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. |
| Id | 838276009 | Status | Primitive |
DHD Diagnosis thesaurus reference set |
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | Target | G12.2 | Term | Ziekte van motorische neuronen |
|
SNOMED CT to Orphanet simple map | 90020 |
SNOMED CT to ICD-10 extended map | Target | G12.2 | Rule | TRUE | Advice | ALWAYS G12.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | Correlation | SNOMED CT source code to target map code correlation not specified |
|
|