Application of Quantitative Motor Assessments in Friedreich Ataxia and Evaluation of Their Relation to Clinical Measures
| Autor: | Matthis Synofzik, Claire Didszun, Imis Dogan, Robin Schubert, Jörg B. Schulz, Thomas Klockgether, Kathrin Reetz, Ludger Schöls, Ralf Reilmann, Christian Hohenfeld, Ilaria Giordano |
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| Rok vydání: | 2019 |
| Předmět: |
Adult
medicine.medical_specialty Ataxia Neurology Adolescent physiopathology [Friedreich Ataxia] Disease physiology [Psychomotor Performance] 050105 experimental psychology Generalized linear mixed model Young Adult 03 medical and health sciences 0302 clinical medicine Physical medicine and rehabilitation diagnosis [Friedreich Ataxia] medicine Humans 0501 psychology and cognitive sciences ddc:610 Cerebellar ataxia business.industry Lift (data mining) 05 social sciences Linear model Middle Aged Friedreich Ataxia Motor Skills Finger tapping Linear Models Female Neurology (clinical) medicine.symptom business Psychomotor Performance physiology [Motor Skills] 030217 neurology & neurosurgery Follow-Up Studies |
| Zdroj: | The Cerebellum 18(5), 896-909 (2019). doi:10.1007/s12311-019-01073-x |
| ISSN: | 1473-4230 1473-4222 |
| DOI: | 10.1007/s12311-019-01073-x |
| Popis: | Friedreich's ataxia (FRDA) is a rare autosomal-recessive slowly progressive neurodegenerative disorder. As common clinical measures for this devastating disease lack sensitivity, we explored whether (a) the quantitative motor assessments of the Q-Motor battery can enhance clinical characterisation of FRDA; (b) clinical measures can predict Q-Motor outcomes and (c) Q-Motor is sensitive to longitudinal change. At baseline 29 patients and 23 controls and in a 1-year follow-up 14 patients and 6 controls were included. The Q-Motor included lift (manumotography), finger tapping (digitomotography) and pronate/supinate (dysdiadochomotography) tasks. To model responses, a search of generalised linear models was conducted, selecting best fitting models, using demographic and clinical data as predictors. Predictors from selected models were used in linear mixed models to investigate longitudinal changes. Patients with FRDA performed worse than controls on most measures. Modelling of the pronate/supinate task was dominated by SCAFI (SCA functional index) subtasks, while tapping task and lift task models suggested a complex relationship with clinical measures. Longitudinal modelling implied minor changes from baseline to follow-up, while clinical scales mainly showed no change in this sample. Overall Q-Motor likely has favourable properties for assessing distinct motor aspects in severe FRDA as it can be administered in wheelchair-bound patients. Further longitudinal research is warranted to fully characterise its relation to routinely used measures and scales for FRDA. |
| Databáze: | OpenAIRE |
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