نتایج جستجو برای: creutzfeldt

تعداد نتایج: 3390  

Journal: :Communicable diseases intelligence quarterly report 2006
Genevieve M Klug Alison Boyd Victoria Lewis Samantha L Douglass Rebecca Argent James S Lee Colin L Masters Steven J Collins

Australia-wide prospective surveillance of human transmissible spongiform encephalopathies (TSEs) has been conducted by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) since October 1993. In addition, the Registry retrospectively ascertained TSE cases within Australia from 1970. Referrals of all suspect cases of human prion diseases or TSEs are investigated by the ANCJDR and...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1978
E E Manuelidis E J Gorgacz L Manuelidis

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 res...

Journal: :Internal medicine 1998
Y Itoh T Amano T Shimizu J Hashimoto A Kubo Y Fukuuchi

A 50-year-old woman presented with progressive visual disturbance, ataxia, and dementia. The cerebral cortex became atrophic, as the disease progressed, and electroencephalography showed periodic synchronous discharges. The patient's prion gene revealed a point mutation (232Met to Arg), and a diagnosis of Creutzfeldt-Jakob disease was made. Iomazenil single-photon emission computed tomography (...

2012
Jacqueline Mikol Jean-Philippe Deslys Wen-Quan Zou Wiangzhu Xiao Paul Brown Herbert Budka Françoise Goutieres

We report a case of iatrogenic Creutzfeldt-Jakob disease(iCJD) in a child with a neonatal growth hormone (GH) deficiency that was treated with native human growth hormone (hGH) between the ages of 9 months and 7 years. Three years after the end of treatment a progressive neurological syndrome consistent with Creutzfeldt-Jakob disease (CJD) developed, leading to death within a year, at age 11. N...

Journal: :Journal of Alzheimer's disease : JAD 2009
Susanne T de Bot H P H Kremer Dennis Dooijes Marcel M Verbeek

In sporadic Alzheimer's disease (AD), cerebrospinal fluid (CSF) analysis is becoming increasingly relevant to establish an early diagnosis. We present a case of familial AD due to a presenilin-1 mutation in which CSF studies suggested appropriate DNA diagnostics. A 38 year old Dutch man presented with dementia, spastic paraparesis, and frontal executive function impairments, mimicking familial ...

Journal: :Journal of neurology, neurosurgery, and psychiatry 1985
L Cartier S Gálvez D C Gajdusek

A family cluster of the ataxic form of Creutzfeldt-Jakob disease with one probable and two autopsy proven cases that occurred in a single generation between 1974 and 1982 is reported. The clinical characteristics of the cases are closely similar to those of kuru patients, with a fair correlation between the prominent truncal ataxia and the intense devastation of the cerebellar cortex most marke...

Journal: :Emerging Infectious Diseases 2017

2015
Peter Rudge Zane Jaunmuktane Peter Adlard Nina Bjurstrom Diana Caine Jessica Lowe Penny Norsworthy Holger Hummerich Ron Druyeh Jonathan D. F. Wadsworth Sebastian Brandner Harpreet Hyare Simon Mead John Collinge

Patients with iatrogenic Creutzfeldt-Jakob disease due to administration of cadaver-sourced growth hormone during childhood are still being seen in the UK 30 years after cessation of this treatment. Of the 77 patients who have developed iatrogenic Creutzfeldt-Jakob disease, 56 have been genotyped. There has been a marked change in genotype profile at polymorphic codon 129 of the prion protein g...

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