Abstract
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.
Highlights
Clinical and neuropathological characteristics of dementia with Lewy bodies Dementia with Lewy bodies (DLB) is the second most common neurodegenerative dementia
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)
[68] Aarsland, D.: Cognitive impairment in Parkinson’s disease and dementia with Lewy bodies
Summary
Bencze János dr.1 ■ Simon Viktória dr.2 ■ Bereczki Erika dr. Majer Réka1, 4 ■ Varkoly Gréta dr.1 ■ Murnyák Balázs Kálmán János dr.5 ■ Hortobágyi Tibor dr. . Majer Réka1, 4 ■ Varkoly Gréta dr.1 ■ Murnyák Balázs Kálmán János dr.5 ■ Hortobágyi Tibor dr. . A betegség fő morfológiai jellemzője a kóros α-szinukleinben gazdag Lewy-test és Lewy-neurit – csakúgy, mint a rokon kórkép Parkinson-kór és az ahhoz társuló demencia esetén. A klinikopatológiai vizsgálatok kiemelten fontosak a patomechanizmus jobb megértése, a pontos diagnózis és a hatékony terápia érdekében. Kulcsszavak: Lewy-testes demencia, neuropatológia, biomarkerek, Parkinson-kór, α-szinuklein. Clinical and neuropathological characteristics of dementia with Lewy bodies Dementia with Lewy bodies (DLB) is the second most common neurodegenerative dementia. The accurate diagnosis is often possible only by neuropathological examination. Clinicopathological studies are important in exploring pathomechanisms, ensuring accurate diagnosis and identifying therapeutic targets. Bencze J, Simon V, Bereczki E, Majer R, Varkoly G, Murnyák B, Kálmán J, Hortobágyi T [Clinical and neuropathological characteristics of dementia with Lewy bodies].
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