autosomaal dominante hereditaire aandoening	degeneratieve aandoening van hersenen	dementie	hereditaire degeneratieve aandoening van centraal zenuwstelsel
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'protein kinase cAMP-dependent type I regulatory subunit beta'-gerelateerde neurodegeneratieve dementie met intermediaire filamenten (aandoening)
	'protein kinase cAMP-dependent type I regulatory subunit beta'-gerelateerde neurodegeneratieve dementie met intermediaire filamenten
	PRKAR1B-gerelateerde neurodegeneratieve dementie met intermediaire filamenten
	PRKAR1B-related neurodegenerative dementia with intermediate filaments
	Protein kinase cAMP-dependent type I regulatory subunit beta-related neurodegenerative dementia with intermediate filaments
	A rare, genetic neurodegenerative disease characterized by dementia and mild parkinsonism with poor levodopa response. Presenting clinical manifestations are memory problems, short attention span, disorientation, language impairment, rigidity, bradykinesia, postural instability and behavioral changes, including apathy, anxiety and delusions.

Id	774069007
Status	Primitive

Associated morphology	degeneratieve afwijking
Finding site	structuur van encephalon

Has interpretation	gestoord
Interprets	cognitieve functie

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	G31.8
Term	Overige gespecificeerde degeneratieve ziekten van zenuwstelsel

SNOMED CT to Orphanet simple map

412066

SNOMED CT to ICD-10 extended map

Target	G31.8
Rule	TRUE
Advice	ALWAYS G31.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified