1.
ATAXIA
Dr. Jyoti Soni (PT)
BPT, MPT (Neurology and Psychosomatic disorders)
Assistant Professor
Apex University,Jaipur

2.
Definition
 Ataxia means absence of order. It denote
disturbance of coordinated muscle activity. It
is caused by disorders of cerebellum and
efferent connections.
 Disease of PNS( neuropathy) may also cause
the ataxia.

3.
 In cerebellum, dysfunction of lower vermis
(vestibulocerebellum) leads to truncal ataxia.
 Spinocerebellar lesion (upper vermis and ant. part of
hemisphere) cause unsteadiness of gait and stance, which
are more evident after eye closure ( positive Romberg ).
 Neocerebellar (cerebellar hemisphere ) damage leads to
ataxia with intended limb movement.
 Ataxia limb movement are irregular and jerky and tend to
overshoot the target (past pointing).
 Accompanied by rhythmic side to side movement as target
is approached (intentional or action tremor).
 Dysarthria with intonation and disturbance of occular
movement (broken of smooth pursuit, gaze evoked
nystagmus).

4.
Classification of ataxia
1. Hereditary ataxia-
 Autosomal recessive ataxia
 Mitochondrial disorders
 Autosomal dominant ataxia
 Spinocerebellar ataxia
 Episodic ataxia
2. Spongiform encephalopathy
3. Nonhereditary ataxia
 Multiple system atrophy, cerebellar type
 Symptomatic ataxia- alcoholic cerebellar degeneration,ataxia due to other toxic causes, acquired
vit.deficiency /metabolic disorders,cerebellar encephalitis or due to physical cues.
 Vascular ataxia-haemorrhage of cerebellum
 Bacterial cerebellitis
 Trauma
 Multiple sclerosis (early and late ataxia)
 Spinal cord / nerve root disorders

5.
Clinical presentation
 Ataxia can have an insidious onset with a chronic and slowly
progressive .
 Ataxia can have an acute onset ,because of cerebellar
infarction, haemorrhage ,infection and have rapid
progression
 Subacute onset (immunological disorders)
 Laterlised cerebellar lesions cause ipsilateral symptoms, in
diffuse cerebellar lesion give rise to more generalised
symmetric symptoms. Lesion in cerebellar hemisphere
produce limb ataxia.
 Lesion of vermis cause truncal and gait ataxia with limb
spared.
 Vestibulocerebellar lesions cause disequilibrium , vertigo,
gait ataxia.

6.
 Stance – impaired stance in absence of motor weakness or gross involuntry
movement-sensory ataxia or cerebellar ataxia
 Gait ataxia- due to incoordination of lower extremities of loweer extremities .
Patient often feel insecure and have to hold onto the wall or furniture and walk
with feet apart.
 Sensory ataxia- positive Romberg sign. Subjects may walk with a high-stepping
gait (due to associated motor weakness) or feet-slapping gait (to assist with
sound-induced sensory feedback).
 Truncal ataxia. Patients may present with truncal instability in the form of
oscillation of the body while sitting (worse with arms stretched out in front) or
standing .
 Limb ataxia. Limb ataxia is often used to describe ataxia of the upper limbs
resulting from incoordination and tremor and can be better described by
functional impairment, such as clumsiness with writing,buttoning clothes, or
picking up small objects.The patient has to slow down the movement to be
accurate in reaching things.
 Dysdiadochokinesia/dysrhythmokinesis.Dysdiadochokinesia/dysrhythmokin
esis is tested by rapidly alternating hand movements or tapping the index finger
on the thumb crease.

7.
 Intention tremor. Intention tremor results from
instability of the proximal portion of the limb and is
manifested by increasing amplitude of oscillation at
the end of a voluntary movement. It is often tested
by finger-to-nose and heel-to-shin maneuvers.
 Dysmetria.The patient misses the targeted object
either due to overshoot (hypermetria) or undershoot
(hypometria). Dysmetria is often tested by a finger-
chasing test
 Dysarthria/scanning speech.
 Upbeat and downbeat nystagmus are defined by
the rapid phase in the up or down direction.

8.
 Common symptoms-
 Lack of coordination
 Slurred speech
 Trouble eating and swallowing
 Deterioration of fine motor skills
 Difficulty walking
 Gait abnormalities
 Eye movement abnormalities
 Tremors
 Heart problems

9.
Diagnostic Procedures
 May include medical history, family history,
and a complete neurological evaluation.
Various blood tests may be performed to rule
out other disorders. Genetic blood tests are
available for many types of hereditary Ataxia.

10.
Treatment
 Speech and language therapy, occupational
therapy, and physical therapy are common
treatment options.They are sometimes used
in conjunction with medication therapy to
manage symptoms.

11.
Physiotherapy
 Rehabilitation for people with ataxia may adopt a
compensatory or restorative approach.
 Compensatory approach - includes orthotics and devices,
movement retraining, reducing the degrees of freedom and
optimising the environment.Valuable for teaching people
practical, everyday strategies and ways of managing the
condition. It may be particularly important for those with
severe upper limb tremor.
 Restorative approaches aim to improve function by
improving the underlying impairment. Despite cerebellar
damage, some improvement in symptoms can occur with
practice in people with chronic and progressive conditions.

12.
Rehabilitation
 Physiotherapy can improve gait, balance and trunk control for people with ataxia, and can reduce
activity limitations and support increased participation.
 The prevention of falls is important to consider in patients with progressive ataxia given their high
frequency and fall-related injuries being common.
 Careful assessment is required to avoid falls.
 For people with cerebellar dysfunction, dynamic task practice that challenges stability, explores
stability limits and aims to reduce upper-limb weight bearing seems an important intervention to
improve gait and balance.
 Strength and flexibility training may be indicated.
 Therapeutic equipment is often provided to support function.
 Intensity of training seems to be important as studies have shown that higher training intensities
are associated with greater improvements in clinical outcome.
 There is some evidence to suggest that improvement is greater in people with less severe ataxia
and it is also related to the ability to learn the task.
 Targeted coordination and gait training over a four-week period resulted in improvements in
people with cerebellar ataxia as measured by the Scale for the Assessment and Rating of
Ataxia (SARA ) that was sustained after one year. Daily training improved outcome.This particular
training showed a more sustained improvement in people with cerebellar dysfunction compared
to people with afferent ataxias such as Friedreich’s ataxia and sensory ataxic neuropathy.
 Balance training exercises undertaken in front of standardised moving visual images resulted in
improvements in balance scores in some patients with SCA6 (a pure cerebellar ataxia) in a pilot
trial but results were mixed.

13.
Specific Interventions for
Balance and Gait
I.Video-game based coordinative training
 eg Intensive coordination training using whole-body controlled videogames can be an
effective and motivational therapy for children with progressive ataxia
II.Treadmill training
 Treadmill training can be an effective intervention for people with ataxia due to brain
injury. Intensity and duration of training seem to be significant factors. Consistent
intensive training over many months combined with over-ground training may be
required.This intervention has not been tested in people with progressive ataxias.
III.Visually guided stepping
 Oculomotor and locomotor control systems interact during visually guided stepping
in that the locomotor system depends on information from the oculomotor system
during functional mobility for accurate foot placement. Marked improvements in
oculomotor and locomotor performance have been seen following eye movement
rehearsal in a small study in patients with mild cerebellar degeneration. Rehearsal of
intended steps through eye movement alone, i.e. looking at foot target placement for
each step, before negotiating a cluttered room, might improve performance and
safety.This simple strategy, although task specific and short lived in nature, is
promising and relatively quick and easy to apply in a functional setting.

14.
IV. Balance and mobility aids
 No studies have specifically evaluated the role of balance and
mobility aids for people with ataxia.Clinical experience suggests
walking aids should be considered on a case-by-case basis.
 In terms of postural control, somatosensory cues from the
fingertips – using light touch contact or a walking aid as a means
of balance – can provide a powerful reference orientation even
when contact force levels are inadequate to provide physical
support for the body. Some individuals with ataxia find light
touch contact more useful as a strategy than a conventional
walking aid.
 Walking aids also have the potential to compromise the ability to
respond to balance disturbances through impeding lateral
compensatory stepping and can thus affect safety, hence ensure
the appropriate walking aid is recommended for each patient.
 Wheelchair sitting

15.
Specific Interventions for
Spasticity
 Physiotherapy has a vital role to play in educating patients and
carers in correct posture, muscle use and the avoidance of
spasticity triggers such as pain and infection.
 Muscle lengthening to maintain and improve range of movement
and prevent the formation of contractures. eg physical exercises
which antagonize the overactive spastic muscle and also improve
muscle strength; passive stretching by the therapist or carer; or
physical positioning techniques. Active exercise is generally more
effective than passive exercise if the patient is able; increased
fitness can also reduce fatigue and permit further exercises.
 Positioning eg splinting, casting, orthoses, standing or the use of
weights, resistive devices, wedges, cushions orT-rolls. More
prolonged splinting can involve firm materials such as metal or
plastic, or softer supportive materials such as foam or sheepskin.
Orthoses should be of good quality, well-fitted and prepared by a
specialist.

16.
Exercise
 The recommended position should be maintained by the
patient.
 The positioning should be comfortable.
 How many sets of the exercises are generally decided
according to the severity of each case because it can vary
from patient to patient.
 Generally, the number of sessions are two times a day.
 Two-three sets of exercises with an interval of 5-6 minutes
in between the sets, in controlled manner is appreciated.
 Progression can be like, starting with 2-3 exercises per
session with repetition of 8-10, and when the task became
easy one can increase the repetition to 12-15 or can add few
more exercises to the program.

17.
Recommended exercise-
 Lying bent knee rotation: this exercises focuses on segmental movement of lower
limb and will help in bed movement and transfers.It can be done by lying face up with
both the knees bent, hip width apart and feet flat,arms can be positioned out wide
away from the body.Now slowly begin to let both the knees rotate from one side of
the body to the other side.(trying keep upper body and back flat). (calm shell).
 Kneeling press up: this can be start in upright kneeling position with knees under hip
and with arms at the sides, slow movement from high kneeling position to hip
straight body upright, to a low kneeling position hip movement down to rest on heels.
 Quadruped weight shifting: start positioned with hand under kneeling and knees
under hips and the spine is neutral. slowly reach an arm forward to shoulder height,
then begin to extend the opposite-side leg backward to hip height. balance for a
moment before lowering both the arm and leg to the ground.
 Vestibular ball: vestibular ball can be used for balance exercises, the external support
of therapist or the person helping to exercise, sitting upright on an exercise ball with
feets apart,the legs are then locked by the therapist in order to avoid any fall as the
patient is have balancing problems. smoothly begins to move the upper body to the
right and then to the left,allow the weight of the trunk to shift from one side to
another.
 Standing heel to toe balance: standing upright position one foot in front of the other
so heel of the front foot is touching the toes of the back foot, as if standing on the
tightrope.