ATAXIA

PRESENTED BY: ZEESHAN AHMAD

ROLL NO 65
DPT BATCH 12
DEFINITION

 Ataxia (Greek word meaning lack of order) is a

neurological disorder consisting of lack of voluntary
coordination of muscle movements.
 Ataxia is a non specific clinical manifestation implying
disfunction of parts of nervous system that coordinate
movement such as CEREBELLUM.
Ataxia comprises the following movement disorders:
 Dysmetria: is demonstrated by inaccurate amplitude of movement and
misplaced force and reflects the impairment in timing of muscle force
typical of cerebellar ataxia. There is excessive extent of movement or
overshooting(hypermetria) or undershooting(hypometria).
 Rebound Phenomenon: “Lack of check” illustrates the dysfunction in
the agonist-antagonist relationship, specifically the problems with
braking of movement.
 Dysdiadochokinesia: denotes difficulty performing rapid alternating
movements.
 Tremor: is an oscillatory movement about a joint due to alternating
contractions of agonists and antagonist.
 Dyssynergia: Demonstrates a lack of coordination between agonist,
antagonist and other synergic muscles resulting in an absence of the
normally smooth, sequential performance of various components of
an action.
 Hypotonia: Typically defined as diminished resistance to passive
movement.
 Dysarthria: Speech disorder where articulation symbols of speech are
normal but mechanical aspects of speech are impaired.
 Nystagmus: Denotes seesaw rhythmical movements of
the eye.
TYPES OF ATAXIA

 FRIEDREICH ATAXIA
 SENSORY ATAXIA
 CEREBELLAR ATAXIA
 FRONTAL ATAXIA
 MOTOR ATAXIA
 VESTIBULAR ATAXIA
FRIEDREICH’S ATAXIA

 FRIEDREICH’S ATAXIA: This is the commonest type of

ataxia having half of all it’s cases.
 Symptoms usually begins to appear in childhood between
8 to 15 years.
 It is transmitted by autosomal recessive inheritance and is
due to an expanded GAA trinucleotide repeat in a non
coding region of an gene of chromosome 9.
CLINICAL FINDINGS

 Initial symptoms is progressive gait ataxia followed by ataxia of all

limbs within 2 years.
 During the same early period, knee and ankle tendon reflexes are
lost and cerebellar dysarthria occurs.
 Joint position sense (Proprioception) and vibration are impaired in
the legs, typically adding sensory component to gait ataxia.
Contd

 Extensor planter responses appear during first 5 years.

 Pes Cavus is widely recognized.
 Severe progressive kyphoscoliosis contributes to functional
disabilities and may lead to chronic restrictive lung disease.
 Cardiomyopathy is sometimes detectable.
 Other abnormalities includes visual impairments including optic
atropy, nystagmus, paresthesia, vertigo, tremor, spasticity and leg
pain.
SENSORY ATAXIA

 Sensory Ataxia (SA) is a form of ataxia caused by the

impairment of the somatosensory nerves, leading to the
interruption of sensory feedback signals. It is
characterized by postural instability and lack of
coordination that worsen when visual input is removed.
 For example when the patient closes the eyes or at night
when the patient cannot rely on visual verification to
know where to place the feet.
CAUSES

• Peripheral nerve: Peripheral neuropathy

(especially Diabetic, Alcoholic and Nutritional), Peripheral
neuritis.
• Dorsal Column Medial Lemniscal Pathway: Tabes dorsalis.
• Posterior column: Subacute combined degeneration of
the cord, Demyelination changes of the posterior column.
CLINICAL FINDINGS

• Kinetic Tremors which appear only on the closure of the

eyes.
• Psuedoathetosis is the random finger movements seen on
outstretched hands with eyes closed and may occur in
sensory neuronopathy affecting the upper limbs.
• Deep Sensory Loss such as - proprioception, kinesthetic
awareness, vibration sense.
• Positive Romberg's Test
CEREBELLAR ATAXIA

 It is a generalized comprehensive terminology denoting

the cerebellar dysfunction comprising problems of
posture, movement patterns and gait.
 Cerebellar ataxias are a group of neurogenerative
disorders characterized by progressive degeneration of
the cerebellum and often accompanied by a variety of
neurological and other systemic symptoms.
CAUSES

 Hemorrhagic or Ischemic lesions

 Tumors
 Progressive sclerosis of cerebellum
 Trauma involving cerebellum
 Cerebrovascular disease
 Hypoplasia of cerebellar vermis
CLINICAL FEATURES

 Aesthenia
 Hypotonicity
 Diminished DTR
 Dysmetria
 Dysdiadochokinesia
 Intentional tremors
 Scanning speech
 Dysphagia
Contd

 Nystagmus
 CARDINAL FEATURES- Nystagmus, Scanning Speech and
Intentional Tremors
GAIT

 The clinical features of cerebellar ataxic gait usually

include a widened base, unsteadiness and irregularity of
steps, and lateral veering.
FRONTAL ATAXIA

 Frontal ataxia (also known as gait apraxia) is observed

when tumors, abscesses, cerebro vascular accidents and
normal pressure hydrocephalus effect the frontal area.
CLINICAL FEATURES

 Patient has difficulties standing erect.

 Even with use of support, patient tends to lean towards
hyperextension.
 Patient's legs are in scissors-cross position during walking
and there is incoordination between the legs and trunk.
 Ataxia is accompanied by frontal dementia, urinary
incontinence, frontal release signs and perseveration.
VESTIBULAR ATAXIA

 Vestibular ataxia develops as a result of peripheral or

central diseases which directly affects the vestibular
nuclei and/or the afferent and efferent connections of the
vestibular nuclei.
CLINICAL FEATURES

 Disturbances of balance in standing and sitting.

 Broad base support and may lean backwards or towards the
side of the lesion in walking.
 Vertigo, nausea, vomiting, blurred vision and nystagmus.
 Decreased Head and trunk motion and subsequently arm
motion.
 Balance is disrupted when performing a head or eye
movement.
MOTOR ATAXIA

 Lack of coordination or an inability to control voluntary

muscle movements during voluntary motions is referred
to as motor ataxia.
CLINICAL FEATURES

 Individuals with motor ataxia often exhibit an unsteady

and uncoordinated walking pattern.
 Individuals with motor ataxia may experience difficulty
controlling eye movements, leading to gaze instability or
nystagmus
 Dysmetria
 Intention Tremor
 Dysdiadochokinesia
 Speech Disturbances
 Postural Instability
CLINICAL ASSESSMENT AND
EVALUATION
Clinical neurological tests
1. Finger to finger and finger to nose tests
2. Heel to shin test
3. Rebound test
4. Test for rapid alternating movements
5. Romberg test for postural sway
Tests for Motor Performance
1. Spiral test and nail test for hand coordination
2. International Cooperative Ataxia Rating Scale(ICARS)
3. BRAIN test(Bradykinesia akinesia incoordination)
4. 6 minute walk test, timed up and go
FINGER-TO-NOSE-TEST

 The patient is asked to bring the tip of the index finger to

the tip of his or her nose.
 Alternations may be made in the initial starting position to
observe performance from different planes of motion.
 Observe intention tremor.
REBOUND TEST

 The patient is positioned in sitting .

 The PT applies sufficient manual resistance to produce an
isometric contraction of biceps.
 Resistance is suddenly released. Normally the opposing
muscle group will contract & “check” movement of limb.
 The patient with dysfunction will be unable to arrest the
progress of the arm, and it will rebound markedly off your
arm.
HEAL ON SHIN

 From a supine position, the heel of one foot is slid up and

down the shin of the opposite LE.
TREATMENT

 The goal of Ataxia treatment is to improve the quality of

life and requires an individualized approach. 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.
 Staying active for as long as possible is an important part
of the treatment plan for people with Ataxia.
PHYSIOTHERAPY

 Physiotherapists will employ a combination of restorative

and compensatory approaches guided by the patient’s
clinical presentation and context.
COMPENSATORY APPROACH

 Compensatory approach includes Introduction to

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 APPROACH

 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.
TRAINING

 Major objective of Physiotherapy is to train optimal and effective

performance of any actions with which the individual is having
difficulty.
 To improve performance of functional movements and develop
skill, specifically during actions such as standing up, sitting down,
walking(including stairs and hills) reaching up to take an object.
 Train everyday actions in standing, with a narrow base of support
and no arm support, in order to improve balance control.
 Train weightbearing exercises for lower limbs involving repetitive flexion-
extension over fixed feet to improve intersegmental control/
coordination, for e.g. standing up and sitting down, squats, step up and
down, heels raise and lower. Emphasis is on smooth continuous
performance, varied weight resistance-body weight, additional weights in
vest.
 Provide exercises for the upper limbs in sitting and standing, involving
picking up and placing games, with arms close to the body if control is
difficult, increasing reaching distance; weightbearing exercises such as
push ups against the wall in standing, pulling games with weighted
objects or resistance.
 Train dynamic stability of the trunk and limbs by
practicing tasks that rely on adapting or developing
strategies to cope with increasingly demanding
conditions. For e.g. tandem walking, walking in narrow
spaces , on different surfaces and under bars or ropes,
walking with eyes open and closed, and starting
movements slowly with increases in movement
complexity, balance demands, or speed.