Dementia With Lewy Bodies Clinical Presentation

  • Author: Howard A Crystal, MD; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA   more...
 
Updated: Apr 25, 2012
 

History

Dementia with Lewy bodies (DLB) is a progressive, degenerative dementia.The following clinical features help to distinguish DLB from Alzheimer disease:

Fluctuations in cognitive function with varying levels of alertness and attention - Clues to the presence of fluctuations include excessive daytime drowsiness (if nighttime sleep is adequate) or daytime sleep longer than 2 hours, staring into space for long periods, and episodes of disorganized speech

  • Visual hallucinations
  • Parkinsonian motor features

Although extrapyramidal features may occur late in the course of Alzheimer disease, they appear relatively early in DLB.

In addition, whereas patients with Alzheimer disease virtually always have anterograde memory loss as a prominent symptom and sign early in the course of the illness, anterograde memory loss may be less prominent in DLB. McKeith et al have suggested that patients with DLB do relatively better on tests of confrontation naming, short and medium recall, and recognition than do patients with Alzheimer disease, whereas patients with Alzheimer disease do better on tests of verbal fluency, visual perception, and performance tasks.[10]

Executive function deficits and visuospatial impairment may be more prominent in persons with DLB than in those with Alzheimer disease (eg, Stroop, digit span backwards).

Other symptoms that may alert clinicians to the diagnosis of DLB (versus Alzheimer disease) include the following:

  • Nonvisual hallucinations
  • Delusions
  • Unexplained syncope
  • Rapid eye movement sleep disorder
  • Neuroleptic sensitivity
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Physical Examination

Patients with DLB usually have impaired cognition consistent with dementia. Cognitive function, as measured by Mini-Mental State Examination (MMSE) scores, appears to be relatively preserved in DLB compared with Alzheimer disease (AD) or AD+DLB (P < .01). Nelson et al evaluated data from 2 large, multicenter data registries (6,340 cases in total) and found that final MMSE scores were 15.6 (+/- 8.7) in persons with DLB, 10.7 (+/- 8.6) in persons with Alzheimer disease, and 10.6 (+/- 8.6) in those with AD+DLB.[11]

An important observation during mental status testing is that the patient has periods of being alert, coherent, and oriented that alternate with periods during which the patient is confused and unresponsive to questions (despite the fact that the patient is awake). This fluctuation is a relatively specific feature of DLB.

Retrieval from memory may be relatively worse than memory storage. Patients may do relatively well with confrontation naming tests and poorly on tests of visuospatial skills (eg, drawing a clock, copying figures).

Patients may have some parkinsonian signs but usually not enough to meet the criteria for a diagnosis of Parkinson disease. Mild gait impairment is relatively frequent and should not be ascribed to old age or osteoarthritis. Resting tremor occurs less frequently than in Parkinson disease. Myoclonus may occur before severe dementia.

Orthostatic hypotension appears to be particularly common in patients with DLB, even when dementia is mild.[12]

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Contributor Information and Disclosures
Author

Howard A Crystal, MD  Professor, Departments of Neurology and Pathology, State University of New York Downstate; Consulting Staff, Department of Neurology, University Hospital and Kings County Hospital Center

Howard A Crystal, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel H Jacobs, MD, FAAN  Associate Professor of Neurology, University of Florida College of Medicine; Director for Stroke Services, Orlando Regional Medical Center

Daniel H Jacobs, MD, FAAN is a member of the following medical societies: American Academy of Neurology, American Society of Neurorehabilitation, and Society for Neuroscience

Disclosure: Teva Pharmaceutical Grant/research funds Consulting; Biogen Idex Grant/research funds Independent contractor; Serono EMD Royalty Speaking and teaching; Pfizer Royalty Speaking and teaching; Berlex Royalty Speaking and teaching

Chief Editor

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA  Professor of Neurology, University of Central Florida College of Medicine; Director of Cognitive Neurology, Director of Stroke Program, James A Haley Veterans Affairs Hospital

Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA is a member of the following medical societies: American Academy of Neurology, American Headache Society, American Heart Association, and American Society of Neuroimaging

Disclosure: Nothing to disclose.

Additional Contributors

Robert A Hauser, MD, MBA Professor of Neurology, Molecular Pharmacology and Physiology, Director, Parkinson's Disease and Movement Disorders Center, University of South Florida; Clinical Chair, Signature Interdisciplinary Program in Neuroscience

Robert A Hauser, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Neuroimaging, and Movement Disorders Society

Disclosure: Adamas Pharmaceuticals Consulting fee Consulting

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

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  2. McKeith IG, Perry EK, Perry RH. Report of the second dementia with Lewy body international workshop: diagnosis and treatment. Consortium on Dementia with Lewy Bodies. Neurology. Sep 22 1999;53(5):902-5. [Medline].

  3. McKeith IG, Ballard CG, Perry RH, et al. Prospective validation of consensus criteria for the diagnosis of dementia with Lewy bodies. Neurology. Mar 14 2000;54(5):1050-8. [Medline].

  4. Verghese J, Crystal HA, Dickson DW, Lipton RB. Validity of clinical criteria for the diagnosis of dementia with Lewy bodies. Neurology. Dec 10 1999;53(9):1974-82. [Medline].

  5. McKeith IG, Dickson DW, Lowe J, et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology. Dec 27 2005;65(12):1863-72. [Medline].

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  9. Perez F, Helmer C, Dartigues JF, Auriacombe S, Tison F. A 15-year population-based cohort study of the incidence of Parkinson's Disease and dementia with Lewy bodies in an elderly French cohort. J Neurol Neurosurg Psychiatry. Dec 3 2009;[Medline].

  10. McKeith IG, Rowan E, Askew K, et al. More severe functional impairment in dementia with lewy bodies than Alzheimer disease is related to extrapyramidal motor dysfunction. Am J Geriatr Psychiatry. Jul 2006;14(7):582-8. [Medline].

  11. Nelson PT, Kryscio RJ, Jicha GA, Abner EL, Schmitt FA, Xu LO, et al. Relative preservation of MMSE scores in autopsy-proven dementia with Lewy bodies. Neurology. Oct 6 2009;73(14):1127-33. [Medline].

  12. Sonnesyn H, Nilsen DW, Rongve A, Nore S, Ballard C, Tysnes OB, et al. High prevalence of orthostatic hypotension in mild dementia. Dement Geriatr Cogn Disord. 2009;28(4):307-13. [Medline].

  13. Clark CM, Xie S, Chittams J, et al. Cerebrospinal fluid tau and beta-amyloid: how well do these biomarkers reflect autopsy-confirmed dementia diagnoses?. Arch Neurol. Dec 2003;60(12):1696-702. [Medline].

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  16. Lim SM, Katsifis A, Villemagne VL, Best R, Jones G, Saling M, et al. The 18F-FDG PET cingulate island sign and comparison to 123I-beta-CIT SPECT for diagnosis of dementia with Lewy bodies. J Nucl Med. Oct 2009;50(10):1638-45. [Medline].

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  19. McKeith I, Del Ser T, Spano P, et al. Efficacy of rivastigmine in dementia with Lewy bodies: a randomised, double-blind, placebo-controlled international study. Lancet. Dec 16 2000;356(9247):2031-6. [Medline].

  20. Fernandez HH, Wu CK, Ott BR. Pharmacotherapy of dementia with Lewy bodies. Expert Opin Pharmacother. Nov 2003;4(11):2027-37. [Medline].

  21. [Best Evidence] Emre M, Tsolaki M, Bonuccelli U, Destée A, Tolosa E, Kutzelnigg A, et al. Memantine for patients with Parkinson's disease dementia or dementia with Lewy bodies: a randomised, double-blind, placebo-controlled trial. Lancet Neurol. Oct 2010;9(10):969-977. [Medline].

  22. Aarsland D, Ballard C, Walker Z, Bostrom F, Alves G, Kossakowski K, et al. Memantine in patients with Parkinson's disease dementia or dementia with Lewy bodies: a double-blind, placebo-controlled, multicentre trial. Lancet Neurol. Jul 2009;8(7):613-8. [Medline].

  23. Woods B, Aguirre E, Spector AE, Orrell M. Cognitive stimulation to improve cognitive functioning in people with dementia. Cochrane Database of Systematic Reviews. 2012;2012.

  24. Boeve BF, Silber MH, Ferman TJ. Melatonin for treatment of REM sleep behavior disorder in neurologic disorders: results in 14 patients. Sleep Med. Jul 2003;4(4):281-4. [Medline].

 
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