This presentation by Dr Lisa Bunn looks at the practical management of ataxia and balance impairment among people with MS. It includes information on assessing ataxia, the rehabilitation of balance, and relevant research studies. It was presented at the MS Trust Annual Conference in November 2013.

1. Practical management of ataxia and
balance impairment: Part 2
Tuesday 5th November 2013
Dr Lisa Bunn

2. What do we mean when we talk about ataxia?
Test yourself on the full spectrum of ataxia signs and symptoms:
B…… I……….
G… I………
St…… B…….
Si….. B…….
In………..
Sp…. I……… (D……..)
Sw….. I…….. (D……)
D……………..
T…..
D…….
Ab…… E.. M………
N…….
S….. P……
D…….. S……
Plus symptoms:
V……
B…… V……. (O……..)

3. What do we mean when we talk about ataxia?
Balance Impairment
• Gait Impairment
• Standing Balance
• Sitting Balance
Incoordination
Speech Impairment
(Dysarthria)
Swallow Impairment
(Dysphagia)
Dysdiadochokinesia
Tremor
Dysmetria
Abnormal Eye Movements
• Nystagmus
• Smooth Pursuit
• Dysmetric Saccades
Plus symptoms:
Vertigo
Blurred Vision (Oscillopsia)

4. Neuro-radiology
SCA8 severe cerebellar atrophy
MS cerebellar plaques
Hain (2012) http://www.dizziness-and-balance.com/disorders/central/ms.htm

5. Functional consequences still remain hard to predict…
Imamizu H et al. PNAS 2003;100:5461-5466

6. Assessment
•
•
•
•
•
•
•
Disease severity
• SARA
Balance impairment
• Kinematics, functional reach, Berg, BESTest,
ABC questionnaire, BalSARA
Gait impairment
• Kinematics, the rivermead gait assessment,
timed 10m walk, 6 minute walk, TUG
Oculomotor
• Oculomotor examination
Inccordination
• 9 hole PEG test
Speech
• PATA test (30s)
Dysphagia
• Swallow assessment

7. Assessment
•
Disease severity
• The Scale for Assessment and Rating of Ataxia (SARA)
http://www.ataxie.nl/wp-content/uploads/diversen/SARA.pdf

8. Ataxia UK’s 2013
(updated guidelines)
are about to be
released…
•
Cover all aspects of management
•
Marsden, Bunn, Kilbride et al., 2013
•
Physiotherapy supplement (may be
published elsewhere this year)
•
Now 20 further therapy studies (20092013) and a systematic review

9. To what types of ataxia do the guidelines refer?
•
MS is only one cause of ataxia
Stroke
Cerebellar / posterior fossa tumours
Foramen magnum compression
Multiple system atrophy
Gluten intoxication
Carbon monoxide poisoning
Sensory loss
Hypothyroidism
from peripheral
Traumatic brain injury
Chronic
neuropathy
alcoholism
Inherited types:
Spino-cerebellar ataxias (SCA1,2,3,6)
Friedrich’s ataxia
Ataxia-telangectasia
Todd and Arbor (2009) http://www.els.net/WileyCDA/ElsArticle/ref1d-a0021592

10. Rehabilitation of balance
The evidence base is accumulating…
•Informed by investigation of balance in people with pure types of
cerebellar disease (Bunn, UCL libraries, 2010)
•Effectiveness evaluated by 4 clinical trials:
» Ilg et al., 2009, 2010 (adult)
» Ilg et al., 2012 (paediatric)
» Miyai et al., 2012 (adult, RCT)
» Bunn et al., 2013 (adult, pilot RCT, in submission)
•Sample size estimates and validation of outcome measures
– Bunn et al., 2013
•Fully powered RCT planned 2015.

11. Ilg et al., 2009-2012
Ilg et al., 2009, 2010 (adult)
•General balance exercises
•Intensive rehab (inpatient)
•Community exercise as follow-up
•1 year follow-up effects reported
Ilg et al., 2012 (paediatric)
•Exer-gaming
•Progressive ataxias
•Improvements observed
•SARA, balance and gait
•Feasibility established
•Underpowered & mixed groups

12. Quantifying balance impairment
Fig. 1: 40s trunk sway
Fig. 2: Correlation between sway speed & SARA
Potential for a continuous measure of disease severity from which
disease progression and intervention effects could be measured.
Bunn et al., Mvt Dis, 2013

13. Applications of ‘quantifying balance’ findings
A continuous measure of:
•Balance impairment – more
sensitive to change than Berg or
Bal-SARA
•Disease severity from which
disease progression could be
measured.
•Intervention effects in future
RCTs
Support for use of othoses
(Ramdharry et al., 2006)

14. Research hypothesis
Sensory processing for balance
control is disrupted in persons with
cerebellar disease pathology.

15. Research approach
Human model of pure cerebellar disease
pathology (SCA6)
Whole body sway responses to sensory stimuli
•Moving visual scenery (MVS)
•Galvanic vestibular stimulation (GVS)
•Muscle vibration (VIB)
• Tibialis anterior and gastrocnemius)

16. Method
Randomised trials:
Direction
Modality
No-stimuli
Measures of body sway:
Trunk markers (C7)
Force responses

17. Results
*p<0.001
p>0.05
p>0.05

18. Translational neuroscience: From lab to
living room…
Can we train avoidance of moving visual cues for
balance in order to increase reliance on vestibular
and proprioceptive control?
•
•
•
•
Pilot of 12 subjects with SCA6
Regime of balance exercises (15min/day)
MVS played during exercise
4 week baseline, 4 week intervention (therapy/control groups)

19. Method

21. Results: Feasibility
• Feasibility: of home-based balance training,
assessment, trial design and technology: iPod touch,
XSens MTx and software for sway measures.
• Dropout: rates ascertained: 2/12 (T=1, C=1).
• Impact: Daily fatigue VAS scores were not affected by
training. Training times documented mean 20mins/day,
x5 per week.
• Adverse events: Advice for those with ear infections to
avoid training.

22. Pilot results

23. Subjective experiences
“I feel stiller and as a result I am fitter-I managed to carry
18 paving slabs from my back garden to the allotment,
which I don’t think I could have done before.”
“I enjoyed the daily training and it’s made me more
motivated to do something else now that the study has
finished”.
“The trial has made me more aware of tying things
together- double vision, losing temper, depression, all
connected with tiredness.”

24. Applications of ‘investigating balance’ findings
•
General exercises could help to restore balance for people with
ataxia
•
Targeted balance interventions could provide additional benefits at
impairment, functional and participation levels
•
We now have the tools to embark on RCTs of therapy options
•
Before effectiveness of targeted interventions is established we can
try general balance exercises and monitor disease progression with
the SARA

25. Clinical advances and remaining questions
•
SARA (Schmidt-Hubsch et al.,
2006)
•
Berg
•
Sway test (Lord, 1998)
•
Balance impairment measures
(Clark et al., GaitPost, 2010;
WeeWiihabilitation, Brady et
al.,2013, in preparation)
•
How do non-ataxia signs affect
our understanding of balance?

26. Questions?
Thanks to:
UCL (sponsor)
NHNN (NHS sponsor) Ataxia UK (funders & sponsors)
Prof Brian Day
Prof Jonathan Marsden
Dr Paola Giunti
All participants and their families.

27. Key Structure References
•
Apps and Garwicz, 2005, Anatomical and Physiological Foundations
of Cerebellar Information Processing. Neuroscience. Nature
reviews. Vol 6. p297-311.
– Available for free download at:
https://www.stanford.edu/group/brainsinsilicon/documents/GarwiczInfoProc.pd
•
Brain atlas:
– http://www.med.harvard.edu/AANLIB/cases/casesNA/pb9.htm
•
Buttner N, Geschwind D, Jen J, Perlman S, Pulst S, Baloh R (1998)
Oculomotor Phenotypes in Autosomal Dominant Ataxias Arch
Neurol. 55:1353-1357
– Available for free download at:
http://archneur.ama-assn.org/cgi/reprint/55/10/1353.pdf
•
Purves D, Augustine GJ, Fitzpatrick D, et al., 2001. Neuroscience.
2nd edition. Chapter 19: Organisation of the Cerebellum.
Sunderland (MA): Sinauer Associates

28. Key Function References
•
Apps and Garwicz, 2005, Anatomical and Physiological Foundations of Cerebellar
Information Processing. Neuroscience. Nature reviews. Vol 6. p297-311.
–
•
•
•
•
•
•
•
Available for free download at:
https://www.stanford.edu/group/brainsinsilicon/documents/GarwiczInfoProc.pdf
Babinski J (1899) De l’asynerhie cerebelleuse. Rev Neurol. Vol.7. p685-686.
Bastian, AJ (2011) Moving, sensing and learning with cerebellar damage. Current Opinion
in Neurobiology. Vol.21. p1-6.
Braitenberg V, Atwood RP (1958) Morphological observations of the cerebellar cortex. J
Comp. Neurol. Vol 109. p1-33.
Boyden ES, Katoh A & Raymond JL (2004) Cerebellum dependent learning: The role of
multiple plasticity mechanisms. Annu Rev Neurosci. Vol 27. p581-609.
Houk JC, Buckingham JT & Barto AG (1996) Models of the cerebellum and motor learning.
Behav Brain Sci. Vol. 19. p368-383.
Thatch WT (1996) On the specific role of the cerebellum in motor learning and cognition:
clues from PET activation and lesion studies in man. Behav Brain Sci. Vol 19. p411-431.
Thatch WT (1998) A role for the cerebellum in learning movement coordination. Neurobiol
Learn Mem. Vol 70. p177-188.

29. Key Assessment References
•
•
•
•
•
•
Schmitz-Hubsch, T, Fimmers, R, Rakowicz M, Rola R., Zdzienicka, E, Fancellu R, Mariotti C, Linnemann C, Schols L,
Timmann D, Filla A, Infante J, Giunti P, Kremer B, van de Warrenburg BPC, Baliko L, Melegh B, Depondt C, Schulz J,
Tenezes de Montcel S, and Klockgether T (2009). Responsiveness of different rating instruments in spino-cerebellar
ataxia patients. In press.
Schmitz-Hubsch, T. Clinical assessment of a patient with spinocerebellar ataxia - the challenge of research. SchmitzHubsch, T. 1-8. 2006. 2006.
Schmitz-Hubsch, T., Tezenas du Montcel, S., Baliko, L., Berciano, J., Boesch, S., Depondt, C., Giunti, P., Globas, C.,
Infante, J., Kang, J. S., Kremer, B., Mariotti, C., Van De Warrenburg, B. P., Melegh, B., Pandolfo, M., Rakowicz, M., Ribai,
P., Rola, R., Schols, L., Szymanski, S., Durr, A., and Klockgether, T. (2006). Scale for the assessment and rating of ataxia:
Development of a new clinical scale. Neurology 66, 1717-1720.
Schmitz-Hubsch, T., Coudert, M., Bauer, P., Giunti, P., Globas, C., Baliko, L., Filla, A., Mariotti, C., Rakowicz, M., Charles,
P., Ribai, P., Szymanski, S., Infante, J., Van de Warrenburg, B. P. C., Durr, A., Timmann, D., Boesch, S., Fancellu, R.,
Rola, R., Depondt, C., Schols, L., Zdienicka, E., Kang, J.-S., Dohlinger, S., Kremer, B., Stephenson, D. A., Melegh, B.,
Pandolfo, M., Di Donato, S., du Montcel, S. Tezenas, and Klockgether, T. (2008). Spinocerebellar ataxia types 1, 2, 3, and
6: Disease severity and nonataxia symptoms. Neurology 71, 982-989.
du Montcel, Sophie Tezenas, Charles, Perrine, Ribai, Pascale, Goizet, Cyril, Bayon, Alice Le, Labauge, Pierre, GuyantMarechal, Lucie, Forlani, Sylvie, Jauffret, Celine, Vandenberghe, Nadia, N'Guyen, Karine, Ber, Isabelle Le, Devos, David,
Vincitorio, Carlo Maria, Manto, Mario Ubaldo, Tison, Francois, Hannequin, Didier, Ruberg, Merle, Brice, Alexis, and Durr,
Alexandra (2008). Composite cerebellar functional severity score: validation of a quantitative score of cerebellar
impairment. Brain awn059.
Duke, J., McEvoy, M., Sibbritt, D., Guest, M., Smith, W., and Attia, J. (2007). Vibrotactile threshold measurement for
detecting peripheral neuropathy: defining variability and a normal range for clinical and research use. Diabetologia 50,
2305-2312.

30. Key Disease References
•
Ataxia UK (2009) Management of the ataxias: towards best clinical practice.
–
•
•
•
•
Bastian AJ, Martin T, Keating J, Thatch W (1996) Cerebellar ataxia: abnormal control of interaction
torques across multiple joints. Journal of neurophysiology. Vol. 76(1). p.492-509vol. 76 no. 1 492-509
Bastian, AJ (2011) Moving, sensing and learning with cerebellar damage. Current Opinion in
Neurobiology. Vol.21. p1-6.
Klockgether, T(Ed) (2000) Handbook of Ataxia. Informa Healthcare Publishers.
Buttner N, Geschwind D, Jen J, Perlman S, Pulst S, Baloh R (1998) Oculomotor Phenotypes in
Autosomal Dominant Ataxias Arch Neurol. 55:1353-1357
–
•
•
•
•
•
Available for free download at: http://www.ataxia.org.uk/pages/resources-and-publications.html
Available for free download at:
http://archneur.ama-assn.org/cgi/reprint/55/10/1353.pdf
Horak FB, Diener HC. (1994) Cerebellar control of postural scaling and central set in stance. J
Neurophysiol 72: 479–493.
Liu et al. (1997) Analysis if action tremor and impaired control of movement velocity in Multiple
Sclerosis during visually guided wrist tracking tasks. Movement Disorders. Vol 12 (6). p992-999.
Marsden, J and Harris, C (2010) Cerebellar ataxia: Pathophysiology and rehabilitation. Clinical
rehabilitation. Vol 25 (3). P195-216.
Mauritz KH, Dichgans J, Hufschmidt A (1979) Quantitative analysis of stance in late cortical cerebellar
atrophy of the anterior lobe and other forms of cerebellar ataxia. Brain. Vol 102. p461-482.
Schmahann and Scherman (1998) The cognitive affective syndrome. Brain. Vol 121. p561-579.

31. Key Management References
• Freeman JA, Gear M, Pauli A, Cowan P, Finnigan C, Hunter
H, Mobberley C, Nock A, Sims R, Thain J. The effect of core
stability training on balance and mobility in ambulant
individuals with multiple sclerosis: a multi-centre series of
replicated single case studies. (Online ahead of publication in
Multiple Sclerosis June 16th 2010)
• Hall, J., Freeman, J. A., Zaijeck, J., Hobart, J. C. & Marsden,
J. F. (2010) 'Factors influencing the applied torque during
manually applied plantarflexor stretches in people with
Multiple Sclerosis In:Chartered society of physiotherapy
(CSP) Congress', Liverpool, 15-16 Ocotber 2010 (UK ), pp.
Chartered Society of Physiotherapy.
• Wardle M and Robertson N (2007) Progressive Late-Onset
Cerebellar Ataxia (Review) ACNR 7:2, p6-12
– Medical orientated review

32. Key Research References
• Bunn L: Sensory mechanisms of balance control in pure
cerebellar disease. University College London; 2011.
• Bunn LM, Marsden JF, Giunti P, Day BL: Stance instability
in spinocerebellar ataxia type 6. Mov Disord 2012, 28:510–
516.
• Ilg W, Synofzik M, Brötz D, Burkard S, Giese M a, Schöls L:
Intensive coordinative training improves motor
performance in degenerative cerebellar disease.
Neurology 2009, 73:1823–30.
• Ilg W, Schatton C, Schicks J, Giese M, Schols L, Synofzik M:
Video game-based coordinative training improves ataxia
in children with degenerative ataxia. Neurology 2012,
79:2056–60.