Dementia with Lewy bodies: prevalence, clinical spectrum and natural history

Author(s): Ransmayr G


The article summarises history, terminology, the clinical and neuropathological diagnostic criteria, neurochemical and genetic findings, sensitivity and specificity of the clinical diagnostic criteria, prevalence, demographical data and nosology, differential diagnosis, and therapy of dementia with Lewy bodies (DLB). DLB shares clinical symptoms of Parkinson's disease and dementia of the Alzheimer-type (DAT). However, DLB is also different to PD and DAT (less tremor and asymmetry of the motor symptoms, more falls, and less favourable response to L-Dopa than PD; in contrast to DAT marked cognitive fluctuations and phases of reduced alertness, hallucinations and delirium). There are genetic similarities to DAT and PD in terms of common genetic risk factors. A genetic cause of the disease has so far not been detected. Whether or not DLB is a disease entity or an association of diseases (Lewy body disease and DAT) has so far not been elucidated. Clinical distinction from DAT and PD has clinical importance because of different therapeutic and prognostic implications. Studies are needed to standardize the treatment of motor, cognitive, psychiatric and vegetative symptoms.

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