May 30, 2024

Cerebral palsy describes a group of permanent disorders of the development of movement and postures, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal and infant brain.

Most common Childhood motor disorder

Cerebral Palsy is accompanied by disturbances of sensation, perception, cognition, communication, and behavior

Etiology of Cerebral Palsy

  • Prematurity
  • Prolonged anoxia
  • Twin/ Triplet pregnancy
  • Congenital brain defect
  • Inborn errors of metabolism
  • Rh factor incompatibility
  • Post-natal causes: Encephalitis, convulsions and head trauma

Clinical Features

History of difficult birth/ illness during early months of pregnancy

Infants:

Late in motor activities (sitting, standing, walking, talking)

Expression less face, speech: difficult

Shoulder: Adducted + IR

Elbow: Flexed

Forearm: Pronated

Fingers: Flexed, thumb adducted in palm

Hip: Flexed, Adducted, IR

Knee: Flexed

Ankle: Plantar flexed

Foot: Equinovarus

Toes: Flexed

Children

The child must be supported while standing

Gait: Clumpsy + Uncoordinated

Lower extremities: Held tightly pressed together, cross in a scissor-like fashion (Heel cannot be brought down to the floor while standing)

Clonus: Seen, DTRs, Hyperactive

Primitive neutral reflexes are Positive

Other common impairments

Motor Impairment

Sensory Impairment: Hearing, tactile, proprioceptive, equilibrium input

Visual: Retinopathy of prematurity, visual cortex involvement, Squint

Cognitive Impairment: Higher brain function affected, Memory and thinking affected

Perpetual Impairment: Capacity to incorporate and interpret sensory and/or cognitive information affected

Communication Impairment: Expressive and/or receptive communication

Behavior Abnormality: Autistic spectrum disorder, ADHD, Sleep disturbances, Mood disorder, Anxiety disorder

Epilepsy: Scarring in the brain, nidus for convulsion

Classification

Area of Involvement

  1. Hemiplegia
  2. Diplegia
  3. Total body involvement

Predominant Neurological Features

  1. Spastic
  2. Dystonic
  3. Hypotonic
  4. Mixed

Motor Performance

Gross motor functional classification System (GMFCS)

Based on patient performance rather than its capacity ( Up to 18 years old)

Classified motor function on 5 points ordinal scale with the description of sills provided for five age group

<2, 2-4, 4-6, 6-12, 12-18

In general, levels are

Level 1Walks without limitation
Level 2Walks with limitation
Level 3Walks using handheld mobility devices
Level 4Self-mobility with limitation; may use powered mobility
Level 5Transport in a manual wheelchair

Factors responsible for abnormal Walking

  1. Neurological Factors
  2. Musculoskeletal Factors

Neurological Factors:

  • Tone abnormalities
  • Defective selective motor control
  • Equilibrium problems
  • Weakness
  • Sensory and perpetual problems

Area of the brain (Control movements)

  • Cerebral cortex: Spasticity
  • Basal Ganglion: Dyskinesia
  • Cerebellum: Ataxia

So children may not sit, stand, or walk without support

Problems in SMC=Clumpsy movements

Musculoskeletal Factors

4 Catagories

  1. Shortened muscles
  2. Elongated muscles
  3. Joint deformities
  4. Bony deformities

Shortened muscles

From birth to adulthood

In the Initial years, spastic muscle behaves short but has normal length when relaxed (Stage of dynamic shortening)

As time advanced, muscles are not adequately stretched and become further short (Stage of muscle contraction)

Advancement from dynamic to contraction depends upon the type of neurological involvement and amount of spasticity

Commonly affected are:

  • Adductors
  • Iliopsoas
  • Hamstring
  • Gastrocnemius
  • Soleus
  • Invertors
  • Peronei

Hampers in Gait and with passage time lead to additional problems like

  • Stretching out the antagonist’s muscles
  • Joint contractures
  • bony deformities (due to abnormal stress)

Elongated and stretched out muscles

When one group of muscle become short: Another group become long

Hamstring (short)—Knee extensors, quadriceps (long)

Long: Not able to exert enough force in the terminal range of movements

Eg. When Quardiceps become long–Knee cannot be extended fully

Results in knee extension lag and Knee Flexion gait

Joint deformities

The hip joint, Knee joint, Ankle, Foot

Hip: Subluxated/ dislocated: Unstable fulcrum

Knee: Flexion: Increase demands of quadriceps

Ankle/ Foot: Planovalgus: Decrease lever arm provided by foot to soleus

Difficult to remain upright in standing/walking

Bone deformities

  • Frontal Plane deformity
    • Valgus of proximal femur
    • Valgus of distal tibia
  • Axial plane deformity
    • Increase femoral anteversion
    • Increase external tibial torsion

Examinations

Examinations are carried out in 9 domains in Cerebral Palsy

  1. Assessment of SMC (Selective motor control)
  2. Strength examinations
  3. Muscle tone assessment
  4. Posture and balance
  5. Muscle length examination for shortening
  6. Muscle length examination for elongation
  7. Joint evaluation for deformities
  8. Assessment of torsional bone deformity
  9. Observation of gait analysis and gait parameters

Assessment of SMC

SMC: Ability to isolate the activation of muscle in a selected pattern in response to the demand of voluntary movement or posture

Normal SMC: Can carry out isolated joint movement without using mass flexors/extensors pattern or undesired movement at other joints

Grading:

Grade 0No ability
Grade 1Partial ability
Grade 2Complete ability to isolate movement

Motor Examination

Muscle length examination for shortening

Due to velocity-dependent spasticity of muscle: Examination should be slowly

[ Decrease anesthesia=> Accurate]

As dynamic contracture: Disappear =managed with botulinum toxin

Static contracture managed with surgery

The following muscles were assessed:

  • Hip flexors
  • Knee flexors
  • Ankle plantar flexors
  • Foor invertors /evertors

Hip Flexors:

Modified Thomas test:

The contralateral hip is flexed till the line joining ASIS and PSIS is vertical

The hip is to be assessed and is allowed to extend by gravity

The angle formed by the thigh and examination table

The angle of hip flexion deformity

Staheli test:

In prone position

Pelvis and lower extremity are off the examination table

Both Hip are flexed

One hand is at PSIS and examined the Hip is extended

Hip extended till the pelvis began to tilt posteriorly

The angle formed by the thigh and an imaginary line extending distally from the torso is a flexion deformity

Hip Abduction:

Hip extended position and knee in 90° flexion as well as in full extension

  • In 90° Knee flexion
    • Gracialis related
    • So, one joint adductor (Adductor longus, brevis, magnus)
    • So, if gracialis contracture is present [ More hip abduction in flexion than in extension]
  • In Extension of Knee
    • Gracialis: Tension (maximum stretch)
    • Two joint

Hamstring Length:

  • 2 joint muscles
  • By SLR
  • The knee is fully extended and the leg is slowly raised causing hip flexion

Popliteal Angle:

Cerebral palsy flexion deformity

Ipsilateral Hip flexed 90° and knee is maximally extended

Angle formed between the longitudinal axis of the leg and vertical extension of the longitudinal axis of femur = Popliteal Angle

Soleus Muscle length:

Assessed by ankle dorsiflexion with extended knee beyond 30°

Knee flexion ensure= relaxation of gastrocnemius muscle

Knee flexed beyond 30° and ankle passively dorsiflexed

If fully dorsiflexion is restricted = Soleus Contracture

Gastrocnemius Muscle length:

Ankle dorsiflexion with extended knee

Knee extended ankle passively dorsiflexed

If dorsiflexed is restricted

Contracture of gastrocnemius /Soleus

If dorsiflexion decreases on knee extension then flexion

Gastrocnemius is short

Combined test for gastrocnemius and soleus (Silfverskoid test)

Invertors/ Evertors length: assessed by taking foot into evertors and invertors

Muscle length examination from elongation

2 Clinical Signs

  • Extension lag
  • Patella Alta

Extension lag:

Supine position, Hip extended and knee flexed (90°)

Then asked to extend the knee joint

If fully extension is not possibly actively but possible passively

Quadriceps lag

(In hamstring contracture, knee flexion deformity cannot be assessed properly)

Patella Alta:

Elongated the patella tendon

Supine: Knee extended

The superior pole of the patella: 1 finger width proximal to the adductor tubercle

Joint Evaluation for deformities

Hip: Subluxed / DIslocate

Knee: Contracture (Posterior contracture)

Assessment of bone deformities

Bone deformity:

  • Femur and tibial torsion: Gait abnormality

Clinically:

  • Femur anteversion: Craig test
  • Tibial torsion: (Difficult to measure)

Bimalleolar axis:

The angle between the transverse plane between the tibial condyle axis and the line joining malleoli

Extended Knee rotated until medial and lateral femoral condyle are horizontal (B)

The line between bimalleolar axis (A)

(A) and (B) angle: tibial torsion

Investigations

X-Rays:

  • Pelvis with Hip -AP
  • Knee – Lateral (Patella Alta)
  • Ankle -AP – Valgus at distal tibia

CT Scan:

  • Torsional abnormality femur/tibia

Treatment Options for Cerebral Palsy

Non Operative

Many specialists

  • Physical and Occupational therapies
  • Speech therapies
  • Convulsive disorder : Anticonvulsive medication
  • Antispasmodic medication: Baclofen/ Diazepam

Botulinum Neurotoxins

Intramuscular toxin A: Spastic muscles:- Injected

MOA:

Blocks release of acetylcholine from neuromuscular end plates

Causing flaccid paralysis of the muscle

Functional improvement is seen in younger children; where muscles tone is the dominant problem

Mostly:- Improvement is temporary

Delays the surgical intervention in walking child

Used before lengthening – of hamstring – to predict the effect of hamstring surgery

Also used to decrease postoperative pain after multilevel surgery

Total dose: Depends upon the number of muscles to be injected and on body weight

Combined with casting/ physiotherapy to stretch the shortened muscle

Muscle activity recovers after about 3 months of the development of a new NM junction

Orthopedics Interventions

  • To improve motor function
  • To prevent deterioration

Orthosis:

Ankle Foot Orthosis (AFO)

  • Dorsiflexor weakness
  • Calf muscle overactivity
  • Plantar flexor overactivity
  • Valgus/ Varus deformity at subtalar joint
  • Plantar flexor weakness

Knee Ankle Foot Orthosis (KAFO)

  • Stretches short hamstring and correct knee contractures

Other Orthosis

Surgical Options

Different for ambulatory ( Improve motor function) and nonambulatory (to improve sitting and standing, to facilitate perineal care and transfer, control pain)

Surgery for Nonambulatory

Goals:

  • Painfree, balancing seat, and easy transfers

Spinal deformities

Scoliosis

Principle for neuromuscular scoliosis (interferes with sitting)

Spinal orthosis: To retard the progression

Surgical Stabilization: When sitting is not possible in spite of modification of wheelchair

Hip deformities

Windswept deformity (one hip adducted and another abducted)-Interferes with perineal care

Hip dislocations

If detected early: managed with adductor and hip flexor lengthening

Hip abduction orthosis and botulinum injection

Not effective in correcting or preventing hip dysplasia

Treatment for hip disorder for

  • Instability
  • Pain
  • Restriction of motion

In child < 5 years:

  • Lateral displacement of Head: Managed by adductor longus, intramuscular tenotomy
  • Gracialis tenotomy
  • Psoas tenotomy

Proximal femoral osteotomy + Supplemented with acetabular osteotomy

Knee deformities

In non-ambulatory child: Knee flexion deformity does not interfere with function:- So one should not be aggressive to correct

Foot and ankle deformities

Non aggressively treatment

Unless it interferes with standing:- transfer surgeries

Surgeries for ambulatory children

Aim:

  • To improve in walking
  • Short muscle is lengthened
  • Long muscle is shortened
  • Bone deformity – Osteotomy
  • Joint contracture – Corrected

Decision for surgery is based upon clinical evaluation

The Hip

Scissoring: Adduction contracture manifested by walking with knees touching each other

Intramuscular lengthening of adductor longus and gracialis myotomy

Flexion overactivity: Does not allow full extension of hip (compensated by lumbar lordosis):

Which is treated by Psoas Muscle lengthening

Improves hip flexion/ extension but is associated with hip flexor weakness (Important for the patient for CP)

So, only indicated in selected cases

Excessive femoral anteversion (Child walks with in-toeing gait)

Corrected by osteotomies (Subtrochanteric, diaphyseal, and supracondylar )

The site is chosen with the concomitant procedure

  • Knee flexion contracture
  • Neck-Shaft angle

Osteotomy is carried out after 8 years (as femoral torsion does not significantly change after 8 years)

Recognize tibial torsion:- If present corrected at the same time

Dislocated /Subluxed hip joint

Shortened femur enough to reduce pressure within joint; Avoid femoral head necrosis than the length

Unilateral LLD can be corrected by contralateral knee (Epiphysiodesis)

The Knee

Flexion and extension deformity of the knee is due to spasticity and/or contracture of the hamstring

If not treated early, it may lead to capsular fibrosis/ contracture

Short hamstring are corrected by lengthening of the medial hamstring

Knee contracture:

Mild: Serial plaster cast/ Wedging of the cast after hamstring release

Severe: Distal femoral extension osteotomy

Child walking with Crouch Gait

It May have elongated the patellar tendon

Corrected by

  • Shortening of the patella tendon
  • Distal transfer of tibialis tuberosity of tibial apophysis has closed

Cospasticity of hamstring and rectus femoris :

Interferes with knee flexion during swing phase

Treatment:

Addressed by transfer of the distal part of the rectus femoris: Sartorius, semitendinous, or iliotibial band

Increasing the dynamic range of motion by about 15-20 degrees is carried out in conjunction with functional hamstring lengthening

Knee Hyperextension can be because of spasticity of the soleus (Which does not allow adequate dorsiflexion):- treatment should be concentrated on the calf (Not the knee)

Foot and Ankle

Euinus by spasticity of gastrocnemius and soleus

The aim is to achieve: Plantigrade foot (Stance phase)

Avoid foot drop in swing phase / Stability

Mild deformity:

Ankle Foot Orthosis

If not achieved = Botulinum toxins with /without stretching cast

Severe deformities

Surgery but calf muscle function is very important for various activities, So caution in lengthening soleus (power may reduce)

Which increases the possibility of crouch gait

Strayer’s Procedure

It is considered to lengthen gastrocnemius only and acceptance of some residual deformities (equinus) which is compensated by raising of the heel

In hemiplegic where both soleus and gastrocnemius are contracted: Achilles tendon lengthening is permissible (Z- lengthening)

Pes Varus

Spasticity of investors also associated with gastrocnemius and soleus spasticity

Mild cases: Orthosis

Moderate cases: Lengthening of calf muscles and tibialis posterior

Severe cases: Soft tissue procedure first then bony procedure later

Pes Valgus

Spasticity of peroneal muscle with or without gastrocnemius and soleus spasm

Mild cases: Lengthening of evertors and calf muscles + Orthosis

Moderate cases: Lateral column; lengthening to correct the eversion of subtalar joint (Osteotomy at anterior calcaneal)

Distracted: Result in lengthening of the lateral column

Severe cases:: Triple fusion for correction

Management Options (Surgical):

Summary of the surgical management of the patient with Cerebral Palsy

External Tibial torsionDerotational osteotomy
Planovalgus footLateral column lengthening
Instability of midfootFusion of calcaneocuboid joint
Hindfoot valgusNot corrected with lateral column lengthening
Needs medial displacement osteotomy
Flexion of kneeCapsulectomy or temporary anterior epiphysiodesis
Limited growth potential: Supra condylar osteotomy
Elongated Patellar tendonPlication of the patellar tendon
Distal transfer of tibial tuberosity
Short muscleLengthened
Weak MusclesStrengthening exercises