Iatrogenic transmission of Creutzfeldt-Jakob disease

Weller, Roy O.

doi:10.1017/s0033291700010965

Cited by 28 publications

(5 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…6 In addition to these pathogenic mutations, PRNP was shown to harbor the methionine (Met)/valine (Val) polymorphism at codon 129 in the general population as well as in familial and sporadic CJD.7s8…”

mentioning

confidence: 99%

Codon 219 lys allele of PRNP is not found in sporadic Creutzfeldt‐Jakob disease

Shibuya

Higuchi

Shin

et al. 1998

Annals of Neurology

148

121

View full text Add to dashboard Cite

The polymorphism at codon 219 of the prion protein gene (PRNP) was found in the general Japanese population with 6% allele frequency. Herein, we examined 85 cases of sporadic Creutzfeldt-Jakob disease (CJD) for the codon 219 polymorphism. The codon 219Glu/Lys heterozygous polymorphism was not found in these CJD cases. In addition, we examined 43 patients with dementia of non-CJD origin, and 4 were found to have the codon 219Glu/Lys heterozygous polymorphism with a similar allele frequency as in the general population. Thus, the codon 219Glu/Lys heterozygous polymorphism might be uniquely excluded from sporadic CJD.

show abstract

mentioning

confidence: 99%

Codon 219 lys allele of PRNP is not found in sporadic Creutzfeldt‐Jakob disease

Shibuya

Higuchi

Shin

et al. 1998

Annals of Neurology

148

121

View full text Add to dashboard Cite

show abstract

“…These include cadaveric pituitary-sourced growth and gonadotrophin hormone injections, coroneal transplantation, intracerebral electrode use, cadaveric dura mater graft use and other neurosurgical procedures. " 19 In 1959 the first clinical use of human growth hormone was described and there were 1908 people in Britain and approximately 10 000 people in the USA who had received growth hormone treatment.7 "The number of pituitary glands used to produce a batch of growth hormone was up to 20 000, with remnants of one batch sometimes being retained and added to future batches;2`0 thus there was the potential to infect many growth hormone vials from only one infected gland.…”

Section: Discussionmentioning

confidence: 99%

A further British case of growth hormone induced Creutzfeldt-Jakob disease.

Ellis

Katifi

Weller

1992

Journal of Neurology, Neurosurgery & Psychiatry

View full text Add to dashboard Cite

Transmission of Creutzfeldt-Jakob disease (CJD) from cadaveric growth hormone injections had previously been reported in 7 cases, including one from Britain. As a result, the treatment was abandoned in 1985 and superceded by safer recombinant DNA growth hormone injections. Recent reports now record the number of cases worldwide as 23, but with the incubation period being measured in years, new cases of CJD can still present. We give a detailed report of one of the recent cluster of British cases and aim to highlight the problem to clinicians who may encounter further patients with cadaveric growth hormone induced CJD. At that stage his general examination was normal. In the neurological examination spontaneous myoclonic jerks were seen in all 4 limbs and he had a marked startle response to loud noises. Bilaterally there were hand and foot primitive grasp reflexes. He was withdrawn but initially orientated in time and place; his speech demonstrated cerebellar dysarthria. In the cranial nerves, he was blind in the right eye, the left could only perceive light.

show abstract

“…however, contagious and case to case spread in humans has only occurred via rather unusual routes. Such 'iatrogenic CJD' cases have resulted from the use of inadequately sterilised intracerebral electrodes, dura mater and corneal grafting and from use of cadaveric pituitary-derived human growth hormone and gonado trophin [10],…”

Section: Introductionmentioning

confidence: 99%

Prion Diseases in Humans and Their Relevance to Other Neurodegenerative Diseases

Collinge¹,

Palmer²

1993

Dement Geriatr Cogn Disord

View full text Add to dashboard Cite

Molecular genetics has led to considerable advances in our understanding of the transmissible spongiform encephalopathies. The identification of pathogenic mutations in the prion protein gene has enabled a molecular reclassification of the familial forms of these diseases, which may now be referred to as inherited prion diseases. Prion diseases of both humans and animals are associated with deposition of an abnormal isoform of a host-encoded protein, the prion protein (PrP). Human prion diseases have inherited, sporadic and acquired forms. A considerable body of evidence now supports the idea that the transmissible agent in these diseases may be an abnormal isoform of the prion protein. The identification of pathogenic mutations in the PrP gene has enabled the identification of cases of inherited prion disease that would not have been recognised using existing clinical and pathological diagnostic criteria. Since marked clinical and neuropathological overlap between the different neurodegenerative disorders is well recognised, PrP gene analysis is of increasing importance in differential diagnosis. Frontal lobe dementia of non-Alzheimer type and Pick''s disease share a number of important clinical and pathological features with prion diseases, and could be considered as candidate prion diseases. However, we have not been able to demonstrate either PrP mutations or the presence of the disease-associated isoform of prion protein in several well-characterised families with these disorders.

show abstract

Iatrogenic transmission of Creutzfeldt-Jakob disease

Cited by 28 publications

References 26 publications

Codon 219 lys allele of PRNP is not found in sporadic Creutzfeldt‐Jakob disease

Codon 219 lys allele of PRNP is not found in sporadic Creutzfeldt‐Jakob disease

A further British case of growth hormone induced Creutzfeldt-Jakob disease.

Prion Diseases in Humans and Their Relevance to Other Neurodegenerative Diseases

Contact Info

Product

Resources

About