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The idea that viral infections may lead to chronic disease, especially of the nervous system, had been entertained since the 1920s, but only in relatively recent years was it rmly established The following indirect and direct evidence supported this view: (1) the demonstration of a slowly progressive nonin ammatory degeneration of nigral neurons long after an attack of encephalitis lethargica; (2) the nding of inclusion bodies in cases of subacute and chronic sclerosing encephalitis; (3) the discovery of chronic neurologic disease in sheep caused by a conventional RNA virus (visna) it was in relation to this disease in sheep that Sigurdsson rst used the term slow infection to describe the long incubation periods during which the animals appeared well; and (4) the demonstration by electron microscopy of viral particles in the lesions of multifocal leukoencephalopathy and, later, isolation of the virus from the lesions The suggestion that the late onset of progressive weakness after poliomyelitis ( postpolio syndrome ) might represent a slow infection has never been veri ed Claims have also been made by numerous groups for a viral causation of multiple sclerosis, amyotrophic lateral sclerosis, and other degenerative diseases, but the evidence is questionable The slow infections of the nervous system due to conventional viruses include subacute sclerosing panencephalitis (measles), progressive rubella panencephalitis, progressive multifocal leukoencephalopathy (JC virus), and visna in sheep These slowly progressive diseases, except for progressive multifocal leukoencephalopathy (PML), are decidedly rare They are caused by conventional viruses and are not to be confused with a group of chronic neurologic diseases that also are sometimes referred to as slow infections but are due instead to nonconventional transmissible agents, now called prions These are accorded a separate section later in this chapter Subacute Sclerosing Panencephalitis (SSPE) This disease was rst described by Dawson in 1934 under the title inclusion body encephalitis and extensively studied by Van Bogaert, who renamed it subacute sclerosing panencephalitis It is now recognized to be the result of chronic measles infection Never a common disease, the condition occurred until recently at a rate of about one case per million children per year With the introduction and widespread use of measles vaccine, it has practically disappeared in the United States Along with the eradication of polio, this has been one of the important advances in preventative neuropediatrics Subacute sclerosing panencephalitis affects children and adolescents for the most part, rarely appearing beyond the age of 10 years Typically there is a history of primary measles infection at a very early age, often before 2 years, followed by a 6- to 8-year asymptomatic period The illness evolves in several stages Initially there is a decline in pro ciency at school, temper outbursts and other changes in personality, dif culty with language, and loss of interest in usual activities These soon give way to a severe and progressive intellectual deterioration in association with focal or. birt pdf 417 Eclipse BIRT PDF417 Barcode Maker add-in makes PDF417 ...
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