Iatrogenic Creutzfeldt-Jakob disease
Synonyms: Iatrogenic MCJ · iCJD
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Iatrogenic Creutzfeldt-Jakob disease | Iatrogenic MCJ · iCJD |
| fr | Maladie de Creutzfeldt-Jakob iatrogène | MCJ iatrogène · i-MCJ |
| de | Creutzfeldt-Jakob-Krankheit, iatrogene | Iatrogene CJD · iCJD |
| es | Enfermedad de CreutzfeldtJakob iatrogénica | iCJD |
| it | Malattia iatrogena di Creutzfeldt-Jakob | MCJ iatrogena · iCJD |
| nl | Iatrogene ziekte van Creutzfeldt-Jakob | Iatrogene CJD · iCJD |
| pt | Doença Creutzfeldt-Jakob iatrogénica | |
| pl | Choroba Creutzfeldta i Jakoba jatrogenna | = en |
| cs | Iatrogenní Creutzfeldtova-Jakobova nemoc | Iatrogenní Creutzfeldt-Jakobova nemoc |
| uk | Ятрогенна хвороба Крейтцфельдта-Якоба | Ятрогенний MCJ · iCJD |
| zh | 医源性克罗伊茨费尔特-雅各布病 | 医源性MCJ |
| tr | İatrojenik Creutzfeldt-Jakob hastalığı | iCJD · İyatrojenik MCJ |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | A81.0 | NTBT | specific | validated |
| ICD-11 | 8E01.0 | E | specific | validated |
| MONDO | 0034976 | E | validated | |
| UMLS | C2349757 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:454700Acquired Creutzfeldt-Jakob disease
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 516 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/576379
- This record as JSON: /data/d/576379.json