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Medicinski podmladak
2018, vol. 69, br. 2, str. 9-15
jezik rada: engleski
vrsta rada: pregledni članak
doi:10.5937/mp69-16325
Creative Commons License 4.0
Klinički spektar frontotemporalne demencije
Clinical Centre of Serbia, Neurology Clinic, Belgrade + University of Belgrade, Faculty of Medicine, Belgrade

e-adresa: goranamandic@yahoo.com

Sažetak

Uvod: Frontotemporalna demencija (FTD) se smatra drugom najčešćom degenerativnom formom demencije sa ranim početkom (DRP). Čini je spektar neurodegenerativnih poremećaja u kojem razlikujemo dva glavna podtipa: FTD sa inicijalnim i preovlađujućim ispadima na planu ponašanja (dezinhibicija, apatija, gubitak empatije, kompulsivno ponašanje..) (FTDbv); i jezičku varijantu - tipove prvenstveno progresivne afazije (PPA), u kojima preovlađuju ispadi u domenu jezičkih funkcija (teškoće u govoru, razumevanju, ponavljanju). Pacijenti sa sporadičnim i genetskim navedenim formama FTD mogu da razviju motorne poremećaje (pre, posle ili istovremeno sa bihevioralnim/kognitivnim poremećajima) u vidu bolesti motornog neurona ili atipičnog parkinsonizma - progresivne supranuklearne paralize i kortikobazalnog sindroma, što sve zajedno čini spektar FTD. Frontotemporalna demencija je još uvek nedovoljno prepoznat entitet, a izražena heterogenost u kliničkoj manifestaciji, rani početak bolesti (najčešće pre 65. godine starosti), kao i preplitanje između ranih FTDbv simptoma i psihijatrijskih manifestacija vodi pogrešnim dijagnozama, predstavljajući izazov kliničarima čak i u tercijarnim centrima. Dug vremenski period u postavljanju adekvatne dijagnoze demencije je najvažniji činilac u razvoju stresa porodice i bolesnika. Rana i precizna dijagnoza FTD je posebno od značaja za sagledavanje prognoze i toka bolesti zajedno sa bolesnikom i porodicom; mogućnost uvođenja simptomatske terapije, čime se poboljšava kvalitet života bolesnika; usmeravanje genetskih testiranja, u interesu članova porodice koji takođe mogu biti u riziku od razvoja bolesti ili prenošenja mutacije na svoje potomke. Cilj: U ovom preglednom radu istakli smo specifičnosti kliničkog ispoljavanja spektra FTD koje bi mogle da budu dragocene u diferencijalno dijagnostičkom razmišljanju kada su u pitanju DRP i skrate ekstenzivnost dijagnostičkih metoda, kao i vreme do postavljanja adekvatne dijagnoze FTD. Zaključak: Rana i precizna dijagnoza spektra FTD važna je za sagledavanje toka bolesti, uvođenje adekvatne simptomatske terapije, kao i za usmeravanje genetskih testiranja, čime se smanjuje stres obolelih i negovatelja.

Ključne reči

spektar frontotemporalne demencije (FTD); kliničke karakteristike; bihevioralna varijanta FTD; primarno progresivne afazije

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