Pathology: Neurodegenerative disorder characterized by Parkinsonism, visual hallucinations and dementia
Lewy bodies (inclusion bodies containing alpha-synuclein and ubiquitin)
Time of onset of cognitive symptoms concurrently or within 1 year of Parkinsonism.
Aetiology: Most cases are sporadic affecting patients between 50 and 85 years.
Symptoms: Cognitive Symptoms: Impaired memory, apathy, executive dysfunction and
visuospatial abilities.
Psychiatric Symptoms: Delusions, visual hallucinations and emotional lability.
Autonomic symptoms: Dry skin, orthostatic hypotension and sexual dysfunction
Other: Frequent falls, transient episodes of depressed consciousness
Signs: Parkinsonian features of rigidity and bradykinesia but fewer resting tremors.
Investigations: Imaging: MRI/CT brain to exclude other causes of dementia
DaT Scan: Reduced uptake of radioactive isotope in the basal ganglia
Treatment: Dopaminergic Therapy: Levodopa for Parkinsonism but response is usually poor
Cholinesterase Inhibitors: Donepezil for cognitive symptoms
Atypical Antipsychotics: Quetiapine for psychiatric symptoms and hallucinations
Avoid neuroleptic drugs such as haloperidol
Complications: Infection, incontinence, falls, self-neglect
Prognosis: Mean survival 8 years from onset.
No disease modifying treatment is currently available.
