University of Washington



ABC-DS Case Consensus ProcessOverviewDetermination of overall Alzheimer’s disease (AD)-related clinical status is based on overall profile of performance on the directly-administered neuropsychological measures and caregiver-reported measures of dementia symptoms, combined with clinical judgment and in consideration of baseline IQ, medical/psychiatric history, neurological exam, and recent life events. All available time points of data (i.e., previous study visits) are reviewed in this process. Staff are blind to imaging and biofluid data.Following the collection of all data for a given test cycle, a summary of the visit information (and prior information) is provided to team members and each team member independently reviews current and past data and determines clinical status. If there is complete agreement among team members, the clinical status is recorded and entered into the database. Conversely, if there is any disagreement among team members regarding the clinical status, a consensus conference meeting is held to discuss the case. It is recommended that each site conduct monthly or bimonthly conferences so that the observations regarding participants are “fresh” in the minds of team position of the Consensus Review Team: Each ABC-DS clinical performance site will have its own Consensus Team that will minimally include three team members such as the site coordinator, PI and neuropsychologist/staff who conducted the neuropsychological assessment. Depending upon how an individual site functions, other team members could include the medical professional who conducted the physical/neurological exam as well as the staff member who conducted the caregiver interview. In addition, there will be at least one outside member of the team from the ABC-DS Clinical Core (an investigator) as well as the Global ABC-DS Consensus Conference Coordinator who will complete all steps of the process (i.e., initial primary rating and conference call if there is not agreement). This system will ensure the process is standardized across rmation Available to the Consensus Review Team: All information from the most recent study visit and any longitudinal data from past test cycles (including data from legacy and U01 study cycles as well as consensus team diagnostic decisions from prior visits) will be available to the consensus team on a summary sheet, with the exception of MRI, PET scan and blood-based/CSF biomarkers data. These summary sheets will be prepared by and distributed to the team by Global ABC-DS Consensus Conference Coordinator. Specific sources of information include:1. Demographic information (age, sex, FSIQ and/or level of functioning)2. Medical and psychiatric history (and family history)3. Physical and neurological exam results4. Clinical Lab results (i.e., thyroid lab results)5. Neuropsychological test results (including all measures)6. Caregiver questionnaire results (e.g., Vineland, DLD, NTG, NPI)7. Summary sheet for all past study visitsClinical Status Categories: Categories are: (a) cognitively-stable (CS), indicating with reasonable certainty that significant impairment was absent, (b) Mild Cognitive Impairment-Down Syndrome (MCI-DS), indicating that there was indication of subtle cognitive and/or functional decline over and above what would be expected with aging, though of insufficient severity to suggest the presence of dementia, (c) possible dementia, indicating that some signs and symptoms of dementia were present, but declines over time were judged to need further evidence of progression, (d) definite dementia, indicating with a high degree of confidence that dementia was present based upon substantial decline over time, (e) status uncertain due to complications, indicating that declines were observed that might be caused by some other concern unrelated to neuropathology, (e.g., psychiatric diagnosis, disruptive life event), and (f) indeterminable, indicating that the individual’s preexisting developmental disability was of such severity that detection of decline indicative of dementia was not possible.Do the above clinical status categorical rating twice. Once for the primary rating and then again for the secondary rating. For cases that clearly fit a category, then the primary and secondary are the same. For borderline cases, this would be different (e.g., CS & MCI-DS). If we combine the primary and secondary ratings, this would result in the following categories: (a) CS, (b) CS but maybe MCI-DS, (c) MCI-DS but maybe CS, (d) MCI-DS, (e) MCI-DS but maybe Possible Dementia, (f) Possible Dementia but maybe MCI-DS, (g) Possible Dementia, (h) Possible Dementia but maybe Definite Dementia, (i) Definite Dementia but maybe Possible Dementia, (j) Definite Dementia.PROCESSEach consensus team member reviews the consensus information and gives a primary rating. If there is no disagreement on primary ratings among the consensus team, nothing else has to be done. In this case, the secondary rating will then be the same as the primary rating. However, if there is disagreement on the primary rating among the consensus team, then they discuss the case on a consensus call. They decide on the primary and secondary ratings (and likely these would differ to capture the uncertainty, but do not have to differ).Consensus Summary Sheet: This form provides a summary of the data used to make the clinical status determination.Step 1: The visit summary sheet is completed and made available to all members of the consensus team. Their clinical status ratings are reviewed by the site coordinator and the case is in consensus conference meeting if there is any disagreement among team members in the primary rating. Weighing Available Information:Consensus Summary TableAreaSource Caregiver-reportCognitive scoresOther (e.g., neuro exam, research team observations)Memory Non-memory, Cognitive Emotional/Behavioral Functional Behavior Notes. For each source and area, put “0“ for no concerns about decline/change, “1” for mild or inconsistent concerns about declines/change, and “2” for moderate/consistent concerns about decline/change. Shaded out boxes indicate no scores in this area from that source. INTERPRETING THE DATAInformation to be used in the determination of diagnostic status will be primarily from the caregiver report and from direct testing/observation of the participant. But there also may be other sources of information, such as a report from a local neurologist who has given the participant an MCI or Dementia diagnosis and review of medical records. We suggest organizing the information using the above table. As there aren’t well-normed tests for adults with Down syndrome, it can be difficult to make a diagnosis of MCI-DS or dementia following a single visit. A key to interpreting any data is to know the individual’s level of functioning and/or premorbid IQ (or prior functioning for those who come with concerns regarding possible dementia).Interpreting Individual Measures: We strive towards using the same evaluators and the same caregivers for all visits. If that is not the case (especially if the caregiver is different), the team may need to wait for data from a subsequent visit before agreeing on an MCI-DS or dementia diagnosis (depending upon how reliable the new information is judged). A second challenge in the interpretation of symptoms involves situations where there have been a worsening of performance but this has occurred in the context of significant environmental changes (e.g., moving to a different home, death of a parent, a major medical issue). In such cases, it may be best to use the “unable to determine” diagnostic category until the participant is seen again. Although we provide guidelines for interpreting performance, it should be noted that determination of Consensus Diagnosis is based on clinical judgement and the overall pattern of findings across measures (think of it as a gestalt interpretation) rather than through the use of cut-off scores or a single measure.a. Dementia Questionnaire for People with Learning Disabilities (DLD): The initial studies by measure authors of the DLD have suggested that a change of >7 points on the Sum of Cognitive Scores (SCS) and a change of >5 points on the Sum of Social Scores (SOS) are associated with a diagnosis of dementia.. Individuals functioning in the moderate to severe ranges of ID will likely have high DLD scores at baseline. Data from our legacy studies suggest changes in the SCS are more predictive of MCI and dementia than change in the SOS. However, as the DLD manual indicates sensitivity and specificity are imperfect for these diagnostic criteria, DLD findings need to be interpreted in the context of other evidence (e.g., change should be compared with change noted in other measures).b. Vineland Adaptive Behavior Scales 3rd ed (VABS-3): The VABS-3 standard scores should generally match the standard scores for overall IQ. So, if an individual has mild ID, then the Vineland standard scores should generally range from 50-70. A decrease of around 10 standard points in the Vineland composite score or subdomain composite scores is likely indicative of meaningful declines in adaptive behavior.c. The Neuropsychiatric Inventory (NPI): The NPI assesses 13 neuropsychiatric areas, which are scored as “not present” and as mild/moderate/severe if present. There is a total score ranging from 0 to 122 points. The emergence of new concerns or increases in the severity of existing neuropsychiatric problems may occur with MCI or dementia, but can also be due to other factors. d. NTG Early Detection Screen for Dementia (NTG): The original data on the NTG suggested that >20 areas of worsening symptoms were suggestive of dementia in adults with DS. No data was available on MCI-DS. Hence, we generally expect an individual with MCI-DS to have >9 and <20 areas of worsening symptoms. Having one or more concern in the language or memory domain had good sensitivity and specificity for MCI-DS.e. Neuropsychological Measures: The DSMSE Total 2 provides a summary mental status score (as well as specific scores on areas such as memory, visual-spatial, etc). This measure tends to be stable across time while individuals are Cognitively Stable. Additionally, our research to date suggests that episodic memory (Cued Recall and Selective Reminding) are the two most sensitive measures for early MCI-DS and dementia. Other measures that tend to change with the onset of MCI-DS and dementia included Purdue Pegboard, Dog and Cat Stroop, and Verbal Fluency.How to Approach a Diagnostic Decision 1. Review the available data in each area using the consensus summary sheet. If data from only a single visit is available, use premorbid level of functioning or IQ to determine if any of the neuropsychological and caregiver measures (e.g., Vineland) are consistent with that level. If there are multiple visits, look for significant change.Memory: DLD (Memory subscale), NTG (Memory items), Neuropsych Testing (Cued Recall, Selective Reminding), NPI, notes from staff (e.g., participant no longer seemed to remember any of the staff who the hospital), notes from neuro exam.Non-Memory, Cognitive: DLD, Neuropsych Testing (Dog & Cat, Verbal Fluency, Expressive One-Word, Block Design), VMI, tinetti, neuro exam, NTG, NPI (psychomotor)Emotional/Behavioral: NTG (Behavioral Items), NPI, notes from staff (e.g., participant frequently refused to comply, tearful) and from neuro exam.Functional: Vineland, NTG (Social items), NPI, notes from staff and neuro exam 2. Complete the Consensus Summary Table, indicating which source is identifying concerns (“0“ for no concerns “1” for mild or inconsistent concerns and “2” for moderate/consistent concerns). Some rules of thumb include:- Could changes be due to major environmental changes (moving, death of parent) or a major illness (see NTG for checklist of recent life stressful events)?-For MCI-DS, we would expect problems in memory and/or non-memory, cognitive domains that could impact other areas (e.g., emotional/behavioral or adaptive/functional). If neuropsychological test results indicate that a participant has improved or stayed the same as baseline in a number of areas, it would be difficult to justify an MCI-DS diagnosis.-For Dementia, we would expect “2” level concerns in multiple areas and across sources. For example, we should expect to see changes in memory and non-memory, cognition on directly-administered neuropsychological assessment, as well as declines in adaptive and functional behavior reported by caregivers.-There will be cases where the caregiver reports no concerns but the neuropsych testing shows a decline. It is possible that the participant was tired or just had a difficult time with the testing. In these cases, it will be important to consider anything that may have impacted testing. All aspects of data should be considered in making the rating. ................
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