Form of communication

1. Non-verbal
a. Gestures
b. Facial expressions
c. Sign language
2. Verbal-prelinguistic
Type Definition Age range
1. Undifferentiated crying Cries without any reason Newborn
2. Differentiated crying Cries for a reason (i.e. hunger) 1 month
3. Cooing Incomprehensible sounds 1 ½ months
4. Babbling Vowels and consonants 3-4 months
5. Lalling Imperfect imitation of words 6 months
6. Echoing Perfect imitation by patient without 9-10 months
the patient knowing the meaning
7. Expressive jargon Creates sentence with 2/o
corresponding rhythm and pause
but is not understandable to the
listener

3. Linguistic
a. 2 words
b. Sentence form

Synergy pattern
1. UE flexor synergy
i. Scapular elevation and retraction
ii. Shoulder abduction and lateral rotation
iii. Elbow flexion
iv. Forearm supination
v. Wrist flexion
vi. Finger flexion and adduction
2. LE flexor synergy
i. Hip FABER
ii. Knee flexion
iii. Ankle dorsiflexion, inversion
iv. Foot inversion
v. Toe extension/dorsiflexion
3. UE mixed synergy
i. Scapular depression and protraction
ii. Shoulder adduction/IR
iii. Elbow flexion
iv. Forearm pronation
v. Wrist flexion
4. LE mixed synergy
i. Hip adduction, extension, and IR
ii. Knee extension
iii. Ankle plantarflexion
5. UE extension synergy
i. Scapular depression and protraction
ii. Shoulder adduction and IR
iii. Elbow extension, forearm pronation
iv. Wrist flexion
v. Finger flexion
6. LE extension synergy
i. Hip extension, adduction, and IR
ii. Knee extension
iii. Ankle plantarflexion, inversion
iv. Foot eversion
v. Toe flexion and adduction

Primitive Reflexes
Reflex Onset Integration Level
Extensor thrust Birth 0-2 Spinal
Moro Birth 0-4 AMR*

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Neck Righting Birth 0-6 Midbrain
ATNR Birth 4-6 Brainstem
STNR Birth 4-6 Brainstem
Foot placement Birth Persists AMR
Parachute 6 months Persists AMR
*AMR – Automatic Movement Reactions

A. Automatic Movement Reactions – These are described as a group of reflexes observed in infants and
young children which are not strictly righting reflexes, but which are reactions produced in the position
of the head, and hypothetically involve either the semicircular canals, labyrinths, or neck proprioceptors.
1. Moro Reflex
i. Patient is in a semi-reclined position
ii. Drop head backward
iii. (-) Is minimal or startle response
iv. (+) Is abduction, extension (or flexion), external rotation of arms, extension and
abduction of the fingers. Followed by cross adduction or crying.
2. Foot placement
i. Pt. is cradled on (B) axilla
ii. Brush dorsum of foot on edge of plinth
iii. (+) Is flexion of LE mimicking stepping reaction
3. Parachute reaction (Protective extensor thrust)
i. Pt. is prone or prone on a vestib ball, arms extended overhead
ii. Suspend patient in air by ankles or pelvis and move head suddenly towards floor
iii. (-) Is arms do not protect head but show primitive reflex reactions such as ATNR or
STNR
iv. (+) Is immediate extension of arms with abduction and extension of fingers to protect
head ((+) saving reaction)
B. Brainstem reflexes - These are static postural reflexes and effect changes in distribution of muscle tone
throughout the body, either in response to a change in position of head and body in space (by stimulation
of labyrinths), or in the head in relation to the body (by stimulation of proprioceptors of the neck
muscles). If these persist beyond 6 mos., indicative of delayed motor maturation of the CNS and results in
an apedal (prone, supine-lying) creature
1. Asymmetric tonic neck reflex
i. Pt. is supine c head in midline and extremities extended
ii. Turn head to one side
iii. (-) If no reaction of limbs on either side
iv. (+) If there is extension of arma dn leg on face side, or increase in extensor tone,
flexion of arm and leg on skull side or increase in flexor tone
2. Symmetric Tonic neck Reflex 1
i. Pt. in quadruped position or over tester’s knees
ii. Ventroflex the head
iii. (-) No change in tone of arms or legs
iv. (+) If arms flex or flexor tone dominates, legs extended or extensor tone dominates
3. Symmetric Tonic Neck Reflex 2
i. Pt. is in quadruped position or over tester’s knees
ii. Dorsiflex the head
iii. (-) No change in tone of arms or legs
iv. (+) Arms extend or extensor tone dominates; legs flex or flexor tone dominates
C. Midbrain Level – Righting reactions interact c each other and work toward establishment of normal
head and body relationship in space as well as in relation to each other. They enable the child to roll
over, sit up, get on his hands nd knees, and make him a quadrupedal creature.
1. Neck Righting Reflex
i. Pt. is in supine, head in mid-position c arms and legs extended
ii. Rotate head to one side actively or passively
iii. (-) Body will not rotate
iv. (+) Body rotates AS A WHOLE in the same direction as the head
D. Spinal Level – these are “phasic” or movement reflexes which coordinate muscles of the extremities in
patterns of either total flexion or extension. Complete domination results in apedal creature.
1. Extensor thrust
i. Pt. is in supine c head in mid-position; on leg extended, one flexed
ii. Stimulate sole of foot of flexed leg
iii. (-) Is controlled maintenance of leg in flexion
iv. (+) Is uncontrolled extension of stimulated leg

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 Special Test
1. Thomas Test – for iliopsoas
a. Pt. is in supine c (B) LE extended
b. Flex one LE in knee and hip (knee to chest)
c. (+) Is flexion or rising off of the plinth of the OPPOSITE knee
d. Indicates iliopsoas contracture of the opposite hip
e. Requires measurement of the angulation of the opposite hip
2. Staheli’s Test – for iliopsoas
a. Pt. is in prone c LE dangling on the edge of examining table
b. Raise or extend one hip keeping the knee straight
c. (+) Is anterior pelvic tilting of tested LE and indicates iliopsoas contracture of the
same hip
d. Requires measurement of the hip extension
3. Popliteal angle test – for hamstring
a. Pt. is in supine with leg to be tested in 90° hip and knee flexion
b. Slowly extend knee maintaining the 90° hip flexion
c. Measure for the value of knee extension
i. Axis = Lateral femoral condyle
ii. Proximal arm = greater trochanter
iii. Distal arm = lateral malleolus
d. Indicates contracture of the hamstrings of the tested leg
4. Silfverskiold Test – gastrocnemius and soleus
a. (+) Test indicates whether gastrocnemius or gastrocsoleus is contracted
b. Pt. is supine c tested leg in extension first. Passively dorsiflex the ankle for any LOM.
Then, flex the ipsilateral knee to 90° and passively dorsilfex the ankle and measure
for LOM
c. LOM of DF when in knee extension indicates gastrocsoleus contracture
d. Decrease in LOM of DF upon flexing knee of tested leg indicates gastrocnemius
contracture only
5. Ely’s Test – rectus femoris
a. Rectus femoris contracture/tightness
b. Prone c knees extended
c. Passively flex tested LE at knee so that heel is touching butt
d. (+) Is when ipsilateral hip flexes or raises off of the examining table
6. Phelp’s Gracilis Test – Adductor spasticity
a. Gracilis contracture
b. Prone c knees extended
c. Passively abduct B hips
d. Heel to buttocks then sudden knee extension
e. (+) crossing of (B) LE
7. Galleazi’s Test
a. LLD or hip dislocation or subluxation
b. Hooklying position
c. (+) When one knee is higher than the other

WeeFIM
1. Adaptation for FIM of adults
2. Assess and track development of functional independence in children c disabilities
3. Consists of 18 items c 6 domains
4. Used between 6 mos and 7y/o
WeeFIM Domains
Self-care
1. Eating
2. Grooming
3. Gathing
4. Dressing – upper body
5. Dressing – lower body
6. Toileting
Sphincter Control
7. Bladder management
8. Bowel management
Mobility
9. Transfer chair, w/c
10. Transfer toilet

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 11. Transfer tub
Locomotion
12. Crawl/walk/wheelchair
13. Stairs
Communication
14. Comprehension
15. Expression
Social Cognition
16. Social interaction
17. Problem solving
18. Memory

Levels of Function for the WeeFIM

No Helper
7 – Complete Independence (timely, safely)
6 – Modified independence (device needed)
Helper
Modified dependence
5 – Supervision
4 – Minimal assist (child =75%-99%)
3 – Moderate assist (child = 50% - 74%)
Complete dependence
2 – Maximal assistance (child = 25% -49%)
1 – Total assistance (child = 0%-24%)
Types of deformities seen in CP pt.:
A. Straphanger
 Scapular retraction
 Shoulder abduction and ER
 Forearm pronation
 Elbow, wrist and finger flexion
B. Birdwing:
 Scapular retraction
 Shoulder abduction and ER
 Forearm supination
 Elbow wrist and finger flexion
C. Windswept
 Hip abduction and ER of one LE
 Hip adduction and IR of opposite LE
D. Pithed frog
 Hip flexion, abduction and ER
 Knee flexion
 Equinus/equinovarus of ankle and foot
E. Frog leg
 Hip flexion, abduction and ER
 Knee flexion
 Ankle dorsiflexion
F. Talipes equinovarus (clubfoot)
 Talocalcaneal plantarflexion
 Subtalar inversion (supinated)
 Forefoot/midtarsal adduction
Eye deformities
A. Nystagmus
 Constant, involuntary cyclical movement of the eyeball
 Rhythmic, quick, oscillitatory, back and forth movement of the eyes
 Seen in CP ataxic patients
B. Strabismus
a. Eye deviation from N conjugate position
b. Loss of ocular alignment
c. CP spastic patients
C. Esotropia
a. Eye pulled inward, crossed eyes
D. Exotropia
a. One or (B) eyes turn outward
E. Hypertropia

4
 a. The visual axis of one eye is higher than the fellow fixating eye
b. The visual axis of one eye is lower than the fellow fixating eye
F. Hypotropia
a. Downward deviation of the visual axis of one eye
G. Poria
a. Unilateral deviation of (B) eyes
H. Homonymous hemianopia
a. Unilateral blindness of (B) eyes
DTRs
1. Biceps – C5, musculocutaneous nerve
2. Brachioradialis – C6, radial nerve
3. Triceps – C7, radial nerve
4. Patellar tendon – L3/L4, femoral nerve
5. Achilles tendon – S1, tibial nerve

Pathologic Reflexes
1. Babinski
a. Stroke the lateral aspect of the sole to the ball of the foot
b. (+) - Extension of big toe and fanning of four small toes
2. Chaddock’s
a. Stroke lateral side of foot beneath lateral malleolus
b. (+) – same as above
3. Oppenheim
a. Stroke anteromedial tibial surface
b. (+) – same response as Babinski
4. Gordon
a. Squeeze calf muscle firmly
b. (+) - same response as Babinski
5. Hoffman’s
a. Flick terminal phalanx of index , middle, or ring finger
b. (+) – flexion of distal phalanx

Grading for Tone Assessment

 0 – no response/flaccid
 1 – decreased response/hypotonic
 2 – normal response/normotonic
 3 – exaggerated response/mild to moderate hypertonia
 4 – sustained response/severe hypertonia

Modified Ashworth Scale for Spasticity Assessment

 0 – no increase in muscle tone
 1 – slight increase in muscle tone, manifested by a catch and release or by minimal resistance at the end
of ROM when the affected part/s is/are moved in flexion/extension
 1+ - Slight increase in muscle tone manifested by a catch, followed by minimal resistance throughout the
remainder (LESS THAN HALF) of ROM
 2 – more marked increase in muscle tone through most of the ROM, but affected part/s easily moved
 3 – considerable increase in muscle tone, passive movement difficult
 4 – affected parts rigid in flexion or extension

Balance Grading
 1 – cannot assume or maintain
 2 – can assume but not maintain or vice versa
 3 – can assume and maintain
 4 – can assume maintain, weight shift and be challenged

Tolerance grading
 Poor – 0-15 minutes
 Fair – 16-30 minutes
 Good – 31-45 minutes
 Normal – 46-60 minutes

RGR Grading
 Poor - (-)RGR, or (+)Reach with (-)Grasp & (-)Release

5
  Fair – (+)Reach, (+)Grasp, DIFFICULTY in Releasing or (+)Reach, DIFFICULTY in Grasp, DIFFICULTY in
Release
 Good – (+)/Complete RGR

Bleck’s 1-2 YEAR OLD prognostication for ambulation

 0 – Good chance for ambulation
o Absent primitive reflexes
 1 – may have a chance/guarded
 2 – poor prognostication

Molnar’s 2-7 YEAR OLD prognostication for ambulation

 Good
o Sits at 2
o Stands at 4
o No primitive reflexes by 18-24 months
 Fair
o Sits at 3
o Stands at 5
 Poor
o Sits at 4
o Stands at 6

FMT Grading
 0 – no ability
 1 – beginning ability, partially achieved, unreliable, insecure, momentary
 2 – reliable with abnormal pattern
 3 – reliably achieved, reliable with good pattern, efficient

Functional Muscle Testing (In order)

1. Head control
2. Trunk control
3. Rolling over
a. Supine to prone
b. Prone to supine
4. Prone on elbows
5. Creeping
6. Quadruped
7. Crawling
8. Sitting (assume)
9. Sitting B/T
10. Kneeling (assume)
11. Kneeling B/T
12. Half-kneeling (assume)
13. Half-kneeling B/T
14. Standing (assume)
15. Standing B/T

Factors That Differentiate Saving From Tilting

1. Speed of Stimulus
a. Saving : fast, tilting : slow
2. Direction of Response
a. Saving : toward the stimulus, tilting : opposite the stimulus
3. Part of the body which responds
a. Saving : upper extremities (extend), Tilting : trunk (arches toward the direction of the stimulus)

CP Triad
 Mental retardation (aka MR)
 Delayed milestones
 Drooling
Triad of Autism (EARLIEST SIGN: NO EYE CONTACT)
 Behavioral impairments
 Social impairments
 Communication impairments
Triad of MR (EARLIEST SIGN: TONGUE THRUSTING/MICROCEPHALY)

6
  Difficulty in acquisition of adapted skills
 Delayed motor development
 Low IQ level
Triad of hydrocephalus
 Crackpot sign
 Sunset eyes
 Cushing’s syndrome
Triad of kernicterus
 Sensorineural hearing loss
 Parinaud’s syndrome (loss of upward gaze)
 Athetosis

Head Circumference
Landmarks:
 Anterior: glabella
 Posterior: occiput

Time Frame Expected Growth Total

0-4 months 0.5 in/mo 2 in
5-12 months 0.25 in/mo 2 in
1-2 years 1 in/year 1 in
3-5 years 0.5 in/year 1.5 in
6-20 years 0.1 in/year 1.5 in

No increase in head circumference at 20 years old. At 20 years old, head circumference no longer increases, but
peaks at a value in between 52.32-58.32 cm.

Formula: x (2.54 cm/1in) ± 3 cm= y

 Where x=head circumference in inches and y=head circumference in centimeters.

The values in the table above are given in inches corresponding to the formula given above. The table below
contains values in centimeters.

Time Frame Expected Growth Total

0-4 months 0.5 in/mo, 1.27cm/mo 2 in, 5.08 cm
5-12 months 0.25 in/mo, 0.635 cm/mo 2 in, 5.08 cm
1-2 years 1 in/year, 2.54 cm/year 1 in, 2.54 cm
3-5 years 0.5 in/year, 1.27cm/year 1.5 in, 3.81cm
6-20 years 0.1 in/year, 0.254 cm/year 1.5 in, 3.81 cm

Formula: z ± 3 cm= y

CP ATHETOID
Athetoid CP/Dyskinetic CP
 Associated with damage to the basal ganglia in the form of lesions that occur during brain development
due to bilirubin encephalopathy and hypoxic ischemic brain injury
 Characterized by bilateral hypertonia and hypotonia due to inability to control muscle tone
 Clinical diagnosis occurs within 18 months of birth

General CP Types
 Spastic
 Dyskinetic
o Athetoid
o Choreoathetoid
o Ataxic
o Dystonic
 Mixed
o Spastic athetoid

Athetoid/dyskinetic CP
 Non-spastic extrapyramidal form of cerebral palsy
Other non-spastic CP
 Ataxic

7
  Dyskinetic
o Choreoathetoid
 Involuntary movements most predominantly found in face and hands
o Dystonic
 Slow, strong contractions which may occur locally or encompass the whole body
Epidemiology
 Pre-natal risk factors
o Prematurity
 Significant risk factor, predisposing to the development of perventricular
leukomalacia (PVL)
 <32 weeks = crucial for the development of surfactant
o Low birth weight
o Intrauterine infections
o Multiple gestations
o Pregnancy complications
 Peri-natal risk factors (10%)
o Birth asphyxia
o Complicated labor and delivery
 Post-natal risk factors
o Non-accidental injury
o Head trauma
o Meningitis/encephalitis
o Cardiopulmonary arrest
 Hypoxic Ischemic Brain Injury (HIBI)
o Form of cerebral hypoxia
 Oxygen cannot perfuse the cells in the brain
 1st cause of of athetoid dyskinetic cerebral palsy
 affectation to the putamen and thalamus caused by HIBI
 Bilirubin encephalopathy
o Also known as kernicterus or neuronal jaundice
o Most common (MC) etiology of CP athetoid
o Unconjugated hyperbilirubinemia (hemolytic disease)
o Risk factors include prematurity and low birth weight
o Main accumulation targets:
1. Basal ganglia
2. Ocular movement nucleus
3. Accoustic nuclei of the brainstem
o Increased bilirubin affinity to gray matter especially to:
1. Globus pallidus
2. Hippocampus
3. Subthalamic nucleus
o Bilirubin -> damages mitochondria
o Inhibits oxidative phosphorylation
o Causes calcium release -> apoptosis
o Stunts axonal and dendritic growth
o Excessive increase in bilirubin -> penetration of the blood brain barrier (BBB) which is
unstable in the neonate
o Mild hyperbilirubinemia
o (L) cognition impairment
o Severe hyperbilirubinemia -> athetoid dyskinetic cerebral palsy
o Bilirubin = waste product of RBC
o RBC life cycle (120 days)

RBC (p 120 days)

V
Macrophage
(phagocytosis)
V
< Hemoglobin >
V V
< Heme > Globin (main protein, conserved for later use)
V V
Biliverdin(free > Toxic bilirubin

8
bilirubin)
V V
Spleen (UB, most UB, Liver (UB)
graveyard of bilirubin)
V V
Glucoronic acid Glucoronic acid
V V
Glucoronyl transferase Glucoronyl transferase
V V
Binds with albumin Binds with albumin making it conjugated and H2O
(however doesn’t go soluble
into conversion & goes
back to circulation)
V V
Kidney Stomach
V V
Urobilinogen Stereobilinogen
V V
Urobilin (yellow color Stereobilin (brown color
of urine of stool)

UB -> Unconjugated bilirubin -> lipid soluble -> attracted to brain cells -> bilipid layer (lipid soluble)

Conjugated bilirubin ->H2O soluble -> Therefore returns to the circulation

Pathophysiology
Direct pathway -> Excitatory in nature (Khan Academy has an excellent video explaining both the direct and
indirect pathways.)

Cortex

Striatum (I) E

Globus pallidus internus (I)

(-) I

Thalamus (E)

*Causes movement

Indirect -> inhibitory; therefore no movement

Cortex

Striatum (I)

9
 I

Globus pallidus ext (I)

(-) E
I

Subthalamic nucleus (E)

Globus pallidus internus (I)

Thalamus (I)

ABO Compatibility
 Type of hemolytic disease in baby
 Occurs usually in first born
 Most common: mOther: type O; bABy: type A or B
During pregnancy -> mother’s blood and baby’s blood do not mix due to presence of placental membrane

Mixture is due to
 Miscarriage
 Trauma at birth
 Unknown cause

Formation of antibodies against type A and B

Penetrates placental membrane

Baby’s circulation

Destruction of baby RBC’s

Increase bilirubin

Rh incompatibility
 Develops when mom is Rh(-) and baby is Rh (+)
 RBC from unborn baby can cross into mother’s blood through placenta
 Mother’s immune system treats Rh(+) as foreigh bodies
 Mother makes antibodies
 First born not affected

Formation of antibodies against Rh(+) baby

10
 Able to penetrate placenta

Developing baby

RBC destruction

Increase bilirubin

Clinical Presentatino (Levitt)

1. Involuntary movements – athetosis
a. Bizarre, purposeless movements which may be uncontrollable
b. May be slow or fast; may be writhing, jerky, tremre, swiping or rotatory
patterns or they may be unpatterned
c. May be present at rest in some children
d. Involvement:
i. May be present in all parts of the body, including the face and
tongue
ii. Dyskinesia may only appear in hands or feet or in proximal
joints or in bilateral distal and proximal joints
iii. Difficulty in being still
e. Triggered by:
i. Heightened emotional state
ii. Excitement
iii. Any form of insecurity
iv. Tackle a mental problem
f. Decreased by:
i. Fatigue
ii. Fever
iii. Drowsiness
iv. Sleep
v. Prone lying
vi. Child’s attention being deeply held
g. Fluctuation of muscle tone
h. Abnormal in timing and directions
i. Spatial characteristics
j. Large motion of proximal joints
2. Posture
a. Unstable; dystonic spasms may throw child off balance
b. Inability to hold posture
c. Difficulty in holding on to an object
d. Abnormal tilt reactions
e. Abnormal standing posture -> compensation for instability
i. Backward lean with hip extension
ii. Lordosis and kyphosis with chin jutting well forward
3. Voluntary movements
a. Possible but with initial delay before the movement is begun
b. Lack of finer movements and weakness
c. Grasp and release have extreme flexion and extension movements which
some older children learn to control for finer grasp or use of large keys on
a computer
4. Athetoid dance

11
 a. Some athetoids are unable to maintain weight on their feet and continually
withdraw their feet either upwards or upwards and outwards
b. They may take weight on one foot whilst pawing or scraping the ground in
a withdrawal motion with the other leg
c. Conflict between grasp and withdrawal reflexes which may also be seen in
hands
5. Paralysis of gaze movements
a. Difficulty to look upwards
b. Difficulty in closing eyes voluntarily
c. Poor head control also disrupts use of the eyes
6. Type of speech: explosive speech
a. Difficulty controlling tongue, breathing and vocal cords
b. Irregular fluctuation of diaphragm
c. Feeding problems, drooling
Differential Diagnosis
1. Huntington’s Disease
a. An autosomal dominant inherited disease
b. Adult onset, most often
c. Death occurs 15-20 years after onset
d. Gene defect on CHROMOSOME 4 -> ENCODES HUNTINGTIN
e. M=F
f. Characteristic signs and symptoms (S/SX)
i. Choreiform movements
1. First appear as involuntary movements of the extremities and twitching of
the face (facial grimacing)
2. Later, patient becomes immobile and unable to speak or swallow
ii. Progressive dementia
1. Occurs with loss of memory and intellectual capacity
2. Sydenham’s Chorea (St. Vitus’ Dance)
a. Disease of childhood
b. Rapid, irregular, involuntary movements of the limbs, face, trunk
c. Associated with rheumatic fever

N Albumin= 3-4mg/dL
16-20 mg/dL = hyperbilirubinemia

Rhoggam
 Treatment for ABO incompatibility
 Administered as early as 5 months of 1st pregnancy and few weeks/days after first delivery
 Boosts up substance which will suppress production of antibodies by the mother to attack antigen’s of
baby’s RBC

CP ATAXIC
CP-> disorder of posture and movement secondary to a non-progressive lesion to the immature brain

Five hallmarks of CP
1. Abnormal pattern of tone
2. Abnormal pattern of posture
3. Abnormal patterns of movement
4. Abnormal patterns of reflexes
5. Delayed motor development

Ataxic CP
1. Affects cerebellum
2. Without order, incoordination
3. Characterized by clumsiness and instability

Epidemiology
 Male > Female of all races
 Pure ataxic: rarest form
o Less vascularized (decrease hemorrhage)
o Decreased affinity to bilirubin
o Located posteriorly
o Small part only

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Anatomy and Physiology
Cerebellum
 Posterior to the FOURTH VENTRICLE, separated from it by AQUEDUCT OF SYLVIUS
 Inferior to OCCIPITAL AND TEMPORAL LOBE, separated by TENTORIUM CEREBELLI
 Primary function
1. Regulation of movement
2. Postural control
3. Muscle tone
 Does not work alone and has connections to the brain and spinal cord
 Regulator of motor output

Layers of the Gray Matter of the Cerebellum

1. Molecular layer -> inhibitory external layer
2. Purkinje layer -> middle layer; controls output of deep cerebellar nuclei; functional unit of the
cerebellum
3. Granular layer -> internal layer; innermost

Lobes of Cerebellum
1. Anterior lobe/Paleocerebellum/Spinocerebellum (APS)
a. Static balance
b. Propulsive and stereotyped (repetitive movements)
c. Receive projections from spinocerebellar pathway
d. Part of motor execution
e. Modulate muscle tone
f. Control of actual execution of movements (corrects deviations or errors of intended movement
with the intended motor command)
2. Posterior lobe/Middle/Neocerebellum/Cerebrocerebellum (PoMiNece)
a. Largest
b. Dynamic balance, coordination
c. Motor planning and programming, preparation of movement
d. Muscle tone and posture
e. Programming the motor cortex for execution of the movement
f. Timing of movements
g. Receive projections from the basal ganglia and cortex
3. Flocculonodular lobe/Archicerebellum/Vestibulocerebellum (FAVe)
a. Oldest lobe
b. Balance and equilibrium, posture
c. Eye movements
d. Receive projections from vestibulocerebellar pathway
e. Controls axial muscles

Zones of the Cerebellum (Cerebellar Homunculus)

1. Vermis (Fastigial Nucleus)
a. Influences long axis of the body (neck, shoulders, thorax, abdomen, and hips)
b. Regulates extensor tone
2. Intermediate (Globose and emboliform)
a. Control muscle contractions of the distal parts of the upper and lower extremities especially
the hands/fingers and feet/toes
b. Direct limb placement and regulates agonist-antagonist muscle pairs to control relative timing,
amplitude, and trajectory of limb movements especially when more precision is required
3. Lateral (Dentate)
a. Bulk of the function of the cerebellum
b. Motor sequence and planning

Cerebellar Peduncles
1. Superior Cerebellar Peduncle (SCP)
a. Also known as the BRACHIUM CONJUNCTIVUM
b. Connects the cerebellum to the midbrain
c. Afferent impulses for LE
d. AFFERENT: VENTRAL SPINOCEREBELLAR TRACT
e. EFFERENT: SEND IMPULSE TO THALAMUS AND SPINAL CORD
2. Middle cerebellar peduncle (MCP)
a. Also known as the BRACHIUM PONTIS

13
 b. Connects the cerebellum to the pons
c. Send signals to LMN from pyramidal tract
d. AFFERENT: TO PONTOCEREBELLAR TRACT
3. Inferior cerebellar peduncle (ICP)
a. Also known as RESTIFORM BODY
b. Connects the cerebellum to the medulla oblongata
c. Receives all impulses from UE
d. AFFERENT: to
1. Spinal cord
2. Dorsal spinocerebellar tract
3. Oligocerebellar
4. Vestibulocerebellar tracts

Deep cerebellar nuclei (Intracerebellar Nuclei)

1. Dentate –largest, most lateral (SCP)
2. Emboliform –medial to the dentate (SCP)
3. Globose – consist of one or more rounded cell groups that lie medial to the emboliform (SCP)
4. Fastigial – near the midline, close to the roof of the 4th VENTRICLE
a. Larger than the globose
b. Inferior cerebellar peduncle

Cerebellar Afferent Fibers From Cerebral Cortex

1. Corticopontocerebellar (CPC)
a. Arise from nerve cells in all lobes of the cerebral cortex
b. Inhibitory on the same side
c. All lobes -> corona radiata -> pontine nuclei -> transverse fibers of the pons -> contralateral
cerebellar hemisphere -> motor cortex -> make exact copy of info -> send to CPC -> receive
info from golgi tendon organs, muscle spindles, smooth and coordinated movement
2. Cerebro-olivocerebellar (COC)
a. Excitatory
b. Arise from all lobes -> corona radiata -> internal capsule -> inferior olivary nuclei -> decussate
-> inferior cerebellar peduncle -> opposite hemispheres
3. Cerebroreticulocerebellar pathway (CRC)
a. Are from nerve cells from many areas of the cerebral cortex (particularly sensorimotor areas)
-> reticular formation (same side) -> Pons and medulla (opposite side) -> SCP and ICP ->
Cerebellar hemisphere (same side)

Cerebellar Afferent Fibers from Spinal Cord

1. Anterior Spinocerebellar Tract
a. Found at all segments of the spinal cord
b. Conveys normal joint information from muscle spindles, tendon organs, joint receptors of
upper extremity and lower extremity
2. Posterior/dorsal Spinocerebellar Tract
a. Receives muscle joint information from the muscle spindles, tendon organs and joint receptors
of the trunk and lower extremities
3. Cuneocerebellar Tract
a. Receives muscle joint information from the muscle spindles, tendon organs, and joint receptros
of upper extremity and upper thorax

Cerebellar Afferent Fibers From the Vestibular Nerve

1. Receives information from the inner ear concerning motion from the semicircular canals and position
relative to gravity from the utricle and saccule
2. Sends afferent fibers to cerebellum via ICP on the same side

Cerebellar Efferent Fibers

1. Globose-emboliform-rubral pathway
a. Influences ipsilateral motor activity
b. Globose and emboliform nuclei -> SCP -> cross to midline in the opposite side in the
decussation of SCP -> synapse with contralateral red nucleus -> gives rise to rubrospinal tract
c. Decussates twice
2. Dentothalamic Pathway
a. Ipsilateral motor activity

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 b. Dentate nucleus -> SCP -> cross the midline to the opposite side in synapse with contralateral
ventrolateral nucleus of the thalamus -> internal capsule -> corona radiata -> primary motor
area of corpus callosum -> corticospinal tract
3. Fastigial Vestibular Pathway
a. Ipsilateral extensor muscle tone
b. Fastigial nucleus -> ICP -> lateral vestibular nucleus on both sides -> vestibulospinal tract
4. Fastigial Reticular Pathway
a. Ipsilateral muscle tone
b. Fastigial nucleus -> ICP -> reticular formation -> reticulospinal tract

Etiology
CEREBELLAR DYSGENESIS -> incomplete/abnormal formation of the cerebellum due to infections or mother’s
exposure to toxins. The fetal brain is vulnerable in the first two trimesters.

Primary cause: Prematurity

 Hypoxia
 Prenatal stroke
o Malformed/weak blood vessel
o Increased blood pressure of mother during labor (PREECLAMPSIA)
 Lack of O2
o Ruptured/torn portion of uterus
o Decreased maternal blood pressure
 Toxin exposure
 Maternal and viral infections -> increase in inflammatory cytokines
 Head trauma
 Congenital hypoplasia -> small cerebellum at birth

Prenatal strokes
SICA: Superior of cerebellum (A&P) -> upper half of dorsal and ventral cerebellum
 Dysmetria of ipsilateral arm -> portion of vermis
 Unsteady walking -> upper portion of intermediate and lateral zone
 Dysarthria -> deep cerebellar nuclei
 Nystagmus -> SCP
AICA: Middle -> mid 10-30% of A&P of cerebellum
 Dysmetria -> Flocculus
 Facial sensory loss -> small portion of lateral hemisphere
 Vestibular sign -> MCP (Pons -> CN 5,6,7,8) and ICP
PICA: Vertigo
 Unsteadiness -> inferior half of A&P of cerebellum
 Walking ataxia -> flocculus, vermis, intermediate and lateral zones (Globose and emboliform nuclei)
 Nystagmus -> Deep cerebellar nuclei

Clinical presentation
Four main motor characteristics of CP ataxia
1. Nystagmus: vertical, horizontal (most common), pendular (quick and slow phase)
2. Hypotonia
3. Voluntary movements are clumsy
4. Disturbance of balance

Other manifestations
 Scanning speech (slow and monotonous)
 Dysdiadochokinesia (rapid alternating movements)
 Dysmetria (distance)
 Hyperextension of knees and elbows -> standing during walking
 High guard to mid guard
 Hypermobile joints
 Fluctuation of muscle tone (low to low normal tone) -> smallest fluctuation -> hypotonic
 Head titubation – vermis and orthostatic tremor
o Cerebellar hemisphere – distal limb ataxia, dysmetria, dysdiadochokinesia, kinetic tremor,
dystonia
o Flocculonodular lobe – gait and posture
 Nystagmus
 Vestibulo-ocular reflex disruption

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Region Vestibulo/archicerebellum Spino/paleocerebellum Cerebro/neocerebellum
Anatomical Site Flocculonodular lobe Anterior lobe Posterior lobe
Zones Flocculonodular lobe Vermis, intermediate Lateral hemisphere
Function Vestibulo Spino Cerebro
Origin Vestibular nuclei Anterior spinocerebellar Corticopontine fibers (DG &
tract/posterior OC)
spinocerebellar tract
Peduncle ICP ICP MCP
Input Mossy fibers Mossy fibers Mossy fibers
Deep Fastigial Globose and emboliform Dentate
Cerebellar
Nuclei
Output FV and FR Globose-emboliform-rubral DT
pathway
Function Balance, head and eye control Regulation of movement Motor planning, motor
(for error) learning
Lesion Nystagmus, disequilibrium, Scanning speech, ataxic gait, Dysarthria, incoordination in
truncal ataxia limb ataxia, dysmetria, muscle tone and posture
dysdiadochokinesia

0-7 years -> cerebral palsy

≥7 years -> not CP; TBI

Most Comon Causes of CP Ataxia by Age Group

<1year old
 Congenital malformation
 Hydrocephalus
 CP
1-3 years old
 Drug ingestion
 Brain tumors
 REFSUM’S DISEASE
8-9 years old
 CP ataxia
8-10 years old
 Drug ingestion
>10 years old
 Friedrich’s ataxia
 MS
 Hereditary

CP SPASTIC
Definition
 CP is a disorder of movement, control, and posture resulting from a non-progressive lesion to an
immature brain, occurring in untero near the time of deliver or within the first three years of life.
 CP is caused by a broad group of developmental, genetic, metabolic, ischemic, infectious, and other
etiologies that produce a common group of neurologic phenotypes
 a.k.a. “Little’s Disease”, infantile paralysis (pertains specifically to CP Spastic diplegia)
 Discovered by William Little
 Pyramidal lesions

Epidemiology
 Male more than female
 80% of all CP cases
 Pregnancy 75%, child birth 5%, birth 15%

Anatomy
The isocortex consists of up to six well-defined layers of cells
1. Molecular – outermost; contains nonspecific afferent fibers that come from
within the cortex or from the thalamus.
2. External granular layer -> dense layer composed of small cells.

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 3. External pyramidal layer -> contains pyramidal cells, frequently in row
formation
4. Internal granular layer -> usually a thin layer within cells similar to those
in the external granular layer, receive specific afferent from the thalamus
5. Internal pyramidal layer -> contains in most areas, pyramidal cells that are
fewer in number, but larger in size from those in the external pyramidal
layer
6. Fusiform (Multiform) layer -> consists of irregular fusiform cells whose
axons enter the adjacent white matter
Cerebrum -> Seat of many of the higher mental functions such as memory and learning, language, and conscious
perception
 Largest part of the brain and consists of two cerebral hemispheres which are connected by the corpus
callosum

Brodman’s area 4 (BA4, Primary motor cortex)

 Initiates/fires movement
 If affected, results in flaccidity
o Since BA4 cannot fire/initiate movement there is no tone (movement)
Brodman’s area 6 (BA6, Premotor cortex)
 Modulates/controls movement
 If affected, result in spasticity
o Since BA4 continues to fire whild BA6 cannot control it (since it lacks inhibitory
neurotransmitters) there is increased tone/spasticity
Pyramidal/Corticospinal tract -> influences motor activity, originates from cortex
Corticobulbar tract -> cranial nerves

Corticospinal tract starts:

Motor cortex (BA4 and 6)

Corona Radiata

Posterior limb of internal capsule

Middle 3/5 of basis pedunculi of the midbrain

Pons (Transverse pontocerebellar fibers)

Medulla oblongata (pyramids)

Cervicomedullary junction

Anterior Corticospinal Tract (10%) Lateral Corticospinal Tract (90%)

(Gross movements) (Fine movements)

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 Diencephalon -> Procencephalon -> Telencephalon -> both to c. hemisphere and cortex

Thalamus -> crucial structure for some types of sensation, pain, temp; regulatory
Subthalamus -> involved in the control of muscle activity
 Regulatory pathway for motor, sensory and reticular functions
Hypothalamus -> controls and integrates function of the autonomic nervous system and the endocrine system
 Maintains body homeostasis; reg. body temperature, drives to eat and drink, sexual
behavior and emotion
Epithalamus -> pineal gland: secretes hormones; circadian rhythm

Etiology
Prenatal factors (from conception to onset of labor):
 TORCH (toxoplasmosis, others, rubella, cytomegalovirus, herpes simplex)
 Intrauterine stroke (ischemic or hemorrhagic)
 Toxemia
 Genetic malformations
 Acquired during gestation
 Diabetes mellitus
 Seizures
 Mom’s age
 MR (mom)
 Multiple births
 Kernicterus
Perinatal factors (birth to fourth week)
 Mechanical in nature
 Fetal asphyxia
 Coiled cord
Postnatal factors (from 1 month-6y/o)
 Traumatic
 Infection
 Toxicity
 Vascular accident
 Cerebral anoxia
 Brain tumors
 Sickle cell anemia
 Congenital disease

Premature birth (prematurity) -> most common and well recognized risk factor for CP. The most common subtype
in premature infants is spastic diplegia.

The underlying brain lesion in premature infants classically manifests as a white matter abnormality on
neuroimaging.
 Consequences of both:
o Arterial hemorrhage from fragile capillaries in the watershed zone next to the lateral ventricle
known as the GERMINAL MATRIX
o Periventricular hemorrhagic infarction of venous origin

Between 25 and 32 weeks’ gestation -> highest risk for periventricular hemorrhage.

Grading of germinal matrix bleeding

 Grade 1 – subependymal and/or germinal matrix hemorrhage
 Grade 2 – subependymal hemorrhage with extension into the lateral ventricle WITHOUT ventricular
enlargement/dilation
 Grade 3 – Subependymal hemorrhage with extension into the lateral ventricles WITH ventricular
enlargment/dilatation
 Grade4 – germinal matrix hemorrhage that extends into the adjacent brain parenchyma, irrespective of
the presence or absence of intraventricular hemorrhage

Hemorrhagic infarction of venous origin in the periventricular region

 Asymmetric
 Located lateral to the external angle of the lateral ventricle
 Healing results in symmetric necrosis of the white matter adjacent to the lateral ventricle resulting in
periventricular leukomalacia (PVL)

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Pathophysiology
Why is PREMATURITY a PRIMARY RISK FACTOR?
 It is because of the physical stress on premature infants and the immaturity of the brain and cerebral
vasculature.
 Before term, the distribution of fetal circulation to the brain results in the tendency for hypoperfusion to
the periventricular white matter.

PREMATURITY

Immature/underdeveloped blood vessels of the brain

Decreased oxygen delivery to the brain.

Hypoperfusion to the periventricular white matter

Either/or
Germinal matrix hemorrhage Periventricular leukomalacia

Between 26th and 34th week of gestation

 The periventricular white matter areas near the lateral ventricles are the most susceptible to injury
 Periventricular white matter areas
o Carry fibers responsible for the motor control and muscle tone of the legs
o If affected, results to CP spastic diplegia

PRENATAL PERINATAL POSTNATAL

Immature/underdeveloped brain and Hypoxia Trauma/inf.
brain parts

Cytokine response (macrophage

release)
Ischemic attack

Kills oligodendrocytes (responsible

for formation of myelin sheathof
Necrosis nerve fibers in the CNS)

Neurologic lesion (cortex and

spinal cord)

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GABA absorption is compromised Partial/complete abnormal pattern Partial/complete abnormal pattern
(primary inhibitory of posture of movement of UE/LE
neurotransmitter)

HYPERTONICITY

SPASTIC CEREBRAL PALSY

Hypoxic-Ischemic Cerebral Injury

 The genesis of hypoxic-ischemic cerebral injury in most cases of CP is prenatal
 The timing of the insult is critical to the evolution of a specific lesion.
 Cerebral ischemia before the 20th wweek of gestation
o Neuronal migration deficit
 Between the 28th and 34th week
o Periventricular leukomalacia
 Between the 34th and 40th week
o Focal or multifactorial cerebral injury

5 Basic Subtypes of Hypoxic-ischemic neuropathology

1. Parasagittal cerebral injury
a. Involves bilateral cortical and adjacent subcortical white matter necrosis of the superior medial
and particularly the posterior aspects of the cerebral convexities
2. Periventricular leukomalacia
a. Occurs in the preterm infant involving bilateral white matter necrosis adjacent to the external
angles of the lateral ventricles affecting centrum semiovale and optic and acoustic radiations
3. Focal and multifocal ischemic brain necrosis
a. Injury to cellular elements caused by an infarcion within a vascular distribution
4. Status marmoratus
a. Nearest lesion and is characterized by injury within the Basal Ganglia
b. CP athetoid
5. Selective neuronal necrosis
a. Most common variety of injury observed in hypoxic-ischemic encephalopathy

Clinical Presentation
Topographical classification
1. Tetraplegia = UE + LE + head + trunk
2. Quadriplegia = all fours, UE+LE
3. Double hemiplegia = all fours, UE>LE
4. Diplegia = all fours LE>UE
5. Hemiplegia = one-sided affectation
6. Triplegia = 3 extremities, one UE + 2 LE
7. Monoplegia = isolated UE/LE involvement

Spastic Diplegia
 Good predictor sign of ambulation = sit by 2 y/o
 Poor prognosis: persistent infantile reflexes beyond 18 months
 Infantile coax valga & femoral anteversionn due to contractures of hip flexors and hip adductors
 Unilateral hip dislocation consequences: pelvic obliquity and scoliosis
 Scissoring gait
 Less reciprocal pattern in LE during mobility
 Combat roll
o Forward progression by reciprocal arm movements while pelvis and legs are dragged on the
floor
 Mermaid crawl due to existing windswept deformity
 Bottom shuffling
 Bunny hopping -> w-sitting
o LE are hiked along without alternating motion

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  Normal to near normal IQ
 Strabismus
 Tight adductors
Spastic Quadriplegia
 Pseudobulbar palsy – sucking, swallowing and chewing difficulties; drooling, dysarthric speech
 ¼ of the population never become independent and are dependent for carrying because of upper
extremity impairment, intellectual deficit, or both
 Highest rate of mental retardation in the rigid/atonic type
 Strabismus – increased rates in spastic diplegia and quadriplegia
 Straphanger or birdwing
 Windswept or pithed frog
 Mental retardation
 Strabismus
Spastic Hemiplegia
 Outlook for ambulation – walk by 3 y/o
 Unequal stride length and stance phase
 Triceps surae spasticity with diminishe or absent heel strike and ineffective toe clearance
 Most frequent complication – heel cord contracture and foot deformities (equinus or equinovarus)
 Cortical sensory deficit – unique feature when child tends to ignore hemiparetic arm
 Parietal damage – astereognosis, defective position sense, two-point discrimination, graphesthesia, and
topognosia
 Contractures of forearm pronators, elbow and wrist flexors, cortical thymb, finger flexion or
hyperextension of interphalangeal joints = spastic hand
 A mild nonstructural and nonprogressive scoliosis d/t unequal leg lengths, asymmetrical function, body
alignment
 Moderate IQ
 Homonymous hemianopsia
o Unilateral blindness
 Growth retardation
 Right aphasia

Characteristics
1. Tone abnormalities
a. Hypotonic age
i. More prolonged as a precursor of dyskinesia than of spasticity
ii. After, spasticity usually appears around 6 months
iii. Long lasting and significantly reduced tone tends to occur a rather severe motor
deficit in either type of neuromuscular dysfunction
iv. Persistent decrease in muscle tone = hypotonic CP
b. Hypertonicity
i. Opisthotonus = signals heightened extensor tone and may be interpreted by the
parents as precocious head raising and rolling over from prone to supine.
ii. Increase in stiffness when pulled to sit = hip extensor spasticity; one of the earliest
signs of spastic CP
Diagnostic Procedures
1. Cranial ultrasonography -> method of choice in neonates and infants to visualize hemorrhage and
hypoxic-ischemic results
2. CT Scan -> for detecting and localizing CNS lesions when brain damage is suspected
3. MRI -> can demonstrate small lesions missed by other methods and also abnormalities of myelination
associated with developmental delay.
4. Bayley Motor Scale -> at one year corrected ge was a highly sensitive indicator of all three diagnostic
categories

Mean age of clinical diagnosis

 Spastic diplegia = 12 ½ months
 Spastic hemiplegia = 21 months
 Spastic quadriplegia = 5 months

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