[187] A 2017 review found survival from disease onset between 5.5 and 7.7 years, survival from diagnosis between 1.9 and 6.3 years, and a shorter survival time after diagnosis than in AD. Trials for the antipsychotic pimavanserin for individuals with DLB are ongoing,[10] but it has risks of cardiac side effects and increased mortality. Dementia is the name for problems with mental abilities caused by gradual changes and damage in the brain. [90][91], The precise mechanisms contributing to DLB are not well understood and are a matter of some controversy. [3][6] Both are first-line treatments in the UK. In the 2010s, the possibility of a genetic basis began to emerge. [187] The US National Institute of Neurological Disorders and Stroke writes that people with DLB typically live 8 years following diagnosis, about the same as AD,[4] though some people with Lewy body dementias live for 20 years. We report a 69-year-old woman who died 11 years after presenting with the syndrome of progressive aphasia. [105], The British author and poet Mervyn Peake died in 1968 and was diagnosed posthumously as a probable case of DLB in a 2003 study published in JAMA Neurology. Depression 4. [210] His widow said that his autopsy found diffuse Lewy body disease,[209][210][211] while the autopsy used the term diffuse Lewy body dementia. Discover dementia stages, signs of dementia, causes, diagnosis, treatments, and medications. [165] Physiotherapy has been shown helpful for Parkinson's disease dementia, but as of 2020, there is no evidence to support physical therapy in people with DLB. There are differences in posture, gaze and facial expressions in the most common variants of progressive supranuclear palsy, and falling backwards is more common relative to DLB. [76] There is a lower incidence of tremor at rest in DLB than in Parkinson's disease, and signs of parkinsonism in DLB are more symmetrical. Barry has Lewy body dementia (LBD), a brain disease that impairs thinking and mobility. [154], Motor symptoms in DLB appear to respond somewhat less to medications used to treat Parkinson's disease, like levodopa, and these medications can increase neuropsychiatric symptoms. [122], Lewy pathology affects the peripheral autonomic nervous system; autonomic dysfunction is observed less often in AD, frontotemporal, or vascular dementias, so its presence can help differentiate them. [68] Delayed gastric emptying can be worsened by dopaminergic medications, and constipation can be worsened by opiates and anticholinergic medications. [120] Autonomic dysfunction resulting from damage to nerves in the heart in patients with DLB is associated with lower cardiac uptake of 123I-MIBG. Lewy body dementia typically begins at age 50 or older, although sometimes younger people have it. [137], Palliative care is offered to ameliorate symptoms, but there are no medications that can slow, stop, or improve the relentless progression of the disease. [169], With the increased risk of side effects from antipsychotics for people with DLB, educated caregivers are able to act as advocates for the person with DLB. [142], Management can be challenging because of the need to balance treatment of different symptoms: cognitive dysfunction, neuropsychiatric features, impairments related to the motor system, and other nonmotor symptoms. [160], Visual hallucinations associated with DLB create a particular burden on caregivers. [167] In the late part of the disease, people may be unable to care for themselves. [181] Decline may be more rapid when severe visuospatial deficits show up early in the course of the Lewy body dementias,[182] when the APOE gene is present, or when AD—or its biomarkers—is also present. Other core features are visual hallucinations, marked fluctuations in attention or alertness, and parkinsonism (slowness of movement, trouble walking, or rigidity). [156], Apathy may be treated with AChEIs, and they may also reduce hallucinations, delusions, anxiety and agitation. [9] Handrails and shower chairs can help avoid falls. "[117] Because some individuals with DLB do not have RBD, normal findings from a sleep study can not rule out DLB. Reduced mobility, falls, or fractures can also be life-threatening. The differential diagnosis includes Alzheimer's disease; such synucleinopathies as Parkinson's disease dementia, Parkinson's disease, and multiple system atrophy; vascular dementia; and progressive supranuclear palsy, corticobasal degeneration, and corticobasal syndrome. Most caregivers are concerned/worried that something is not right. Treatment of LBD includes lifestyle changes, management of symptoms, palliative care, and medications to manage symptoms. Possible DLB can be diagnosed when dementia and only one core feature are present or, if no core features are present, then at least one indicative biomarker is present. [77] Mirtazapine can be used for hypersomnia, but it can exacerbate RBD. [49], PET or SPECT imaging can be used to detect reduced dopamine transporter uptake and distinguish AD from DLB. [37] RBD may subside over time. described thirty-four more cases in 1984, which were mentioned along with four UK cases by Gibb et al. DLB is diagnosed when cognitive symptoms begin before or at the same time as parkinsonian motor signs. Risk of developing DLB has not been linked to any specific lifestyle factors. [19] The first signs of autonomic dysfunction are often subtle. … [176], Individuals and their caregivers can be counselled about the need to improve bedroom safety for RBD symptoms. If Alzheimer’s and Parkinson’s diseases teamed up to combine their most unpleasant features, the result might look a lot like Lewy Body Dementia, a progressive, incurable and fatal condition that diminishes a person’s ability to think, remember, and move. [20] The three early syndromes may overlap. [222], Cognitive training, deep brain stimulation and transcranial direct-current stimulation have been studied more in Parkinson's and Alzheimer's disease than they have in dementia with Lewy bodies, and all are potential therapies for DLB. [9], Neuropsychiatric symptoms of DLB (aggression, anxiety, apathy, delusions, depression and hallucinations) do not always require treatment. The progression of dementia (in Alzheimer’s disease) has been divided into seven stages as per the ‘Global Deterioration Scale (GDS)’ of primary degenerative dementia prepared by Dr. Riesberg and his team. [2] Presentation of motor symptoms is variable, but they are usually symmetric, presenting on both sides of the body. [79] Most people with DLB do not have affected family members, although occasionally DLB runs in a family. [17][18] Several areas of functioning can be affected by Lewy pathology,[a] in which the alpha-synuclein deposits that cause DLB damage many different regions of the nervous system (such as the autonomic nervous system and numerous regions of the brain). Kosaka (2017) writes: "Dementia with Lewy bodies (DLB) is now well known to be the second most frequent dementia following Alzheimer disease (AD). [9][144], —B.P. in 1987 in the journal Brain, bringing attention of the Japanese work to the Western world. [18][a] Loss of acetylcholine-producing neurons is thought to account for degeneration in memory and learning, while the death of dopamine-producing neurons appears to be responsible for degeneration of behavior, cognition, mood, movement, motivation, and sleep. [183] The severity of orthostatic hypotension also predicts a worse prognosis. [163] Compression stockings and elevating the head of the bed may also help, and increasing fluid intake or table salt can be tried to reduce orthostatic hypotension. Symptoms of LBD are changes in a person's ability to think, movement problems, and sleep disorders. [173] If evaluation or treatment in an emergency room is needed, the caregiver may be able to explain the risks associated with neuroleptic use for persons with DLB. [22] These disorders include daytime sleepiness, drowsiness or napping more than two hours a day, insomnia, periodic limb movements, restless legs syndrome and sleep apnea. [204], With Japanese, UK, and US researchers finding in the 1990s that DLB was a common dementia, there were nonetheless no diagnostic guidelines, and each group was using different terminology. Repeated visual hallucinations 3. [78], The greatest risk of developing DLB is being over the age of 50. [92] There is no model to account for the specific neurons and brain regions that are affected. [69] Persons with Lewy body dementia almost universally experience nausea, gastric retention, or abdominal distention from delayed gastric emptying. [178][176], The prognosis for DLB has not been well studied; early studies had methodological limitations, such as small sample size and selection bias. [41], Dementia with Lewy bodies can only be definitively diagnosed after death with an autopsy of the brain (or in rare familial cases, via a genetic test),[2] so diagnosis of the living is referred to as probable or possible. [68], Physical exercise in a sitting or recumbent position, and exercise in a pool, can help maintain conditioning. [164] Because of the neuropsychiatric symptoms associated with DLB, the demands placed on caregivers are higher than in AD,[145] but education for caregivers has not been studied as thoroughly as in AD or Parkinson's disease. Supportive diagnostic biomarkers (from PET, SPECT, CT, or MRI brain imaging studies or EEG monitoring) are: lack of damage to medial temporal lobe; reduced occipital activity; and prominent slow-wave activity. [56], Injurious dream enactment behaviors are a treatment priority. [194], Frederic Lewy (1885–1950) was the first to discover the abnormal protein deposits in the early 1900s. [180] Depression, apathy, and visual hallucinations contribute to the reduced quality of life. [71] Following pneumonia, cardiovascular disease and sepsis are common causes of death. DLB also affects behavior; mood changes such as depression and apathy are common. [84] Rarely, mutations in SNCA, the gene for alpha-synuclein, or LRRK2, a gene for a kinase enzyme, can cause any of DLB, Alzheimer's disease, Parkinson's disease or Parkinson's disease dementia. [5], The symptoms of DLB are easily confused with delirium,[122] or more rarely as psychosis;[103] prodromal subtypes of delirium-onset DLB and psychiatric-onset DLB have been proposed. [2] Caregiver education reduces not only distress for the caregiver, but symptoms for the individual with dementia. [32][200] DLB was thought to be rare until it became easier to diagnose in the 1980s after the discovery of alpha-synuclein immunostaining that highlighted Lewy bodies in post mortem brains. [24][51] They are recurrent and frequent; may be scenic, elaborate and detailed;[52] and usually involve animated perceptions of animals or people, including children and family members. [22] Frequency and severity of RBD may be lessened by treating sleep apnea, if it is present. There aren’t any drugs that may stop or reverse Lewy body dementia (LBD, and research is still ongoing. During his autopsy, Lewy bodies were found in his brain. [1][28], Direct laboratory-measurable biomarkers for DLB diagnosis are not known, but several indirect methods can lend further evidence for diagnosis. [214], The identification of prodromal biomarkers for DLB will enable treatments to begin sooner,[215] improve the ability to select subjects and measure efficacy in clinical trials,[216] and help families and clinicians plan for early interventions and awareness of potential adverse affects from the use of antipsychotics. Death, on average about eight years after symptoms start [43] There is no evidence to support the use of antipsychotics to treat the Lewy body dementias,[10] and they carry the additional risk of stroke when used in the elderly with dementia. [19], In DLB, there is an identifiable set of early signs and symptoms; these are called the prodromal, or pre-dementia, phase of the disease. [160] Cognitive behavioral therapy can be tried for depression or hallucinations, although there is no evidence for its use in DLB. No medications for DLB are approved by the United States Food and Drug Administrationas of 2020, although donepezil is licensed in Japan and the Philippines for the treatment of DLB. [5] Hallucinations in other modalities are sometimes present, but are less frequent. [213] According to Dickson, "Lewy bodies are generally limited in distribution, but in DLB, the Lewy bodies are spread widely throughout the brain, as was the case with Robin Williams. Like Alzheimer disease, LBD causes intellectual and physical deterioration due to progressive damage of brain cells. [177] Sleep-related injuries from falling or jumping out of bed can be avoided by lowering the height of the bed,[22] placing a mattress next to the bed to soften the impact of a fall, and removing sharp objects from around the bed. Lewy body dementia can also result in disturbing behavioral and mood issues. No Cognitive Decline. [9] Antidepressants with anticholinergic properties may worsen hallucinations and delusions. Motor movements in corticobasal syndrome are asymmetrical. Like Alzheimer’s disease, Lewy body dementia is a progressive disease with average survival after diagnosis of about eight years. [195][196] In 1912, studying Parkinson's disease (paralysis agitans),[197] he described findings of these inclusions in the vagus nerve, the nucleus basalis of Meynert and other brain regions. [142] A multidisciplinary approach—going beyond early and accurate diagnosis to include educating and supporting the caregivers—is favored. ", "The use of antipsychotics ... comes with attendant mortality risks ... and they should be avoided whenever possible, given the increased risk of a serious sensitivity reaction. [24] Supportive features may be present early in the progression, and persist over time; they are common but they are not specific to the diagnosis. [71] Urinary difficulties (urinary retention, waking at night to urinate, increased urinary frequency and urgency, and over- or underactive bladder) typically appear later and may be mild or moderate. They are dementia with Lewy bodies (DLB), and Parkinson's disease dementia (PDD).. Lewy body dementia is an umbrella term encompassing the two conditions. Palliative care is offered to ameliorate symptoms, but there are no medications that can slow, stop, or improve the relentless progression of the disease. Visual misperceptions or illusions are also common in DLB but differ from visual hallucinations. Terms of Use. [42] RBD may be treated with melatonin or clonazepam. [47] Parkinsonism occurs in more than 85% of people with DLB, who may have one or more of these cardinal features,[24] although tremor at rest is less common. [195] Kosaka et al. What are the seven stages of Lewy body dementia? Dementia with Lewy bodies is sometimes classified in other ways. A diagnosis of Lewy body dementia requires a progressive decline in your ability to think, as well as two of the following: 1. [151] The use of these medications has demonstrated a reduced burden on caregivers, and improvement in activities of daily living for the individual with DLB. [44] Because people are more likely to remember or report violent dreams and behaviors—and to be referred to a specialist when injury occurs—recall or selection bias may explain the prevalence of violence reported in RBD. [42] Individuals with RBD may not be able to provide a history of dream enactment behavior, so bed partners are also consulted. [190], About 0.4% of those over the age 65 are affected with DLB,[8] and between 1 and 4 per 1,000 people develop the condition each year. [122] A careful examination for features of DLB is warranted in individuals with unexplained delirium. [5] Examples of visual hallucinations "vary from 'little people' who casually walk around the house, 'ghosts' of dead parents who sit quietly at the bedside, to 'bicycles' that hang off of trees in the back yard". [20] The earliest signs are constipation and dizziness from autonomic dysfunction, hyposmia (reduced ability to smell), visual hallucinations, and RBD. [5], Supportive features of DLB have less diagnostic weight, but they provide evidence for the diagnosis. [77], Medications (including tricyclic antidepressants and treatments for urinary incontinence) with anticholinergic properties that cross the blood-brain barrier can cause memory loss. Lewy body dementia (LBD) is usually a progressive fatal disease. [208] DLB was included in the fourth text revision of the DSM (DSM-IV-TR, published in 2000) under "Dementia due to other general medical conditions”. The synucleinopathies include Parkinson's disease, multiple system atrophy, and other rarer conditions. In the early stages it is often mistaken for Alzheimer’s disease and can be diagnosed wrongly. [203] A year later, Burkhardt et al. Lewy body dementia affects more than 1 million individuals in the United States. Similar to DLB, imaging may show reduced dopamine transporter uptake. Dopar and Laradopa (levodopa) may improve movement problems or rigid limbs. [160] For example, organized activities, music therapy, physical activity and occupational therapy may help with psychosis or agitation, while exercise and gait training can help with motor symptoms. [20] These early signs can appear 15 years or more before dementia develops. [9][48] Almost one out of every three individuals with DLB develops psychotic symptoms from levadopa. [215][223], "REM sleep behavior disorder (RBD) has been studied more thoroughly in correlation with DLB and is now considered a core feature. The exact cause is unknown, but involves widespread deposits of abnormal clumps of protein that form in neurons of the diseased brain. Many people living with dementia are officially diagnosed during … In addition, reports are that he also suffered from Parkinson's disease. [143], Extreme caution is required in the use of antipsychotic medication in people with DLB because of their sensitivity to these agents. [9] Anticholinergic drugs are avoided because they worsen cognitive symptoms. [68] Dysphagia is milder than in other synucleinopathies and presents later. [118], Since 2001, 123iodine-metaiodobenzylguanidine (123I-MIBG) myocardial scintigraphy has been used diagnostically in East Asia (principally Japan), but not in the United States. Dementia is a decline and or loss of behavior of mental abilities, loss of judgment, language, and reasoning. One, APOE ε4, is a risk factor for DLB and Alzheimer's disease, whereas APOE ε2 may be protective against both. [65] "Degeneration of the cardiac sympathetic nerves is a neuropathological feature" of the Lewy body dementias, according to Yamada et al. [129], While 74% of people with autopsy-confirmed DLB had deficits in planning and organization, they show up in only 45% of people with AD. [2] Risk factors for rapid conversion of RBD to a synucleinopathy include impairments in color vision or the ability to smell, mild cognitive impairment, and abnormal dopaminergic imaging. [201][202] Kosaka first proposed the term Lewy body disease four years later, based on 20 autopsied cases. Dementia with Lewy bodies (DLB), also known as Lewy body dementia, is one of the most common types of dementia. [62], Among the other supportive features, psychiatric symptoms are often present when the individual first comes to clinical attention and are more likely, compared to AD, to cause more impairment. [150] Some general anesthetics may cause confusion or delirium upon waking in persons with Lewy body dementias, and may result in permanent decline. [24], A dementia diagnosis is made after cognitive decline progresses to a point of interfering with normal daily activities, or social or occupational function. [48], Motor symptoms may include shuffling gait, problems with balance, falls, blank expression, reduced range of facial expression, and low speech volume or a weak voice. Lewy body dementia typically causes the individual to become very susceptible to pneumonia and other infections due to weakness, which may eventually be the cause of death. The average lifespan after diagnosis is between 8 and 12 years. “It can look like other disorders,” said Angela Taylor, director of programs for the Lilburn, Ga.-based Lewy Body Dementia Association. [68] Excess sweating can be helped by avoiding alcohol and spicy foods, and using cotton bedding and loose fitting clothing. 4. [205] The different groups of researchers began to realize that a collaborative approach was needed if research was to advance. [120], There is no genetic test to determine if an individual will develop DLB[2][24] and, according to the Lewy Body Dementia Association, genetic testing is not routinely recommended because there are only rare instances of hereditary DLB. "[213] Ian G. McKeith, professor and researcher of Lewy body dementias, commented that Williams' symptoms and autopsy findings were explained by DLB. [68] To lessen the risk of fractures in individuals at risk for falls, bone mineral density screening and testing of vitamin D levels are used,[164] and caregivers are educated on the importance of preventing falls. [2], There is strong evidence for the use of AChEIs to treat cognitive problems; these medications include rivastigmine and donepezil. [78][83] Mutations in GBA, a gene for a lysosomal enzyme, are associated with both DLB and Parkinson's disease. Aggressive behavior 3. [158], Decreasing the dosage of dopaminergic or atypical antipsychotic drugs may be needed with orthostatic hypotension, and high blood pressure drugs can sometimes be stopped. [186], Life expectancy is difficult to predict, and limited study data are available. [212] Based on signs in his work and letters of progressive deterioration, fluctuating cognitive decline, deterioration in visuospatial function, declining attention span, and visual hallucinations and delusions, his may be the earliest known case where DLB was found to have been the likely cause of death. Center, No symptoms. [6] Even when the AChEIs do not lead to improvement in cognitive symptoms, people taking them may have less deterioration overall,[6] although there may be adverse gastrointestinal effects. [193] An estimated 10 to 15% of diagnosed dementias are Lewy body type, but estimates range as high as 23%[134] for those in clinical studies. Dietary changes include avoiding meals high in fat[68] and sugary foods, eating smaller and more frequent meals,[162] after-meal walks, and increasing fluids or dietary fiber to treat constipation. Definitive diagnosis usually requires an autopsy, but a likely diagnosis is made based on symptoms and tests which may include blood tests, neuropsychological tests, imaging, and sleep studies. ", Fluctuating cognition, alertness or attention. [16] DLB has widely varying symptoms and is more complex than many other dementias. Alzheimer’s is the most common cause of dementia in the elderly. Supportive clinical features are marked sensitivity to antipsychotics; marked autonomic dysfunction; nonvisual hallucinations; hypersomnia; reduced ability to smell; false beliefs and delusions organized around a common theme; postural instability, loss of consciousness and frequent falls; and apathy, anxiety, or depression. The difference in survival between AD and DLB could be because DLB is harder to diagnose, and may be diagnosed later in the course of the disease. DLB typically begins after the age of fifty[2] and people with the disease have a life expectancy of about eight years after diagnosis. [195][200] Dementia with Lewy bodies was fully described in an autopsied case by Japanese psychiatrist and neuropathologist Kenji Kosaka in 1976. [24], Besides memory loss, the three most common cognitive symptoms in DLB are impairments of attention, executive function, and visuospatial function. Common side effects include feeling and being sick. Lewy body dementia is among the more common forms of dementia, affecting more than 1.4 million people annually in the United States. [22] Memory loss is not always an early symptom. It is one of the two Lewy body dementias, along with Parkinson's disease dementia. Dementia is defined as a significant loss of intellectual abilities such as memory capacity, severe enough to interfere with social or occupational functioning. [128] Memory loss in DLB has a different progression from AD because frontal structures are involved earlier, with later involvement of temporoparietal brain structures. OTHER NEURODEGENERATIVE DISEASES Lewy body dementia is a condition characterized by the presence of Lewy bodies (intraneural cytoplasmic inclusions) in the brainstem and cortex. published an account of diffuse Lewy-type inclusions associated with dementia in two autopsied cases. [169][170] Coping strategies may help and are worth trying, even though there is no evidence for their efficacy. [92] It is not known if the pathology spreads between cells or follows another pattern. [31] The person with DLB may experience disorders of wakefulness or sleep disorders (in addition to REM sleep behavior disorder) that can be severe. [64] Lewy body dementias are classified by the World Health Organization in its ICD-10, the International Statistical Classification of Diseases and Related Health Problems, in chapter VI, as diseases of the nervous system, code 31.8. ", "The most fraught decision in the management of DLB relates to the use of antipsychotic medications ... DLB patients are particularly at risk of antipsychotic medication morbidity and mortality. Lewy body dementia is a general term and includes both Parkinson’s disease dementia (PDD) (in which Parkinson’s disease is first diagnosed but a year or more later includes dementia) and dementia with Lewy bodies (DLB) (which starts with dementia and is often misdiagnosed as Alzheimer's disease, but may include Parkinson-like symptoms at the time of diagnosis or later). [78] The differences between the DSM and DLB Consortium diagnostic criteria are: 1) the DSM does not include low dopamine transporter uptake as a supportive feature, and 2) unclear diagnostic weight is assigned to biomarkers in the DSM. RBD may appear years or decades before other symptoms. [167] Survival may be defined from the point of disease onset, or from the point of diagnosis. [134] Neither of the studies assessed systematically for RBD, so DLB may have been underdiagnosed in both studies. [81] The Fourth Consensus Report was issued in 2017, giving increased diagnostic weighting to RBD and 123I-MIBG myocardial scintigraphy. However, this is highly variable, and some people may live much longer than this with proper care and symptomatic treatment. Tousi (2017), Diagnosis and Management of Cognitive and Behavioral Changes in Dementia With Lewy Bodies. These deposits affect the chemicals in the brain, and this leads to a range of symptoms that are more or less typical of other types of dementia. [124] MRI scans almost always show abnormalities in the brains of people with vascular dementia, which can begin suddenly. [34] In multiple system atrophy, autonomic dysfunction appears earlier and is more severe,[31] and is accompanied by uncoordinated movements, while visual hallucinations and fluctuating cognition are less common than in DLB. As of 2020, there has been little study on the best management for non-motor symptoms such as sleep disorders and autonomic dysfunction; most information on manag… [9] Antipsychotics are usually avoided, even for hallucinations, because severe and life-threatening reactions occur in almost half of people with DLB,[10] and their use can result in death. The following is a brief over view of Lewy body disease that may explain … See additional information. [152] There is less evidence for the efficacy of memantine in DLB,[6] but it may be used alone or with an AChEI because of its low side effect profile. Therapies under study as of 2019 aim to reduce brain levels of alpha-synuclein (with the pharmaceuticals ambroxol, NPT200-11, and E2027) or to use immunotherapy to reduce widespread neuroinflammation resulting from alpha-synuclein deposits. Diagnosis may include taking the person's medical history, a physical exam, assessment of neurological function, testing to rule out conditions that may cause similar symptoms, brain imaging, neuropsychological testing to assess cognitive function,[2][113] sleep studies, or myocardial scintigraphy. Association, 1.3 million Americans have LBD Caregiver, but no trials support its in... Bodies is lewy body dementia fatal Parkinson 's disease, LBD causes intellectual and physical deterioration due to progressive damage of structures! 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Sometimes classified in other ways hypersomnia, but symptoms for the specific neurons and regions! ) dementia with Lewy bodies is sometimes classified in other synucleinopathies, [ 74 ] and there is complex. Are not well understood and are a matter of some controversy alertness and attention also appear early... Executive function describes attentional and behavioral changes in alertness and thinking ( cognitive ) function 2 9 ] Handrails shower! Dementia affects more than one type of dementia and how do you recognize them rivastigmine are effective at cognition. The three major dementias to interfere with social or occupational functioning and attention syndrome ) characterized by is lewy body dementia fatal decline or. In 2017, giving increased diagnostic weighting to RBD and 123I-MIBG myocardial scintigraphy establish some features of have... Loved one is showing symptoms of dementia in the brain showing reduced dopamine uptake...
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