The International Lewy Body Dementia Conference closed its doors having brought together researchers and clinicians in the field from across the world, as well as people living with Lewy Body Disease (LBD) and their carers. I have to admit that I was initially sceptical about the choice of venue in Las Vegas, but the weather was pleasant (less than 40 degrees), and the facilities luxurious and surprisingly quiet; despite having to pass through the noisy and smoky casino to reach it, the conference centre was calm and organised.
The conference itself more than lived up to our expectations. The first day opened by James Leverenz, Cleveland clinic who highlighted the exciting developments in LBD since the last conference was held in 2017: the publication of new diagnostic criteria, the inclusion of LBD in the National Alzheimer’s Project, the DIAMOND Lewy programme and many others. Susan Schneider-Williams followed with a touching, personal account of the impact of the disease on her husband Robin Williams and herself as carer. She concluded by highlighting the areas of LBD where she felt more research is needed: improving accuracy of diagnosis, better symptom management and preventing suicide in people with LBD.
Following this inspirational opening was a series of plenary sessions that provided a thorough update on all aspects of LBD. Ian McKeith, Newcastle University, updated us on the diagnostic criteria for LBD. These were published in 2017 and have received 281 citations so far, being amongst the top 1% of papers in Neurodegeneration; this highlights the increased importance of LBD in neurodegeneration research. However, challenges still exist. Although both DSM V and ICD 11 recognise LBD separately from Parkinson’s disease (PD) they still include inconsistencies regarding the presentation and diagnosis of LDB. In DSM V for example, patients can meet the criteria for diagnosis for both DLB and PD dementia. Rectifying these inconsistencies in the next DSM and ICD will be an important challenge.
Taris Ferman, Mayo Clinic, followed giving a thorough update on cognition in LBD including predicting progression to LBD from Mild Cognitive Impairment and key differences in neuropsychology and imaging between LBD and Alzheimer’s disease (AD). Next, Daniel Weintraub, University of Pennsylvania, highlighted the importance of psychiatric symptoms in both LBD and PD dementia which is reflected in the updated diagnostic criteria and updated us on available treatment options. Jennifer Goldman, University of Chicago, then, updated us on motor symptoms in LBD; she highlighted the reduced prevalence of resting tremor in LBD compared to PD and the emergence of wearable technologies for quantifying and objectively monitoring motor symptoms. Finally she reported the results of a recent randomised controlled trial of zonisamide in LBD which showed improvement in UPDRS Part III with 50mg dose (compared to placebo, addition to levodopa) without worsening cognition.
Simon Lewis, University of Sydney, gave the last talk of the day on Sleep and Autonomic symptoms in LBD. He walked us through the phenomenology and diagnosis of REM sleep behaviour disorder (RBD) as well as the significant role of idiopathic RBD as a prodromal stage to synucleopathies. He highlighted the predictors of conversion to synucleopathy from iRBD: abnormal quantitative motor testing, objective motor examination, olfactory deficit, MCI, erectile dysfunction, abnormal DAT and abnormal colour vision.
After coffee and pastries, we continued our update on all things LBD with Kejal Kantarci, Mayo Clinic, talking about imaging. She highlighted the value of surrogate neuroimaging markers of synuclein pathology including DAT and SPECT and pointed the importance of better quantifying and recognising mixed AD and LBD pathology. Next, Douglas Galasko, University of California, discussed other biomarkers of LBD, including CSF and blood a-synuclein. Although these biomarkers have significantly improved and exciting developments like RTQuick are in the pipeline, there is need for standardisation before these can be used in practice. Debby Tsuang, University of Washington, then updated us on genetics focusing on the significant genetic correlation between AD and PD with Dementia with Lewy Bodies but the very small correlation between AD and PD; this provides further evidence for the importance of AD pathology in LBD. Dennis Dickson, Mayo Clinic, further expanded on this topic, including how the pathological criteria for LBD have changed and the recognition of new forms such as amygdala predominant LBD. Last but not least in this session, Jordan Gladman, NINDS and Angela Taylor, LBD Association, gave an update on the ADRD Summit giving us hopeful news of increased funding for research in LBD.
Following lunch, we proceeded to an engaging debate on Consensus criteria for Prodromal Dementia with Lewy Bodies. This included lively discussion on Biomarkers, Mild Cognitive Impairment, Delirium, Psychiatric presentations and RBD which will inform the work of the committee in the months to come.
The rest of the conference included 30 selected presentations on Clinical aspects, Imaging, Therapeutics, Biomarkers, Pathology, Genetics and Epidemiology. These are too many to do them justice in this report but they covered the breadth and depth of LBD at the highest of standards. Highlights included the emerging importance of visual dysfunction as a predictor of cognitive decline, novel neuroimaging techniques such as network lesion mapping and thalamic nuclei segmentations, and the development of new biomarkers such as neurofilament light and EEG.
The conference ended with another lively debate on Controversies. Firstly on whether patients with Dementia with Lewy Bodies and PD dementia should be included in the same clinical trials where John Paul Taylor, Newcastle argued against and Dag Aarsland, Kings College London arguing for. Then followed a debate on whether protein aggregates should be the primary focus for disease modifying therapies; Alberto Espay, University of Cincinnati, argued against and David Irwin, University of Pennsylvania, argued for this. All speakers made their arguments well and a thoughtful and interesting discussion followed with the audience with opinions remaining relatively divided until the end.
Overall, the ILBD meeting this year highlighted the extraordinary advancements in this field of neurodegeneration which has been for so long overlooked. Most importantly, it brought together clinicians and patients from across the globe in a beautiful venue, promoting further discussion and collaborations.
The next ILBD meeting will not be for another 2 years, but everyone in the field should watch for it!
|Top 5 highlights from ILBD 2019|
|1||The 2017 Revision of LBD criteria: Lumbar punctures are back in diagnosis of MS|
|2||The DIAMOND Lewy programme: Assessment toolkit available to download for free: https://research.ncl.ac.uk/diamondlewy/toolkitsvideos/|
|3||Zonisamide can be a useful add-on to levodopa for motor symptoms in LBD without cognitive side effects.|
|4||Prodromal LBD is a growing field including not only RBD but also MCI, late onset psychosis and possibly delirium.|
|5||New neuroimaging techniques and biomarkers are likely to improve diagnosis and patient stratification in LBD.|