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Dementia is often associated but is relatively mild There are characteristic spongiform changes, as in SSE Brain tissue from patients with this disease, when inoculated into chimpanzees, has produced a spongiform encephalopathy (Masters et al) Molecular genetic studies of affected members demonstrate a mutation of the prion protein gene This syndrome should be considered as a small familial subset of SSE, of slowly progressive type Fatal Familial Insomnia This is another very rare familial disease; it is characterized by intractable insomnia, sympathetic overactivity, and dementia, leading to death in 7 to 15 months (see also page 340) The pathologic changes, consisting of neuronal loss and gliosis, are found mainly in the medial thalamic nuclei Studies of a few families have shown a mutation of the prion protein gene, and brain material was found to contain a protease-resistant form of the gene Transmission of the disease by inoculation of infected brain material has not been accomplished (Medori et al) Kuru This disease, which occurs exclusively among the Fore linguistic group of natives of the New Guinea highlands, was the rst slow infection due to a nonconventional transmissible agent to be documented in human beings Clinically the disease takes the form of an afebrile, progressive cerebellar ataxia, with abnormalities of extraocular movements, weakness progressing to immobility, incontinence in the late stages, and death within 3 to 6 months of onset In some ways it is similar to the ataxic (Brownell-Oppenheimer) variant of Creutzfeldt-Jakob disease The remarkable epidemiologic and pathologic similarities between kuru and scrapie were pointed out by Hadlow (1959), who suggested that it might be possible to transmit kuru to subhuman primates This was accomplished in 1966 by Gajdusek and coworkers; inoculation of chimpanzees with brain material from affected humans produced a kuru-like syndrome in chimpanzees after a latency of 18 to 36 months Since then the disease has been transmitted from one chimpanzee to another and to other primates by using both neural and nonneural tissues The pioneering work in this eld led to the awarding of a Nobel Prize Histologically there is a nonin ammatory loss of neurons and spongiform change throughout the brain, but predominantly in the cerebellar cortex, with astroglial proliferation and PAS-positive stellate plaques of amyloid-like material ( kuru plaques ) The transmissible agent has not been visualized, however Kuru has gradually disappeared, apparently because of the cessation of ritual cannibalism by which the disease had been transmitted In this ritual, infected brain tissue was ingested and rubbed over the body of the victim s kin (women and young children of either sex), permitting absorption of the infective agent through conjunctivae, mucous membranes, and abrasions in the skin. barcode generator excel 2013 Barcode Add-In for Word & Excel Download and Installation
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ADAMS H, MILLER D: Herpes simplex encephalitis: A clinical and pathological analysis of twenty-two cases Postgrad Med J 49:393, 1973 AICARDI J, GOUTHIERES F, ARSENIO-NUNES HL, LEBON P: Acute measles encephalitis in children with immunosuppression Pediatrics 55:232, 1977 ANDERSON NE, WILLOUGHBY EW: Chronic meningitis without predisposing illness A review of 83 cases Q J Med 63:283, 1987 ANDERSON NE, WILLOUGHBY EW, SYNEK BK: Leptomeningeal and brain biopsy in chronic meningitis Aust N Z J Med 26:703, 1995 ANLAR B, SAATCI I, KOSE, et al: MRI nding in subacture sclerosing panencephalitis Neurology 47:1278, 1996 ASNIS DS, CONETTA R, TEIXEIRA AA, et al: The West Nile virus outbreak of 1999 in New York: The Flushing Hospital experience Clin Infect Dis 30:413, 2000 how to generate barcode in asp.net c#, ean 128 excel font, crystal reports qr code generator, crystal reports code 39, crystal reports qr code generator free, qr code c# open source barcode in excel 2003 erstellen Get Barcode Software - Microsoft Store
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