that some cases of familial Alzheimer's disease may be associated with a transmissible dementia. Animal experiments show that presymptomatic carriers of "slow virus" agents can transmit disease. Because of these findings, we have extended the precautions previously delineated to include those at risk of acquiring transmissible dementia, specifically, to the descendants of those affected with familial Alzheimer's disease or familial Creutzfeldt-Jakob's disease. Blood donation from such persons may pose a danger, because transmissible spongioform encephalopathy has been passed from animal to animal by blood serum and by the WBC layer of frozen whole blood.
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PMID:Precautions in familial transmissible dementia: including familial Alzheimer's disease. 36 3

A 51-year-old man who died of Creutzfeldt-Jakob disease (CJD), had transient dyskinesias with intention myoclonus and exaggerated startle reaction in early life. This may suggest a link between myoclonic encephalopathy of infants and CJD, and an incubation period of more than 40 years of the transmissible agent of CJD.
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PMID:Creutzfeldt-Jakob disease. Infection of infancy or childhood? 37 67

This is a report on three cases of subacute spongioid encephalopathy (Jakob-Creutzfeldt syndrome) where an extended cerebral process had been diagnosed clinically. However, a final and correct diagnosis could not be established until after the postmortem examination. The patients were 1 woman and 2 men; the duration of disease was between 5 weeks and 2 1/2 years. In 2 of the patients, initial pseudoneurasthenic complaints were followed, in the further course of disease, by manifestations of an organic psychosyndrome accompanied by neurological and, moreparticularly, coordination disorders. The neuropathological conditions manifested themselves especially in the frontal and occipital cortices as spongioid states, astroglial changes, ganglion-cell failures and pathobioses.
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PMID:[Space-occupying cerebral process as a clinical misdiagnosis in subacute spongioid encephalopathy (Jakob-Creutzfeldt syndrome). Clinical-morphological report on 3 cases]. 77 28

Some aspects of the epidemiology of Creutzfeldt-Jakob disease in England and Wales in the decade 1964-73 were studied with the object of detecting evidence of natural transmission of this slow virus encephalopathy. Some geographical clustering and possibility of contact between cases was found.
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PMID:Epidemiology of Creutzfeldt-Jakob disease in England and Wales. 109 99

Intracerebral and intraperitoneal inoculation of two guinea pigs with biopsy material from a patient with Creutzfeldt-Jakob disease produced a similar fatal encephalopathy characterized by status spongiosus 422 and 512 days after inoculation. Serial transmission of this disease from guinea pig to guinea pig was achieved in subsequent passages.
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PMID:Transmission of Creutzfeldt-Jakob disease from man to the guinea pig. 110 89


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