progressieve supranucleaire verlamming (aandoening)
progressieve supranucleaire verlamming
progressieve supranucleaire paralyse
Progressieve supranucleaire paralyse (PSP) is een aandoening die lijkt op de ziekte van Parkinson, echter zonder beverigheid. Kenmerken zijn stijfheid en verminderde beweeglijkheid, stoornissen in oogbewegingen en uiteindelijk dementie.
Progressive supranuclear palsy
PSP - progressive supranuclear palsy
A rare late-onset neurodegenerative disease with characteristics of supranuclear gaze palsy, postural instability, progressive rigidity, and mild dementia. Five clinical variants have been described with clinicopathological correlations, with Richardson's syndrome the most common clinical variant. The disease has neuropathological manifestations of neuronal loss, gliosis with astrocytic plaques and accumulation of tau-immunoreactive neurofibrillary tangles in specific brain areas. The differences in the rate and areas of accumulation of phosphorylated tau protein correlate with the five clinical variants. The disease is a 4R tauopathy composed of a preponderance of four-repeat (exon 10 positive) tau isoforms and a characteristic biochemical profile (doublet tau 64 and tau 69). The MAPT H1-clade specific sub-haplotype, H1c, is a risk factor for this disease.
Associated morphologydegeneratieve afwijking
Causative agentTau protein
Finding sitestructuur van cerebrum
Clinical courseprogressief
Has interpretationafwezig
InterpretsMovement observable
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TermProgressieve supranucleaire oftalmoplegie [Steele-Richardson-Olszewski]
SNOMED CT to Orphanet simple map683
SNOMED CT to ICD-10 extended map
AdviceALWAYS G23.1
CorrelationSNOMED CT source code to target map code correlation not specified