Physiotherapy management of cerebellar ataxia

The Romberg test targets

1. Sensory disorders

2. Cerebellar disorders

A lesion to the right cerebellar hemisphere will cause ataxic signs on the :

1. Right arm

2. Left arm

Isometrataxia is a typical sign of ataxia. What does it mean ?

1.Difficulty performing symetrical actions with both arms

2. Difficulty maintaining a constant force during an action

Physiotherapy management of cerebellar ataxia

3-step assessment

1. Confirming your patient has ataxia and which signs are prevalent

- Pronation/Supination test (dysdiadochokinesia)

- Finger-Nose test (dysmetria & intention tremor)

- Look for typical ataxic signs in tandem walk / gait

- Romberg test (differential diagnosis)

References

What is cerebellar ataxia ?

2. Evaluate your patient's functional level

Non ataxia specific functional assessment tools:

TUG

6MWT

10MWT

Dynamic gait index

There are 3 types of ataxia (lack of coordination):

QUIZ

3. Use validated ataxia-specific scales :

- For research

- For multiprofessional communication

The rating scales (clinimetrics) :

SARA – Scale for the assessment and rating of ataxia

ICARS – International cooperative ataxia rating scale

FARS – Friedreich’s ataxia rating scale

The symptoms :

1. Limb movement disorder (general dyscoordination, tonus abnormality, dysmetria, tremor)

2. Postural and gait disorders (unstable inefficient gait)

3. Oculomotor deficit

4. Dysarthria & dysphagia

5. Cognitive and Affective deficits

Review your knowledge of the cerebellum

Lawrence E, Coshall C, Dundas R, Stewart J, Rudd A, Howard R, Wolfe C. Estimates of the prevalence of acute stroke impairments and disability in a multiethnic population. Stroke 2001; 32:1279–1284.

Levin H, Williams D, Valastro M, Eisenberg H, Crofford M, Handel S. Corpus callosum atrophy following closed head injury: Detection with magnetic resonance imaging. J Neurosurg 1990; 73:77–81.

Cassidy E, Killbridge C, Holland A. Management of the Ataxias: towards best Clinical Practice. Physiotherapy Supplement. 2009.

Armutlu K, Karabudak R, Nurlu G. Physiotherapy approaches to the treatment of ataxic multiple sclerosis: a pilot study. Neurorehabilitation and Neural Repair. 2001;15

(3):203–11.

Miyai I, Ito M, Hattori N, Mihara M, Hatakenaka M, Yagura H, Sobue G, Nishizawa M. Cerebellar Ataxia rehabilitation Trialists Collaboration. Cerebellar ataxia rehabilitation trial in degenerative cerebellar.Neurorehabil

Neural Repair. 2012;26(5):515-22.

Brown TH, Mount J, Rouland BL, Kautz KA, Barnes RM, Kim J. Body weight-supported treadmill training versus conventional gait training for people with chronic traumatic brain injury. J Head Trauma and Rehab. 2005 ; 20 (5):

402-15.

Wiles CM, Newcombe RG, Fuller KJ, Shaw S, Furnival-Doran J, Pickersgill TP, et al.Controlled randomised crossover trial of the effects of physiotherapy on mobility in chronic multiple sclerosis. Journal of Neurology

Neurosurgery and Psychiatry. 2001;70(2):174–9.

Jones L, Lewis Y, Harrison J. The effectiveness of occupational therapy and physiotherapy in multiple sclerosis patients with ataxia of the upper limb and trunk. Clin Rehab. 1996 ; 10, 277-282.

Lord SE, Wade DT, Halligan PW. A Comparison of two physiotherapy treatment approaches to improve walking in multiple sclerosis: a pilot randomized controlled study. Clinical Rehabilitation. 1998;12:447–86.

Schmitz-Hübsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, et al. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology. 2006 ;66:1717–20.

Subramony SH, May W, Lynch D, Gomez C, Fischbeck K, Hallett M, et al." Measuring Friedreich ataxia: interrater reliability of a neurological rating scale neurologic rating scale. Neurology. 2005 ;64:1261–2.

Fahey MC, Corben L, Collins V, Churchyard AJ, Delatycki MB. How is disease progress in Friedreich’s ataxia best measured? A study of four rating scales. J Neurol Neurosurg Psychiatry. 2007;78:411.

Storey E, Tuck K, Hester R, Hughes A, Churchyard A.Inter-rater reliability of the International Cooperative Ataxia Rating Scale (ICARS). Mov Disord. 2004 ;19:190–2.

Weyer A, Abele M, Schmitz-Hübsch T, Schoch B, Frings M, Timmann D, et al. Reliability and validity of the scale for the assessment and rating of ataxia: a study in 64 ataxia patients. Mov Disord. 2007 ;22:1633–7.

What our review taught us

Functions of the cerebellum:

Coordination of voluntary movements

Maintainance of balance

Muscle tone tuning

Cognitive function

But ...

We found

6 RCTs about physiotherapy interventions

5 scientific articles about rating scales

Our research question was very general.

We have a limited amount of articles.

(5 assessment and 6 intervention)

The assessment part of our review only covers scales specific to ataxic syndromes and we did not evaluate these articles systematically.

The general recommendations based on our review

Our conclusions

What does our PAP consist of

Overall, we now know there is:

- Limited good quality evidence about the effectiveness of physiotherapy for ataxia

- Limited poor quality evidence to choose between different treatment options

- No scientific data about the use of other non specific assessment tools

- Scientifically approved grading scales specific to ataxia

What causes those lesions to the cerebellum / cerebellar pathways ?

Our recommendations

During Semester 3, we missed some information about Ataxia so we decided to fill in the gap :

We defined 8 different categories of etiologies for ataxia :

Course material to teach 2nd year ESP student :

A didactic course manual

A Powerpoint presentation for a class of 1.40 h

- General approach : Focus on working around ataxia since the cerebellum function itself most likely will not improve with physiotherapy

- Treatment choice : Favor the tools available to physiotherapists which received some scientific evidence

- Assessment : Use the ataxia specific scales, as well as, functional scales not specific to ataxia

- Researchers should focus on the long term effect of physiotherapy compared to no treatment before focusing on the comparison between treatment options.

Intervention

1. Cerebral Vascular Accidents (mostly affecting the cerebellar posterior-inferior arteries)

2. Immune disorders (mostly multiple sclerosis)

3. Tumors in the posterior fossa

4. Traumatic brain injury in the cerebellar region

5. Autosomal recessive cerebellar ataxias are a group of ataxias (ACARs) such as Friedreich's ataxia.

6. Autosomal dominant ataxias also called SpinoCerebellar Ataxias (SCAs)

7. Development disorders affecting the cerebellum (Chiari malformation, Cerebellar hypoplagia)

8. Intoxications mostly due to alcohol (such as in the Wernicke-Korsakoff syndrome)

We based the course material on a literature review where our research question was : What is the best evidence for

In the absence of good quality evidence to guide our physiotherapy practice we can consider the following clues.

- the effectiveness of physiotherapy compared to no treatment for cerebellar ataxia (any etiology)

- the effectiveness of specific physiotherapy treatments compared to standard physiotherapy for cerebellar ataxia (any etiology)

- the validity, responsiveness and reliability of clinimetrical tools used to assess cerebellar ataxia (any etiology)

It is important for physiotherapist to know about ataxia because it is a misunderstood disorder common among neurological patients:

- Ataxia is present in at least :

20 % of stroke patients

40 % of MS patients

10 % of TBI patients

- Specific ataxic syndromes have a higher prevalence than well known diseases such as Huntington's and motor neuron diseases .

(Lawrence 2001, Kraft 1986, Levin 1990)

Estimate: 10 000 adults affected in the UK (Cassidy et al. 2005)

The interventions that lower quality evidence tends to deter:

For lack of evidence despite many attempts:

Axial weighting

For lack of evidence + possible adverse effects:

Mobility aid with strong weight bearing on UE

Lycra garnment

Wrist weighting

The interventions that lower quality evidence

tends to encourage :

Balance training

Core training

Strength & flexibility

Gait training : treadmill and /or over ground

Cold therapy to reduce tremor

(very short term)

Keep in mind your role is to help the patient, not to treat ataxia.

Isabelle Gontard, Caroline Hiensch, Lisa Zinkwe

PAP 2014

Thank you for your attention

Time for your questions