CEREBELLAR ATAXIA

NITHIN NAIR

DIAGNOSTIC INVESTIGATIONS• Haematology: CBC, Creatinine, Liver

Enzymes, Electrophoresis, ESR, CRP, TFT, Vitamin B12, Cholestrol – helps to evaluate overall health and detect a range of disorders inculding infection and heavy metal poisoning

DIAGNOSTIC INVESTIGATIONS• Urine tests: Urine analysis may suggest –

systemic abnormalities related to some forms of ataxia. Wilson’s disease – screen 24 hrs urine collection for copper.

DIAGNOSTIC INVESTIGATIONS• Imaging studies: A computerized

tomography (CT) scan or Magnetic Resonance Imaging (MRI) of brain may help to determine potential causes.

DIAGNOSTIC INVESTIGATIONS• Genetic testing: It is done to determine

whether a patient, particularly a child has the gene mutation that causes one of the hereditary ataxic conditions. Chromosomal studies are performed on peripheral blood lymphocytes and cultured skin fibroblasts

DIAGNOSTIC INVESTIGATIONS• Brainstem Auditory Potential (BAEP or BAER):

Helpful to determine the presence of intact central pathways and may also provide some information about central (brainstem) projection pathways associated with hearing.

DIAGNOSTIC INVESTIGATIONS• Lumbar Puncture: CSF analysis is

helpful primarily to determine the presence of inflammatory diseases.

ASSESSMENT• HISTORY: History taking needs following consideration – Duration (Acute, subacute, chronic) Symmetry Rate of progression (static, episodic, progressive) Associated features (headache, vomiting,

dystonia/chorea, proprioceptive dysfunction, visual deficits, auditory involement)

Medical history (infection, medications/intoxications, environmental exposures)

Family history (suggest genetic disorder – autosomal recessive transmission/ autosomal dominant inheritence)

ASSESSMENT• ON OBSERVATION• PAIN ASSESSMENT• ON EXAMINATIONHIGHER CENTRES (COGNITION, MEMORY,

ORIENTATION, SPEECH, PROBLEM SOLVING)

CRANIAL NERVES (II, III, IV, VI, VIII)SENSORY ASSESSMENTMOTOR ASSESSMENT (TONE, REFLEXES,

ROM, MUSCLE POWER, VCA, TREMOR)

ASSESSMENT BALANCE (BBS, FUNCTIONAL REACH TEST, TIMED UP

& GO, FRIEDREICH ATAXIA RATING SCALE) GAIT (DGI, OBSERVATIONAL GAIT ANALYSIS-SPEED,

SYMMETRY, LEVEL OF INDEPENDENCE) CO-ORDINATION (EQUILIBRIUM, NON-EQUILIBRIUM) CARDIOVASCULAR (SUBMAXIMAL GRADED EXERCISE

TESTING) FATIGUE (MODIFIED FATIGUE IMPACT SCALE) FUNCTION AND DISABILITY (FIM, BARTHEL INDEX) SPECIFIC SCALES (INTERNATIONAL COOPERATIVE

ATAXIA RATING SCALE, SCALE FOR ASSESSMENT AND RATING OF ATAXIA)

TYPICAL CLINiCAL TEST• HYPOTONIA:

SPECIFIC TEST POSITIVEMUSCLE PALPATION Reduced firmness

PASSIVE SHAKING OF LIMBS

Moves through greater arc of motion

HOLD OBJECTS WHILE CONVERSING

Drops when distracted

VOL.FLEXION-EXTENSION OF KNEE OR ELBOW (SUPPORTED/UNSUPPORTED)

Ataxic when unsupported, controlled when supported

FLEX ONE FINGER ONLY All fingers flex

TYPICAL CLINiCAL TEST• ASTHENIA

SPECIFIC TEST POSITIVEMAINTAIN ARM IN 90° POSITION OF FLEXION OR ABDUCTION

Arm(s) tire quickly

MAXIMAL RESISTED MUSCLE CONTRACTION FOR MAJOR MUSCLE GROUPS

Weaker on involved side or unable to work against resistance

REPEATED SUBNMAXIMAL MUSCLE CONTRACTIONS –RISING ON TOES, PUSHUPS, SQUEEZING TENNIS BALL

Tires quickly

TYPICAL CLINiCAL TEST• BALANCE AND POSTURAL CONTROL

SPECIFIC TEST POSITIVEOBSERVATION-STANDING POSTURE

Feet apart, trunk flexed slightly, needs to hold for stability, postural tremor of legs

HOLD LIMB AGAINST PULL OF GRAVITY

Postural tremor

NUDGE CLIENT UNEXPECTEDLY WHEN SITTING OR STANDING

Loses balance

STAND ON ONE FOOT OR WALK BACKWARD

Loses balance

TYPICAL CLINiCAL TEST• DYSMETRIA

SPECIFIC TESTS POSITIVEFLEX ARMS TO 90°, QUICKLY ELEVATE OVERHEAD AND THEN RETURN TO 90° POSITION

Not able to resume 90° position without initial error

PUT PEG IN A HOLE, TRACE CIRCLE WITH PENCIL, TRACE CIRCLE ON FLOOR WITH GREAT TOE, SLIDE HEEL DOWN SHIN SLOWLY, PLACE FEET ON WALKERS WHEN WALKING

Intention tremor, undershoots or overshoots target

THERAPIST RESISTS CLIENT’S ELBOW FLEXION AND RELEASES UNEXPECTEDLY

Arm rebounds

TYPICAL CLINiCAL TEST

SPECIFIC TESTS POSITIVE

MARCH TO CADENCE Unable to follow rhythm

WALK ON HEELS OR TOES Loses balance and rhythm

WALK CLOCKWISE AND COUNTERWISE

Stumbles in one direction

WALK ON UNEVEN GROUND Cannot compensate and stumbles

OBSERVATION- TYPICAL GAIT PATTERN

Slow, stumbles easily, not rhythmical, step length irregular

GAIT DISTUBANCE

TYPICAL CLINiCAL TEST• DYSDIADOCHOKINESIA

SPECIFIC TESTS POSITIVE

TAP HAND ON KNEE OR TOES ON FLOOR

Rapidly loses rhythm and range

WALK AS FAST AS POSSIBLE

Gait become impaired when fast

OBSERVATION –ADL’S Unable to brush teeth, stir food...

TYPICAL CLINiCAL TEST• MOVEMENT DECOMPOSITION:

SPECIFIC TESTS POSITIVE

SUPINE- CLIENT TOUCHES HEEL TO OPPOSITE KNEE

Movement broken into separate phases – does not flow

OBSERVATION – TYPICAL MOVEMENT

Activity appears as if in a slow motion – mechanical like a puppet

TYPICAL CLINiCAL TEST• OCULOMOTOR PERFORMANCE

TYPES PROCEDURESMOOTH PURSUIT (MOVING TARGET)

Sit Head still Follow pen tip with ⇨ ⇨eyes Test in all movement planes ⇨and directions Vary speed.⇨

SACCADES(STATIONARY TARGET)

Verbally promted Client alternately ⇨fixes gaze on a pen tip and the examiner’s nose Vary target ⇨locations Observe for dysmetria⇨

GAZE EVOKED NYSTAGMUS Client maintains gaze in variety of locations including near end ranges of lateral gaze Observe for nystagmus ⇨

REFERENCE....Neurological Rehabilitation -

Darcy Umphred (5th and 6th edition)

Neurorehabilitation (Neurogen) – Dr. VC Jacob

Text book of Neurology – Navneet kumar