Often forgotten and misdiagnosed Lewy body dementia

  Within the spectrum of dementia syndromes, dementia with Lewy bodies (DLB) is arguably the most clinically dramatic, most misdiagnosed, and relatively recently singled out type of dementia. From the name of DLB, it can be inferred that Lewy Body (LB) is one of its main pathological features. These round, homogeneous microscopically pink vesicles were first identified and named by a British neurologist, Friedrich Lewy, in 1913 in the midbrain substantia nigra of patients with Parkinson’s disease and were later confirmed to be one of the most important pathological features of Parkinson’s disease (see figure below).  In 1961, the Japanese scholar Okazak also observed the presence of Lewy’s vesicles in large numbers in the cerebral cortex and brainstem of a group of Alzheimer’s patients with significant extrapyramidal and psychobehavioral symptoms, and first proposed the concept of DLB. Given that the clinical symptoms and pathological features of these patients overlap between Alzheimer’s disease and Parkinson’s disease, it has been named in a variety of ways, including Alzheimer’s disease Lewy body variant, Alzheimer’s disease with Parkinson’s changes, dementia with cortical Lewy body disease, diffuse Lewy body disease, etc., until 1996 when the “International Working Group on Dementia with Lewy Bodies “It was not until 1996 that the International Working Group on Dementia with Lewy Bodies (IWGLLB), in its joint report, unified the clinical manifestations of progressive dementia, extrapyramidal symptoms and fluctuating mental behavior abnormalities under the name of dementia with Lewy bodies (DLB).  It is now internationally recognized that DLB is a common type of dementia second only to Alzheimer’s disease among neurodegenerative dementias, accounting for approximately 15% to 25% of post-mortem dementia patients, second only to Alzheimer’s disease. Despite the lack of authoritative incidence statistics, the prevalence of DLB in Chinese people over 60 years of age should be between 0.11% and 1.18%, and the clinical prevention and treatment situation of DLB is quite serious due to the huge population base, the increasing life expectancy of the population and the accelerating trend of aging in China!  Although the distinctive symptoms of DLB do not seem to be easily misdiagnosed just from its clinical features and the latest international diagnostic criteria (see the chart below). Unfortunately, however, the misdiagnosis rate of DLB is high both nationally and internationally. A recent medical care survey sponsored by the DLB Association showed that close to 80% of DLB patients were misdiagnosed with other neurological disorders at the first visit, and more than half of them were misdiagnosed with Alzheimer’s disease.  There are five reasons for the low detection rate and high misdiagnosis of DLB: 1. Public awareness is extremely low. Especially in the background that most domestic people still have difficulty in calling Alzheimer’s disease by its full name, public awareness of DLB is almost a blank, and at the same time, the widespread phenomenon of “attaching importance to physical symptoms and neglecting cognitive symptoms” has led to A large number of DLB patients have missed the opportunity of early diagnosis.  2. The consultation mechanism is too diffuse and spontaneous, and the psychology of seeking quick results leads DLB patients to change hospitals, specialties and doctors frequently, and they cannot receive systematic, continuous and dynamic observation, which also makes the clinical information of DLB patients with variable and diverse clinical symptoms become fragmented. Survey results, also from the DLB Association, show that patients see an average of three physicians before being properly diagnosed, and 15% of patients have seen more than five physicians. Only 27% of patients are correctly diagnosed within 3 months of symptom onset, and more than 50% of patients take more than a year to be correctly diagnosed, with 20% of those diagnosed after 3 years.  3. Many clinicians lack both the knowledge of DLB and the psycho-psychological expertise and skills necessary for diagnosis. Since patients often have real and vivid visual hallucinations, fluctuating cognitive impairment and Parkinson’s-like symptoms, non-specialists without special training are either overwhelmed by them, or misdiagnose them as various psychiatric disorders and push them to psychiatrists or misdiagnose them as refractory Parkinson’s disease and keep changing anti-Parkinson’s drugs, and even a considerable number of family members and doctors suspect that DLB patients are pretending to be sick.  4.The equipment and treatment level of domestic medical institutions vary, especially in remote and backward rural areas, and there is a lack of appropriate professionals and examination equipment (such as MRI, PET, SPECT, etc.).  5. In addition, because the etiology and pathogenesis of DLB are not very clear, there is still a big controversy in academic circles about the understanding of DLB. In particular, the diagnostic criteria for DLB are many and complex, and many early symptoms (such as decreased attention, poor sleep status, and delayed movement) are easily ignored by patients and families, resulting in poor clinical practicability of diagnostic criteria is also one of the reasons for misdiagnosis and underdiagnosis.   In view of the above reasons, strengthening public awareness of DLB and training of professional knowledge in the industry through various forms will greatly improve the phenomenon of underdiagnosis and misdiagnosis, and increase the rate of early diagnosis. Since the earlier the diagnosis is confirmed, the better the management of the disease, it is important for patients to have a specialist who is familiar with dementia and DLB to follow them through the entire process. In order to maximize the use of existing medical resources and save medical costs, it is necessary to set up a national DLB diagnosis center with regional branches, establish a hierarchy of diagnosis experts and a shareable patient information database, and make full use of the developed Internet network and visual communication tools to establish a remote consultation mechanism. At the same time, for patients with clinically suspected DLB, clinical information should be collected from caregivers as much as possible, and a uniform caregiver questionnaire should be created and distributed to help confirm early diagnosis. Of course, the solution to the high misdiagnosis rate of DLB also depends on a more comprehensive, clearer and unified understanding of its etiology, pathology, pathogenesis, natural evolution of the disease and clinical symptoms by the academic community, and it is imperative for the government to increase the investment in scientific research in this field.  (Note: The hallucinations of Lewy body dementia are distinct and vivid, mainly visual hallucinations, often disturbing and even terrifying hallucinations. Patients are convinced of their hallucinations and often have expressions, movements and emotional reactions that match the content of the hallucinations. (For example, when the patient describes that someone is breaking into the house in the middle of the night, the patient will show frightened expressions and resistance or escape movements, which often make the family or the elderly in the same dormitory fearful, bored and miserable!)