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complex van amyotrofische laterale sclerose, parkinsonisme en dementie (aandoening)
complex van amyotrofische laterale sclerose, parkinsonisme en dementie
ALS-PD-complex van Guam
lytico-bodig
Amyotrophic lateral sclerosis, parkinsonism, dementia complex
Amyotrophic lateral sclerosis, parkinsonism, dementia of Guam syndrome
Lytico Bodig disease
PDALS (parkinsonism, dementia, amyotrophic lateral sclerosis) complex
Guam disease
A rare neurodegenerative disease characterized by extrapyramidal symptoms (rigidity, tremor, bradykinesia) and dementia, typically beginning in the fifth or sixth decade of life and progressing to a vegetative state with pelvicrural flexion contractures within few years. Oculomotor signs, olfactory dysfunction, and autonomic disturbances may also be observed. Neuropathological hallmarks are frontotemporally accentuated cerebral atrophy, as well as neurofibrillary tangles and neuronal loss in a characteristic distribution in cortical and subcortical regions. The disease is endemic to the Pacific Island of Guam.
Id838276009
StatusPrimitive
Has interpretationgestoord
Interpretscognitieve functie
Clinical courseprogressief
Has interpretationlangzaam
InterpretsMovement
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetG12.2
TermZiekte van motorische neuronen
SNOMED CT to Orphanet simple map90020
SNOMED CT to ICD-10 extended map
TargetG12.2
RuleTRUE
AdviceALWAYS G12.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified