OBJECTIVES To compare, in a retrospective clinicopathological study, the presentation features of patients with dementia and cortical Lewy bodies (Lewy body dementia) with those of patients with Alzheimer's disease. METHODS From a population of 426 cases from the dementia brain bank, 39 cases of Lewy body dementia and 61 cases of Alzheimer's disease with presentation details were identified. ...

The relationship between clinicopathologic diagnosis and 123I-FP-CIT SPECT in 18 patients with dementia (12 with Lewy body disease) from one center in the United States was assessed. The sensitivity and specificity of abnormal 123I-FP-CIT SPECT with reduced striatal uptake on visual inspection for predicting Lewy body disease were 91.7% and 83.3%, respectively. The mean calculated putamen to oc...

1 McKeith IG, Fairbairn AF, Perry RH, Thompson P. The clinical diagnosis and misdiagnosis of senile dementia of Lewy body type (SDLT). Br J Psychiatry 1994;165: 324-32. 2 Crystal HA, Dickson DW, Lizardi JE, Davies P, Wolfson LI. Antemortem diagnosis of diffuse Lewy body disease. Neurology 1990;40: 1523-8. 3 Byrne EJ, Lennox GG, Godwin-Austen RB, et al. Dementia associated with cortical Lewy bod...

BACKGROUND Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. OBJECTIVE To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. METHODS Review clinical records and pathological ...

OBJECTIVE To further elucidate the relation between diffuse Lewy body disease and Parkinson's disease. METHODS AND RESULTS The clinical features of nine cases of pure diffuse Lewy body disease without pathological evidence of coexisting Alzheimer's neuritic pathology were reported. All patients were aged less than 70 years at onset (mean 62 years). Five patients presented with clinical featur...

Lewy body diseases include dementia with Lewy bodies and Parkinson's disease. Whereas dementia with Lewy bodies and Parkinson's disease can be distinguished as separate clinical entities, the pathological picture is very often identical. α-synuclein aggregation is a key event in the pathogenesis of Lewy body diseases and β-synuclein inhibits α-synuclein aggregation in vitro and in vivo. Recentl...

BACKGROUND ATP13A2 (PARK9) loss of function mutations are a genetic cause of an early-onset form of Parkinson's disease (PD), with in vitro studies showing that ATP13A2 deficits lead to lysosomal and mitochondrial dysfunction and α-synuclein accumulation, while elevated ATP13A2 expression reduces α-synuclein toxicity. The three human brain tissue studies assessing changes in ATP13A2 expression ...

Human ?-synuclein is a 140 amino acid protein with little or no secondary structure. The ?-synuclein is expressed at high levels in the brain and enriched in neural synaptic terminals but its physiological function remains largely unknown. More recently, ?-synuclein has been shown to be one of the principal componets of Lewy bodies, neuronal inclusions that are found in diverse human neurodegen...