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Query: UMLS:C0004134 (
ataxia
)
15,886
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The squirrel monkey (Saimiri sciureus) has been shown to be nearly as susceptible as the chimpanzee to experimentally induced Creutzfeldt-Jakob disease (CJD), and has been used extensively in diagnostic and pathogenetic studies. However, no information is available concerning the clinicopathological characteristics of different strains of human transmissible spongiform encephalopathy (TSE) in this species, in particular, strains of sporadic and variant CJD (
sCJD
and vCJD, respectively). Brain homogenates from patients with
sCJD
or vCJD were inoculated intracerebrally at dilutions of 10(-1) or 10(-3) into the left frontal cortex of squirrel monkeys. Animals were kept under continuous clinical surveillance during the preclinical and clinical phases of disease, and regularly underwent standardized behavioural testing. Brains from three animals in the
sCJD
and vCJD groups were examined histopathologically and immunohistochemically for the presence of pathognomonic misfolded protein (PrP(TSE)). Overall, incubation periods and durations of illness were slightly shorter in monkeys infected with
sCJD
than in those infected with vCJD, but the earliest signs of illness (
ataxia
and tremors) were the same in both groups. Clinical disease in the
sCJD
monkeys was somewhat more severe and of shorter duration. Post-mortem examinations revealed distinctive patterns of spongiform change and PrP(TSE) distribution in the brains of
sCJD
and vCJD animals, similar to those seen in humans except that amyloid plaques were not present. PrP(TSE) was uniformly absent from peripheral lymphoid tissues in both groups of animals. Human strains of
sCJD
and vCJD cause distinguishable clinicopathological features in the squirrel monkey that can provide a baseline for the evaluation of future therapeutic studies.
...
PMID:Clinical, neuropathological and immunohistochemical features of sporadic and variant forms of Creutzfeldt-Jakob disease in the squirrel monkey (Saimiri sciureus). 1725 88
We report the case of a 71-year-old woman with progressive dementia over the course of 4 years, characterized by prominent pyramidal signs and by the lack of
ataxia
and other cerebellar signs. Creutzfeldt-Jakob disease (CJD) was not suspected during the patient's life. Autopsy brain tissue showed severe spongiform encephalopathy with kuru-like, but not florid, plaques in neocortex and cerebellum. Massive synaptic diffuse and plaque-like PrP(Sc) deposition was found in the cerebral cortex, striatum, cerebellum and brainstem. Genetic analysis revealed no PRNP gene mutations and methionine/valine heterozygosity (MV) at codon 129. The pathogenic scrapie prion protein (PrP(Sc)) pattern detected by Western blot was Type 2. However, this pattern showed a single unglycosylated band in contrast to the doublet described for MV2 subtype of
sCJD
with kuru plaques. In summary, this is an autopsy case report of a particular presentation of MV2 subtype of
sCJD
.
...
PMID:A patient with MV2 subtype of sporadic Creutzfeldt-Jakob disease and atypical clinical presentation. 1913 Jul 39
