familiaire Alzheimer-achtige prionziekte (aandoening) | | familiaire Alzheimer-achtige prionziekte | | Familial Alzheimer-like prion disease | | Familial Alzheimer-like prion disease is an exceedingly rare form of prion disease characterized by the neuropathological features of Alzheimer disease including memory impairment and depression, related to abnormal prion protein (PrP) caused by a gene mutation in PRNP. Patients present with a prolonged, atypical course (absence of myoclonus or ataxia) unlike other forms of prion disease with severe neurofibrillary tangle pathology and high levels of cerebral amyloidosis. |
| Id | 721219005 | Status | Primitive |
DHD Diagnosis thesaurus reference set |
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | Target | A81.8 | Term | Overige gespecificeerde atypische virusinfecties van centraal zenuwstelsel |
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SNOMED CT to Orphanet simple map | 280397 |
SNOMED CT to ICD-10 extended map | Target | A81.8 | Rule | TRUE | Advice | ALWAYS A81.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | Correlation | SNOMED CT source code to target map code correlation not specified |
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