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Lewy Body Dementia Diagnostic Criteria

When Should I See My Healthcare Provider About Lewy Body Dementia

Updated Diagnostic Criteria and Management of Lewy Body Dementia

If you or a loved one are experiencing symptoms of Lewy body dementia, talk to your healthcare provider.

If youve been diagnosed with Lewy body dementia, youll need to see your healthcare team regularly to monitor your health and symptoms and to make sure your medications are working.

A note from Cleveland Clinic

Learning early that you have a diagnosis of Lewy body dementia allows you and your family to plan for a meaningful quality of life together and enables you to get your legal, financial and healthcare plans and desires in order. Your healthcare team will be ready to provide education, support and care for you or your loved one. Ask your team for information on local LBD support groups as well. Support groups can be very helpful for sharing care tips and providing comfort in knowing youre not alone.

Data Synthesis And Statistical Analysis

Each outcome measure reported in this study was used in at least 3 of the 17 included studies. The primary outcome measures of efficacy were cognitive function, behavioral disturbances, and motor function. Cognitive function was assessed by MMSE, modified MMSE , or Montreal Cognitive Assessment . Behavioral disturbances were assessed by Neuropsychiatric Inventory and Brief Psychiatric Rating Scale . Motor function was assessed by Unified Parkinsons Disease Rating Scale-motor . Secondary outcome measures included ADL, global function, all-cause discontinuation, discontinuation due to adverse events, and incidence of individual adverse events. ADL was assessed by Alzheimers Disease Co-operative Study-Activities of Daily Living Inventory , Unified Parkinsons Disease Rating Scale-Activities of Daily Living , and Zarit Caregiver Burden Interview . Global function was assessed by Clinicians Interview-Based Impression of Change plus Caregiver Input and Alzheimers Disease Cooperative Study-Clinical Global Impression of Change .

We based our analyses on intent-to-treat or modified ITT data . However, we analyzed the complete set of data for ensuring that the maximum possible information was included .

Professional Brief: New Dlb Diagnostic Criteria

Newly updated in 2017, the diagnostic criteria for dementia with Lewy bodies now allows healthcare providers the ability to diagnose the disorder based on their patients individualized clinical presentation and biomarker profile.LBDAs newest publication, Professional Brief: New DLB Diagnostic Criteria, highlights

  • Key changes in the criteria
  • Core and supportive clinical features of DLB
  • Indicative and supportive biomarkers of DLB
  • Criteria for probable and possible DLB
  • Exclusion criteria
  • How to differentiate between DLB and Parkinsons disease dementia
  • Tips for assessing symptoms of DLB in the primary care setting

Read Also: What Happens To The Neurons In Alzheimer’s

Lewy Body Composite Risk Score

The LBCRS evaluates the presence of four motor and six non-motor symptoms within the last 6 months. Motor signs include slowness, rigidity, postural instability, and resting tremor. Non-motor symptoms are the following: excessive daytime sleepiness, illogical thoughts, frequent episodes of staring, visual hallucinations , enacted dreaming, and autonomic dysfunctions.

The symptoms were considered as present if they occurred at least three times during the 6 months preceding the clinical investigation.

A global score equal or superior to 3 indicates a probable DLB diagnosis , whereas a score ranging from 0 to 2 is not suggestive of DLB diagnosis .

Research Criteria For The Diagnosis Of Prodromal Dementia With Lewy Bodies

New Diagnostic Criteria Published for DLB

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How Is Lewy Body Dementia Related To Alzheimers Disease And Parkinsons Disease

Dementia with Lewy bodies and Parkinsons disease dementia are two related clinical disorders that make up the general broader category of Lewy body dementia. Sometimes, providers first diagnose LBD as Parkinsons disease or Alzheimers disease based on its symptoms.

  • Parkinsons disease dementia : Some people are diagnosed with Parkinsons disease if they first have symptoms of a movement disorder typical to Parkinsons. But their diagnosis is changed to PDD if dementia symptoms develop over the years.
  • Alzheimers disease : Some people who have a memory or cognitive disorder are diagnosed with AD. But other distinctive symptoms may suggest co-existing dementia with Lewy bodies. Distinctive symptoms of LBD include changes in attention, alertness and cognitive ability movement issues visual hallucinations and more.

Diagnostic Threshold In Dlb

The majority of participants with DLB in East Anglia exceeded the threshold for probable DLB as set out in the 2005 criteriaReference McKeith, Dickson, Lowe, Emre, O’Brien and Feldman8 at the time of final diagnosis, whereas only 35% exceeded this threshold in the North East .

Fig. 2 Diagnostic threshold in dementia with Lewy bodies .

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How Does Prodromal Dlb Usually Present

One or more of the core clinical features characteristic of fully developed DLB may develop before dementia and are usually accompanied by mild cognitive complaints. Spontaneous parkinsonism often develops within the predementia stage but is not present in all patients., RBD is a parasomnia that typically occurs years, and even decades, before the onset of dementia or parkinsonism and may presage any of the -synucleinopathies.,,e4 In patients with mild cognitive deficits who later develop dementia, both parkinsonism and RBD strongly predict a later transition to DLB rather than to AD or other dementia types., Delirium can occur during the predementia stage of DLB as can fluctuations of cognition and arousal that may give rise to a diagnosis of delirium. Visual hallucinations either spontaneous or provoked by illness or medication are more likely to occur compared with normal controls or prodromal AD., There are also case reports of delusions, hallucinations, depression, and anxiety as presenting features of DLB. Based on such observations, 3 prototypic prodromal DLB syndromes have been proposed as mild cognitive impairment , delirium-onset, or psychiatric-onset.

A Hypothetical Biomarker Profile Of Prodromal Dementia With Lewy Bodies

Lewy Body Dementia Update Diagnosis, Management, and Future Directions

Figure 5

Hypothetical timelines of biomarker development. Biomarker development in dementia with Lewy bodies . This hypothesis mirrors that proposed by Jack and colleagues in Alzheimers disease . The first biomarkers of DLB will be markers of alpha-synuclein deposition . Syn deposition probably decreases later in the disease process following cell death . This would then be followed by markers of cell damage or death and then clinical symptoms/signs . Biomarkers in two different sites. In this hypothetical representation, Syn deposition, cell damage and loss and the development of symptoms all occur in the olfactory bulb prior to the development of Syn deposition in the neocortex.

Evidence supporting the presence of LB disease may need to be weighed against evidence for the presence of other diseases such as AD. However, amyloid deposition is often seen in DLB , and DLB can develop from amnestic MCI . The presence of amnestic MCI and a positive amyloid PET scan, sufficient for a diagnosis of prodromal AD or of MCI due to AD intermediate likelihood , is therefore also consistent with prodromal DLB.

Also Check: Symptoms Of End Stage Alzheimer’s

What Is The Life Expectancy For People With Lewy Body Dementia

The average life expectancy of Lewy body dementia is five to eight years after the initial diagnosis. But some people with LBD live up to 20 years after their diagnosis.

This short average life expectancy could be due to a lack of knowledge regarding LBD among healthcare providers and the population and difficulty in distinguishing it from other similar conditions. This often leads to a delay in diagnosis, which delays the onset of specific therapy.

Treatments For Dementia With Lewy Bodies

There’s currently no cure for dementia with Lewy bodies or any treatment that will slow it down.

But there are treatments that can help control some of the symptoms, possibly for several years.

Treatments include:

Recommended Reading: Assessment For Alzheimer’s Disease

Outlook For Dementia With Lewy Bodies

How quickly dementia with Lewy bodies gets worse varies from person to person.

Home-based help will usually be needed, and some people will eventually need care in a nursing home.

The average survival time after diagnosis is similar to that of Alzheimer’s disease around 6 to 12 years. But this is highly variable and some people live much longer than this.

If you or a loved one has been diagnosed with dementia, remember that you’re not alone. The NHS and social services, as well as voluntary organisations, can provide advice and support for you and your family.

Who Does Lewy Body Dementia Affect

Research criteria for the diagnosis of prodromal dementia with Lewy ...

Lewy body dementia typically affects people over the age of 50. The older you are, the more at risk you are for developing the condition. Men and people assigned male at birth are more likely to have Lewy body dementia than women and people assigned female at birth.

A family history of LBD and Parkinsons disease also increases your risk of developing it.

Also Check: Prevalence Of Dementia In Us

Comparison Of Diagnostic Pathways In Dlb

A comparison of the diagnostic pathways of the DLB and non-DLB dementia groups was carried out . Before a final diagnosis was made, those in the DLB group received significantly more alternate diagnoses, clinical assessments at home and imaging tests brain single-photon emission computerised tomography imaging) than those in the non-DLB group.

Table 2 Comparison of the diagnostic pathways for dementia with Lewy bodies group versus non-DLB groupa

FP-CIT, 123I-N–fluoropropyl-2beta-carbomethoxy-3beta-4-iodophenyl nortropane brain single-photon emission computerised tomography.

a. How the DLB and non-DLB group reached their final diagnosis was compared, including the number of alternative diagnoses received before their final diagnosis.

When mild cognitive impairment , which accounted for about a fifth of these alternate initial diagnoses across both groups, was removed the differences were even greater with a mean of 0.65 alternate prior diagnoses for the DLB group compared with 0.17 for the non-DLB group .

The mean time between the date of final diagnosis and initiation of treatment for dementia was also significantly different between groups. Treatment on average was started before a final diagnosis in the DLB group and afterwards in the non-DLB group . We did not, however, find a significant difference between the time of onset of cognitive impairment and initiation of treatment .

How Is Lewy Body Dementia Treated

Theres no cure for Lewy body dementia . Medications and nonmedical therapies, like physical, occupational and speech therapies, manage symptoms as much as possible.

Medications that can help manage the symptoms of LBD include:

  • Cholinesterase inhibitors: This type of medication, which includes rivastigmine, galantamine and donepezil, helps manage the cognitive symptoms of LBD.
  • Carbidopa-levodopa: Symptoms of parkinsonism, like tremors, are usually treated with levodopa, a drug commonly used to treat Parkinsons disease. However, it has serious side effects and can lead to delusions, hallucinations and confusion.
  • Pimavanserin: This medication can be used to treat psychosis in people with Parkinsons disease dementia.
  • Clonazepamandmelatonin: These medications can help treat REM sleep behavior disorder.
  • Antidepressants: Depression is common in people with LBD and often requires antidepressant therapy with selective serotonin reuptake inhibitors .
  • Memantine: This drug is typically used to treat dementia caused by Alzheimers disease, but its been investigated in clinical trials and may work in people with LBD who are in the early phases of the condition.

People with LBD can take part in different therapies to improve their quality of life, including:

  • Individual and family psychotherapies .

Recommended Reading: What Differentiates Alzheimer Disease From Senile Dementia

Diagnosing Dlb On Clinical Symptoms Alone

  • Dementia is required, which simply means a decline in thinking skills that interferes with everyday life. In early DLB memory may be relatively normal in comparison to Alzheimers disease. Instead, a person with DLB experiences problems with other cognitive skills, which may need a neuropsychologist for assessment:
  • Paying attention
  • Reasoning and problem solving, called executive function
  • Understanding how objects relate in three-dimensional space, called visuospatial skills.
  • At least two of the following clinical symptoms are required:
  • Delirium-like fluctuating cognition: unpredictable changes in thinking, attention and alertness
  • Repeated visual hallucinations
  • REM sleep behavior disorder
  • Parkinsonism, specifically slowed movements, tremor when limbs are at rest, and muscle rigidity
  • DLB also has other symptoms that support a diagnosis, but are not so common that they help make the diagnosis. Two new supportive symptoms added to this list are the loss of smell and excessive daytime sleepiness.

    Who Can Diagnose Lbd

    Characterizing the prodromal phase of dementia with Lewy bodies

    Many doctors and other health care professionals are not familiar with LBD, so people may see several physicians before receiving a correct diagnosis. A general practitioner is usually the first professional visited by persons who are encountering changes in thinking, behavior, or movement. But, neurologists more frequently have the knowledge required to diagnose LBD. Geriatric psychiatrists, neuropsychologists, and geriatricians may also be skilled in diagnosing the condition.

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    Advances In Dementia With Lewy Bodies

    • Therapeutic Advances in Neurological DisordersAug 2012
    • Therapeutic Advances in Neurological DisordersJul 2019
    • Therapeutic Advances in Neurological DisordersDec 2019
    • Therapeutic Advances in Neurological DisordersOct 2012
    • Therapeutic Advances in Neurological DisordersOct 2009
    • Australian & New Zealand Journal of PsychiatryMar 2019
    • Journal of Geriatric Psychiatry and NeurologySep 2016
    • Therapeutic Advances in Neurological DisordersJul 2018
    • Therapeutic Advances in Neurological DisordersJun 2017
    • The SAGE Encyclopedia of Abnormal and Clinical Psychology2017
    • The SAGE Encyclopedia of Abnormal and Clinical Psychology2017
    • The SAGE Encyclopedia of Abnormal and Clinical Psychology2017
    • The SAGE Encyclopedia of Abnormal and Clinical Psychology2017
    • CBT for Older People: An Introduction2015
    • The SAGE Encyclopedia of Stem Cell Research2015
    • The SAGE Handbook of Healthcare2008
    • Adult Palliative care for Nursing, Health and Social Care2019

    What Are The Symptoms Of Lewy Body Dementia

    Lewy body dementia symptoms may resemble those of other neurological disorders, like Alzheimers disease and Parkinsons disease. Symptoms fluctuate over time and vary from person to person.

    There are several possible symptoms of LBD, which can be grouped into the following categories:

    • Movement issues.
    • Smaller handwriting than whats normal for the person.

    Cognitive symptoms of Lewy body dementia

    Fluctuating cognitive function is a relatively specific feature of Lewy body dementia. A person with LBD may experience periods of being alert and coherent in between periods of being confused and unresponsive to questions. This can change from day to day or within the same day.

    Other cognitive symptoms include a decline in:

    • Planning abilities.
    • Problem-solving skills.
    • Ability to focus.
    • Understanding information in visual form.

    Visual hallucinations, or seeing things that arent there occur in up to 80% of people with LBD and often early on in the condition. Other types of hallucinations, such as hearing or smelling things that arent there, are less common than visual ones but may also occur.

    Visuospatial difficulties, including decreased depth perception, trouble recognizing familiar objects and impaired hand-eye coordination, are also common in people with LBD.

    Sleeping problems in Lewy body dementia

    Other sleep issues associated with LBD include:

    • Excessive daytime drowsiness.

    Dysautonomia in Lewy body dementia

    • Changes in body temperature.

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    Assessment Toolkit For Dementia With Lewy Bodies

    The Assessment Toolkit for DLB is based on a series of specific questions carried out to identify core and suggestive features of DLB. Beyond the evidence of global cognitive decline, four domains are investigated : CF, VH, RBD, and parkinsonism. The toolkit includes a questionnaire that is administered by the rater to the patient and the caregiver, and a short neurological exam to determine the 5-item Unified Parkinsons Disease Rating Scale score. Moreover, the presence of dopaminergic deficit in basal ganglia on SPECT/PET, low uptake on metaiodobenzylguanidine myocardial scintigraphy, or polysomnography confirmation of RBD is evaluated. These features are considered as indicative biomarkers. A diagnosis of probable DLB is made if either two core features or one core and one indicative feature are identified. A diagnosis of possible DLB is considered if one feature is satisfied.

    Diagnosing Lewy Body Dementia: For Professionals

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    Lewy body dementia can be difficult to diagnose. Talking to both patients and caregivers helps doctors make a diagnosis. It is important to ask the patient and their care partners about any symptoms involving thinking, movement, sleep, behavior, or mood. Certain medications can worsen LBD symptoms be aware of all current medications and supplements the patient is taking.

    Dementia with Lewy bodies is often hard to diagnose because its early symptoms may resemble those of Alzheimer’s disease or a psychiatric illness. As a result, it is often misdiagnosed or missed altogether. As additional symptoms appear, making an accurate diagnosis may become easier.

    The good news is that doctors are increasingly able to diagnose LBD earlier and more accurately, as researchers identify which symptoms and biomarkers help distinguish it from similar disorders.

    Visiting a family doctor is often the first step for people who are experiencing changes in thinking, movement, or behavior. If a persons primary doctor is not familiar with LBD, they may have patients seek second opinions from specialists, like a geriatric psychiatrist, neuropsychologist, or a geriatrician to help diagnose LBD. If a specialist cannot be found in your community, ask the neurology department at a nearby medical school for a referral. Neurologists generally have the expertise needed to diagnose LBD.

    Difficult as it is, getting an accurate diagnosis of LBD early on is important so that a person:

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    Future Directions To Improve The Diagnostic Criteria For Dlb

    To further improve the 2017 revised diagnostic criteria for DLB, future directions include 1) the evaluation of the sensitivity and specificity of the 2017 revised criteria by pathologically confirmed cases 2) the development of the criteria for prodromal DLB, and, further, preclinical DLB to detect early-stage disease and 3) the establishment of new biomarkers as well as further characterization of clinical features specific to DLB.

    Concerning the pathological evaluation of the criteria for clinical diagnosis, mixed pathologies, especially the frequent co-occurrence of AD-related pathology, complicate the pathophysiological and clinical manifestations of DLB . In the diagnostic criteria, the likelihood that the pathological findings are associated with a typical DLB clinical syndrome is assessed on the basis of the staging of Lewy-related pathology and AD pathology . It is recommended to measure reliable biomarkers for AD pathology for the precise clinical diagnosis of DLB. In addition, the distribution of the Lewy-related pathology responsible for each cognitive/psychiatric symptom should be further elucidated with careful clinicopathological studies .

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