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Neurology 1999;53:902
© 1999 American Academy of Neurology
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Report of the second dementia with Lewy body international workshop

Diagnosis and treatment

I. G. McKeith, MD, FRCPsych, E. K. Perry, PhD, DSc, R. H. Perry, RH, DSc, FRCPath and for the Consortium on Dementia with Lewy Bodies

From the Department of Old Age Psychiatry, Institute for the Health of the Elderly (Dr. McKeith), the MRC Neurochemical Pathology Unit (Dr. E.K. Perry), and Department of Neuropathology (Dr. R.H. Perry), Newcastle General Hospital, UK, for the Consortium on DLB.

Address correspondence and reprint requests to Dr. I.G. McKeith, Department of Old Age Psychiatry, Institute for the Health of the Elderly, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, UK.

BACKGROUND/OBJECTIVE: The second International Workshop of the Consortium on Dementia with Lewy Bodies (DLB) met to review developments since publication of consensus guidelines for the clinical and pathologic diagnosis of DLB in 1996. The specificity of a clinical diagnosis of probable DLB, made using consensus criteria, is generally high (>85%), but sensitivity of case detection is lower and more variable. Inter-rater reliability for the core clinical features—recurrent visual hallucinations and spontaneous motor features of parkinsonism—is acceptable, but reliable identification of fluctuating cognition remains problematic. Depression and REM sleep behavior disorder may be additional features supportive of a diagnosis of DLB that were not included in the original guideline.

RESULTS: It is recommended that the clinical consensus criteria continue to be used in their current format with research efforts focused on increasing sensitivity of case detection. Antiubiquitin immunocytochemistry is the method of choice for routine detection of Lewy bodies for diagnostic purposes in research and clinical practice. The use of {alpha}-synuclein antibodies to label Lewy bodies and Lewy neurites represents a major methodologic advance since the first DLB workshop. {alpha}-Synuclein–based methods are likely to be most useful in research laboratories, particularly for clinicopathologic correlative studies.

CONCLUSION: Clinical management of DLB patients usually centers on the treatment of noncognitive features. There is now a pressing need to establish appropriately designed randomized controlled trials in DLB. Collaboration between dementia and movement disorder specialists is essential for rapid progress in research and clinical management protocols.

Key words: Dementia—Lewy body—{alpha}-Synuclein—Diagnosis—Treatment.




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