eMedicine Specialties > Neurology > Behavioral Neurology and Dementia

Dementia With Lewy Bodies: Differential Diagnoses & Workup

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
Contributor Information and Disclosures

Updated: Dec 10, 2008

Differential Diagnoses

Alzheimer Disease
Lacunar Syndromes
Cortical Basal Ganglionic Degeneration
Parkinson-Plus Syndromes
Frontal and Temporal Lobe Dementia
Prion-Related Diseases
Hydrocephalus
Progressive Supranuclear Palsy

Other Problems to Be Considered

Dementia in Parkinson's disease
Dementia in progressive supranuclear palsy

Workup

Laboratory Studies

  • Laboratory studies should include those usually ordered in a dementia evaluation, including tests of the following:
    • Chemistry panel
    • Complete blood cell count
    • Thyroid studies
    • Vitamin B-12 levels
    • Syphilis, Lyme disease, or HIV testing, when appropriate
  • No sensitive or specific blood, cerebrospinal fluid (CSF), or urine tests are currently available for DLB.

Imaging Studies

  • Because vascular dementia can cause symptoms and signs similar to those of dementia with Lewy bodies (DLB), brain MRI is indicated to distinguish DLB from vascular dementia.
  • Patients with vascular dementia often have white matter lesions on MRIs, whereas patients with DLB do not.
  • MRI is superior to CT scanning in identifying hippocampal atrophy.
  • Patients with DLB usually have less hippocampal atrophy than patients with Alzheimer's disease (but more than control subjects). Whether this difference is clinically useful is under investigation, as is the diagnostic utility of functional imaging.
  • Single-photon emission CT scanning or positron emission tomography scanning may show decreased occipital lobe blood flow or metabolism in DLB but not in Alzheimer's disease.
  • Reduced dopamine transporter activity in the basal ganglia is seen with positron emission tomography scanning or single-photon emission CT scanning.
  • PET imaging with Pittsburgh Compound B showed that amyloid deposition in the clinically diagnosed patients with DLB was similar to that in the patients with Alzheimer's disease. However, amyloid binding was less in patients with dementia in Parkinson's disease.
  • Until disease-modifying therapies that are specific to DLB or Alzheimer's disease are developed, metabolic imaging studies to enhance accuracy of the diagnosis are rarely needed.

Other Tests

  • In certain circumstances, neuropsychological testing is helpful to differentiate DLB from Alzheimer's disease and to establish a baseline for future comparison.
  • Patients with DLB may have changes on electroencephalography earlier than patients with Alzheimer's disease, but whether this difference is diagnostically useful is not clear.
  • CSF examination is not required in routine cases.
    • Patients with Alzheimer's disease have higher levels of tau protein in their CSF than patients with DLB.
    • Patients with both LBV-AD have intermediate values.
    • CSF levels of beta-amyloid are lower than normal in DLB, Alzheimer's disease, and LBV-AD. However, CSF beta-amyloid levels in DLB, LBV-AD, and Alzheimer's disease do not differ from each other.

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, dorsal raphe, and the dorsal motor nucleus of cranial nerve X. LBs are found in nonpyramidal cells in layers V and VI of the cortices (especially limbic and transitional cortex).

Other findings 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. The primary constituent of LBs is alpha-synuclein, a presynaptic protein, the function of which is unknown. Neurofilament proteins and ubiquitin are other important constituents of LBs. Numerous neurotransmitters, including ACh, are diminished in DLB. The decrease in ACh may be more severe than in Alzheimer's disease.

More on Dementia With Lewy Bodies

Overview: Dementia With Lewy Bodies
Differential Diagnoses & Workup: Dementia With Lewy Bodies
Treatment & Medication: Dementia With Lewy Bodies
Follow-up: Dementia With Lewy Bodies
References
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References

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Further Reading

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Keywords

Lewy body dementia, DLB, LB, Lewy body variant of Alzheimer disease, diffuse Lewy body disease, senile dementia of the Lewy body type, idiopathic Parkinson disease, Parkinson's disease, Parkinson disease with dementia, PD with dementia, parkinsonian dementias, Alzheimer disease, Alzheimer's disease, dementia with Lewy bodies, dementia with LBs, progressive degenerative dementia, nonvisual hallucinations, neuroleptic sensitivity, unexplained syncope, delusions, rapid eye movement sleep disorder, myoclonus, apolipoprotein genotype E subtype 4, apoE4 genotype

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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: Pfizer Honoraria Speaking and teaching; Myriad Honoraria Consulting

Medical Editor

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: Allergan Sales, LLC Honoraria Speaking and teaching; Bayer Shering Pharma AG Honoraria Consulting; Boehringer Ingelheim France Honoraria Consulting; Centapharm Honoraria Speaking and teaching; Genzyme Corporation Honoraria Consulting; GlaxoSmithKline Honoraria Consulting; IMPAX Laboratories, Inc.  Consulting; Kyowa Pharmaceuticals, Inc. Honoraria Consulting; Novartis Pharmaceuticals Corp. Honoraria Consulting; Prestwick Pharmaceuticals, Inc. Honoraria Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Richard J Caselli, MD, Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale
Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD, Professor, Department of Neurology, Oregon Health and Science University; Associate Director, Oregon Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association
Disclosure: Co-Axia Consulting fee Review panel membership; Talecris Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Boehringer Ingelheim Honoraria Speaking and teaching; Boston Scientific Honoraria Speaking and teaching; Concentric Medical None Review panel membership; Northstar Neuroscience  Review panel membership; ev3 Consulting fee Review panel membership

 
 
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