Neuro

Dementia With Lewy Bodies

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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.

Signs

Parkinsonian features of rigidity and bradykinesia but fewer resting tremors.

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 

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.

Prognosis

Mean survival 8 years from onset.
No disease modifying treatment is currently available.
Infection, incontinence, falls, self-neglect

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