Quantitative motor phenotype assessment in pre-manifest and symptomatic Huntington's disease: tongue force analysis differentiates between disease stages and provides high phenoty…
M Say, N Bechtel, A Sturrock, S van den Bogaard, C Jauffret, S Bohlen, DR Langbehn, JA Mills, TP Archarya, H Johnson, B Borowsky, A Durr, BR Leavitt, RAC Roos, SJ Tabrizi, B Landwehrmeyer, R Reilmann · Aktuelle Neurologie · 2009 · Heft 10
Bibliografische Angaben
Zusammenfassung
Background: Assessment of motor phenotype and diagnosis of Huntington's Disease (HD) are based on the Unified Huntington's Disease Rating Scale-Total Motor Score (UHDRS-TMS), a categorical clinical scale. Therefore, results of clinical trials are limited by (1) subjective error, (2) sensitivity of the categorical scale, and (3) insensitivity in pre-manifest subjects. More objective and quantitative measures of motor phenotype are warranted. In a pilot study, we found increased variability of tongue force (TF) in pre-manifest and symptomatic HD, and correlation with genotype and UHDRS-TMS (Reilmann et al. 2005).…
