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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