Angela Taylor

Diagnosis and management of dementia with Lewy bodies Fourth consensus report of the DLB Consortium

The Dementia with Lewy Bodies (DLB) Consortium has refined its recommendations about the clinical and pathologic diagnosis of DLB, updating the previous report, which has been in widespread use for the last decade. The revised DLB consensus criteria now distinguish clearly between clinical features and diagnostic biomarkers, and give guidance about optimal methods to establish and interpret these. Substantial new information has been incorporated about previously reported aspects of DLB, with increased diagnostic weighting given to REM sleep behavior disorder and 123iodine-metaiodobenzylguanidine (MIBG) myocardial scintigraphy. The diagnostic role of other neuroimaging, electrophysiologic, and laboratory investigations is also described. Minor modifications to pathologic methods and criteria are recommended to take account of Alzheimer disease neuropathologic change, to add previously omitted Lewy-related pathology categories, and to include assessments for substantia nigra neuronal loss. Recommendations about clinical management are largely based upon expert opinion since randomized controlled trials in DLB are few. Substantial progress has been made since the previous report in the detection and recognition of DLB as a common and important clinical disorder. During that period it has been incorporated into DSM-5, as major neurocognitive disorder with Lewy bodies. There remains a pressing need to understand the underlying neurobiology and pathophysiology of DLB, to develop and deliver clinical trials with both symptomatic and disease-modifying agents, and to help patients and carers worldwide to inform themselves about the disease, its prognosis, best available treatments, ongoing research, and how to get adequate support.

Lewy Body Dementia: Caregiver Burden and Unmet Needs

Lewy body dementia (LBD) is a common cause of dementia but to date, little is known about caregiver burden. The Lewy Body Dementia Association (www.LBDA.org) conducted a web-based survey of 962 caregivers (mean age 56 y; 88% women). The most common initial symptoms were cognitive (48%), motor (39%), or both (13%). Caregivers expressed concerns about fear of future (77%), feeling stressed (54%), loss of social life (52%), and uncertainty about what to do next (50%). Caregivers reported moderate-to-severe burden; 80% felt the people around them did not understand their burden and 54% reported feelings of isolation with spousal caregivers reporting more burden than nonspousal caregivers. Only 29% hired in-home assistance, whereas less than 40% used respite or adult day care, geriatric case managers, or attended a support group meeting. Lack of service utilization occurred despite two-thirds of caregivers reporting medical crises requiring emergency services, psychiatric care, or law enforcement. Caregivers reported preferences for web-based information, directories of LBD expert providers, information on LBD research, and location of local support groups. These findings highlight significant unmet needs for LBD caregivers and provide targets for intervention to reduce caregiver burden. Community resources such as the Lewy Body Dementia Association may serve this end, while also providing practical information and support for caregivers.

Lewy body dementia: The caregiver experience of clinical care

BACKGROUND Lewy body dementia (LBD) is the second most common cause of dementia, however, little is known about how the clinical diagnosis of LBD is obtained in the community or the caregiver experience while seeking the diagnosis. METHODS The Lewy Body Dementia Association (www.LBDA.org) conducted a web-based survey of 962 caregivers over a 6-month period. RESULTS The mean age of respondents was 55.9y; 88% were female and 64% had daily contact with patients. The mean age of LBD patients was 75.4y; 62% were male and 46% lived with a caregiver. The most common presentation of symptoms as reported by LBD caregivers was cognitive (48%), motor (39%) or both (13%). The first diagnoses given to the patients were Parkinson disease or other movement disorder (39%), Alzheimer disease or other cognitive disorder (36%), or mental illness (24%). Fifty percent of patients saw >3 doctors for more than 10 visits over the course of 1 year before an LBD diagnosis was established. Neurologists diagnosed most cases (62%), while primary care providers diagnosed only 6% of cases. No differences were found between the presentation of disease and the number of physicians, number of office visits, length of time to establish diagnosis, or type of doctor who finally made an LBD diagnosis. Caregivers viewed physicians as knowledgeable about disease manifestations and treatment options, but not about disease course/prognosis and available community resources and referrals. CONCLUSIONS These data highlight a need for increasing physician awareness and knowledge of LBD, which will facilitate accurate diagnosis and treatment. Community resources such as the Lewy Body Dementia Association may serve this end, while also providing practical information and support for caregivers.

Arguing against the proposed definition changes of PD.

As members of the Lewy Body Dementia Association Scientific Advisory Council, we aim to address some of the issues raised in the article titled “Time to Redefine PD? Introductory Statement of the MDS Task Force on the Definition of Parkinsons Disease.” In particular, we suggest that the 1‐year rule distinguishing Parkinsons disease dementia from dementia with Lewy bodies is worth maintaining because it serves an important purpose in clinical practice and clinical and basic science research and when helping the lay community understand the complexity of these different clinical phenotypes. Furthermore, we believe that adding an additional diagnostic label, “PD (dementia with Lewy bodies subtype),” will confuse rather than clarify the distinction between dementia with Lewy bodies and PD or PD dementia, and will not improve management or expedite therapeutic development. We present arguments supporting our contentions.

Axenic culture of the rodent tapeworms Hymenolepis dìminuta and H. nana.

Abstract 1. 1. Cysticercoid larvae of Hymenolepis diminuta have been maintained in vitro for 6 days at 25 ° C in simple media containing proteose peptone, Ringers solution, and glucose. The larvae were still infective to rats at the end of this time. When rat serum was added to the medium the larvae remained infective for 6 days at 30 ° C but not at 25 ° C. 2. 2. Cysticercoid larvae of H. nana survived for 5 days in vitro at 25 ° C in a simple salt solution with glucose and they were still infective to mice at the end of this period. 3. 3. The early beetle stages of H. diminuta and H. nana were maintained in a healthy condition in vitro for 7 days and some development was obtained with H. diminuta larvae (30 ° C). 4. 4. The juveniles of H. diminuta have been kept alive for 7 days in vitro at 38 ° C in a mixture of horse serum and medium 199; they retained their infectivity to rats only for the first 4 days. 5. 5. The juveniles of H. nana have survived for 9 days in vitro at 38 ° C in a mixture of horse serum and medium 199 supplemented with amino acids; they retained their infectivity to mice for the first 8 days when the horse serum was replaced by calf serum. Some growth in length occurred when an extract of mouse intestine was added to the medium.

Alzheimer's Disease-Related Dementias Summit 2016: National research priorities

Goal 1 of the National Plan to Address Alzheimer’s Disease is to prevent and effectively treat Alzheimer disease and Alzheimer disease–related dementias by 2025. To help inform the research agenda toward achieving this goal, the NIH hosts periodic summits that set and refine relevant research priorities for the subsequent 5 to 10 years. This proceedings article summarizes the 2016 Alzheimers Disease–Related Dementias Summit, including discussion of scientific progress, challenges, and opportunities in major areas of dementia research, including mixed-etiology dementias, Lewy body dementia, frontotemporal degeneration, vascular contributions to cognitive impairment and dementia, dementia disparities, and dementia nomenclature.

Cause of Death and End-of-Life Experiences in Individuals with Dementia with Lewy Bodies: END OF LIFE IN DEMENTIA WITH LEWY BODIES

To investigate the natural history, cause of death, and end‐of‐life experiences of individuals diagnosed with dementia with Lewy bodies (DLB).

THE LEWY BODY DEMENTIA ASSOCIATION RESEARCH CENTERS OF EXCELLENCE PROGRAM: TOWARD OPTIMIZING CLINICAL CARE AND CLINICAL TRIAL INFRASTRUCTURE

Age of Conversion to DLB 77.6 68.2 75.3 68.2 78.3 67.8 81.2 67.7 Years between Baseline and DLBConversion 3.1 62.2 1.3 60.4 3.1 60.9 7.3 6 1.7 Education 15.8 63.7 16.1 63.4 15.8 63.8 15.2 64.0 Sex 71.2% Male 75.3% Male 65.1% Male 76.7% Male Age of Cognitive Decline 71.7 68.8 70.2 69.0 72.1 68.2 74.0 69.8 Age of Behavioral Decline 71.2 6 10.4 64.5 62.6 66.5 6 8.9 80.3 6 8.4 Age of Motor Function Decline 72.1 68.9 66.3 62.9 67.8 67.0 79.4 67.9