Abstract Details

Title Prevalence of Neurocognitive Disorder in a Pre-manifest Huntington’s disease Cohort

Topic Movement Disorders

Presentation(s) P1 - Poster Session 1 (8:00 AM-9:00 AM)

Poster/Presentation
Number 5-014

Objective To determine the prevalence of mild and major neurocognitive disorder in premanifest HD patients using DSM-5 criteria.

Background Huntington’s disease (HD) is a neurological disorder characterized by progressive dysfunction across three domains: movement, cognition, and behavior. The diagnosis of manifest HD is based primarily on motor dysfunction, however cognitive decline typically begins in the pre-manifest (preHD) phase and often exerts an understated functional impact. The Diagnostic and Statistical Manual of Mental Disorders (DSM-5) has established criteria for both mild and major neurocognitive disorder (NCD), however, these are not routinely applied in HD.

Design/Methods The following cognitive battery was administered to both preHD (CAG repeat > 36) and control participants: Stroop Color and Word Test (SCWT), Symbol Digit Modalities Test (SDMT), and Verbal fluency (Category). DSM-5 criteria for NCD were then applied to these test results to determine diagnostic prevalence of NCD.

Results 23 preHD participants and 26 Healthy Controls (HC) were enrolled. PreHD participants' age was (40.7±11.5) (mean±SD), years of education (15.7±2.7), CAG repeat length (42.1±2.7). The UHDRSTM total motor score was (2.0±2.2). HC age was (37.1 ± 12.0), and education of (16.6 ± 2.1). Group differences in age and education were not statistically significant, however, group differences were observed for Verbal Fluency (p = 0.03) and Word Reading (p = 0.004). Using DSM-5 criteria, the prevalence of NCD-mild was 35% in preHD vs. 3% in HC, NCD-major was 13% in preHD versus 0% in HC.

Conclusions The prevalence of NCD in preHD is surprisingly elevated. Establishing a clinical diagnosis of NCD prior to motor manifestation can be critical for access to services, anticipating longitudinal care, expanding recruitment for disease-modifying trials, and validating patient concerns when appropriate. Consideration should be given to incorporating NCD criteria into clinical practice.

Authors/Disclosures
Luis A. Sierra, Jr. (Beth Israel Deaconess Medical Center) PRESENTER	Mr. Sierra has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Samuel A. Frank, MD, FAAN (Beth Israel Deaconess Medical Center/Harvard Medical School)	Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for uniQure. Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Teva. Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. The institution of Dr. Frank has received research support from Huntington's Disease Society of America. The institution of Dr. Frank has received research support from Roche/Genentech. The institution of Dr. Frank has received research support from CHDI Foundation. The institution of Dr. Frank has received research support from Huntington Study Group. The institution of Dr. Frank has received research support from Cerevel.
Simon Laganiere, MD (Beth Israel Deaconess Medical Ctr)	Dr. Laganiere has nothing to disclose.

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