Highlights & Basics
- Dementia with Lewy bodies (DLB) is a neurodegenerative disorder with parkinsonism, progressive cognitive decline, prominent executive dysfunction, behavioral and sleep disturbances, and visuospatial impairment.
- Core clinical features are: cognitive fluctuations; recurrent visual hallucinations; rapid eye movement (REM) sleep behavior disorder; and one or more spontaneous cardinal motor features of parkinsonism: bradykinesia, rest tremor, or rigidity. Supportive clinical features include sensitivity to antipsychotic agents, postural instability and falls, syncope, autonomic dysfunction, delusions and nonvisual hallucinations, apathy, anxiety, and depression.
- Diagnosis is made clinically and can only be confirmed pathologically by the presence of Lewy bodies. Many patients have concomitant Alzheimer disease-type pathology.
- Treatment requires a multidisciplinary approach, with emphasis on behavioral and psychological symptoms. This includes both nonpharmacologic/behavioral and pharmacologic interventions, as well as supportive care. The main goal of treatment is to improve or stabilize cognition, behavior, and activities of daily living, and to maintain safety.
- First-line pharmacologic treatment for cognitive impairment and behavioral symptoms is cholinesterase inhibitors. Clonazepam or melatonin is effective for REM sleep behavior disorder. Levodopa/carbidopa can be used for motor symptoms, although adverse effects may be restrictive and response limited.
Quick Reference
History & Exam
Key Factors
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Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Citations
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