autosomaal dominante hereditaire aandoening
familiaire aandoening
hereditaire degeneratieve aandoening van centraal zenuwstelsel
prionziekte
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familiaire Alzheimer-achtige prionziekte (aandoening)
familiaire Alzheimer-achtige prionziekte
Familial Alzheimer-like prion disease
An exceedingly rare form of prion disease with characteristics of 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.
Id721219005
StatusPrimitive
Associated morphologyspongiforme degeneratie
Causative agentprion
Finding sitestructuur van encephalon
Pathological processinfectieus proces
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetA81.8
TermOverige gespecificeerde atypische virusinfecties van centraal zenuwstelsel
SNOMED CT to Orphanet simple map280397
SNOMED CT to ICD-10 extended map
TargetA81.8
RuleTRUE
AdviceALWAYS A81.8
CorrelationSNOMED CT source code to target map code correlation not specified