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  1. Article ; Online: Using the LASSI-L to Detect Robust Interference Effects in Premanifest Huntington Disease.

    Sierra, Luis A / Ullman, Clementina J / Frank, Samuel A / Laganiere, Simon

    Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology

    2023  Volume 36, Issue 2, Page(s) 100–107

    Abstract: Background: Diagnosis of manifest Huntington disease (HD) is based primarily on motor symptoms, but premanifest HD (preHD) is often associated with subtle cognitive decline. The Loewenstein-Acevedo Scales for Semantic Interference and Learning (LASSI-L) ...

    Abstract Background: Diagnosis of manifest Huntington disease (HD) is based primarily on motor symptoms, but premanifest HD (preHD) is often associated with subtle cognitive decline. The Loewenstein-Acevedo Scales for Semantic Interference and Learning (LASSI-L) is a validated verbal learning test that can be used to detect early cognitive decline.
    Objective: To determine the utility of the LASSI-L for detecting early cognitive decline in individuals with preHD and to compare the results of the LASSI-L with those of commonly used neuropsychological tests in HD.
    Method: We administered the LASSI-L to 13 individuals with preHD and 13 healthy controls matched for age, sex, and education as part of a longitudinal study of disease progression. For comparison purposes, we administered the Mini-Mental State Examination; Stroop Color and Word Test; Symbol Digit Modalities Test; Trail-Making Test, Parts A and B; and category fluency (animals) task.
    Results: Five of the seven sections on the LASSI-L captured group differences: Proactive Semantic Interference (PSI; P < 0.001), Failure to Recover From PSI ( P = 0.038), Retroactive Semantic Interference (RSI; P = 0.013), Delayed Recall ( P < 0.001), and B1 Cued Recall Intrusions ( P = 0.036). Using a false discovery rate of <0.05, PSI, RSI, and Delayed Recall remained significant.
    Conclusion: The LASSI-L is a sensitive instrument for detecting early interference effects in individuals with preHD that outperforms commonly used neuropsychological tests. The LASSI-L could be a useful addition to clinical and research protocols involving individuals with preHD.
    MeSH term(s) Humans ; Huntington Disease/diagnosis ; Longitudinal Studies ; Cognitive Dysfunction/diagnosis ; Cognitive Dysfunction/psychology ; Memory ; Learning ; Neuropsychological Tests
    Language English
    Publishing date 2023-06-01
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2108112-8
    ISSN 1543-3641 ; 1543-3633
    ISSN (online) 1543-3641
    ISSN 1543-3633
    DOI 10.1097/WNN.0000000000000329
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Prevalence of neurocognitive disorder in Huntington's disease using the Enroll-HD dataset.

    Sierra, Luis A / Ullman, Clementina J / Baselga-Garriga, Clara / Pandeya, Sarbesh R / Frank, Samuel A / Laganiere, Simon

    Frontiers in neurology

    2023  Volume 14, Page(s) 1198145

    Abstract: Background: Cognitive decline in Huntington's disease (HD) begins early in the disease course, however the reported prevalence and severity of cognitive impairment varies based on diagnostic approach. A Movement Disorders Society Task Force recently ... ...

    Abstract Background: Cognitive decline in Huntington's disease (HD) begins early in the disease course, however the reported prevalence and severity of cognitive impairment varies based on diagnostic approach. A Movement Disorders Society Task Force recently endorsed the use of standardized DSM-5-based criteria to diagnose neurocognitive disorder (NCD) in Huntington's disease.
    Objectives: To determine the prevalence and severity of cognitive impairment across different stages of HD by applying NCD criteria (mild and major) to participant data from the Enroll-HD database.
    Methods: Enroll-HD participants were triaged into either premanifest (preHD), manifest or control groups. PreHD was further dichotomized into preHD near or preHD far based on predicted time to diagnosis using the scaled CAG-age product score (CAPs). Embedded cognitive performance and functional independence measures were used to determine prevalence of NCD (mild and major) for all groups.
    Results: Prevalence of NCD-mild was 25.2%-38.4% for manifest HD, 22.8%-47.3% for preHD near, 11.5%-25.1% for preHD far, and 8.8%-19.1% for controls. Prevalence of NCD-major was 21.1%-57.7% for manifest HD, 0.5%-16.3% for preHD near, 0.0%-4.5% for preHD far, and 0.0%-3.0% for controls.
    Conclusion: The prevalence of NCD in HD is elevated in preHD and demonstrates a sharp rise prior to diagnosis. In manifest HD, the vast majority of participants meet criteria for NCD. These findings are important for optimizing clinical care and/or anticipating the need for supportive services.
    Language English
    Publishing date 2023-07-14
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2564214-5
    ISSN 1664-2295
    ISSN 1664-2295
    DOI 10.3389/fneur.2023.1198145
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: LASSI-L detects early cognitive changes in pre-motor manifest Huntington's disease: a replication and validation study.

    Sierra, Luis A / Hughes, Shelby B / Ullman, Clementina J / Hall, Andrew / Pandeya, Sarbesh R / Schubert, Robin / Frank, Samuel A / Halko, Mark A / Corey-Bloom, Jody / Laganiere, Simon

    Frontiers in neurology

    2023  Volume 14, Page(s) 1191718

    Abstract: Background and objectives: Cognitive decline is an important early sign in pre-motor manifest Huntington's disease (preHD) and is characterized by deficits across multiple domains including executive function, psychomotor processing speed, and memory ... ...

    Abstract Background and objectives: Cognitive decline is an important early sign in pre-motor manifest Huntington's disease (preHD) and is characterized by deficits across multiple domains including executive function, psychomotor processing speed, and memory retrieval. Prior work suggested that the Loewenstein-Acevedo Scale for Semantic Interference and Learning (LASSI-L)-a verbal learning task that simultaneously targets these domains - could capture early cognitive changes in preHD. The current study aimed to replicate, validate and further analyze the LASSI-L in preHD using larger datasets.
    Methods: LASSI-L was administered to 50 participants (25 preHD and 25 Healthy Controls) matched for age, education, and sex in a longitudinal study of disease progression and compared to performance on MMSE, Trail A & B, SCWT, SDMT, Semantic Fluency (Animals), and CVLT-II. Performance was then compared to a separate age-education matched-cohort of 25 preHD participants. Receiver operating curve (ROC) and practice effects (12 month interval) were investigated. Group comparisons were repeated using a preHD subgroup restricted to participants predicted to be far from diagnosis (Far subgroup), based on CAG-Age-Product scaled (CAPs) score. Construct validity was assessed through correlations with previously established measures of subcortical atrophy.
    Results: PreHD performance on all sections of the LASSI-L was significantly different from controls. The proactive semantic interference section (PSI) was sensitive (
    Discussion: The LASSI-L is a sensitive, reliable, efficient tool for detecting cognitive decline in preHD. By using a unique verbal learning test paradigm that simultaneously targets executive function, processing speed and memory retrieval, the LASSI-L outperforms many other established tests and captures early signs of cognitive impairment. With further longitudinal validation, the LASSI-L could prove to be a useful biomarker for clinical research in preHD.
    Language English
    Publishing date 2023-07-18
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2564214-5
    ISSN 1664-2295
    ISSN 1664-2295
    DOI 10.3389/fneur.2023.1191718
    Database MEDical Literature Analysis and Retrieval System OnLINE

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