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  • 1 Debreceni Egyetem, Debrecen
  • 2 Debreceni Egyetem, Debrecen
  • 3 Semmelweis Egyetem, Budapest
  • 4 Szegedi Tudományegyetem, Szeged
  • 5 Szegedi Tudományegyetem, Szeged, Állomás u. 1., 6725
  • 6 King’s College London, London, Egyesült Királyság
Open access

Absztrakt:

A Lewy-testes demencia (DLB) és a Parkinson-kórhoz társuló demencia (PDD) számos klinikai, patofiziológiai és morfológiai átfedést mutató neurodegeneratív kórkép. Neuropatológiai szempontból meghatározó a kóros α-szinuklein-aggregátumokat tartalmazó Lewy-testek (LB) jelenléte. DLB-ben nagyszámú LB található a corticalis régiókban, míg PDD-ben elsősorban a subcorticalis területek érintettek. Továbbá, DLB-ben gyakoribb az Alzheimer-kórra (AD) jellegzetes β-amyloid-plakkok és neurofibrillaris kötegek megjelenése. A mai napig vitatott, hogy valóban két külön betegségről vagy egyazon kórkép különböző megnyilvánulásairól van-e szó. A klinikai diagnózis alapja a motoros és a kognitív tünetek megjelenése között eltelt idő: DLB-ben a demencia gyakran a parkinsonizmus előtt jelentkezik, míg PDD-ben a motoros tünetek alakulnak ki korábban. Az egyre pontosabb képalkotó módszerek alapján DLB-ben nagyobb mértékű a corticalis károsodás, a kolinerg deficit, valamint a társuló AD-patológia, mint PDD-ben. Terápiás szempontból a PDD-ben gyakran használt levodopa DLB-ben kevésbé hatékony, sőt növelheti a pszichózis kialakulásának kockázatát, a betegség magtüneteihez tartozó hallucinációk és viselkedési tünetek kezelése pedig a jelentős antipszichotikum-hiperszenzitivitás miatt ütközik nehézségekbe. Összefoglalónkban részletesen tárgyaljuk a kórképek jellegzetességeit a patológia, a radiológia és a klinikai tünetek vonatkozásában, külön figyelmet fordítva az átfedések, valamint a különbségek bemutatására. Orv Hetil. 2020; 161(18): 727–737.

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