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 & it’s management

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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 non-

motor 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 ataxiasSome 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. 2006, Valkovic et al. 2008)

Static balance

External Perturbation Test - Pull test (Hunt&Sethi 2006, Munhoz et al. 2004, Horak et al. 2005)

Static balance in different sensory conditions

Clinical Sensory Integration Test (Smania et al. 2008, Chaudry et Dynamic balance in different sensory

al. 2004) conditions

Sensory Integration Test of Computarised Dynamic Posturography (Mirka&Black 1990, Jackson et al. 1995, Cham et al. 2006)

Static and dynamic balance

Static and Dynamic Posturography (Mohan et al. 2008, Federica et al. 2008, Buatois et al. 2006)

Static balance

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. 2008)

Gait and functional 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 Rating Scale (D'Abreu et al. 2007)

Evaluating truncal and extremity ataxia, gait ataxia, nystagmus and talking

Scale for Assessment and Rating of Ataxia (Yabe et al. 2008)

Evaluating truncal and extremity ataxia, gait ataxia and talking

Ataxia Functional Composite Scale (Assadi et al. 2008)

Evaluating gait speed, upper extremity ataxia and visual acuity

Nine Hole Peg Test (Lynch et al. 2005)

Evaluating upper extremity ataxia

Computer Graphics Tablet (Erasmus et al. 2001)

Evaluating upper extremity ataxia

Brief Ataxia Rating Scale (Schmahmann 2009)

Evaluating truncal and extremity ataxia, gait ataxia, nystagmus and talking

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-therapist’s finger: The patient and therapist sit opposite each

other.The therapist’s 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 therapist’s index finger.The position of the

therapist’s 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 therapist’s finger with the index finger.The position of the

therapist’s 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 therapist’s.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 examiner’s 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-therapist’s 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,

Trimethoprim–sulfamethoxazole,

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

GOALS 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, three-

quarter 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 O’Sullivan Schmitz.

2. Brain And Bannister’s 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):1173-

84.

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

Discription Page no.

1 Cerebellum 2

2 Cerebellum and its surrounding regions

3

3 Altered walking gait of a woman with cerebellar disease

4 Rapid alternating movements

5 Finger-to-therapist’s finger

6 Heel on shin

7 Tendom walking

8 Rhomberg test

9 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