Iatrogenic Creutzfeldt-Jakob disease

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Iatrogenic Creutzfeldt-Jakob disease

Synonyms: Iatrogenic MCJ | iCJD

A rare acquired human prion disease characterized by progressive invariably fatal neurodegeneration resulting from accidental transmission of CJD prions in the course of medical procedures or treatments (treatment with human pituitary growth hormone or gonadotrophin human dura mater or corneal graft exposure to contaminated neurosurgical instruments). Patients present rapidly progressive cognitive impairment as well as myoclonus visual or cerebellar problems pyramidal or extrapyramidal features and/or akinetic mutism. EEG examination may show characteristic generalized periodic sharp wave complexes. Neuropathologic analysis reveals spongiform change neuronal loss and gliosis and deposition of abnormal prion protein.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version November 2023

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Advocacy Organizations

Creutzfeldt – Jakob Foundation Israel

Our mission is to advance the research for the treatment and prevention of Creutzfeldt-Jakob disease (CJD), a neurodegenerative, rare, and fatal disease, that causes rapid deterioration and certain death (in a couple of months). We aim to accelerate research by founding a database and biobank of our families and genetic patients for researchers worldwide in a collaborative way.

Clinical Trials

For a list of clinical trials in this disease area, please click here.