A neurodegenerative disorder with parkinsonism, progressive cognitive decline, prominent executive dysfunction, and visuospatial impairment.
Core clinical features include: recurrent visual hallucinations; cognitive fluctuations; REM sleep behavior disorder; and one or more spontaeous cardinal 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 (AD)-type pathology.
Pharmacologic treatment is off-label in the US. Meta-analyses support the use of cholinesterase inhibitors for the cognitive and the neurobehavioral symptoms. Memantine may be of some value in the treatment of dementia and behavioral symptoms.
The use of antipsychotic medication must be minimized due to the increased sensitivity to these medications' adverse effects, including increased mortality.
Levodopa/carbidopa can be used for the motor symptoms, though adverse effects may be restrictive and response limited; clonazepam is effective for REM sleep behavioral disorder.
Essential for a diagnosis of dementia with Lewy bodies (DLB) is dementia, defined as a progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational functions, or with usual daily activities. DLB is characterized by the following core clinical features (the first 3 typically occur early, and may persist throughout the course): fluctuating cognition; recurrent visual hallucinations; REM sleep behavior disorder; and one or more spontaneous cardinal symptoms of parkinsonism: bradykinesia, rest tremor, or rigidity. Prominent or persistent memory impairment may not necessarily occur in the early stages but is usually evident with progression. Deficits on tests of attention, executive function, and visuoperceptual ability may be especially prominent and occur early.Disease course is progressive, although treatment may help some cognitive, sleep, motor, and behavioral symptoms. DLB should be distinguished from neurocognitive disorder associated with Parkinson disease, although there are areas of clinical overlap.
In the DSM-5, DLB is classified as a major neurocognitive disorder with Lewy bodies.
History and exam
Alan J. Lerner, MD
Professor of Neurology
Department of Neurology
University Hospitals Case Medical Center
Case Western Reserve University
AJL receives research grant support from NIH, TauRx, Eli Lilly, and Axovant, and has been a medicolegal consultant in several legal cases, but he declares that he has no competing interests related to this topic.
Dr Alan J. Lerner would like to gratefully acknowledge Dr Brian S. Appleby, Dr Ethan Gore, Dr Rajeet Shrestha, Dr Timothy Wuerz, Dr Julie Schneider, Dr Viola Fahmy, and Dr Sube Banerjee, previous contributors to this topic. BSA, EG, RS, TW, JS, VF, and SB declare that they have no competing interests.
Bryan Bernard, PhD
Assistant Professor and Clinical Neuropsychologist
Department of Neurological Sciences
Rush University Medical Center
BB declares that he has no competing interests.
James E. Galvin, MD, MPH
Neurology, Psychiatry and Neurobiology
Memory Diagnostic Center and Alzheimer Treatment Unit
Education and Community Outreach
Alzheimer Disease Research Center
Washington University School of Medicine
JEG declares that he has no competing interests.
Ian McKeith, MBBS
Professor of Old Age Psychiatry
Institute for Ageing and Health
IM is the author of several references cited in this topic.
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