Bencze János, Simon Viktória, Bereczki Erika, Majer Réka, Varkoly Gréta, Murnyák Balázs, Kálmán János, Hortobágyi Tibor
Patológiai Intézet, Neuropatológiai Tanszék, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen, Nagyerdei krt. 98., 4032.
Pszichiátriai és Pszichoterápiás Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.
Orv Hetil. 2017 Apr;158(17):643-652. doi: 10.1556/650.2017.30735.
Dementia with Lewy bodies (DLB) is the second most common neurodegenerative dementia. The accurate diagnosis is often possible only by neuropathological examination. The morphologic hallmarks are the presence of α-synuclein-rich Lewy bodies and Lewy neurites, identical to those seen in Parkinson's disease (PD) and Parkinson's disease dementia (PDD). Neurotransmitter deficits, synaptic and ubiquitin-proteasome system (UPS) dysfunction play major role in the pathomechanism. Characteristic symptoms are cognitive fluctuation, parkinsonism and visual hallucinations. Due to the often atypical clinical presentation novel imaging techniques and biomarkers could help the early diagnosis. Although curative treatment is not available, therapies can improve quality of life. Clinicopathological studies are important in exploring pathomechanisms, ensuring accurate diagnosis and identifying therapeutic targets. Orv Hetil. 2017; 158(17): 643-652.
路易体痴呆（DLB）是第二常见的神经退行性痴呆。通常只有通过神经病理学检查才能进行准确诊断。形态学特征是存在富含α-突触核蛋白的路易小体和路易神经突，这与帕金森病（PD）和帕金森病痴呆（PDD）中所见的相同。神经递质缺乏、突触和泛素-蛋白酶体系统（UPS）功能障碍在发病机制中起主要作用。特征性症状是认知波动、帕金森综合征和视幻觉。由于临床表现通常不典型，新型成像技术和生物标志物有助于早期诊断。虽然尚无治愈性治疗方法，但治疗可改善生活质量。临床病理研究对于探索发病机制、确保准确诊断和确定治疗靶点很重要。《匈牙利医学周报》。2017年；158（17）：643 - 652。
