M arkus Quandt

1. Move and improve - how coordinative training helps ataxia Elektroschwache und Kosmische Strings Ludger Schöls Department of Neurology and Hertie-Institute for Clinical Brain Research University of Tübingen Markus Quandt San Antonio, 16.03.2012

2. Physikalische Eigenschaften Presenter disclosures • Ludger Schöls has no relationships to disclose or list

3. Outline • Cerebellum and motor learning • Does physiotherapy help in ataxias? • Which physiotherapy concept is successful? • A flash in the pan or longterm effects?

4. Cerebellum is the place of motor learning Marr (1969) & Albus (1971): Cerebellum is the „motor learning machine“ Examples: Motor use of new tools (Imamizu et al., 2000) Fast recalibration of internal motor programs - e.g. saccadic adptation (Barash et al., 1999) - e.g. adaptation to new force fields (Maschke et al, 2004) - e.g. adaptation of anticipated motor prediction

5. Cerebellar degeneration in ataxias Regeneration of cerebellar function after focal lesions is well established: • e.g. after tumor or stroke • limited regeneration if cerebellar nuclei are affected Ataxias are degenerative diseases • Degeneration is a generalized rather than a focal process • No healthy regions left that can take over for affected parts • Doubts that the cerebellum can still learn motor functions with a degenerative ataxia This matches with the experiences of some patients that physiotherapy was not particular helpful to them

6. Which physiotherapy? But: Does that proof physiotherapy ot be ineffective? If a pain killer does not help against high glucose levels this does not mean that drugs are ineffective in diabetes So: Which physiotherapy did not help? • Vojta? • Bobath? • Isometric training? • Massage / relaxation? • Balance? • Coordinative training? Which physiotherapy concepts do help? No physiotherapy concept had been evaluated in ataxia !!!

7. Active coordinative training Neurology 2009; 73:1823-1830 Concept of Doris Brötz (Tübingen): • Active release of „fixed“ movement patterns • Traning of static balance • Training of dynamic balance • Whole body movements • Falling strategies and Steps to prevent falling • Movements to treat and prevent contractures • Rather few exercises but frequent repetitions

8. Study design Physio Follow up Longterm Intrinsic control W60 W 0 W 8 W12 W20 V1 V2 V3 V4 V5 Intervention: 4 week course with 3 physiotherapy sessions a week = 12 x physiotherapy per patient

9. Read out • Goal attainment score (Patient) • Berg balance score (Physiotherapist) • Ataxia rating scale (Neurologist) • Movement analysis (Computer)

10. Goal attainment score (GAS) GAS addresses indivudal goals in daily life selected by the patient 0: Stage at entry of study 1: Less than expected 2: Expected outcome 3: Better outcome than expected 4: Much better than expected 0 1 2 3 4 Kiresuk et. al., 1994; Lawrence Erlbaum Associates Inc.

11. Berg balance score (BBS) The BBS rates balance in a physiotherapeutic examination 14 items addressed • Sitting • Stance • Gait • Timed movements Berg et. al., 1989; Physiotherapy Canada

12. SARA Scale for the assessment and rating of ataxia (SARA) Higher scores indicate more severe problems 8 items, maximum sum score: 40 Item 1: Gait (8 points) Item 2 Stance (6 points) Item 3: Sitting (4 points) Item 4: Speech (6 points) Item 5: Finger chase (4 points) Item 6: Finger pointing (4 points) Item 7: Diadochokinesia (4 points) Item 8: Heel-shin slide (4 points) Schmitz-Hübsch et. al., 2006; Neurology

13. Computerized movement analysis • Registration of three-dimensional movement trajectories by 41 reflecting markersusing a VICON motion capture system with 10 infrared cameras • Analysis of complex whole body movmements for variability in room and time • Stance: sway is assessed as path length of the center of gravity while standing with feet together • Gait: Analysis of intra-limb coordination by the angle-angle plotsof the hip and knee joints  temporal variability measure: vbt • Dynamic balance on a treadmill with sudden backward move

14. Results • Improvement after training: - 5.2 SARA points ~ progression of 2 – 4 years • Goal attainment: 2.5 = more than expected • Gait velocity, intra-limb coordination, static and dynamic balance all improved • More benefit with cerebellar rather than afferent ataxia • Persistent effects after 8 weeks but better with regular training at home: - 0.4 vs +1.0 Ilg et al, Neurology 2009

15. Case S.T. - stance 48 year old kindergarten teacher Idiopathic cerebellar ataxia Disease duration 1.5 years Physiotherapy before the study: • Stabilisation exercises • Isometric training Pre Post

16. Case S.T. - gait 48 year old kindergarten teacher Idiopathic cerebellar ataxia Disease duration 1.5 years Physiotherapy before the study: • Stabilisation exercises • Isometric training Pre Post

17. Case S.T. – complex movements 48 year old kindergarten teacher Idiopathic cerebellar ataxia Disease duration 1.5 years Physiotherapy before the study: • Stabilisation exercises • Isometric training Pre Post

18. Case S.T. – stairs 48 year old kindergarten teacher Idiopathic cerebellar ataxia Disease duration 1.5 years Physiotherapy before the study: • Stabilisation exercises • Isometric training Pre Post Goal attainment score 0: Climbing stairs only with banister 1: Intermittend use of the banister 2: No banister required for 2 steps in both directions 3: Staircase upwards without banister 4: Staircase up and down without banister

19. Longterm effects • Even after 1 year SARA was better than baseline especially in the cerebellar group • Goal attainment: After 1 year still better than expected • Improvement in intra-limb coordination persisted over 1 year in the cerebellar group • Patients performing continuous exercises were doing better than those without training Ilg et al, Mov Disord 2010

20. Conclusions • Physiotherapy is able to improve ataxia even in cerebellar degeneration • Active, coordinative training is a successful physiotherapy concept in ataxia • Effects are visible not only as a group mean but also on an individual basis • Effects persist over long term especially if a continuous training is performed • Patients with afferent ataxia do profit but cerebellar ataxia is likely to respond even better • Improvements meet individual goals in every day life Move and improve!

21. Thank you … … and the dream team! Doris Brötz Matthis Synofzik Winfried Ilg