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Abstract Details

Prevalence of Neurocognitive Disorder in a Pre-manifest Huntington’s disease Cohort
Movement Disorders
P1 - Poster Session 1 (8:00 AM-9:00 AM)
5-014
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.  
To determine the prevalence of mild and major neurocognitive disorder in premanifest HD patients using DSM-5 criteria.
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.
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.
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 Alberto Sierra, Jr. (Beth Israel Deaconess Medical Center)
PRESENTER
Mr. Sierra has nothing to disclose.
Clementina Julia Ullman (Beth Israel Medical Center) Miss Ullman has nothing to disclose.
Clara Baselga-Garriga, Other Ms. Baselga-Garriga has nothing to disclose.
Karen Hildebrand, Other Miss Hildebrand has nothing to disclose.
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 Novartis. Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage Therapeutics. Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for uniQure. 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 Triplet Therapeutics.
Simon Laganiere, MD (Beth Israel Deaconess Medical Ctr) Dr. Laganiere has received research support from HDSA.