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Garima Gupta

MPT Neurology
Indian Institute Of Rehabilitation Sciences
New Delhi
Contents
◙ Standing and walking
◙ Role of cerebellum
◙ What is gait ataxia and its causes?
◙ Assessments of ataxia
◙ Specific scales for Ataxia and their validity & reliability
◙ Goals in Gait training in Ataxia
◙ Interventions and evidences.
◙ References

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Standing and Walking

Muscle power

Postural sensibility

Central co-ordinating mechanism

Brain & Bannister’s clinical neurology 7th edition

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Role of cerebellum

MOTOR
CORTEX

PERIPHERAL
FEEDBACK
MECHANISM

CEREBELLUM

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Role Of Cerebellum Cont…

 Hence cerebellum acts as : Comparator


Error correcting mech.

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Role Of Cerebellum Cont…
 The cerebellum is important for movement control and plays a
particularly crucial role in balance and locomotion.
 Recent work suggests that it plays a role in the generation of
appropriate patterns of limb movements, dynamic regulation
of balance, and adaptation of posture and locomotion through
practice.

______________________________________________________________________
Cerebellar control of balance and locomotion.
Morton SM, Bastian AJ.
Kennedy Krieger Institute and Department of Neurology, Johns Hopkins University School of Medicine,
Baltimore, MD 21205, USA.

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Role Of Cerebellum Cont…

• posture, equilibrium, and locomotion (vermis and


Medial fastigial nuclei)
cerebellum

Intermediate • control of discrete, ipsilateral limb movements.


Cerebellum

Lateral • control of complex, visually guided limb


movements and the planning of those movement
Cerebellum (lateral hemisphere and dentate nuclei)

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Role Of Cerebellum Cont…

 Animal studies also confirmed the fact that the control of balance in
stance and locomotion is dependent on the medial part of the
cerebellum (Thach and Bastian, 2004) while the control of goal-
directed movements and perturbed or visually guided walking is
influenced by the intermediate and lateral parts of the cerebellum.
(Cooper et al.2000)

______________________________________________________________
Relative Contributions of Balance and Voluntary Leg-Coordination Deficits toCerebellar Gait Ataxia
Susanne M. Morton1 and Amy J. Bastian
J Neurophysiol 89: 1844–1856, 2003;
Specific influences of cerebellar dysfunctions on gait
Heidrun Golla et al.
Brain (2007), 130, 786^798

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Ataxic Gait and
position in standing
& Gait

a. Sways to the right in


standing position.

b. Steady on the right

leg.

c. Unsteady on the left

leg.

d. Ataxic gait.

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Gait Ataxia

Cerebellar ataxic gait is typically characterized by an instable


stumbling walking path, increased step width and high variability of
gait (Diener and Dichgans, 1996; Morton and Bastian, 2004).

Ataxia is a common sign in a variety of disorders.


Some conditions displaying ataxia are inherited
Insidious, or congenital
Stroke
Traumatic brain
Metabolic disorders.

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Cerebellar infarcts, hypoxia
Dorsal spinal cord compression from vertebral fractures
Alcohol
Drug abuse
Vestibular dysfunction may result in ataxia.

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What are the bases for cerebellar recovery??
Recovery after cerebellar lesions or disease in humans is poorly
documented.

These is, however, strong evidence of recovery after cerebellar lesions in


experimental animals, which suggests that if the cerebellum is not totally
destroyed, neighboring areas of the cerebellum can adapt or compensate
for the impaired region.

Possible mechanisms of recovery after central nervous system lesions may


include:
Neural sprouting,
Vicarious functions,
Functional reorganization,
Substitution.
____________________________________
Kathleen M Gill-Body et al
Physical therapy 1997

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Assessment
History

Systems review: cardiovascular system, musculoskeletal


system(such as foot and spine deformities)

Cranial nerves: ocular movements, visual field, acuity deficits,


hearing loss, dysarthria, dysphagia.

Motor functions: symmetry, ROM, muscle strength, spasticity may


present later in the disease.

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Deep tendon reflexes and superficial reflexes: decrease or
absent.

Positive babinski: later in disease.

Sensory integrity: sensory neuropathy may present.

Coordination tests:

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 Balance measures:
Functional reach test,
Pediatric balance scale,
Timed “Up and Go”,
Timed Up and Down stairs test and
measurement of static standing.

 Gait assessment: wide base of support, unequal step length,


decreased velocity etc…
______________________________________________
Presentation and Progression of Friedreich Ataxia and Implications for
Physical Therapist Examination Joyce R Maring, Earllaine Croarkin
Volume 87 Number 12 Physical Therapy

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Composite Performance Measures
A. International Cooperative Ataxia Rating Scale:

100 point scale


10-15 mins
Domains:
i. Posture and gait disturbance
ii. Kinetic functions
iii. Speech disorder
iv. Oculomotor disorder

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B. Friedreich’s Ataxia Rating Scale:
30 mins
Domains:
I. Functional staging of ataxia
II. Activities of daily living
III. Neurologic examination
1. Bulbar
2. Upper limb coordination
3. Lower limb coordination
4. Peripheral nervous system
5. Upright stability

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C. Ataxia Clinical Rating Scale
D. Functional Ataxia Scoring Scale
E. Inherited Ataxia Progression Scale
F. Inherited Ataxia Clinical Rating Scale
G. Northwestern University Disability Scale

_________________________________________________
Presentation and Progression of Friedreich Ataxia and Implications for
Physical Therapist Examination
Joyce R Maring, Earllaine Croarkin
Volume 87 Number 12 Physical Therapy

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History Vestibular Cerebelllar Sensory
Vertigo Present ++
Paroxysmal,
constant, or
waxing and
waning Sometimes Absent
may be worse present
with head
movements,
+/- noise, or
Valsalva
maneuvers
Limb May be present
paresthesia or Absent with brainstem Present
numbness involvement
Ataxia worse in Only if
the dark bilateral Absent or rare Present
vestibulo-
pathy

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History Vestibular Cerebelllar Sensory
Cerebellar
signs
•Tremor
•Ataxia
•Dysmetria Absent Present Absent
•Dysynergia
•Dysdiadoch
okinesis
•Dysarthria
•Titubation
•Impaired
eye pursuit
Nystagmus Often present Often present
acutely central type Absent
peripheral type
Peripheral
proprioceptive Absent Absent Present
sensory deficit
Romberg's test May be
present if Absent Present
there is a
bilateral
vestibulo
pathy
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Gait Training In Ataxia
Goals:
Minimize disability, deformity and pain.
Prolonging locomotor skills.
Maintaining or improving patient’s ability to continue to participate in
all environmental contexts for as long as possible.
Patient and family education about the effect of disease progression
on function and life style, potential therapeutic interventions and
realistic expectation about those interventions.
Once the patient is on established home exercise program ongoing
clinical evaluation with changes in the home program as needed.

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Interventions
1. Proximal muscle stabilization exercises & Pre gait training
2. Strengthening exercises
3. Stretching exercises
4. Coordination exercises
5. Balance exercises
6. Vestibular Rehabilitation
7. Aerobic fitness and treadmill training
8. Body weight supported treadmill training
9. Maintenance of biomechanical alignment

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10.Weight bearing ex’s & gait training in parallel bar
11.Biofeedback
12.Hydrotherapy
13. Adaptive devices:
Walker / Cane

 Power scooter
 Wheel chair
14.Newer concepts in training of gait ataxia
15. Home exercises

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Proximal muscle stabilization exercises & Gait training
:

 To improve postural stability.


 Prone

 Forearm supported prone lying

 Reaching and B/L and U/L weight bearing activities and weight
shifting activities.

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 Quadruped position weight transfer and reach outs.

Kneeling position weight transfer and reach outs.

Half kneeling weight transfer and reach outs.

Standing

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 Standing in a 1st with wide BOS gradual narrow BOS
parallel bar

Placing foot forward on marked point.

Arm swing with foot placement.

Reduce support & gait with assistive devise.

Gradual increase in step length & distance walked.

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Strengthening exercises:

For hip and shoulder muscles – posture and functional use of arms
and legs.
For trunk and low back muscles- trunk control and helps to reduce
pain from scoliosis
PNF techniques like rhythmic stabilization can promote trunk
stabilization.
Kabat, in 1955, described proprioceptive neuromuscular facilitation
including resistive exercises to help improve strength. coordination,
endurance, balance, and gait, but no research studies of the efficacy of PNF for
patients with cerebellar disorders have been reported.**
Avoid over fatigue
Low repetition, low weights, with rest period in between.
________________________________________
**Physical Therapy . Volume 77 . Number 5 . May 1997

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Stretching exercises:
Gasrtocnemius/soleus and foot arch : for foot deformities such as
pes cavus.
Stretching of spinal musculature is beneficial to tightened muscles
as a result of scoliosis.
In wheel chair bound patient: hamstring and hip flexor stretching is
beneficial to prevent contractures.

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Coordination exercises:
 All the coordination test can be used as the treatment exercises to
improve coordination,
 complexity can be increases by varying:
Support condition
Timing constraints
Environmental context

 Closing eyes, altering the speed, direction and force, withdrawal of


external cues & guidance, increasing the amplitude of movement.
Reduce the attentional demands of action to encourage the
automaticity (eg. by speaking during the performance)

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Balance exercises:

What is the rational for balance exercise? :

 Promote use of VOR & COR* for gaze stability.


 Promote use of saccadic eye movements for gaze stability.
 Promote VOR cancellation
 Improve ability to use somatosensory and vestibular inputs for
postural control.
 Improve ability to use vestibular and visual inputs for postural
control.
 Improve postural control using all sensory inputs.
 Improve postural control using visual and vestibular inputs.

* VOR- Vestibulo ocular reflex


COR- Cervico ocular reflex

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Exercises :

Visual fixation, slow Visual fixation at


Visual fixation,
and fast head various speeds,
slow/self paced head
movements, EO,
movements, simple complex static and
static background, dynamic background

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Static stance, EO/EC, Semi tandem stance
Semi tandem stance,
feet together, arms with EC
EO and EC , arms
closed to body, head continuously , firm
crossed
movements and padded surface

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Gait with
Gait with narrow BOS, Walking progressively
EO, wide turns, sideways/backward, narrowed BOS, sharp
March in place, EO/EC, slow/ fast turns bending and
firm/ foam surfaces head movements reaching activities

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Vestibular Rehabilitation:

 Cawthoren first describe the concept in 1944.


 Based on central mechanisms of neuro plasticity known as
adaptation, familiarization and substitution for obtaining vestibular
compensation .
 The VR exercises seek to
Improve the vestibulovisual interaction during cephalic
movement.
Increase the static and dynamic postural stability .
Reduce individual sensitivity to cephalic movement.
_________________________________________
Decreased ataxia and improved balance after vestibular rehabilitation
Helen S. Cohen
Otolaryngology- Head & Neck surgery
Vol 130;4:418-425 2004
Vestibular Rehabilitation
Arq Neuropsiquiatr 2009;67(2-A):219-223

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Gaze stability Eye- Head coordination ex’s protocol-

1. Visual fixation on stationary target


2. Active eye head movement between two stationary targets
3. Visual fixation on a moving target
4. Visual fixation on a moving target – Gaze Stability
5. Imaginary visual fixation

_____________________________
Kathleen M Gill-Body et al
Physical therapy 1997

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 Cawthorne’s head exercises.
 Ear- Eye coordination exercises.
 Ear- body coordination exercises.

_________________________________
Guidelines from Michigan Ear Institute

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Aerobic fitness:

Case report:
Patient’s with FRDA may improve aerobic fitness by participating in
stationary cycling for 20 to 25 mins at 70% to 85% of their maximum
heart rate. Large increase in cardiorespiratory and work measure
demonstrated clinically important physiologic adaptation to aerobic
conditioning in this patient. Peak VO2 increased 27% and peak
ventilation increased 21%. Total exercise time increased 5 mins,
reflecting a 50 watt increase in maximum work load. In addition, the
patient experienced a 4.75Kg weight loss.
_______________________________________
Endurance exercise training in friedreich ataxia .
Archive Physical medical rehabilitation
1989;70:786-788

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Treadmill training:

A woman (25 years) and a man (53 years) with chronic ataxia due to
head trauma. Three 20-minute treadmill training sessions each week
with progression in velocity and step length. Both individuals
demonstrated gains in all parameters over initial baseline and
subsequent phases, with performance increases ranging from 26% to
233% when first and last assessments were compared. Significantly
superior effects of treadmill training over baseline conditions on
cadence were detected (P < 0.05). Gains in walking speed were not
significantly better during intervention, but intervention withdrawal
produced deceleration of performance gains.
_________________________________________________
Treadmill training for ataxic patients: a single-subject experimental design
Clinical Rehabilitation 2008;22:234.

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Body weight supported treadmill training:
BWST has several advantages:
The body-weight support harness allows a progressive increase in the
demands for postural control
The treadmill allows systematic control and progression of the speed
at which walking is performed,
The repetitive training of a complete gait cycle enables a more
appropriate pattern of sensory input associated with the different
phases of gait to stimulate the locomotor pattern.
In addition, locomotor training using BWST allows the therapist
to provide manual assistance to help the patient simulate a more
normal walking pattern.

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Body weight supported treadmill training:

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 Locomotor training using a BWS system both on the treadmill and during over
ground walking was implemented 5 days a week for 4 weeks in a clinic.
Locomotor training using BWS on a treadmill was continued 5 days a week for 4
months at home.
 Locomotor training using BWS on a treadmill in conjunction with over ground
gait training may be an effective way to improve ambulatory function in
individuals with severe cerebellar ataxia, but the intensity and duration of
training required for functionally significant improvements may be prolonged.
_______________________________________________________
Locomotor Training Using Body-Weight Support on a Treadmill in
Conjunction With Ongoing Physical Therapy in a Child With Severe
Cerebellar Ataxia Kristin Cernak, Vicki Stevens, Robert Price, Anne Shumway-Cook
Volume 88 Number 1 Physical Therapy

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Newer Approach:
 Some researches says that BWS treadmill do not sufficiently
challenge the balance function as it provide the support with
harness.
 BWS training there is absence of associated postural
adjustments(APAs).
 This restriction may limit the full advantage of unload of gait
training.

_________________________________________________________
A rehabilitation tool for functional balance using altered gravity and virtual reality
Lars IE Oddsson et al
Journal of NeuroEngineering and Rehabilitation 2007, 4:25
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Maintenance of biomechanical alignment:

Maintaining biomechanical alignment is an important


therapeutic consideration. Orthopedic problems such as foot
deformities and scoliosis are often treated with orthoses or
surgery and may result in a temporary improvement in function.

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 Orthopedics shoe:
Case report:
This study reports the case of a 26-year-old woman with FA.
The patient suffered from:
Pain on soles and dorsal side of toes, when walking with
Standard shoes; its rating was 70/100 mm on the visual analogue
pain scale
Fatigability, with an estimated 400 m walking distance
Falls happening many times per day
Ankle sprains occurring once a week.

__________________________________________________
 Orthopedic shoes improve gait in Friedreich’s ataxia: a clinical and quantified case study
C. GOULIPIAN, L. BENSOUSSAN et al
EUR J PHYS REHABIL MED 2008;44:93-8

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 Impairments:
Equinovarus .
Claw toes.
Hallux valgus.

 Rehabilitation program:
To maintain ankle ROM and balance.
Orthopedic shoe to improve stability and hold the foot deformities.
Avoid friction

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Biofeedback :
 Sensory information can be augmented by using a biofeedback (BF)
system.
 Visual, acoustic, and tactile BF systems have been used successfully to improve
stance balance in subjects lacking vestibular, visual, and somatosensory
information.

____________________________________________________________
 Effects of practicing tandem gait with and without vibrotactile in subjects with unilateral vestibular loss.
Marco Dozzaa et al.
J Vestib Res. 2007 ; 17(4): 195–204.

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Hydrotherapy :
 In early rehabilitation, hydrotherapy can be used to increase alertness by using
Halliwick techniques or stimulating the input by using Watsu with lots of
movement and turbulence.
 A hydrotherapy advantage in this patient group is the ease of handling in the
water in comparison to handling on dry land.

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Adaptive Devices

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The use of reverse –
break system walker
has been reported to
reduce fall frequency.

Some people use


lateral stepping
strategies to prevent
falling; for these people
walker may reduce
ambulatory safety.

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•Some researches says that the use
of upper extremity as weight
bearing may impede the
improvement of balance in gait
training..

•Brandt et al suggested
progressively increasing body
instability to activate “ sensori
motor rearrangement”.

_________________________________________________________
Retraining of functional gait through the reduction
of upper extremity weight bearing in chronic
cerebellar ataxia
Internal rehabilitation medicine
1987

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Power scooter

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Newer concepts for gait training in Ataxia:
 Motor control theory (Dynamic Action Theory).

 Walk as fast as possible. Treatment showed 3 fold improvement in gait velocity and
2 fold improvement in stride length and single leg support time improved.
 Automatic spinal program over take the control, suppressing the misleading false
cerebellar inflow.

 Task oriented training.


 Virtual reality
 Auditory feedback

__________________________________________________________
 cerebellar stroke with speed dependent gaot ataxia Stroke journal.
 Neurorehabilitation & neural repair 18;2:117-124 2004
Rehabilitation management of fridreich ataxia: LE force control variability & gait performance
 Neurology 2006 Jan 24;66(2):178-81
Virtual reality cues for improvement of gait in patients with multiple sclerosis
 J Neurol Phy Ther 2005 Mar;29(1):34-42
Locomotor training and virtual reality – based balance training for an individual with multiple sclerosis: a case report
 J. Neurol Sci. March 15; 254(1-2)2007
Auditory feedback control for improvement of gait in patients with multiple sclerosis

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Home exercise:
Balance
1. Sitting unsupported.
2. High kneeling with bench for upper-extremity support
3. Knee walking with walker and without walker
4. Standing balance:
● Feet apart
● Feet together
● Split stance
● Weight shifting
● Stepping without assistive device

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Mobility
1. Crawling on forearms
● On extended arms
2. Transfers
● Wheel to chair and back
● Wheelchair to floor
● Floor to wheelchair
● Sit to stand and back to sitting
3. Gait
● Treadmill with harness
● Gait with 4-wheeled walker
● Gait with U-Step walker
Strength
1. Progressive resistive exercises for core and extremities

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References:
 Principle of internal medicine: Harrison16th edition; Vol II
 Brain & Bannister’s clinical neurology. 7th edition.
 Clinical Neuroanatomy Richard S. Snell 6th Edition
 Physical rehabilitation assessment & management. 4th edition.
 Cerebellar control of balance and locomotion. Morton SM, Bastian
AJ..Kennedy Krieger Institute and Department of Neurology, Johns
Hopkins University School of Medicine, Baltimore, MD 21205, USA.
 Specific influences of cerebellar dysfunctions on gaitHeidrun Golla
et al. Brain (2007), 130, 786^798
 Presentation and Progression of Friedreich Ataxia and Implications
for Physical Therapist Examination Joyce R Maring, Earllaine
Croarkin Volume 87 Number 12 Physical Therapy
 Decreased ataxia and improved balance after vestibular
rehabilitation Helen S. Cohen Otolaryngology- Head & Neck
surgery Vol 130;4:418-425 2004
1/24/2010 Garima Gupta ISIC New Delhi 60
References cont…
 Vestibular Rehabilitation Arq Neuropsiquiatr 2009;67(2-A):219-223
 Endurance exercise training in friedreich ataxia . Archive Physical
medical rehabilitation 1989;70:786-788
 Treadmill training for ataxic patients: a single-subject experimental
design. Clinical Rehabilitation 2008;22:234.
 Locomotor Training Using Body-Weight Support on a Treadmill in
Conjunction With Ongoing Physical Therapy in a Child With Severe
Cerebellar Ataxia. Kristin Cernak, Vicki Stevens, Robert Price, Anne
Shumway-Cook Volume 88 Number 1 Physical Therapy
 Retraining of functional gait through the reduction of upper
extremity weight bearing in chronic cerebellar ataxia Internal
rehabilitation medicine 1987
 Cerebellar stroke with speed dependent gaot ataxia Stroke journal.
 Orthopedic shoes improve gait in Friedreich’s ataxia: a clinical and
quantified case study C. GOULIPIAN, L. BENSOUSSAN et al EUR J
PHYS REHABILGarima
1/24/2010 MED 2008;44:93-8
Gupta ISIC New Delhi 61
References cont…
 Neurorehabilitation & neural repair 18;2:117-124 2004 Rehabilitation
management of fridreich ataxia: LE force control variability & gait
performance Neurology 2006 Jan 24;66(2):178-81
 Virtual reality cues for improvement of gait in patients with multiple
sclerosis J Neurol Phy Ther 2005 Mar;29(1):34-42
 Locomotor training and virtual reality – based balance training for an
individual with multiple sclerosis: a case report J. Neurol Sci. March
15; 254(1-2)2007
 Auditory feedback control for improvement of gait in patients with
multiple sclerosis.Baram Y,Miller A. Jneurol Sci 2007 Mar 15;254
(1-2):90-4
 Relative Contributions of Balance and Voluntary Leg-Coordination
Deficits to Cerebellar Gait Ataxia Susanne M. Morton and Amy J.
Bastian J Neurophysiol 89: 1844–1856, 2003;
 Hydrotherapy in adult neurology By Johan Lambeck PT. EWAC Medical
http://www.ewac.com
 Effects of practicing tandem gait with and without vibrotactile in
subjects with unilateral vestibular loss. Marco Dozzaa et al. J Vestib
Res. 2007 ; 17(4): 195–204.

1/24/2010 Garima Gupta ISIC New Delhi 62

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