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Lewy Body Disease – A Partially Remediable Cause of Dementia

The features of dementia with Lewy bodies (DLB) are not sufficiently widely known or recognised in clinical practice. DLB can be mistaken for Alzheimer’s disease, vascular cognitive impairment, other dementia syndrome, late-onset delusional or schizophreniform disorder, psychotic depression, mania or Charles Bonnet syndrome. Misdiagnosis is unfortunate, as this common disorder often responds, sometimes dramatically, to the cholinergic drugs: donepezil, rivastigmine and galantamine. This has been demonstrated in open label studies for all three, but in placebo controlled studies only for rivastigmine. Patients with this disorder may develop marked, even catastrophic adverse reactions to psychoactive drugs, including antidepressants, but particularly antipsychotic drugs. Recently a consensus group provided revised criteria for the clinical diagnosis of dementia with Lewy bodiesthese criteria will be outlined, with some elaboration. They include: progressive cognitive decline often with relatively retained memory functions; deficits on tests of attention, executive function, and visuospatial ability; fluctuating cognition, often with marked ‘sundowning’ and sometimes transient unexplained loss of consciousness; visual hallucinations which are typically well formed and detailed; sometimes features of parkinsonism; a marked propensity to delirium. Several brief case studies will be presented which illustrate aspects of the diagnosis and relatively successful treatment of symptoms including hallucinations and cognitive impairment. Lewy body disease is associated with pathological aggregations of alpha-synuclein in the brain stem, limbic system and cerebral cortex. Post mortem and other studies have shown considerable overlap of Lewy body, Alzheimer and vascular pathology.