Dementia With Lewy Bodies Workup

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

Imaging Studies

Because vascular dementia can cause symptoms and signs similar to those of DLB, brain magnetic resonance imaging (MRI) is indicated to distinguish DLB from vascular dementia. Patients with vascular dementia often have white matter lesions on MRI scans, whereas patients with DLB do not.[14]

Patients with DLB usually have less hippocampal atrophy than do patients with Alzheimer disease (but more than control subjects), although whether this difference is clinically useful is under investigation, as is the diagnostic utility of functional imaging. MRI is superior to computed tomography (CT) scanning in identifying this atrophy.

SPECT or positron emission tomography (PET) scanning may show decreased occipital lobe blood flow or metabolism in DLB but not in Alzheimer disease. SPECT using ligands that bind to the dopamine transporter molecule (eg,123 I-beta-CIT) has been used to suggest the diagnosis of DLB. Abnormal dopamine transporter scans have been shown to have a sensitivity of over 75% and a specificity of over 90% for DLB.[15]

A study by Lim et al using SPECT scanning with123 I-beta- carbomethoxy-3beta-(4-fluorophenyl) tropane (123 I-beta-CIT), as well as PET scanning with18 F-fluorodeoxyglucose (18 F-FDG), in 14 patients with a clinical diagnosis of DLB and 10 with Alzheimer disease found that relative preservation of the mid- or posterior cingulate gyrus (cingulate island sign) had 100% specificity for DLB. SPECT and PET scanning each appeared useful for the diagnosis of DLB, but SPECT provided more robust results than did PET.[16]

PET imaging with Pittsburgh Compound B showed that amyloid deposition in clinically diagnosed patients with DLB was similar to that in the patients with Alzheimer disease. However, amyloid binding was less in patients with dementia in Parkinson disease. Findings of these studies suggest that the presence of amyloid accelerates dementia in Lewy body disorders but has little influence on its nature.[17, 18]

Until disease-modifying therapies that are specific to DLB or Alzheimer disease are developed, metabolic imaging studies to enhance the accuracy of the diagnosis are rarely needed.

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Histologic Findings

The characteristic lesion is the LB, an eosinophilic (hematoxylin and eosin staining), round inclusion found in the cytoplasm of substantia nigra cells and in the nucleus basalis of Meynert, locus ceruleus, and dorsal raphe, as well as in the dorsal motor nucleus of CNX. LBs are found in nonpyramidal cells in layers V and VI of the cortices (especially the limbic and transitional cortex).

Other histologic findings in DLB are minimal atrophy, occasional vacuolization in deep layers of the temporal cortex, and abnormal neurites in cells of CA2/3 of the hippocampus and various brainstem nuclei.

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Approach Considerations

No sensitive or specific blood or urine tests are currently available for DLB. Laboratory studies in patients with dementia with Lewy bodies (DLB) should include those usually ordered in a dementia evaluation, including the following:

  • Chemistry panel
  • Complete blood count (CBC)
  • Thyroid studies
  • Vitamin B-12 levels
  • Syphilis, Lyme disease, or human immunodeficiency virus (HIV) testing, when appropriate

Cerebrospinal fluid (CSF) examination is not required in routine cases. However, CSF findings in DLB include the following:

  • Patients with Alzheimer disease have higher levels of tau protein in their CSF than do patients with DLB
  • Patients with LB variant of Alzheimer disease (LBV-AD) have intermediate values
  • CSF levels of beta amyloid are lower than normal in DLB, Alzheimer disease, and LBV-AD; however, CSF beta-amyloid levels in DLB, LBV-AD, and Alzheimer disease do not differ from each other[13]

Patients with DLB may have changes on electroencephalography earlier than do patients with Alzheimer disease, but whether this difference is diagnostically useful is not clear.

In certain circumstances, neuropsychological testing is helpful in differentiating DLB from Alzheimer disease and in establishing a baseline for future comparison.

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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].

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  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].

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