Report of the second dementia with Lewy body international workshop - Diagnosis and treatment

Background/Objective: The second International Workshop of the Consortium o n Dementia with Lewy Bodies (DLB) met to review developments since publicat ion of consensus guidelines for the clinical and pathologic diagnosis of DL B in 1996. The specificity of a clinical diagnosis of probable DLB, made us ing consensus criteria, is generally high (>85%), but sensitivity of case d etection is lower and more variable. Inter-rater reliability for the core c linical features-recurrent visual hallucinations and spontaneous motor feat ures of parkinsonism-is acceptable, but reliable identification of fluctuat ing cognition remains problematic. Depression and REM sleep behavior disord er may be additional features supportive of a diagnosis of DLB that were no t included in the original guideline. Results: It is recommended that the c linical consensus criteria continue to be used in their current format with research efforts focused on increasing sensitivity of case detection. Anti ubiquitin 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 neurite s represents a major methodologic advance since the first DLB workshop. alp ha-Synuclein-based methods are likely to be most useful in research laborat ories, particularly for clinicopathologic correlative studies. Conclusion: Clinical management of DLB patients usually centers on the treatment of non cognitive features. There is now a pressing need to establish appropriately designed randomized controlled trials in DLB. Collaboration between dement ia and movement disorder specialists is essential for rapid progress in res earch and clinical management protocols.