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Instrumented Objective Clinical Examination of Cerebellar Ataxia: the Upper and Lower Limb—a Review

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Abstract

Cerebellar dysfunction results in impairments in co-ordination or ‘ataxia’. Bedside examination of cerebellar function has changed little since the early nineteenth century with the exception being the oculomotor examination which has become instrumented. Otherwise, competence and confidence in performing the clinical assessment relies heavily on the skill and experience of the clinician. Potentially, instrumented objective measurement will more accurately assess the severity of ataxia and the changes brought about by advancing therapies in pharmaceutical trials and in rehabilitation intervention. This study describes instrumented versions of several bedside tests of cerebellar function, including rhythmic tapping of the hand (RTH), finger-nose test (FNT), dysdiadochokinesia (DDK), ramp tracking (RMT), ballistic tracking (BT), rhythmic tapping of the foot (RTF) and the heel shin (HST) examination which were validated against scores from Ataxia Rating Scales (ARS) such as the Scale of Assessment and Rating of Ataxia (SARA). While all of the instrumented tests accurately distinguished between ataxic subjects and controls, there was a difference in performance, with the best four performing upper limb tests being RTH, FNT, DDK and BT. A combination of BT plus RTH provided the best correlation with the SARA and outperformed a combination of all the bedside tests (Spearman 0.8; p < 0.001 compared to 0.68; p < 0.001 for the combined set) in identifying the presence and severity of ataxia. This indicates that there is redundancy in the information provided by the bedside tests and that adding other tests to BT plus RTH does not add accuracy to the assessment of ataxia. This analysis highlighted the need for metrics that could be generalised to each of the assessments of ataxia, so, in turn, domains of stability, timing, accuracy and rhythmicity (STAR domains) were developed and compared to the SARA. The STAR criteria could potentially influence the future of instrumented assessment in CA and pave the way for further research into the objective measurement of the cerebellar examination.

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Notes

  1. It must be emphasised that the relevant publications must be followed for the details of model building for each test as each followed a slightly different path.

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Funding

This research is supported by the Royal Victorian Eye and Ear Hospital (RVEEH) and the Florey Institute of Neuroscience and Mental Health, Melbourne, Australia, through the National Health and Medical Research Council (NHMRC) (Grant: GNT1101304 and APP1129595) and CSIRO Data61.

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Correspondence to Laura Power.

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Power, L., Pathirana, P., Horne, M. et al. Instrumented Objective Clinical Examination of Cerebellar Ataxia: the Upper and Lower Limb—a Review. Cerebellum 21, 145–158 (2022). https://doi.org/10.1007/s12311-021-01253-8

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