ATAXIA PDF

ATAXIA DR/RAGHDA NASSAR  Introduction The cerebellum, also refered to as the little brain, plays a vital role in the control of movement. It is comprised of three main lobes;  1- The vestibulocerebellum (archicerebellum) flocculonodular lobe  2- The spino-cerebellum (paleocere...

ATAXIA DR/RAGHDA NASSAR  Introduction The cerebellum, also refered to as the little brain, plays a vital role in the control of movement. It is comprised of three main lobes;  1- The vestibulocerebellum (archicerebellum) flocculonodular lobe  2- The spino-cerebellum (paleocerebellum) anterior lobe  3- The cerebrocerebellum (neocerebellum)posterior lobe.  Connections of the cerebellum  The vestibulocerebellum is responsible for maintaining balance and assists in postural control.  The spinocerebellum aids in error detection and modulating muscle tone,  Cerebrocerebellum is involved in coordination,fine movement,,action preparation and timing.  The cerebellum receives input from cortical, sensory and proprioceptive areas, then projects this information to the thalamus. Damage to the cerebellum can cause significant motor impairment. CEREBELLUM AND CONTROL SYSTEMS  The cerebellum receives extensive sensory input, and it appears to use this input to guide movements in both a feed back and feed forward control manner.  Feedback control systems(Closed loop systems)  In a feed back controller ,a desired output is compared continuously with the actual output, and adjustments are made during the execution of the movement until the actual movement matches the desired movement. Feed forward control systems (Open loop systems)  In a pure feed forward system, once the commands are sent, there is no way to alter them (i.e.,there is no feed back loop).  The advantage of a feed forward system is that it can produce the precise set of commands for the effect or without needing to constantly check the output and make corrections during the movement itself.  Coordination  Act as a comparator (Error- Correction Mechanism) : (closed Loop model ) Cerebellum compares voluntary commands of signals with the sensory signals produced by the involving movement. If they are not matched, cerebellum will provide corrective feedback to motor pathways. In cerebellar lesion, cerebellum may not automatically correct movement errors and movement becomes dysmetric.  Act as a compensator : It performs predictive compensatory modification of reflexes in preparation of movement  Act as adaptive feed control system (open loop model ) It programs or model voluntary movement skill based on memory of previous sensory input and motor output  Functions of cerebellum  1. Regulation of Equilibrium.  2. Maintenance of tone. Through connections with spinal cord, cerebellum regulates the tone and contraction of the axial and proximal limb ms. Lesion leads to hypotonia.  3. Regulation (or Coordination) of Voluntary Movements.  4. Motor learning(Adaptive feed forward control system):).  3. Regulation (or Coordination) of Voluntary Movements.  The cerebellar role in coordination of movements is carried out by a No. of mechanisms, including:-  a) Comparator and Error- Correction Mechanism.  b) Damping Mechanism.  c) Planning the Sequence and Timing of Movements  Damping Mechanism.  All movements of the body tend to overshoot due to momentum. The cerebellum sends braking signals to stop the movement at intended point, thereby preventing the overshoot.  If the cerebellum is damaged, the cerebral cortex will recognize the overshooting only after it occurs and corrects it, this leads to oscillation of the limb around the intended point i.e. intention kinetic tremor.  What is ataxia?  In-coordination of voluntary movement activity with or without disequilibrium in absence of motor weakness  Co-ordination  Definition: Integration between motor and sensory system in a harmonical manner to produce purposeful movement  or the ability to use right muscles at right time with proper intensity to reach certain goal.  Structures Responsible For Balance Development And Coordination 1. Intact cerebellum. 2. Intact motor system. 3. Intact sensory cortex. 4. Intact proprioceptors. 5. Intact vestibular system. 6. Intact vision.  Types of ataxia  1- Cerebellar ataxia  2- Sensory ataxia  3-Vestibular ataxia  4- Mixed ataxia  5-Hysterical ataxia.  1-Cerebellar ataxia  Cerebellar ataxia develops as a result of lesions to the cerebellum, and/or the afferent and efferent connections of the cerebellum.  Vestibulo-cerebellar dysfunction  Spino-cerebellar dysfunction  Cerebro-cerebellar dysfunction  Causes:  1. Idiopathic which may be related to age.  2. Symptomatic due to vascular, inflammatory, traumatic,or tumor.  3. Heridofamilial (very common) as Friedreich's or Marie's ataxia.  Age of Friedreich's ataxia Marie's ataxia onset Cerebellar 1st decade 2nd and 3rd decades manifestati Mainly neocerebellar , Pyramidal tract on Mainly (incoordination in form of archicerebellar(disturb nystigmus, dysartheria, nodding of ance of equilibrium- head, titubation of trunk, deviation swaying. during standing- toward affected side in unilateral walk wide base or drunken gait) side or zigzag in bilateral lesions) Spinocerebellar,(Pyramid al tract, peripheral nerves and posterior column) Deep Diminished Exaggerated reflexes sensations Impaired Preserved sensations superficial and deep sensations  2-Sensory Ataxia:  Can be observed in types of hereditary ataxia such as Friedreich's ataxia.  Sensory ataxia may also be observed in diseases such as  Peripheral neuropathy: diabetic-alcoholic- nutritional  Posterior column  Brainstem lesion  Thalamic syndrome  Parietal lobe lesion  3- Vestibular Ataxia  It is ataxia due to lesions of the vestibular division of the eighth nerve.  CAUSES: 1) Meniere's disease. 2) Labyrinthitis. 3) Acoustic neuroma.  CLINICALLY: There is:  - Vertigo, tinnitus, deafness. Cerebellar syndromes( clinical pictures) :  a) Archicerebellum (vestibulocerebellum syndrome): It is manifested by:- 1) Swaying during standing, with a tendency to fall down. 2) Unsteady (staggering) gait→ wide-based in order to provide better equilibrium during walking  b) Paleocerebellum-spinocerebellum syndrome: It is manifested by:- Hypotonia and hyporeflexia that occur also in both Archicerebellar and Neocerebellar syndromes  c)Neocerebellum (cerebro cerebellum) syndrome: It is manifested by:- -Hypotonia and hyporeflexia This occurs in acute cerebellar lesions, but it is rarely seen in chronic lesions. Hypotonia is distinct particularly in proximal and antigravity muscles.  Asthenia( is not a common symptom, only 10% of patients)  This describes a generalized non-specific weakness as a feature of cerebellar dysfunction. This occurs more often with extensive and deep lesions and is most apparent in the proximal musculature. Fatigue has also been noted as a common feature of cerebellar dysfunction.  Incoordination;  1) Dysmetria→ inability to judge (errors) in the range and direction of the movement (hypometria or hypermetria)  2) Intention Tremors (Kinetic Tremors): They appear when the patient performs a voluntary motor act, not seen when the ms are at rest.  Kinetic tremor, which is oscillation that occurs during the course of the movement  Intention tremor, which is the increase in tremor towards the end of the movement N.B: patients has head nodding, trunk titubation and intention kinetic tremors of extremities. Titubation, which is tremor affecting the head and upper trunk typically after lesion of the vermis Postural truncal tremor, which affects the legs and lower trunk, is seen in anterior cerebellar lobe lesions  3) Decomposition of Complex Movements: The patient performs the movement in a distinct sequences rather than smooth movement 4) Rebound Phenomenon  The cerebellar patient is unable to stop the ongoing movement rapidly due to failure of the damping functions of the cerebellum.When there is a flexion of the forearm against resistance (provided by the examiner's hand), the cerebellar patient cannot stop the resultant inward movement of his limb in due time following its release, and the forearm flexes forcibly and may strike his body with considerable violence. 5) Dysdiadochokinesia  Difficulty to perform rapid alternating opposite movements e.g. rapid repetitive pronation and supination of forearm.The movements are slow and irregular.  Diadochokinesia = ability to perform RAM  Dysdiadochokinesis = slow, irregular, clumsy movements  6) Nystagmus Nystagmus of cerebellar disorders is a tremor of the eye balls, oscillatory movements of the eye in a horizontal, vertical , rotatory or mixed direction  7) Dysarthria: Staccato speech →slow, explosive, interruptive speech. Or scanning speech →slow and decomposed speech.  8) Unsteady Gait The gait is unsteady and broad-based due to dysmetria and kinetic tremors of the lower limb ms. In case of archicerebellum lesion, it will lead to wide base or drunkening gait. In case of neocerebellum lesion, it will lead to deviation to one side (affected side) if the lesion is unilateral, but if the lesion is bilateral, it will lead to zigzag gait.  PROBLEMS OF ATAXIC PATIENTS  Lack of balance:  postural sway and poor equilibrium may be disturbed by changes in proprioceptive control loops of the cerebellum.  Inco-ordination of movement  Instability around shoulder & pelvis which interfere with: -Control of the arm and difficulty in sustaining activity against gravity. -Manipulation of objects at a distance from the object.  Instability around the pelvis interferes with control of the pelvis on the extended leg in weight bearing.  Orofacial dysfunction: nystagmus, dysarthria  Poor visual fixation & eye hand contact due to tremor, nystagmus &nodding of the head  Inadequate head and trunk control effect on the postural control  Gait problems (wide base gait-drunken gait)  Fear of fall  Wide BOS  Decomposition of movement  Visual feedback  Attention and  concentration  Hypermetria  Time  Activities of muscles  Certain Principles to be considered during Assessment:  Determine of basic functional capabilities.  Assessment must be done bilaterally even in  unilateral lesion  Examination of functional activities must include:  Assistance needed  Time to complete to the activity  Precautions should be taken in consideration during assessment of ataxic patients:  - Assessment must be done bilaterally even in unilateral lesion  - Assessment must be done in quiet place to avoid distraction  - Age and psychological state must be considered  Assessment of ataxic patients  - Motor assessment including muscle tone and muscle test  - Sensory assessment including superficial and deep sensation  - ROM  - Orofacial function assessment including facial expression, lip & jaw closure  - Coordination of respiration with swallowing and speech  Coordination assessment (the most important item)  including non equilibrium and equilibrium subtypes.  Coordination tests focus on assessment of movement capabilities in five main areas:  1- Alternate or reciprocal motion.  2- Movement composition, or synergy.  3- Movement accuracy.  4- Fixation, or limb holding.  5- Equilibrium and postural holding.  A)Equilibrum co-ordination :-  1 ) static :  - Prone on elbows. - Prone on hands.  - Quadruped. - Half kneeling.  - Sitting on the edge of bed with arms infront of the patient, arms in abduction, arms backward.  - Standing with feet apart, with feet together, one foot forward and other backward, on one foot.  2) Dynamic :-  - From supine to side lying. - From side lying to prone.  - Sitting with leaning forward then regain.  - Sitting with leaning side-ways.  - Sitting with leaning backward then regain.  - Sitting to standing - Walk forward.  - Walk sideward. - Walk backward.  - Walk in a circle. - Walk on heels or toes  Grading system :-  1: Normal performance.  2: Minimal impairment : able to accomplish with slightly less than normal speed and skill.  3: Moderate impairment: able to accomplish activity but movements are slow and unsteady.  4: severe impairment: only able to initiate activity without completion.  5: Activity impossible.  B) Non-Equilibrum co-ordination tests:  1- Finger-to- nose test:  The patient brings the tip of his forefinger from a distance on to the trip of his nose.The test is conducted with the eyes open then closed.  2- Finger-to-finger test: The patient brings the tips of his forefinger from the distance of his outstretched arms to meet each other in the midline.  3- Finger-to-doctor's finger test: The patient brings the tip of his forefinger from a distance on to the tip of doctor's forefinger.  In test 1, 2 and 3 we see the following:  -Reciprocal of movement.  -Decomposition of movement  - Tremor.  – Fixation or limb holding.  - Accuracy ( speed and distance (i.e) dysmetria in the form of hypo or hypermetria )  4- Adiadokokinesis or Dysdiadokokinesis: The patient is asked to do rapidly alternating movement.  e.g : pronation and supination of the forearm  5- Rebound phenomenon :- The patient with his elbow fixed , flexes it against resistance , when the resistance is suddenly released the patient's forearm flies upwards and may hit his face or shoulder.  6- Heel-to-knee test : For lower limb The patient raises his leg , brings down his heel onto the knee of his other leg and slides it down along the shaft of the tibia.  7- Walking along a straight line , foot close to foot :- There is deviation to the affected side in cases of unilateral cerebellar lesion. Tandem gait  Romberg’s Test  Pt is standing on a wall (prevent falling) & ask pt to close his eyes & maintain his position in standing  If swaying occurs indicates the presence of sensory ataxia  Aim of this test:To differentiate between cerebellar and sensory ataxia.  Gait assessment  Type of gait according to site of lesion:  - Archi-cereballar lesion :wide base or drunken.  - Neo cerebellar lesion deviation to one side or zigzag.  Evaluation of gait: walk slowly, change direction and different speeds.  The patient progression in ambulation can be determined by the number of times they lose their balance in a treatment session, frequency of a specific error, the distance ambulated, or the level of assistance needed.  Treatment: Goals:  1.Postural stability.  2. Functional gait.  3. Accuracy of limb movement.  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 (i.e. eye hand coordination)  4- Through developing independent and functional gait, improving the life quality of the patient by increasing the patient's independence while performing ADL.  Main principles of training  1- simple then complex  2- eyes open then closed  3- proximal tonus and stabilization then distal segments  4- compensation methods and supportive aids and equipment should be employed when necessary  5- home exercise program and sports activities Approaches for improving proprioception  Methods of treatment:  PNF technique  1- Rhythmic stabilization to improve proximal stability  2-Reversal tech. to improve ability to alternate movement from agonist and antagonist  3-Approximation to improve proximal fixation  - Resistive ex.  - gait exercises on different surfaces (hard, soft, inclined surfaces) with eyes open and closed  - 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.  - Tapping and weight bearing increasing stability  - Using ankle and wrist weight to decrease tremor  - Balance can improved in antigravity position (sitting, standing) in normal base of support  - balance board ,ball  Methods to improve coordination:  1-Frenkel's exercises: a series of gradual progressive exercises designed to increase coordination  Aim : Establishing control of movement by use of any part of sensory mechanism which remain intact as sight & hearing to compensate for the loss of kinesthetic sensation  Graduations:  Fast then slow  Big joint then small joint  One joint then more than one joint  One direction then more than one direction  Unilateral then bilateral  Symmetrical then asymmetrical  Continuous then interrupted  on the bed then off the bed  Wide BOS then narrow BOS (Supine then sitting then standing)  Steps of exercises from supine:  1- Flex & extend one leg, heel sliding down a straight line on table.  2- Abduct & adduct hip smoothly with knee bent, heel on table.  3- Abduct & adduct leg with knee & hip extended, leg sliding on table.  4- Flex & extend hip & knee with heel off the table.  5- Place one heel on knee of opposite leg & slide heel smoothly down toward ankle & back to knee.  6- Flex & extend both legs together heels sliding on table.  7- Flex one leg while extending other leg.  8- Flex & extend one leg while abducting &adducting the other.  Sitting :  1-Slide heel to reach a mark on the floor  2-change standing and sit again  Standing :  1-transfer weight from foot to foot  2- walking side ways  3-placing foot on specific marks  For arms :  Sitting with arm supported on a table and placing hand at specific mark  Try to reach an object  Picking up objects  Put the hand in a ring or hole  2. Specific techniques of PNF  a- Slow reversal: - It is isotonic contraction of antagonist followed by isotonic contraction of agonist.  b- Slow reversal hold: - It is isotonic contraction of antagonist followed by isometric contraction of antagonist, followed by the same sequence of agonist.  3. Repetition of non equilibrium tests with specific modification (Interruption of the range, with eye open then closed, and change speed)  4. Combined pattern of PNF  a- Bilateral symmetrical  B-Bilateral asymmetrical  c- Reciprocal same diagonal  D-Reciprocal opposite diagonal.  Training of coordination should include (5Ps):  1- Perception : To tell whether or not performance is occurring as desired through proprioceptive pathways and reinforced by visual and tactile perception.  2- Precision : Breaking down activity to units which are simple so that they can be practiced more precisely  3- Perceptual practice : Repetition of activity at frequently intervals.  4- Peak performance : The patient practice the movement below peak performance which is determined according to complexity , muscular effort and repetition in order to avoid occurrence of fatigue.  5- Progression: Revision of peak performance as improvement occur and transition of exercises into functional goal.  VERTIGO Treatment (vestibular Exercises)  Vertigo: -  It is the sense of rotation of the body in steady surrounding or the reverse  Designed to promote vestibular adaptation and substitution. The goals are to enhance gaze stability, provide postural stability, improve vertigo, and improve ADLs.  -Vestibular habituation exercises  - Cawthorne Cooksey exercises  1. Vestibular habituation training: -  Is based on the hypothesis that a response decline occurs as a result of repeated stimulation of those receptors which elicit vertigo.  Steps  1. Determine what position or movement elicit vertigo and note intensity and duration of vertigo.  2. Select the exercises or movement which elicit the vertigo as intensely as possible.  3.Repeat each exercise.five times twice daily.  4. After each repetition of each exercise instruct the patient to stay in the position that elicit vertigo as long as vertigo persist.  2. The cawthorne-cooksey exercises  Is a series of graduated exercise that encourage head and eye movement which train visual and somato-sensory systems to compensate for vestibular dysfunction. The exercises are graduated from easily tolerated movement to movement that is more likely to elicit vertigo.  Each ex is repeated 10-20-times per day and progress from slow to fast  Methods to decrease The Tremor of limbs - Tonic holding - Alternative isometric ex. - Weight bearing exercises. - Approximation. - Weights in ankle & wrist.  Methods to decrease nodding of the head and Titubation of the Trunk  1. Rythmic Stabilization  2. Alternative isometric ex.  Asthenia:-  1. Graduated Resistive exercises to antigravity muscles.  2, Endurance Ex  Gait training  1. Walk in parallel bar  2. Walk by a walker  3. Walk by crutches or canes to allow reciprocal movement of arms and legs with appropriate timing.  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.  Orofacial training: (Using a mirror)  Movement of laryngeal wall.  Tapping under jaw to stimulate swallowing.  Vibration to the inferior surface of tongue.  Application of ice on facial ms to gain relaxation.  Take a deep breath before he speaks in order to ↑ volume of sound