Barkatullah University Bhopal

Career Institute of Medical Sciences

Bhopal
A
Project on
Management of Cerebellar Ataxia
Session 2011-2012
Submitted by:

Guided By:

Akanksha Dixit

Dr.Namrata shrivastava

B.P.T. IV Year

(M.P.T. in ORTHO)

Career Institute of Medical Sciences

Bhopal

Certificate
This is to certify that a project on Management of
cerebellar ataxia is submitted by Ms. Akanksha Dixit, a
student of final year in partial fulfillment of the
requirements for Bachelor of Physiotherapy, submitted to
Physiotherapy Department of Career College of Batch
2011-12.

Project Guide:

H.O.D.

Dr.Namrata Shrivastava

Dr. Rakhi Wadhwa

(P.T.)

(P.T.)

Career Institute of Medical Sciences

Bhopal

Certificate
This is to certify that a project on PT Management
of cerebellar ataxia is submitted by Ms. Akanksha Dixit, a
student of final year in partial fulfillment of the
requirements for Bachelor of Physiotherapy, submitted to
Physiotherapy Department of Career College of Batch
2011-12.

Internal Examiner

ExternalExaminer

Acknowledgement
The moment of acknowledgement gives pride that gives me a feeling
to cherish about; I take this opportunity to express my sincere gratitude to all
who contributed in making this work possible within a very limited time.

I express my deep sense of gratitude to Mr. P.N. Tiwari, Principal,

Career College of physiotherapy, who has given permission to carry out this
project.

My sincere thanks to Mr. Vishnu Rajoriya, Chairman, Career College

of Physiotherapy, who stood as a pillar of strength and gave his valuable help
and cooperation in completion of this project.

My heartiest indebtedness to the head of Department, Dr. Rakhi

Wadhwa who Patronized me at all times. I also wish to express my deep
sense of gratitude to Dr. Namrata , guide and lecturer for her continuous and
tireless support and advice, not only during the course of my project making,
but also during other times.

I am indebted to Dr. Swapnil, Dr. Namrata, Dr. Sneha, Dr. Saurav who
imbibed in me the inspiration and zeal to complete the task.

Last but not the least; I would like to thank my parents, brother,
colleagues as well as my well wishers for their sincere wishes and kind
cooperation.

AKANKSHA DIXIT

Cerebellar ataxia & its management

hgCONTENTjkjlkdjhkjklhgh

Content

S.no. Title

page no.

1.

Introduction ....

2.

Anatomy of cerebellum......

3.

Functions of cerebellum......................

4.

Types of cerebellar ataxia ......

5.

Causes.......................................................

6.

Signs and symptoms

7.

Physical examination................

8.

Coordination tests

9.

Investigations...............

10.

Diagnosis.............................................................

11.

Differential diagnosis..

12.

PT Management(Treatment principles).......................

13.

Conclusion....

14.

References.

Introduction

Many diseases involve the cerebellum and produce ataxia, which is characterized
by incoordination of balance, gait, extremity and eye movements, and dysarthria.
Cerebellar lesions do not always manifest with ataxic motor syndromes, however. The
cerebellar cognitive affective syndrome (CCAS) includes impairments in executive,
visual-spatial, and linguistic abilities, with affective disturbance ranging from emotional
blunting and depression, to disinhibition and psychotic features.

The cognitive and psychiatric components of the CCAS, together with the ataxic
motor disability of cerebellar disorders, are conceptualized within the dysmetria of
thought hypothesis.
This concept holds that a universal cerebellar transform facilitates automatic
modulation of behavior around a homeostatic baseline, and the behavior being modulated
is determined by the specificity of anatomic subcircuits, or loops, within the
cerebrocerebellar system. Damage to the cerebellar component of the distributed neural
circuit subserving sensorimotor, cognitive, and emotional processing disrupts the universal
cerebellar transform, leading to the universal cerebellar impairment affecting the lesioned
domain.Cerebellar ataxia is a type of ataxia that is related to improper functioning of
cerebellum Ataxia is basically related to unbalanced and uncoordinated body movements.
Cerebellum ataxia is a rare disorder, that is related to improper functioning of the
cerebellum, an important brain anatomy, which controls the coordination of body
movements.

Fig no 1:-Anatomy of cerebellum

Fig no :-2 Cerebellum and surrounding regions; sagittal view of one hemisphere. A: Midbrain.
B: Pons. C: Medulla. D: Spinal cord. E:Fourth ventricle. F: Arbor vitae. G: Tonsil. H: Anterior
lobe. I: Posterior lobe.

The cerebellum (Latin for little brain) is a region of the brain that plays an
important role in motor control. It is also involved in some cognitive functions such
as attention and language,

and

probably

in

some emotional functions

such

as

regulating fear and pleasure responses Its movement-related functions are the most clearly
understood, however
The cerebellum does not initiate movement, but it contributes to coordination,
precision, and accurate timing. It receives input from sensory systems and from other parts
of the brain and spinal cord, and integrates these inputs to fine tune motor activity.
Because of this fine-tuning function, damage to the cerebellum does not cause paralysis,
but instead produces disorders in fine movement, equilibrium, posture, and motor learning

Functions of cerebellum

The strongest clues to the function of the cerebellum have come from examining the
consequences of damage to it. Animals and humans with cerebellar dysfunction show,
above all, problems with motor control. They continue to be able to generate motor
activity, but it loses precision, producing erratic, uncoordinated, or incorrectly timed
movements. A standard test of cerebellar function is to reach with the tip of the finger for a
target at arm's length: A healthy person will move the fingertip in a rapid straight
trajectory, whereas a person with cerebellar damage will reach slowly and erratically, with
many mid-course corrections.
Deficits in non-motor functions are more difficult to detect. Thus, the general
conclusion reached decades ago is that the basic function of the cerebellum is not to
initiate movements, or to decide which movements to execute, but rather to calibrate the
detailed form of a movement.
Prior to the 1990s, the function of the cerebellum was almost universally believed
to be purely motor-related, but newer findings have brought that view strongly into
question. Functional imaging studies have shown cerebellar activation in relation to
language, attention, and mental imagery; correlation studies have shown interactions
between the cerebellum and non-motoric areas of the cerebral cortex; and a variety of nonmotor symptoms have been recognized in people with damage that appears to be confined
to the cerebellum.
Kenji Doya has argued that the function of the cerebellum is best understood not in
terms of what behaviors it is involved in but rather in terms of what neural computations it
performs; the cerebellum consists of a large number of more or less independent modules,
all with the same geometrically regular internal structure.

Types of cerebellar ataxia

TH E GENETIC ATAXIAS:
These diseases are often classified on the basis of inheritance. Clinical diagnosis
has now been aided by molecular genetic testing. This may pinpoint a specific
genetic cause, which allows a precise diagnosis, or may exclude some types of
ataxia, helping to refine the diagnostic possibilities.

Autosomal dominant cerebellar ataxias

This group of genetic ataxias looks similar clinically, usually having a permutation of the
clinical features outlined above. Onset can be from infancy to old age but most commonly
is between the ages of 20-60. More than half of families have a mutation in a known gene.
Most mutations have taken the form of a triplet repeat expansion (a stretch of DNA 2-3
times normal) existing in different genes. The responsible
genes are numbered SCA1, SCA2, SCA3 etc, and over 25 subtypes are recognized,
of which around 40% can be fairly easily tested for in the NHS. Testing for the
remainder is complex; some known types may only be available as research, others
may not yet have an identified gene, so testing may not be possible. Clinically, the
gene SCA7 is important because visual failure may precede or accompany the
ataxia, whereas vision is often preserved in the other ataxias.
Prognosis is variable but patients with later onset usually experience slower
progression. As a guide, the ability to walk independently is lost approximately 15
years after onset.

Autosomal recessive ataxias

The commonest autosomal recessive ataxia is Friedreich's ataxia (FA). However, there are
other rare autosomal recessive ataxias. Clinically they can be distinguished from FA by the
presence of reflexes, (usually lost in FA), and genetically by the absence of a mutation in
the FA gene. Other recessive ataxias include ataxia telangiectasia, ataxia with ocular motor
apraxia, ataxia with vitamin E deficiency and the recently identified ataxia with CoQ10
deficiency (which appears to respond to treatment with CoQ10 tablets).

Episodic ataxias
These are also known as paroxysmal ataxias. They are characterised by episodes of
Ataxia including dysarthria, tremor and nystagmus lasting minutes to hours. The Episodic
ataxias are subdivided into two disorders on clinical and genetic grounds. In Both
disorders episodes are suppressed by acetazolamide. Patients need to be Warned about the
risk of nephrolithiasis on long term acetazolamide therapy; the Incidence of this
complication is estimated at 20%. Some patients may experience a Progressive ataxia
underlying the short lived episodes.
.

Mitochondrial disorders
These involve mutations in the genes that are found in the mitochondria, the
nergyproducing compartments of cells. As each person inherits their mitochondria from
Their mother, this means that these disorders can only be passed down the maternal Line.
Most of the genes found in the mitochondria are involved in the production of Energy, so
generally mitochondrial disorders result from an incapacity to produce Sufficient energy
within cells, preventing them from doing normal functions. Some Mitochondrial disorders
have ataxia as a main symptom. Examples of mitochondrial ataxia disorders are: NARP
(neuropathy,

ataxia,

and

Retinitis

pigmentosa),MELAS

(mitochondrial

encephalomyopathy, lactic acidosis with Stroke-like episodes) and Myoclonus epilepsy

with ragged red fibres (MERRF).

Cerebellar degenerations of unknown cause:

This syndrome has also been termed idiopathic cerebellar ataxia (ILOCA). Clinically
this disease resembles the autosomal dominant cerebellar ataxias, but with No family
history or detectable mutation. Extrapyramidal and autonomic features May be more
prominent and may be improved by symptomatic treatment (although Anti-parkinsonian
drugs seldom help much). Course and prognosis varies markedly Between patients.
Some patients initially diagnosed with this type of ataxia may then be given a Specific
diagnosis. For example they may be told they have multiple system atrophy (with
cerebellar symptoms), or may be diagnosed as having gluten ataxia (which may respond to
a gluten-free diet).

Fig no 3:- Altered walking gait of a woman with cerebellar disease

Causes of cerebellar ataxia

Damage, degeneration or loss of nerve cells in the part of brain that controls muscle
coordination (cerebellum), results in loss of coordination or ataxia.cerebellum comprises
two pingpong-ball-sized portions of folded tissue situated at the base of brain near
brainstem. The right side of cerebellum controls coordination on the right side of body;
the left side of cerebellum controls coordination on the left side of body.
Diseases that damage the spinal cord and peripheral nerves that connect cerebellum to
muscles also may cause ataxia. Ataxia causes include:

Head trauma. Damage to brain or spinal cord from a blow to head, such as
might occur in a car accident, can cause sudden-onset ataxia, also known as acute
cerebellar ataxia.

Stroke. When the blood supply to a part of brain is interrupted or severely

reduced, depriving brain tissue of oxygen and nutrients, brain cells begin to die.

Transient ischemic attack (TIA). Caused by a temporary decrease in blood supply

to part of brain, most TIAs last only a few minutes. Loss of coordination and other signs
and symptoms of a TIA are temporary.

Cerebral palsy. This is a general term for a group of disorders caused by

damage to a child's brain during early development before, during or shortly
after birth that affects the child's ability to coordinate body movements.

Multiple sclerosis (MS). MS is a chronic, potentially debilitating disease that

affects your central nervous system, which comprises brain and spinal cord.

Chickenpox. Ataxia can be an uncommon complication of chickenpox and other

viral infections. It may appear in the healing stages of the infection and last for
days or weeks. Normally, the ataxia resolves completely over time.

Paraneoplastic syndromes. These are rare, degenerative disorders triggered

by your immune system's response to a cancerous tumor (neoplasm), most
commonly from lung, ovarian, breast or lymphatic cancer. Ataxia may appear
months or years before the cancer is diagnosed.

Tumor. A growth on the brain, cancerous or noncancerous (benign), can damage the
cerebellum.

Toxic reaction. Ataxia is a potential side effect of certain medications, such as

barbiturates, such as phenobarbital, and sedatives, such as benzodiazepines. Alcohol and
drug intoxication; heavy metal poisoning from lead or mercury, for example and
solvent poisoning from paint thinner, for example also can cause ataxia.
For some adults who develop sporadic ataxia, no specific acquired or genetic cause can be
found. This is known as sporadic degenerative ataxia, which can take a number of forms,
including multiple system atrophy (MSA), a progressive, degenerative disorder.

Hereditary ataxias
Some types of ataxia and some conditions that cause ataxia are hereditary. If someone
have one of these conditions, they were born with a defect in a certain gene that makes
abnormal proteins. The abnormal proteins hamper the ability of nerve cells, primarily in
your cerebellum and spinal cord, to function properly and cause them to degenerate over
time. As the disease progresses, coordination problems worsen.
we can inherit a genetic ataxia from either a dominant gene from one parent (autosomal
dominant disorder) or a recessive gene from each parent (autosomal recessive disorder). In
the latter case, it's possible neither parent has the disorder (silent mutation), so there may
be no obvious family history.
Different gene defects cause different types of ataxia, most of which are progressive. Each
type causes poor coordination, but each has specific signs and symptoms.

Autosomal dominanat ataxia

These include:

Spinocerebellar ataxias. Researchers have labeled 28 autosomal dominant

ataxia genes with the designation SCA1 through SCA28, generally numbered

according to their order of discovery, and the number continues to grow. Cerebellar
ataxia and cerebellar degeneration are common to all types, but other signs and
symptoms, as well as age of onset, differ depending on the specific gene mutation.

Episodic ataxia: There are six recognized types of ataxia that are episodic
rather than progressive EA1 through EA6. All but the first two are rare. EA1
involves brief ataxic episodes that may last seconds or minutes; are triggered by
stress, being startled or sudden movement; and often are associated with muscle
twitching. EA2 involves longer episodes, usually lasting from 30 minutes to six
hours, that also are triggered by stress symptoms are dizziness (vertigo), fatigue
and muscle weakness during

episodes. In some cases of episodic ataxia,

symptoms resolve in later life. Episodic ataxia doesn't shorten life span, and
symptoms may respond to medication, such as acetazolamide (Diamox), which
also is used to treat seizures, or the anticonvulsant phenytoin (Dilantin).

Autosomal recessive ataxias

These include:

Friedreich's ataxia This neurological disorder involves damage to cerebellum,

spinal cord and peripheral nerves. Peripheral nerves carry signals from brain and
spinal cord to muscles. In most cases, signs and symptoms appear between the
ages of 5 and 15, but may occur as early as 18 months or as late as 30 years of age.
The rate of disease progression varies. If patient have Friedreich's ataxia, however,
he or she likely to rely on a wheelchair within 15 years of the appearance of
symptoms, and life span may be affected if the disorder includes significant heart
disease.

The first indication generally is difficulty walking (gait ataxia). The condition typically
progresses to the arms and trunk. Muscles weaken and waste away over time, causing
deformities, particularly in feet, lower legs and hands. Other signs and symptoms that

may develop as the disease progresses include slow, slurred speech (dysarthria); fatigue;
rapid, involuntary eye movements (nystagmus); spinal curvature (scoliosis); and heart
disease, including heart enlargement (cardiomyopathy) and heart failure.

Ataxia-telangiectasia. This rare, progressive childhood disease causes

degeneration in the brain and other body systems. Signs and symptoms usually
appear

by

age

10.

The

disease

causes

immune

system

breakdown

(immunodeficiency disease), which increases susceptibility to other diseases. It

affects various organs.
Telangiectasias are tiny red "spider" veins that may appear in the corners of child's eyes
or on the ears and cheeks. Although they're characteristic of the disease, child may or may
not develop them. Delayed development of motor skills, poor balance and slurred speech
are typically the first indications of the disease. Recurrent sinus and respiratory infections
are common. About 1 in 5 children with ataxia-telangiectasia develops leukemia or
lymphoma because of dysfunction in the immune system. Most people with the disease
need a wheelchair by their teens and die in their teens or early 20s.

Congenital cerebellar ataxia. This type refers to ataxia that results from
damage to the cerebellum that's present at birth.

Wilson's disease. People with this condition accumulate copper in their brains,
livers and other organs, which can cause neurological problems, including ataxia.

Signs and symptoms

Clinical presentation
These diseases can manifest mainly in adult life, but also in adolescence or childhood.
Certain features differ between patients and may help in making a precise diagnosis.
The following features are common presenting or early manifestations:

Progressive ataxia of gait (usually broad based)

Progressive limb ataxia including tremor

Progressive slurring dysarthria

Nystagmus

Later on during the course of these diseases:

Ophthalmoplegia

Dysphagia

Parkinsonian features

A minority of patients may encounter:

Decrease in visual acuity

Cognitive decline

Ataxia may affect movement of the middle part of the body from the neck to the hip area
(the trunk) or the arms and legs (limbs). When the person is sitting, the body may move
side-to-side, back-to-front, or both. Then the body quickly moves back to an upright
position. When a person with ataxia of the arms reaches for an object, the hand may sway
back and forth.
Common symptoms of ataxia include:

Clumsy speech pattern (dysarthria)

Repetitive eye movements (nystagmus)

Uncoordinated eye movements

Walking problems (unsteady gait)

Physical examination

Measurement and assessment

In the treatment of ataxia, it is essential to determine treatment programs suitable for the
patient and his/her needs in order to attain the desired goal of the physiotherapy and
rehabilitation program. This can be achieved through the use of appropriate measurement
and assessment methods, and the interpretation of the findings. Measurement and
assessment is not only significant in terms of preparing a suitable treatment program but
also in the follow-up of the changes in the patient's condition over a period of time and
the observation of the effects of the treatment.
Standardization problems in measurement and assessment which are one of the most
distressing aspects of neurological rehabilitation applications become more troubling in
cases of ataxia. In the literature, there are more scales, observational methods and
computerized systems developed to assess balance than to evaluate in-coordination.
Although the observational methods and scales mostly designed to assess balance are
easy to use and can be readily utilized in the clinic, their ability to provide standardized
measurements is limited, and the results can vary depending on the person who has done
the observation. Though computerized systems are highly reliable, they are costly
systems which require working within the laboratory environment. Balance assessment
tools frequently used by physiotherapists are shown in Table 1.

Table 1: Methods of Balance Assessment

Assessment Tool

Purpose of Tool

External Perturbation Test - Push and Release test (Jacobs et al.

Static balance
2006, Valkovic et al. 2008)
External Perturbation Test - Pull test (Hunt&Sethi 2006, Munhoz Static balance in different
et al. 2004, Horak et al. 2005)
sensory conditions

Dynamic balance in
Clinical Sensory Integration Test (Smania et al. 2008, Chaudry et
different sensory
al. 2004)
conditions
Sensory Integration Test of Computarised Dynamic
Static and dynamic
Posturography (Mirka&Black 1990, Jackson et al. 1995, Cham et
balance
al. 2006)
Static and Dynamic Posturography (Mohan et al. 2008, Federica
Static balance
et al. 2008, Buatois et al. 2006)
Single Leg Stance Test (Soyuer et al. 2006, Mann et al. 1996)

Static balance

Functional Reach Test (Martin et al. 2006, Jacobs et al. 2006)

Functional static and

dynamic balance

Berg Balance Scale (Yelnik&Bonan 2008, Ryerson et al. 2008,

Enberg et al. 2008)

Functional static and

dynamic balance

Five Times Sit to Stand Test (Buatois et al. 2008)

Functional dynamic
balance and gait

Time Up and Go Test (Zampieri& Di Fabio 2008, Vereeck et al. Gait and functional
2008)
dynamic balance
Dynamic Gait Index (Herman et al. 2008, Chang et al. 2008)

Dynamic balance and gait

Tandem Walking (Ravdin et al. 2008)

Dynamic balance

Four Square Step Test (Blennerhassett&Javalath 2008)

Dynamic balance

Measurements such as gait duration, step length, step width can be used apart from these
tests. Moreover, self-perception scales filled in by the patient such as Dizziness Handicap
Inventory, Activity Specific Balance Confident Scale and scales for daily living activities
such as FIM and Barthel Index can be employed to assist in assessment methods
(Wrisley & Pavlou 2005).
There are a limited number of scales which have been developed to assess truncal ataxia
and extremity ataxia together, and tested for validity and reliability. (Table 2)

Table 2: Scales of Ataxia

Assessment Tool

Purpose of Tool

International Cooperative Ataxia Evaluating truncal and extremity ataxia, gait ataxia,
Rating Scale (D'Abreu et al. 2007) nystagmus and talking
Scale for Assessment and Rating of Evaluating truncal and extremity ataxia, gait ataxia
Ataxia (Yabe et al. 2008)
and talking
Ataxia Functional Composite Scale Evaluating gait speed, upper extremity ataxia and
(Assadi et al. 2008)
visual acuity
Nine Hole Peg Test (Lynch et al.
Evaluating upper extremity ataxia
2005)
Computer
Graphics
(Erasmus et al. 2001)

Tablet

Brief
Ataxia
Rating
(Schmahmann 2009)

Scale Evaluating truncal and extremity ataxia, gait ataxia,

nystagmus and talking

Evaluating upper extremity ataxia

Friedreich's ataxia impact scale

(Cano 2009)

Speech, upper limb functioning, lower limb

functioning, body movement, complex tasks,
isolation, mood, self perceptions

Composite cerebellar functional

severity score (du Montcel 2008)

Upper limb functions

Coordination tests

1. Finger-to-nose: The shoulder is abducted to 90 with the elbow extended.The

patient is asked to bring the tip of the index finger to the tip of his or her nose.Alterations
may be made in the initial starting position to observe performance from different planes
of motion.

2.Finger-to-therapists finger: The patient and therapist sit opposite each other.The
therapists index finger is held in front of the patient.The patient is asked to touch the tip
of his or her index finger to the therapists index finger.The position of the therapists
finger may be altered during testing to observe ability to change distance,direction,and
force of movement.

3.Finger-to-finger : Both shoulders are abducted to 90 with the elbow extended.The

patient is asked to bring both hands toward the midline and approximate the index finger
from opposing hands.

4.Alternate nose to-finger: The patient alternately touches the tip of his or her
nose and the tip of the therapists finger with the index finger.The position of the
therapists finger may be altered during testing to observe ability to change
distance,direction,and force of movement.

5.Finger opposition: The patient touches the tip of the thumb to the tip of each finger
in sequence.Speed may be gradually increased.

6.Mass grasp: An alteration may be made between opening and closing fist (from
finger flexion to full extention).Speed may be gradually increased.

7.Pronation/supination: With the elbows flexed to 90 and held close to body,the

patient alternately turns the palms up and down.This test also may be performed with
shoulders flexed to 90 and elbows extended.Speed may be gradually increased.The ability
to reverse movements between opposing muscle groups can be examined at many
joints.Examples include active alternation between flexion and extension of the
knee,ankle,elbow,fingers,and so forth.

8.Rebound test: The patient is positioned with the elbow flexed.The therapist applies
sufficient manual resistance to produce an isometric contraction of biceps.Resistance is
suddenly released.Normally,the opposing muscle group(triceps) will contract and check
movement of the limb.Many other muscle groups can be tested for this phenomenon,such
as the shoulder abductors or flexors,elbow extensors,and so forth.

9.Tapping(hand): With the elbows flexed and the forearm pronated,the patient is
asked to tap the hand on the knee.

10.Tapping(foot): The patient is asked to tap the ball of one foot on the floor without
raising the knee ; heel maintains contact with floor.

11.Pointing and past pointing : The patient and therapist are opposite each
other,either sitting or standing.Both patient and therapist bring shoulders to a horizontal
position of 90 of flexion with elbows extended.Index fingers are touching or the patients
finger may rest lightly on the therapists.The patient is asked to fully flex the
shoulder(fingers will be pointing towards ceiling) and then return to the horizontal
position such that index fingers will again approximate.Both arms should be tested,either
seprately or simultaneously.A normal response consists of an accurate return to the starting
position.In an abnormal response,there is typically a past pointing,or movement beyond
the target.Several variations to this test include movements in other directions such as
toward 90 of shoulder abduction or toward 0 of shoulde flexion(finger will point toward
floor).Following each movement,the patient is asked to return to the initial horizontal
starting positon.

12.Alternate heel-to-knee;heel-to-toe: From a supine position, the patient is

asked to touch the knee and big toe alternately with the heel of the opposite extremity.

13.Toe to examiners finger: From a supin position, the patient is instructed to touch
the great toe to the examiners finger.The position of finger may be altered during testing
to observe ability to change distance,direction ,and force of movement.

14.Heel on shin: From a supine position,the heel of one foot is slide up and down the
shin of the opposite LE.

15.Drawing a circle : The patient draws an imaginary circle in the air with either UE
or LE (a table or the floor also may be used). This also may be done using a figure-eight
pattern.This test may be performed in the supine position for the LE.

16.Fixation or position holding:

Upper extrimity:The patient holds arms horizontally in front(sitting or standing).LE:The
patient is asked to hold the knee in an extended position(sitting).

Fig no 4:- Rapid alternating movements

Fig no5:- Finger- to-therapists finger

Fig no 6:- Heel on shin

Fig no 7:-Tendum walking

Fig no 8:- Rhomberg test

Fig no 9:- Rise from sitting position

Investigations

ENG or rotatory chair testing may show specific signs of a cerebellar disorder. In general,
one must be very careful in using these studies as the audiologists who commonly
interpret ENG tests, generally are unfamiliar with central disorders, and often simply say
that the patient has a "central vestibular disorder", rather than indicate that they don't find
anything wrong with their patient's ears.

Brain Imaging
Brain imaging is always obtained in cerebellar disorders. MRI imaging, with the highest
filed strength that is available, should be undertaken. Researchers do not recommend
"open MRI" testing in MRI's, or "low field". At the present writing - -2008 -- researchers
recommend 3T MRI testing, preferably with T1, T2, diffusion, and Flair. T1 saggital
images must be obtained to diagnose the Chiari malformation.
In general, MRI scanning often shows shrinkage of part or all of the cerebellum and/or
shrinkage of the brainstem. While great progress has been made recently in the
identification of genetic causes of cerebellar atrophy, neverthless the most common
situation is for genetic testing (if available) to be negative. This means that genetic testing
is frequently unhelpful, and the role of cerebellar genetic testing should not be
overemphasized. Some general references about radiological diagnosis are Huang, Tuason
et al. 1993; Wullner, Klockgether et al. 1993.
Because genetic testing is usually negative in progressive cerebellar disorders, the
"diagnosis" of cerebellar disorders basically means separation of patients into overlapping
groups -- genetically identified degenerations, progressive hereditary conditions without
an identified gene, and undiagnosed causes of cerebellar symptoms. Generally the
"undiagnosed" group is the largest one.
Broadly speaking, there are many disorders that cause shrinkage of the cerebellum -- such
as hereditary degenerations or toxins. There are very few disorders that cause shrinkage of
the brainstem - -these are mainly hereditary degenerations . Severe cerebellar
symptomswith a normal MRI scan suggest a paraneoplastic cerebellar problem.

Lumbar puncture (spinal tap)-removal of a small amount of fluid that surrounds

the brain and spinal cord

Blood tests

Ultrasound-a test that uses sound waves to examine the head

Urine analysis

Tests to detect other possible diseases that are causing the symptoms:
Nerve conduction study -a test that measures the speed and degree of electrical activity in
a nerve to determine if it is functioning normally
Electromyography (EMG)-a test measures and records the electrical activity that muscles
generate at rest and in response to muscle contraction

Diagnosis of Cerebellar
Ataxia

Since many neurological disorder have similar symptoms; sometimes, it is difficult to

diagnose cerebellar ataxia. The neurologist may need a number of tests to diagnose
sporadic cerebellar ataxia. Following are some tests that are done to diagnose the disease:

Brain scans: Magnetic Resonance Imaging (MRI) scans give the images of
cerebellum and help to determine whether the cerebellum is damaged.

Patient and Family history: These factors help in understanding, whether the
ataxia is caused due to alcohol, tumor or hereditary reasons. If the parents and
grandparents have ataxia, the patient is likely to be suffering from autosomal dominant
inheritance; while, autosomal recessive inheritance can be diagnosed, if parents are not
affected, but at least one child has ataxia.

Genetic tests: These tests help to diagnose the type of inherited cerebellar ataxia.
The main clinical features of cerebellar disorders include incoordination,
imbalance, and troubles with stabilizing eye movements. There are two distinguishable
cerebellar syndromes -- midline and hemispheric.
Midline cerebellar syndromes are characterized by imbalance. Persons are unsteady, they
are unable to stand in Romberg with eyes open or closed, and are unable to well perform
tandem gait. Severe midline disturbance causes "trunkal ataxia" a syndrome where a
person is unable to sit on their bed without steadying themselves. Some persons have
"titubation" or a bobbing motion of the head or trunk. Midline cerebellar disturbances also
often affect eye movements. There may be nystagmus, ocular dysmetria and poor pursuit.

Fig no 10:- CT scan

Differential diagnosis

1) Brain tumors, including cerebellar astrocytoma, medulloblastoma, neuroblastoma

2) Cerebellar contusion
3) Subdural hematoma
4) Toxins, including ethanol or anticonvulsants
5) Cerebellar infarction or hemorrhage
6) Meningitis
7) Encephalitis
8) Acute disseminated encephalomyelitis
9) Multiple sclerosis

Medical Management

Some drug treatments that have been used to control ataxia include:
5-hydroxytryptophan (5-HTP),
Idebenone,
Amantadine,
Physostigmine,
L-carnitine or derivatives,
Trimethoprimsulfamethoxazole,
Vigabatrin,
Phosphatidylcholine,
Acetazolamide,
4-aminopyridine,
Buspirone, and a combination of coenzyme Q10 and vitamin E

PT management &
Treatment principles

Main principles of training

1. Throughout the whole training program, exercises should be practiced consciously
at first, and in later stages should be followed by automatic exercise activities
2. Exercises should progress from simple to complex
3. Activities should be practiced first with the eyes open and later with the eyes
closed
4. After achieving proximal tonus and stabilization, the coordinated movement of the
distal segments should be taken into consideration
5. Compensation methods and supportive aids and equipment should be employed
when necessary
6. Treatment should be supported by an appropriate home exercise program and
sports activities

GOAL
S OF TREATMENT
The goal of the physiotherapist in the rehabilitation of ataxia resulting from defects in
neurological structures and effecting the functions of the patient, is to improve the
functional level of the patient through restorative techniques. When this is not possible,
the therapist makes use of compensatory strategies to make the patient perform as
independent as possible within the present functional level. The goals of restorative
physical treatment can be briefly described as:
1. Improving balance and postural reactions against external stimuli and gravitational
changes
2. Improving and increasing postural stabilization following the development of joint
stabilization
3. Developing upper extremity functions

4. Improving balance and postural reactions against external stimuli and gravitational
changes
5. Improving and increasing postural stabilization following the development of joint
stabilization
6. Developing upper extremity functions
7. Through developing independent and functional gait, improving the life quality of
the patient by increasing the patient's independence while performing daily life
activities

Physical therapy approaches

A physical treatment program is prepared from the interpretation of the
measurement and assessment results. The contents of the treatment program can vary
depending on the type and characteristics of ataxia. For instance, while approaches which
improve proprioception and incorporate visual aids are used more commonly in patients
with sensory ataxia, stabilization training is more important to reduce truncal and
extremity ataxia in patients with cerebellar ataxia. The patient with vestibular ataxia
should be given habitation exercises in order to reduce vertigo, and also vestibulo-ocular,
vestibulo-spinal reflexes should be stimulated to improve balance. In some cases, a
problematic condition which requires the use of a number of approaches, such as mixed
ataxia, may arise. In such cases, the experience of the physiotherapist and the patient's
effort plays an important role in determining the program.
When preparing the treatment prescription, it should be kept in mind that the
proprioceptive, vestibular and visual systems, and the cerebellum are in close relation, and
that balance and coordination result from this relation. For example, proprioceptive
exercises contribute to balance while improving proprioception. The opposite of this is
also true. Approaches in the treatment of extremity ataxia may enable proprioceptive input
to increase and the balance to develop by establishing stabilization. Therefore, it is not

possible to classify the methods used in the rehabilitation of ataxia as approaches directed
merely towards proprioception or balance, since all of these interact with each other.
The classification of treatment applications can be briefly described as follows:

Approaches for improving proprioception

The aim is to increase proprioceptive input by mechanically stimulating the joint
surfaces, muscles and tendons, and decreasing postural instability by improving body
awareness. There are many approaches that can be used for this purpose. These are:
Proprioceptive Neuromuscular Fascilitation (PNF), rhythmic stabilization, slow reversal
techniques (Adler et al. 2000, Gardiner 1976), resistive exercises (DeSouza 1990, Arai et
al. 2001), use of Johnstone pressure splints (Armutlu et al. 2001), gait exercises on
different surfaces (hard, soft, inclined surfaces) with eyes open and closed, plyometric
exercises (Risberg et al. 2001), balance board-ball and minitrampoline exercises
(Diracoglu et al. 2005).
Recently, vibration has been a frequently used application. Vibration can directly be
applied to the muscle and tendon, and also is applied by exposing the whole body to
vibration (Schunfried et al. 2007, Hatzitaki et al. 2004, Semenova 1997).
Another method is the suit therapy. The suit is made up of a vest, shorts, knee pads
and special shoes attached by using bungee type bands that are used to correctly align the
body and provide resistance as movements are performed. Its major goals are to improve
proprioception (sensation from joints, fibers, and muscles), and to increase weight-bearing
for normalized sensory input regarding posture and movement (Semenova 1997).
In addition, methods which develop body awareness, such as the Feldenkrais and
Alexandre Techniques (Jain et al. 2004), yoga, and body awareness exercises can be
included in the program.

Activities for improving balance

Firstly, the proximal muscles and stabilization of the trunk should be improved
(Edwards 1996). For this purpose, it is appropriate to use the mat activities of the PNF
techniques. Following the neuro-developmental order, the patient should be trained to
come to the bridge position from lying on the back, onto the forearms from lying face
down, to crawl, and to come onto the knees, half knees and into a sitting position, and to
establish static and dynamic stability in these positions. Initially, the patient should be
maintained in the required position by approximation and verbal directions, and then static
stabilization should be strengthened through external perturbation (pushing and pulling in
different directions).
Afterwards, the patient should be trained in these positions for weight transferring
and functional extension so as to be prepared for dynamic stabilization. Subsequently, the
patient should be trained in positions in which the support surface is narrowed or the
center of gravity is changed in order to make the balance activities difficult. (e.g.
establishing balance on two or three extremities in the crawling position or shifting the
center of gravity upwards by the elevation of the arms in the sitting-on-the-knees position)
(Addler et al. 2000).
In the standing position, following the transferring of weight onto the front, back and
sides, narrowing the support surface and balance training in tandem position, balance
training on one leg should be performed. This is a position with which ataxic patients have
great difficulty.
Another option is to perform balance training on the posturography device in order to
benefit from visual feedback obtained from observing the patient's ability to sustain
his/her postural oscillation in the center of gravity (Qutubuddin et al. 2007).
The best indicator of dynamic stabilization/balance is gait. Therefore, gait training should
be given including the following applications : walking on two narrow lines, tandem gait,

backward gait, slowed down gait (soldier's gait), stopping and turning in response to
sudden directions, flexion, extension and left-right rotations of the head.
Disciplines such as Tai Chi (Hackney&Earhart 2008) and Yoga consist of activities which
develop balance.

Vestibular exercises
Since dizziness accompanies balance dysfunction in vestibular problems, repetitive head
movements and Cawthorne and Cooksey exercises (Dix 1979) are of great importance. A
vestibular exercise program consists of repetitive, progressively more difficult, eye, head
and body movements designed to encourage movement and facilitate sensory substitution.
Many components of this exercise program are used by physical and occupational
therapists today (Ribeiro et al. 2005, Corna et al. 2003, Jauregui-Renaud et al. 2007,
Brown et al. 2006).

Approaches to extremity ataxia

Exercises designed for the treatment of extremity ataxia are utilized to provide fixation
by establishing balance between the eccentric and concentric contractions within the
multi-joint movements of lower extremities and the upper extremities in particular. During
the performance of these exercises, it is important to establish slow, controlled and
reciprocal multi-joint movement and stabilization. Freenkel's coordination exercises were
developed for this purpose (Edwards 1996, Danek 2004). Actively repeated contractions
similar to PNF can be utilized on their own or by combining them with Freenkel's
coordination exercises (Armutlu et al. 2001). While these two types of exercise are
effective in cases with mild extremity ataxia, they can be insufficient in severe cases. In
such cases, rhythmic stabilization and combination of isotonic techniques are more
effective than PNF (Adler et al. 2000).
Coordination Dynamics Therapy (CDT) was developed by Dr. Giselher Schalow. This
therapy, he says, "improves the self-organization of the neuronal networks of the CNS for
functional repair by exercising extremely exact coordinated arm and leg movements on a

special device (GIGER MD) and, in turn, the coordinated firing of the many billions of
neurons of the human CNS" (Schalow 2006, Schalow 2004, Schalow 2002).

Use of supportive aids

In cases which restorative physical treatment applications are insufficient, use of
supportive devices enables the patient to function more easily within his present functional
level. In cases of severe ataxia, suspending weights from the extremities and the use of
weighted walkers can be preferred (Gibson-Horn 2008).

Sports activities
Horse riding, swimming, playing billiards, golf and darts are suitable for this type of
patient (Bertoti 1988, Hammer et al. 2005).

Conclusion of treatment
According to the physiotherapist, mobility and upper extremity functions are the most
important functions of the patient. Ataxia is a neurological problem with major effect on
both functions and it, when compared to other symptoms of neurological diseases (muscle
weakness, spasticity), is sometimes more persistent and difficult to cope with. Therefore,
physical therapy applications play an important part in the management of ataxia.
Evaluation of the patient, determination of suitable treatment methods and problem
solving approach, as well as performing the exercises regularly; are of major importance
for the success of treatment programme.

Frenkel's Exercises for Ataxic Conditions

These exercises prepared by Curative Services -- Courage Center
This program consists of a planned series of exercises designed to help you compensate
for the inability to tell where your arms and legs are in space without looking.
The exercise routine takes about 1/2 hour and should be done 2 times daily.

1. Exercises are designed primarily for coordination; they are not intended for
strengthening.
2. Commands should be given in an even, slow voice; the exercises should be done to
counting.
3. It is important that the area is well lit and that you are positioned so that you can watch
the movement of your legs.
4. Avoid fatigue. Perform each exercise not more than four times. Rest between each
exercise.
5. Exercises should be done within normal range of motion to avoid over-stretching of
muscles.
6. The first simple exercise should be adequately performed before progressing to more
difficult patterns.

Exercises While Lying:

Starting position: Lie on bed or couch with a smooth surface along which the feet may be
moved easily. Head should be raised on a pillow so that patient can watch every
movement.

1. Bend one leg at the hip and knee sliding heel along the bed. Straighten the hip and knee
to return to the starting position. Repeat with the other leg.
2. Bend one leg at the hip and knee as in #1. Then slide leg out to the side leaving heel on
the bed. Slide leg back to the center and straighten hip and knee to return to the starting
position. Repeat with the other leg.
3. Bend one leg at the hip and knee with the heel raised from the bed. Straighten leg to
return to the starting position. Repeat with the other leg.
4. Bend and straighten one leg at the hip and knee sliding heel along the bed stopping at
any point of command. Repeat with the other leg.

5. Bend the hip and knee of one leg and place the heel on the opposite knee. Then slide
heel down the shin to the ankle and back up to the knee. Return to starting position and
repeat with the other leg.
6. Bend both hips and knees sliding heels on the bed keeping ankles together. Straighten
both legs to return to starting position.

7. Bend one leg at the hip and knee while straightening the other in a bicycling motion.

Exercises While Sitting:

Starting position: Sit on a chair with feet flat on the floor.

1. Mark line, raising just the heel. Then progress to alternately lifting the entire foot and
placing the foot firmly on the floor upon a traced foot print.
2. Make two cross marks on the floor with chalk. Alternately glide the foot over the
marked cross: forward, backward, left and right.
3. Learn to rise from the chair and sit again to a counted cadence. At one, bend knees and
draw feet under the chair; at two, bend trunk forward; at three, rise by straightening the
hips and knees and then the trunk. Reverse the process to sit down.

Exercises While Standing:

Starting position: Stand erect with feet 4 to 6 inches apart.
1. Walk sideways beginning with half steps to the right. Perform this exercise in a counted
cadence: At one, shift the weight to the left foot; at two, place the right foot 12 inches to
the right; at three, shift the weight to the right foot; at four, bring the left foot over to the

right foot. Repeat exercise with half steps to the left. The size of the step
taken to right or left my be varied.
2. Walk forward between two parallel lines 14 inches apart placing the right foot just
inside the right line and the left foot just inside the left line. Emphasize correct placement.
Rest after 10 steps.
3. Walk forward placing each foot on a footprint traced on the floor. Footprints should be
parallel and 2 inches from a center line. Practice with quarter steps, half steps, threequarter steps and full steps.
4. Turn to the right. At one, raise the right toe and rotate the right foot outward, pivoting
on the heel; at two, raise the left heel and pivot the left leg inward on the toes; at three,
completing the full turn, and then repeat to the left.
5. Walk up and down the stairs one step at a time. Place the right foot on one step and
bring the left up beside it. Later practice walking up the stairs placing one foot on each
step. At first use the railing, then as balance improves dispense with the railing.

Upper Extremity Exercises:

When the arms are affected use a blackboard and chalk. Change a minus sign to a plus
sign; copy simple diagrams (straight lines, circles, zig-zag lines, etc.) Various coordination
boards may be used to improve eye-hand coordination.

Fig no 11:- Lines for walking

Fig no.12:- Foot prints for walking

Fig no 13:-Exercise for legs in standing

Fig no 14: Transference of weight from foot to foot

Fig no 15:- One leg stretching to slide heel to a position indicated by a mark on the floor

Fig no 16:- gait training in parallel bar

Conclusion

Patients with cerebellar dysfunction may struggle with depression and other forms of
psychological distress, limitations in cognitive ability and flexibility, slowed reaction
times and impaired attentional modulation, as well as less ability to do "multitasking"
automatically. These important aspects of higher order behavior have an impact on quality
of life, employment, and personal relationships and need to be recognized by the medical
profession as well as by patients and their families. By working with available treatments
and novel cognitive rehabilitation strategies, adults and children with inherited or acquired
cerebellar disorders could benefit from the new recognition that the cerebellum is not only
a motor control device, but it is also an essential component of the brain mechanisms for
personality, mood, and intellect.

References

Reference books:1. Physical Rehabilitation Fifth edition OSullivan Schmitz.

2. Brain And Bannisters Clinical Neurology Seventh edition.
3 .Roberta B Shepherd Third edition.
4. Corna S, Nardone A, Prestinari A, et al. 2003. Comparison of Cawthorne-Cooksey
exercises and sinusoidal support surface translations to improve balance in patients with
unilateral vestibular deficit. Archives of Physical Medicine and Rehabilitation 84(8):117384.
5.

Edwards S. 1996. Abnormal tone and movement as a result of neurological

impairment: considerations for treatment. In: Edwards S editor. Neurological

Physiotherapy. New York: Churchill Livingstone
6. Guyton AC. 1976. Nervous System. Textbook of Medical Physiology. Philadelphia:
WB Saunders.
7. Herdman SJ. 1998. Vestibular disorders and rehabilitation. In: Lazar RB editor.
Principles of Neurologic Rehabilitation. United States: The McGraw-Hill Companies

Figure caption
Figure
1

Discription

Page
no.

Cerebellum
2

Cerebellum and its surrounding

regions
3

Altered walking gait of a

woman with cerebellar disease

Rapid alternating movements

Finger-to-therapists finger

Heel on shin
7 Tendom walking
8 Rhomberg test

Rise from sitting position

10

CT scan

11

Lines for walking

12

Foot prints for walking

13

Exercise for legs in standing

14

Transference of weight from

foot to foot

15

One leg stretching to slide heel

to a position

16

Gait training in parallel bar