MANAGEMENT OF CEREBRAL PALSY

Definition

Cerebral palsy is a group of disorder of the development of movement and posture, causing activity limitation, that are attributed to non progressive disturbance that occurred in the developing brain . The motor disorder of CP  is  often accompanied by disturbance of sensation , cognition communication perception and/or by a seizure disorder.

(American academy of Cerebral palsy and development medicine)

Diagnosis

1.Delayed motor milestones

2.Abnormal muscle tone

3.Hyperreflexia

4.Absence of regression or evidence of a more specific diagnosis

 

1.Intervention never should be delayed awaiting diagnosis or etiological assessment

2 families do the best when informed up front that cerebral palsy is the possibility

Spasticity  Vs dystonia

Spasticity: Spasticity- velocity dependent increase in tonic stretch reflex with exaggerated tendon jerks.

Other motor phenomena of upper motor neuron: phasic stretch reflex (clonus and hyperreflexia),

flexor and extensor spasms,  co-contraction, dystonia

Dystonia:  involuntary muscles contraction that cause twisting ,repetitive movements and abnormal postures

SPASTICITY

—Present in most patient with Cerebral palsy (65 % )

—When reduced patients may :

– perform integrated muscle movement

– develop muscle strength

– function at a higher level

Measurement of spasticity

—Modified Ashworth scale

—GMFCS

1.Ambulatory in all settings

2.Walks without aides but has limitations in community settings

3.Walks with aides

4.Mobility requires wheelchair or adult assist

5.Dependant for mobility

—GMFCS

—GMFCS

—Motor impairment

1.primary: muscle tone, balance,  strength and selectivity problems that are directly related to damage in the CNS

2.Secondary:muscle contractures and deformity that develop over time in response to primary problem and musculoskeletal growth

3.tertiary:adaptive mechanism and coping responses that the child develops to adapt to the primary and secondary impairment.

Goals of Spasticity:  Management

—Decrease spasticity

—Improve functional ability and independence

—Decrease pain associated with spasticity

—Prevent or decrease incidence of contractures

—Improve ambulation

—Facilitate hygiene

—Ease rehabilitation procedures

—Save caregivers’ time

—Near full participitation in society

 

Treatment of spasticity and motor tone

—Physical therapy

—Orthotics

—Assistive devices

—Control of spasticity

—Orthopedic surgery

—Timing of rehabilitative measures

PHYSIOTHERAPY

—Conventional PT :

goal: bring child to erect posture, give independent mobility and prevent deformity

works peripheral on muscles, tendons, and ligaments

– Active exercises,

– Passive ROM exercises

– Passive stretching

– Bracing

CNS modifications :

applying external stimuli / effectiveness not proven

e.g.  – Neuro-developmental therapy

– Sensory integration therapy

– Patterning

– Conductive education

– Pressure point stimulation

 

NDT(NeuroDevelopment therapy)

—According to the Bobaths – the motor problems of cerebral palsy arise fundamentally from central nervous system (CNS) dysfunction which interferes with the development of normal postural control against gravity and impedes normal motor development.

Their goal was the establishment of normal motor development and function and/or the prevention of contractures and deformities.

—Effects of physiotherapy

—Maintenance Physical Therapy

Goals :

– balance and gait training

– maximize voluntary muscle usage

– prevent joint contractures

-essential after orthopedic procedures

and pubertal spurts.

ORTHOTICS

to hold extremity in stable and functional position.

1.Ankle foot orthosis :provide ankle stability, put foot I more functional position for gait & slow down contracture at ankles

2.Spinal orthosis: slow progression of scoliosis

PHARMACOTHERAPY

—Baclofen

—Diazepam

—Dantrolene Sodium

—Tizanidine

—Other

MECHANISM OF ACTION

Drug

Baclofen:

Diazepam:

Dantrolene Sodium:

Tizanidine:

Site of action

GABAb receptors in spinal cord

Central nervous system

Skeletal muscles beyond the myoneural junction

Central acting (spinal and supraspinal) at alpha2 – adrenergic receptor sites

BACLOFEN

GABA agonist – inhibits release of excitatory neurotransmitter at level of spinal cord

—Oral : mixed reports/ side effects/ not selective

—Continuous intrathecal – implantable pump

—Good results in releasing spasticity, and improving function

—Complications of pump and catheter

—Needs specialized centers

Botulinum-A toxin

Acts at myo-neural junctions

Inhibits exo cytosis of Acetylcholine

—Inject selected muscles at multiple sites

—Spasticity reduction may last up to 6 months

—Reversible , painless , minimal side effects

—Most patients still require lengthening for permanent correction

—Role :   – Facilitates physiotherapy and mobilization

– Delays surgical management

– Trial to determine effects of  specific

proposed surgical treatment

SELECTIVE DORSAL RIHZOTOMY[SRD]

Cut 30 – 50 % of abnormal dorsal rootlets  L2 – S1

—Followed by intensive physiotherapy

—Results encouraging

—May cause hyperlordosis / hip subluxation

—Best for : spastic diplegia, 4-8 yrs, no previous surgery, no contractures, no extra pyramidal signs

—? Not enough alone

—Orthopedic procedures obtain similar results

—Orthopedic procedures

Goal: 

ambulatory: straight spine, stable hip and horizontal pelvis and extended knee

non ambulatory: facilitate sitting ,prevent pain and improve hygiene

Depending on CNS  maturation ,ambulation potential and rate of deformity development

SOFT TISSUE PROCEDURES

—Reduce muscle tone by lengthing the spastic muscle and reducing stretch reflex

—Tenotomy

—Tendon lengthening

—Myotomy

—Tendon transfers

—Orthopedic procedures

1)Contracture management: soft tissue procedures

2)Joint dislocations: osteotomies , arthrodeses

3)Bony deformities

Timings:

Soft tisses procedures-4-7 yrs

Hand surgeries- 6-12yrs

Bony procedures-aft 8 yrs

—Hip surveillance

—Non ambulatory  and who can walk fewer than 10 steps hip xray 30 month

—Repeat every 6-12 month until 7 yrs

—Spinal radiographs 5 and 10 yrs

(dev med child neruo 2004)

Oromotor Dysfunction

—Especially common in persons with Extrapyramidal Cerebral palsy and Spastic quadriplegia

—Language delay/Speech delays

—Drooling

—Dysphagia

—Aspiration

Gastrointestinal Dysmotility

—Delayed gastric emptying

—Gastroesophageal reflux

—Pain

—Chronic aspiration

—Constipation

These disorders are interrelated and compound one another.

Associated problems

—Mental Retardation

—Communication Disorders

—Neurobehavioral

—Seizures

—Vision Disorders

—Hearing loss

—Somatosensation (skin sensation, body awareness)

—pain

—Temperature instability

—Nutrition

—Drooling

—Dentition problems

—Gastroesophageal reflux

—Dysphagia

—Autonomic dysfunctio