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Garima Gupta MPT Neurology
Indian Institute Of Rehabilitation SciencesNew 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
Brain & Bannister’s clinical neurology 7th edition
3
Muscle power
Postural sensibility
Central co-ordinating mechanism
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Role of cerebellum
CEREBELLUM
PERIPHERAL FEEDBACK
MECHANISM
MOTOR CORTEX
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Role Of Cerebellum Cont…
Hence cerebellum acts as : ComparatorError 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 fastigial nuclei)Medial
cerebellum
• control of discrete, ipsilateral limb movements.Intermediate Cerebellum
• control of complex, visually guided limb movements and the planning of those movement (lateral hemisphere and dentate nuclei)
Lateral Cerebellum
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Role Of Cerebellum Cont…
Animal studies also confirmed the fact that the control of balance instance and locomotion is dependent on the medial part of thecerebellum (Thach and Bastian, 2004) while the control of goal-directed movements and perturbed or visually guided walking isinfluenced by the intermediate and lateral parts of the cerebellum.(Cooper et al.2000)
______________________________________________________________Relative Contributions of Balance and Voluntary Leg-Coordination Deficits toCerebellar Gait AtaxiaSusanne M. Morton1 and Amy J. Bastian J Neurophysiol 89: 1844–1856, 2003;Specific influences of cerebellar dysfunctions on gaitHeidrun 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 instablestumbling walking path, increased step width and high variability ofgait (Diener and Dichgans, 1996; Morton and Bastian, 2004).
Ataxia is a common sign in a variety of disorders. Some conditions displaying ataxia are inheritedInsidious, or congenital StrokeTraumatic brain Metabolic disorders.
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Cerebellar infarcts, hypoxiaDorsal spinal cord compression from vertebral fracturesAlcohol Drug abuseVestibular 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 poorlydocumented.
These is, however, strong evidence of recovery after cerebellar lesions inexperimental animals, which suggests that if the cerebellum is not totallydestroyed, neighboring areas of the cerebellum can adapt or compensatefor the impaired region.
Possible mechanisms of recovery after central nervous system lesions mayinclude:
Neural sprouting, Vicarious functions,Functional reorganization,Substitution.
____________________________________Kathleen M Gill-Body et alPhysical therapy 1997
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AssessmentHistory
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 forPhysical Therapist Examination Joyce R Maring, Earllaine CroarkinVolume 87 Number 12 Physical Therapy
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Composite Performance Measures A. International Cooperative Ataxia Rating Scale:
100 point scale10-15 minsDomains:
i. Posture and gait disturbance ii. Kinetic functionsiii. Speech disorderiv. Oculomotor disorder
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B. Friedreich’s Ataxia Rating Scale:30 minsDomains:
I. Functional staging of ataxiaII. Activities of daily livingIII. Neurologic examination
1. Bulbar2. Upper limb coordination3. Lower limb coordination4. Peripheral nervous system5. Upright stability
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C. Ataxia Clinical Rating ScaleD. Functional Ataxia Scoring ScaleE. Inherited Ataxia Progression ScaleF. Inherited Ataxia Clinical Rating ScaleG. Northwestern University Disability Scale
_________________________________________________Presentation and Progression of Friedreich Ataxia and Implications forPhysical Therapist ExaminationJoyce R Maring, Earllaine CroarkinVolume 87 Number 12 Physical Therapy
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History Vestibular Cerebelllar Sensory Vertigo Present ++
Paroxysmal, constant, or waxing and waning may be worse with head movements, +/- noise, or Valsalvamaneuvers
Sometimes present
Absent
Limb paresthesia or numbness
Absent May be present with brainstem involvement
Present
Ataxia worse in the dark
Only if bilateral
vestibulo-pathy
Absent or rare Present
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History Vestibular Cerebelllar Sensory Cerebellar signs •Tremor •Ataxia •Dysmetria •Dysynergia •Dysdiadochokinesis•Dysarthria •Titubation •Impaired eye pursuit
Absent Present Absent
Nystagmus Often present acutely
peripheral type
Often present central type Absent
Peripheral proprioceptive sensory deficit
Absent Absent Present
Romberg's test May be present if there is a bilateral vestibulopathy
Absent Present
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Gait Training In AtaxiaGoals:
Minimize disability, deformity and pain.Prolonging locomotor skills.Maintaining or improving patient’s ability to continue to participate inall environmental contexts for as long as possible.Patient and family education about the effect of disease progressionon function and life style, potential therapeutic interventions andrealistic expectation about those interventions.Once the patient is on established home exercise program ongoingclinical evaluation with changes in the home program as needed.
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Interventions1. Proximal muscle stabilization exercises & Pre gait training2. Strengthening exercises 3. Stretching exercises4. Coordination exercises5. Balance exercises6. Vestibular Rehabilitation7. Aerobic fitness and treadmill training8. Body weight supported treadmill training 9. Maintenance of biomechanical alignment
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10.Weight bearing ex’s & gait training in parallel bar11.Biofeedback12.Hydrotherapy 13. Adaptive devices:
Walker / Cane Power scooter Wheel chair
14.Newer concepts in training of gait ataxia15. 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 BOSparallel 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 armsand legs.For trunk and low back muscles- trunk control and helps to reducepain from scoliosisPNF techniques like rhythmic stabilization can promote trunkstabilization.Kabat, in 1955, described proprioceptive neuromuscular facilitationincluding resistive exercises to help improve strength. coordination,endurance, balance, and gait, but no research studies of the efficacy of PNF forpatients with cerebellar disorders have been reported.**
Avoid over fatigueLow 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 conditionTiming constraintsEnvironmental 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 reflexCOR- Cervico ocular reflex
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Exercises :
Visual fixation, slow/self paced head
movements, EO,
Visual fixation, slow and fast head
movements, simple static background,
Visual fixation at various speeds,
complex static and dynamic background
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Static stance, EO/EC, feet together, arms
closed to body, head movements
Semi tandem stance, EO and EC , arms
crossed
Semi tandem stance with EC
continuously , firm and padded surface
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Gait with narrow BOS,EO, wide turns,March in place,
firm/ foam surfaces
Walking sideways/backward,
EO/EC, slow/ fast head movements
Gait with progressively
narrowed BOS, sharp turns bending and 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 vestibularcompensation .
The VR exercises seek toImprove 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 rehabilitationHelen S. CohenOtolaryngology- Head & Neck surgeryVol 130;4:418-425 2004Vestibular RehabilitationArq Neuropsiquiatr 2009;67(2-A):219-223
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Gaze stability Eye- Head coordination ex’s protocol-
1. Visual fixation on stationary target2. Active eye head movement between two stationary targets3. Visual fixation on a moving target4. Visual fixation on a moving target – Gaze Stability 5. Imaginary visual fixation
_____________________________Kathleen M Gill-Body et alPhysical 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 instationary cycling for 20 to 25 mins at 70% to 85% of their maximumheart rate. Large increase in cardiorespiratory and work measuredemonstrated clinically important physiologic adaptation to aerobicconditioning in this patient. Peak VO2 increased 27% and peakventilation increased 21%. Total exercise time increased 5 mins,reflecting a 50 watt increase in maximum work load. In addition, thepatient 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 individualsdemonstrated 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 thedemands for postural controlThe treadmill allows systematic control and progression of the speedat which walking is performed,The repetitive training of a complete gait cycle enables a more
appropriate pattern of sensory input associated with the differentphases of gait to stimulate the locomotor pattern.In addition, locomotor training using BWST allows the therapistto provide manual assistance to help the patient simulate a morenormal 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 inConjunction With Ongoing Physical Therapy in a Child With SevereCerebellar Ataxia Kristin Cernak, Vicki Stevens, Robert Price, Anne Shumway-CookVolume 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 withharness.
BWS training there is absence of associated posturaladjustments(APAs).
This restriction may limit the full advantage of unload of gaittraining.
_________________________________________________________A rehabilitation tool for functional balance using altered gravity and virtual reality Lars IE Oddsson et alJournal of NeuroEngineering and Rehabilitation 2007, 4:25
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Maintenance of biomechanical alignment:
Maintaining biomechanical alignment is an importanttherapeutic consideration. Orthopedic problems such as footdeformities and scoliosis are often treated with orthoses orsurgery 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 withStandard shoes; its rating was 70/100 mm on the visual analoguepain scaleFatigability, with an estimated 400 m walking distanceFalls happening many times per dayAnkle sprains occurring once a week.
__________________________________________________Orthopedic shoes improve gait in Friedreich’s ataxia: a clinical and quantified case studyC. 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 walkerhas been reported toreduce fall frequency.
Some people uselateral steppingstrategies to preventfalling; for these peoplewalker may reduceambulatory 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 ataxiaInternal rehabilitation medicine1987
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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 and2 fold improvement in stride length and single leg support time improved.
Automatic spinal program over take the control, suppressing the misleading falsecerebellar inflow.
Task oriented training. Virtual reality Auditory feedback
__________________________________________________________ cerebellar stroke with speed dependent gaot ataxia Stroke journal. Neurorehabilitation & neural repair 18;2:117-124 2004Rehabilitation management of fridreich ataxia: LE force control variability & gait performance Neurology 2006 Jan 24;66(2):178-81Virtual reality cues for improvement of gait in patients with multiple sclerosis J Neurol Phy Ther 2005 Mar;29(1):34-42Locomotor training and virtual reality – based balance training for an individual with multiple sclerosis: a case report J. Neurol Sci. March 15; 254(1-2)2007Auditory feedback control for improvement of gait in patients with multiple sclerosis
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Home exercise:Balance1. Sitting unsupported.2. High kneeling with bench for upper-extremity support3. Knee walking with walker and without walker4. Standing balance:● Feet apart● Feet together● Split stance● Weight shifting● Stepping without assistive device
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Mobility1. Crawling on forearms● On extended arms2. Transfers● Wheel to chair and back● Wheelchair to floor● Floor to wheelchair● Sit to stand and back to sitting3. Gait● Treadmill with harness● Gait with 4-wheeled walker● Gait with U-Step walkerStrength1. 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 Gollaet al. Brain (2007), 130, 786^798
Presentation and Progression of Friedreich Ataxia and Implications for Physical Therapist Examination Joyce R Maring, EarllaineCroarkin 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
601/24/2010 Garima Gupta ISIC New Delhi
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 REHABIL MED 2008;44:93-8 611/24/2010 Garima Gupta ISIC New Delhi
References cont… Neurorehabilitation & neural repair 18;2:117-124 2004 Rehabilitation
management of fridreich ataxia: LE force control variability & gaitperformance Neurology 2006 Jan 24;66(2):178-81
Virtual reality cues for improvement of gait in patients with multiplesclerosis J Neurol Phy Ther 2005 Mar;29(1):34-42
Locomotor training and virtual reality – based balance training for anindividual with multiple sclerosis: a case report J. Neurol Sci. March15; 254(1-2)2007
Auditory feedback control for improvement of gait in patients withmultiple sclerosis.Baram Y,Miller A. Jneurol Sci 2007 Mar 15;254(1-2):90-4
Relative Contributions of Balance and Voluntary Leg-CoordinationDeficits 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 Medicalhttp://www.ewac.com
Effects of practicing tandem gait with and without vibrotactile insubjects with unilateral vestibular loss. Marco Dozzaa et al. J VestibRes. 2007 ; 17(4): 195–204.
621/24/2010 Garima Gupta ISIC New Delhi