Lewy body dementia (LBD)

Definition:

Lewy Body dementia (LDB) is neurodegenerative disease characterized by dementia, mild parkinsonism, and fluctuations in attention and alertness. It is the second most-common degenerative dementia after Alzheimer's disease. Progressive accumulation of alpha-synuclein- and ubiquitin-positive Lewy Bodies is seen in the brainstem, the limbic system or cortical and subcortical regions, depending on disease subtype. The genetic risk factors include alpha-synuclein mutations and SNCA locus triplication. Mutations of leucine-rich repeat kinase 2 (LRRK2) and the glucocerebrosidase (GBA) gene have also been implicated in familial DLB.

References:

[1]. D W Dickson, et al. Alpha-synuclein and the Lewy body disorders. Curr Opin Neurol. 2001 Aug;14(4):423-32. [Content Brief]

[2]. Dag Aarsland, et al. Are Parkinson's disease with dementia and dementia with Lewy bodies the same entity?. J Geriatr Psychiatry Neurol. 2004 Sep;17(3):137-45. [Content Brief]

[3]. David J Burn, et al. Update on dementia with Lewy bodies. Curr Neurol Neurosci Rep. 2005 Sep;5(5):339-44. [Content Brief]

[4]. Francesca M Brett, et al. Familial diffuse Lewy body disease, eye movement abnormalities, and distribution of pathology. Arch Neurol. 2002 Mar;59(3):464-7. [Content Brief]

[5]. Ignacio F Mata, et al. Glucocerebrosidase gene mutations: a risk factor for Lewy body disorders. Arch Neurol. 2008 Mar;65(3):379-82. [Content Brief]

[6]. Makoto Hashimoto, et al. The Role of alpha-synuclein assembly and metabolism in the pathogenesis of Lewy body disease. J Mol Neurosci. 2004;24(3):343-52. [Content Brief]

[7]. Manuel B Graeber, et al. Dementia with Lewy bodies: disease concept and genetics. Neurogenetics. 2003 Aug;4(4):157-62. [Content Brief]

[8]. Owen A Ross, et al. Lrrk2 and Lewy body disease. Ann Neurol. 2006 Feb;59(2):388-93. [Content Brief]

[9]. T Obi, et al. Clinicopathologic study of a SNCA gene duplication patient with Parkinson disease and dementia. Neurology. 2008 Jan 15;70(3):238-41. [Content Brief]

[10]. Takeshi Ikeuchi, et al. Patients homozygous and heterozygous for SNCA duplication in a family with parkinsonism and dementia. Arch Neurol. 2008 Apr;65(4):514-9. [Content Brief]

[11]. Vincenzo Bonifati, et al. Recent advances in the genetics of dementia with lewy bodies. Curr Neurol Neurosci Rep. 2008 May;8(3):187-9. [Content Brief]

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