Motor training in degenerative spinocerebellar disease: ataxia-specific improvements by intensive physiotherapy and exergames

Abstract

The cerebellum is essentially involved in movement control and plays a critical role in motor learning. It has remained controversial whether patients with degenerative cerebellar disease benefit from high-intensity coordinative training. Moreover, it remains unclear by which training methods and mechanisms these patients might improve their motor performance. Here, we review evidence from different high-intensity training studies in patients with degenerative spinocerebellar disease. These studies demonstrate that high-intensity coordinative training might lead to a significant benefit in patients with degenerative ataxia. This training might be based either on physiotherapy or on whole-body controlled videogames ("exergames"). The benefit shown in these studies is equal to regaining one or more years of natural disease progression. In addition, first case studies indicate that even subjects with advanced neurodegeneration might benefit from such training programs. For both types of training, the observed clinical improvements are paralleled by recoveries in ataxia-specific dysfunctions (e.g., multijoint coordination and dynamic stability). Importantly, for both types of training, the retention of the effects seems to depend on the frequency and continuity of training. Based on these studies, we here present preliminary recommendations for clinical practice, and articulate open questions that might guide future studies on neurorehabilitation in degenerative spinocerebellar disease.

Figures

Figure 1

Coordinative physiotherapy. (a) Exemplary exercise of the training protocol: training of dynamic balance and multijoint coordination. (b) Group data of the clinical ataxia score SARA before training intervention (BT), after the four weeks training intervention (AT) and for follow-up assessment (F1J) after one year. Stars indicate significant differences between examinations (*P < 0.05). SARA: scale for the assessment and rating of ataxia ([35] reproduced with permission from Wiley).

Figure 2

Exergame-based training. (a–c) Screenshots from the three XBOX Kinect games used in the training protocol. (a) 20000 leaks practice whole-body coordination and interaction with a dynamic environment; (b) table tennis practices goal-directed upper limb movements and dynamic balance, as well as movement timing; (c) light race practices goal-directed lower limb movements, fast movements, and dynamic balance. (d) Snapshot from the “Light Race” game. Patient C1 performs dynamic stepping movements in order to control the avatar to step onto the highlighted areas on the floor (figures reproduced with permission from Microsoft Xbox Kinect (a), (b) and Ubisoft (c), (d)). (e, f) Group comparisons of the clinical ataxia scores (SARA) and lateral sway in gait at examinations E1–E4. Patients were examined four times: two weeks before intervention (E1), immediately before the first training session (E2), after the two-week lab-training period (E3), and after the six-week home-training phase (E4) [43]. Stars denote significance: *P < 0.05, **P < 0.01.