A clear pathologic hallmark like that identified in sporadic Parkinson’s disease (PD) is lacking in many of the monogenic causes of PD. In leucine-rich repeat kinase 2 (LRRK2) mutations (PARK8), alpha-synuclein pathology in the form of Lewy bodies (LBs) is frequently, but not consistently, observed. Other pathologies reported in LRRK2 cases include tau inclusions of the Alzheimer, progressive s...

A case of a 60-year-old man with biopsy-proven diffuse Lewy body disease was presented with clinical and serial neuropsychological data. Findings showed a rapid decline over a 2-year period characterized by an atypical degenerative dementia, including a global mental decline with preserved orientation, profound perceptual deficits, and no specific relative progressive memory impairment. Few ext...

A 67-year-old male patient initially showed memory disturbance followed by tremors a year later. The symptoms rapidly aggravated to dementia and Parkinsonian symptoms, and the patient died 2 years and 6 months after the onset at the age of 69 years and 5 months. Autopsy revealed numerous senile plaques in the cerebral cortex and Alzheimer's neurofibrillary tangles in the inferior temporal lobe ...

The authors assessed the accuracy of published clinical criteria and their own modifications of those criteria in diagnosing Lewy body disease (LBD). Clinical diagnoses were made by two clinicians, blinded to neuropathologic diagnoses, using the Rochester Alzheimer's Disease Center database and traditional medical records. Neuropathologic diagnoses were made according to published guidelines. R...

Lewy bodies, neuropathological hallmarks of Parkinson's disease and dementia with Lewy bodies, comprise alpha-synuclein filaments and other less defined proteins. Characterization of Lewy body proteins that interact with alpha-synuclein may provide insight into the mechanism of Lewy body formation. Double immunofluorescence labeling and confocal microscopy revealed approximately 80% of cortical...