Format

Send to

Choose Destination
Cerebellum. 2019 Aug 22. doi: 10.1007/s12311-019-01073-x. [Epub ahead of print]

Application of Quantitative Motor Assessments in Friedreich Ataxia and Evaluation of Their Relation to Clinical Measures.

Author information

1
Department of Neurology, RWTH Aachen University, Pauwelsstraße 30, 52074, Aachen, Germany.
2
JARA-Brain Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich GmbH/RWTH Aachen University, Aachen, Germany.
3
George-Huntington-Institute, Technology-Park, Münster, Germany.
4
Department of Neurodegenerative Diseases and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
5
German Centre for Neurodegenerative Diseases (DZNE), Tübingen, Germany.
6
Department of Neurology, University Hospital Bonn, Bonn, Germany.
7
German Centre for Neurodegenerative Diseases (DZNE), Bonn, Germany.
8
Department of Clinical Radiology, University of Münster, Münster, Germany.
9
Department of Neurology, RWTH Aachen University, Pauwelsstraße 30, 52074, Aachen, Germany. kreetz@ukaachen.de.
10
JARA-Brain Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich GmbH/RWTH Aachen University, Aachen, Germany. kreetz@ukaachen.de.

Abstract

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.

KEYWORDS:

Cerebellar ataxia; Friedreich ataxia; Motor activity; Upper extremity

PMID:
31441004
DOI:
10.1007/s12311-019-01073-x

Supplemental Content

Full text links

Icon for Springer
Loading ...
Support Center