Authors: Bazarian, Jeffrey J. | Beck, Christopher | Blyth, Brian | von Ahsen, Nicolas | Hasselblatt, Martin
Article Type: Research Article
Abstract: Purpose: To validate a correction factor for the extracranial release of the astroglial protein, S-100B, based on concomitant creatine kinase (CK) levels. Methods: The CK- S-100B relationship in non-head injured marathon runners was used to derive a correction factor for the extracranial release of S-100B. This factor was then applied to a separate cohort of 96 mild traumatic brain injury (TBI) patients in whom both CK and S-100B levels were measured. Corrected S-100B was compared to uncorrected S-100B for the prediction of initial head CT, three-month headache and three-month post concussive syndrome (PCS). Results: Corrected S-100B resulted in a statistically …significant improvement in the prediction of 3-month headache (area under curve [AUC] 0.46 vs 0.52, p=0.02), but not PCS or initial head CT. Using a cutoff that maximizes sensitivity (⩾90%), corrected S-100B improved the prediction of initial head CT scan (negative predictive value from 75% [95% CI, 2.6%, 67.0%] to 96% [95% CI: 83.5%, 99.8%]). Conclusions: Although S-100B is overall poorly predictive of outcome, a correction factor using CK is a valid means of accounting for extracranial release. By increasing the proportion of mild TBI patients correctly categorized as low risk for abnormal head CT, CK-corrected S100-B can further reduce the number of unnecessary brain CT scans performed after this injury. Show more
Keywords: Mild traumatic brain injury, cerebral concussion, serum markers, post concussive syndrome
Citation: Restorative Neurology and Neuroscience, vol. 24, no. 3, pp. 163-172, 2006
Authors: Bull, Michael T. | Darwin, Kristin | Venkataraman, Vinayak | Wagner, Joseph | Beck, Christopher A. | Dorsey, E. Ray | Biglan, Kevin M.
Article Type: Research Article
Abstract: Background: Virtual visits through web-based video conferencing can increase access to specialty care for individuals with Huntington disease (HD) and facilitate research participation. Objective: To determine the feasibility of conducting virtual visits directly into the homes of individuals with HD, to assess the reliability of conducting remote versus in-person motor assessments, and to determine the test-retest reliability of conducting motor assessments remotely. Methods: Individuals with mild to moderate HD underwent baseline in-person clinic assessments and completed a HD care survey. Participants were randomized to receive three virtual visits from one of two physicians over four months that included a modified …Unified Huntington's Disease Rating Scale motor examination (excluding rigidity and balance assessments) via web-based video conferencing. Intraclass coefficients (ICC) were calculated to determine the level of agreement between remote and in-person assessments. Participants also completed a survey on their interest in telemedicine. Results: Thirteen individuals underwent baseline assessments, eleven (85%) participants completed at least one virtual visit, and 27 (82%) of 33 total virtual visits were completed. Remote motor scores demonstrated good reliability (ICC = 0.78; n = 11) compared to in-person motor scores. Test-retest reliability of motor scores conducted remotely was excellent (ICC = 0.90; n = 11). Participants expressed moderate future interest in using virtual visits to participate in research and to receive care. Conclusion: In this pilot study, virtual visits into the home were feasible and reliable for conducting motor assessments in HD. Larger scale studies need to confirm and generalize these findings to a broader population of participants. Show more
Keywords: Telehealth, Huntington disease, feasibility, reliability, remote assessment
DOI: 10.3233/JHD-140102
Citation: Journal of Huntington's Disease, vol. 3, no. 2, pp. 189-195, 2014
Authors: Dorsey, E. Ray | Brocht, Alicia F.D. | Nichols, Paige E. | Darwin, Kristin C. | Anderson, Karen E. | Beck, Christopher A. | Singh, Sonal | Biglan, Kevin M. | Shoulson, Ira
Article Type: Research Article
Abstract: Background: Tetrabenazine, a treatment for chorea in Huntington disease, carries a boxed warning due to safety, especially related to suicidality. Objective: To compare the frequency of depressed mood and suicidality among a closely monitored cohort of individuals with Huntington disease who were exposed and not exposed to tetrabenazine. Methods: A longitudinal prospective study involving 1360 individuals with HD evaluated at 48 research centers in Australia, Canada, and the United States was examined for frequency of depressed mood that triggered a risk assessment, suicidal thoughts, suicide attempts, and completed suicide among individuals with prior, new, and no exposure to tetrabenazine.. Seventy-seven …individuals were on tetrabenazine at study enrollment (prior exposure), 64 individuals were exposed to tetrabenazine during the study's course (new exposure), and 1219 individuals had no exposure to tetrabenazine. Results: The hazard ratio for depressed mood among those with prior exposure to tetrabenazine compared to no exposure was 0.9 (95% CI, 0.5–1.6) and for those with new exposure compared to no exposure was 1.2 (95% CI, 0.8–1.9). One individual (1.3%) with prior exposure, one individual (1.6%) with new exposure, and 35 individuals (2.9%) with no exposure to tetrabenazine reported suicidal thoughts. The hazard ratio for suicidal ideation among those with prior exposure to tetrabenazine compared to no exposure was 0.5 (95% CI, 0.1–3.8) and for those with new exposure to tetrabenazine compared to no exposure was 0.6 (95% CI, 0.1–4.4). Among individuals with prior or new exposure to tetrabenazine, no suicide attempts or suicides occurred. Among those with no exposure to tetrabenazine 17 suicide attempts (1.4%) and four suicides (0.3%) occurred. Conclusions: In a large observational study with close clinical supervision, tetrabenazine treatment was not associated with an increased risk of depressed mood, suicidal ideation, suicide attempts, or suicide. Show more
Keywords: Depression, Huntington disease, suicide, tetrabenazine, U.S. Food and Drug Administration
DOI: 10.3233/JHD-130071
Citation: Journal of Huntington's Disease, vol. 2, no. 4, pp. 509-515, 2013
Authors: Anderson, Karen E. | Arbatti, Lakshmi | Hosamath, Abhishek | Feigin, Andrew | Goldstein, Jody | Kayson, Elise | Kinsler, Brett L. | Falanga, Lauren | Denise, Lynn | Carlozzi, Noelle E. | Frank, Samuel | Jackson, Katie | Kostyk, Sandra | Purks, Jennifer L. | Serbin, Kenneth P. | Kinel, Shari | Beck, Christopher A. | Shoulson, Ira
Article Type: Research Article
Abstract: Background: Direct-to-participant online reporting facilitates the conduct of clinical research by increasing access and clinically meaningful patient engagement. Objective: We assessed feasibility of online data collection from adults with diagnosed Huntington’s disease (HD) who directly reported their problems and impact in their own words. Methods: Data were collected online from consenting United States residents who self-identified as 1) having been diagnosed with Huntington’s disease, 2) able to ambulate independently, and 3) self-sufficient for most daily needs. Data for this pilot study were collected using the Huntington Study Group myHDstory online research platform. The Huntington Disease Patient Report of Problems (HD-PROP), …an open-ended questionnaire, was used to capture verbatim bothersome problems and functional impact. Natural language processing, human-in-the-loop curation of verbatim reports involving clinical and experience experts, and machine learning classified verbatim-reports into clinically meaningful symptoms. Results: All 8 questionnaires in the online pilot study were completed by 345 participants who were 60.9% men, 34.5±9.9 (mean±SD) years old, and 9.5±8.4 years since HD diagnosis. Racial self-identification was 46.4% Caucasian, 28.7% African American, 15.4% American Indian/Alaska Native, and 9.5% other. Accuracy of verbatim classification was 99%. Non-motor problems were the most frequently reported symptoms; depression and cognitive impairment were the most common. Conclusions: Online research participation was feasible for a diverse cohort of adults who self-reported an HD diagnosis and predominantly non-motor symptoms related to mood and cognition. Online research tools can help inform what bothers HD patients, identify clinically meaningful outcomes, and facilitate participation by diverse and under-represented populations. Show more
Keywords: Patient-reported outcomes, Huntington’s disease, machine learning, self-report
DOI: 10.3233/JHD-231520
Citation: Journal of Huntington's Disease, vol. 13, no. 2, pp. 237-248, 2024