Avtor/Urednik		Jankovic, J
Naslov		Progressive supranuclear palsy
Prevedeni naslov		Progresivna supranuklearna paraliza
Tip		članek
Vir		Zdrav Vestn
Vol. in št.		Letnik 65, št. Suppl 3
Leto izdaje		1996
Obseg		str. III-25-30
Jezik		eng
Abstrakt		After idiopathic parkinsonism (Lewy body Parkinson's disease or PD), the parkinsonism plus syndromes (PPS) constitute the second largest group of parkinsonian disorders. Progressive supranuclear palsy (PSP) is the most frequent PPS accounting for about 8% of all parkinsonian patients in the Baylor College of Medicine Movement Disorders Clinic. First described by Steele, Richardson and Olszewski in 1964, PSP is now considered to be a distinct clinical-pathological entity. The diagnosis should be considered in any patient with progressive parkinsonism and disturbance of oclular motility, but more precise diagnostic criteria are currently being formulated in a multi-center NIH-sponsored clinical-pathological study. In a review of 126 PSP patients, we found unsteadiness of gait, frequent falling, monotonous speech, loss of eye contact, slowness of movement and of mentation, sloppy eating, and non-specific visual complaints to be the most typical presenting symptoms. Some PSP patients present with the syndrome of "pure akinesia". Supranuclear aphthalmoparesis, typically manifested by paralysis of downgaze, is the most distinguishing feature, although pathologically documented cases of PSP without ophthalmoparesis have been reported. Pseudobulbar palsy with dysarthria, dysphagia and "emotinal incontinence", axial rigidity and bradykinesia are also typical features. PSP occurs more often in men and its mean age at onset, 63 years, is about 10 years later than the typical onset of PD. The relentlessly progressive course leads to death, usually from aspiration, within 10 years of onset. (Abstract truncated at 2000 characters.)
Izvleček		Ob idiopatski Parkinsonovi bolezni so sindromi parkinsonizem plus (PPS) druga največja skupina parkinsonskih motenj. Najpogostejša med PPS je progresivna supranuklearna paraliza (PSO); ugotovljena je bila pri 8% vseh bolnikov, ki so jih zaradi parkinsonizma obravnavali v ekstrapiramidni kliniki na Baylor College of Medicine. PSP so l. 1964 prvi opisali Steele, Richardson in Olszewski; danes je to razpoznavna kliničnopatološka entiteta. Diferencialnodiagnostično prihaja v poštev pri vsakem bolniku s progresivnim parkinsonizmom in motnjami očesnih gibov, natančnejša diagnostična merila pa določa kliničnopatološka študija, ki pod okriljem ameriškega Nacionalnega inštituta za zdravje (NIH) nastaja v več centrih. Pri pregledu 126 bolnikov s PSP so bili najznačilnejši nasledji znaki: nestabilnost hoje, pogosti padci, monoton govor, izguba očesnega kontakta, upočasnitev gibov in misli, "nemarno" prehranjevanje in nespecifične vidne težave. Nekateri bolniki so imeli sindrom "čiste akineze". Najznačilnejša je supranuklearna oftalmopareza, zlasti nezmožnost pogleda navzdol; so pa znani primeri s patološko potrjeno diagnozo PSP brez pareze očesnih gibov. Med tipične znake štejemo tudi psevdobulbarno paralizo z dizartrijo, disfagijo in "emocialno inkontinenco", aksialno rigidnost in bradikinezo. PSP se pogosteje javlja pri moških. Povprečna starost ob nastopu bolezni je 63 let, torej 10 let več kot pri Parkinsonovi bolezni. Bolezen napreduje nezadržno. Bolnik umre po približno desetih letih, običajno zaradi aspiracije. PSP je sporadična idiopatska bolezen, dobro pa so bile dokazane tudi žilne (multiinfarktne) oblike. (Izvleček prekinjen pri 2000 znakih.)
Deskriptorji		SUPRANUCLEAR PALSY, PROGRESSIVE