CEREBRAL PALSY

•	Definition.
•	Etiology.
•	Classification.
•	Associated problems.
•	History.
•	Clinical examination.
•	Management.

Definition

1.	Cerebral palsy is a term used to describe a spectrum of diseases characterized by:

a.	Brain damage.

b.	Occurring antenatally, perinatally or during the first few years of life, resulting in

c.	Non-progressive disease characterized predominantly by

d.	motor dysfunction or posture problems.

2.	Prevalence

a.	2 – 2.5 / 1000 live births.

b.	8 – 10% of very premature babies.

Etiology

1.	Antenatal

a.	Fetal:

i.	TORCH infections.

ii.	cerebral malformations.

iii.	fetal coagulation and autoimmune disorders.

iv.	chromosomal abnormalities.

v.	CVA.

b.	Maternal:

i.	diabetes mellitus.

ii.	toxoplasmosis.

c.	Placental: placental insufficiency. 

2.	Perinatal

a.	Prematurity.

b.	Hypoxia.

c.	Hypoglycaemia.

d.	Intrapartum infection. 

e.	Intracranial haemorrhage.

3.	Postnatal:

a.	Kernicterus.

b.	Trauma.

c.	CVA.

d.	Postnatal intracranial infections.

4.	Criteria have been developed defining acute intrapartum events sufficient to cause permanent neurological impairment:

a.	Evidence of metabolic acidosis in fetal umbilical arterial cord or early neonatal blood (pH < 7.00; base deficit > 12 mmol/L).

b.	Early onset of moderate to severe neonatal encephalopathy in infants 34 weeks’ gestation or over.

c.	Splastic quadriplegic or dyskinetic types of CP.

5.	CP types and causes

a.	Spastic hemiplegia: cerebrovascular occlusion.

b.	Spastic diplegia: prematurity.

c.	Choreoathetotic: kernicterus.

Classification

1.	Types of CP

a.	Spastic (70%): 

i.	quadriplegia.

ii.	hemiplegia.

iii.	diplegia.

b.	Dyskinetic (15%):

i.	choreoathethoid.

ii.	dystonic.

c.	Ataxic (15%).

d.	Mixed (10%).

e.	Hypotonic: early stages of spastic, dyskinetic and ataxic forms.

2.	Spastic CP

a.	Most common type of CP: 70%.

b.	Clinical features are those of pyramidal realease:

i.	posture.

ii.	tone.

iii.	deep tendon reflexes.

iv.	contractures.

c.	Pattern of involvement: hemi-, di- or quadriplegia.

d.	Persistence of primitive reflexes.

e.	Pseudobulbar palsy with dysfunctional swallowing.

3.	Spastic hemiplegic CP

a.	Clinical features are those of pyramidal release involving one side of the body:

i.	posture and gait.

ii.	tone and deep tendon reflexes.

iii.	contractures and deformities.

iv.	wasting of affected limbs.

b.	Note any facial muscle weakness:

i.	cortical sensory loss.

ii.	visual field defect.

iii.	speech defects.

c.	Posture:

i.	shoulders: adducted + internally rotated.

ii.	elbows: flexed + pronated.

iii.	wrists: flexed.

iv.	fingers: thumb adducted, fingers clenched over thumb (spontaneous Waternburg’s sign).

v.	hips: adducted + internally rotated + extended; scissoring on ventral suspension.

vi.	knees: extended.

vii.	ankles: plantar-flexed + talipes equino varus.

d.	Gait: circumduction of affected leg, ankle plantar-flexed and enhanced by running.

e.	Contractures and deformities:

i.	heel cord contractures.

ii.	scoliosis.

iii.	flexor deformities of elbow and wrist with pronation of forearm and ulnar deviation of wrist.

iv.	adduction of thumb.

v.	scoliosis of spine due to spastic contraction of flexors of trunk on affected side.

vi.	equinovarus and valgus of ankle with shortening of Achilles tendon.

f.	Neurological assessment:

i.	reflex hyperexcitability: increased tone, reflexes and clonus.

ii.	wasting of paretic extremities.

iii.	homonynous hemianopia.

iv.	UMN facial muscle weakness on one side.

v.	hemisensory deficit on one side.

vi.	loss of cortical sensation: stereognosis, 2-point discrimination, etc.

g.	Developmental assessment: abnormal persistence of neonatal reflexes. 

4.	Spastic diplegic CP

a.	Spasticity of LL >> UL.

b.	Posture:

i.	hip and knee flexion.

ii.	hip adduction and inward rotation of legs.

iii.	equino varus / valgus of feet.

iv.	kyphosis of dorsal spine.

v.	lordosis of lumbar spine.

c.	Normal IQ.

d.	Normal speech.

e.	Associated with low birth weight and prematurity.

5.	Dyskinetic CP

a.	Hypotonia in early infancy with hypermotility of joints.

b.	Later in life:

i.	dystonia: fluctuations of muscle tone with intermittent spasms.

ii.	athetosis: slow, alternating rhythmic movements, e.g. flex & extend, supinate & pronate.

iii.	chorea: irregular, jerking and writhing movements, e.g. of tongue, face, shoulders.

c.	These involuntary movements increase during stress.

d.	Speech abnormalities: dysarthria.

e.	Hearing impaired.

f.	Mild mental deficit.

g.	Due to kernicterus.

6.	Ataxic CP

a.	Rare form of CP, but commonest cause of floppy infant.

b.	Hypotonic and hyporeflexic in infancy.

c.	Later on:

i.	ataxic gait.

ii.	tremors: intentional and incoordination.

iii.	nystagmus is uncommon.

d.	Mild mental deficit.

Associated medical problems in CP

1.	Seizures

a.	25 – 35% of all CP.

b.	Commonest in spastic tetraplegia followed by hemiplegic.

2.	Mental retardation

a.	Range from 25 – 75% of all CP.

b.	Mixed CP most retarded followed by spastic quadraplegics.

c.	Dyskinetic and ataxic CPs are quite normal.

3.	Speech retardation

a.	Occurs in more than 50% of CPs.

b.	Due to:

i.	mental retardation.

ii.	expression aphasia.

iii.	poor coordination.

iv.	dysarthria.

4.	Visual abnormalities

a.	Strabismus.

b.	Refractive error.

c.	Visual field defects.

d.	Blindness.

5.	Deafness.

6.	Psychological:

a.	Behavior.

b.	School problems.

History

1.	Presenting complaint: reason for current admission.

2.	Overall assessment

a.	Type of CP.

b.	Intellectual ability.

c.	Functional ability.

d.	Associated medical problems.

e.	Associated social problems.

3.	Intellectual ability

a.	Mental status: 

i.	evidence of mental retardation.

ii.	current placement regarding education, domestic, employment.

b.	Vision, speech and hearing.

4.	Functional ability

a.	Activities of daily living.

b.	Feeding and nutrition:

i.	sucking and swallowing ability.

ii.	tube feeds.

iii.	gastrostomy.

iv.	gastro-esophageal reflux.

v.	failure to thrive.

vi.	aspiration.

c.	Mobility:

i.	degree of ambulation.

ii.	use of walking aids, wheelchairs.

iii.	skeletal problems: contractures, kyphoscoliosis.

iv.	abnormal posturing.

5.	Associated medical problems

a.	Seizures:

i.	type.

ii.	duration.

iii.	frequency.

iv.	usual treatment including side-effects, compliance and drug levels.

v.	last seizure.

b.	Urinary incontinence.

c.	Constipation.

d.	Management of menses.

e.	Chest infections.

f.	Pressure sores. 

6.	Birth and developmental history

a.	Maternal past history of miscarriages or infertility.

b.	Pregnancy:

i.	hypertensive disease of pregnancy.

ii.	teratogenic medications.

iii.	placental problems.

iv.	clinical evidence of, or exposure to, infection.

c.	Delivery:

i.	presentation.

ii.	instrumental delivery.

iii.	Apgar score.

iv.	resuscitation required.

v.	birth weight.

vi.	need for oxygen, nursery care.

d.	Neonatal period:

i.	respiratory distress.

ii.	feeding difficulties.

iii.	seizures.

iv.	hyperbilirubinaemia.

v.	intraventricular haemorrhage.

vi.	hydrocephalus.

vii.	retinopathy of prematurity.

e.	Developmental history: age at which milestones achieved.

7.	Management

a.	Recent management in hospital.

b.	Usual treatment at home.

c.	Daily routine.

d.	Frequency of therapies: physiotherapy, occupational and speech therapies.

e.	Usual doctors seen.

f.	Compliance with treatment.

8.	Social history

a.	Schooling.

b.	Impact on family.

c.	Social support available. 

d.	Primary care-giver: understanding and possible neglect. 

Clinical examination

1.	Introduction

a.	Describe overall picture of patient.

b.	e.g. A profoundly intellectually handicapped microcephalic girl with spastic quadriplegia.

2.	General observations

a.	Dysmorphic features.

b.	Parameters:

i.	often obvious microcephaly with sunken temples.

ii.	height and weight; plot values on progressive percentile charts.

c.	UL Posture:

i.	opisthotonus: arching of back.

ii.	shoulders: adducted and internally rotated.

iii.	elbow: extended / flexed; pronated.

iv.	wrist flexed.

v.	clenched fist.

d.	LL posture:

i.	hip: adducted, internally rotated, extended; scissoring of legs.

ii.	knees: extended.

iii.	ankle: plantar flexed.

iv.	foot: talipes equino varus.

e.	Movements:

i.	paucity.

ii.	involuntary.

iii.	which limb is moving.

iv.	muscle atrophy of extremities.

f.	Behavior: lack of interaction with environment.

g.	Eye signs: squint, nystagmus.

h.	Bulbar signs:

i.	dysarthria.

ii.	drooling.

I.	Interventions:

i.	nasogastric tubes.

ii.	gastrostomy tube.

iii.	scars of orthopaedic procedures.

j.	Clothing: nappies in older child.

k.	Peripheral aids: wheelchair, splints, orthoses.

3.	Neurological assessment of motor function

a.	Inspect carefully for:

i.	muscle bulk, atrophy.

ii.	fasciculations.

b.	Palpate muscle bulk in each group.

c.	Tone: hypertonic / clasp-knife rigidity.

d.	Reflexes:

i.	hyper-reflexia with adductor reflex and cross extensor reflex.

ii.	up-going plantar reflex.

iii.	sustained clonus.

e.	Power: 

i.	voluntary movement.

ii.	functional power.

f.	Gait:

i.	perform a standard gait examination.

ii.	if the child cannot walk, but can crawl, look for abnormal crawling. 

4.	Cranial nerves

a.	Ocular muscles: any squint, paralysis.

b.	Jaw jerk: increased implies lesion above brainstem.

c.	Facial asymmetry.

d.	Hearing.

e.	Swallowing.

5.	Development assessment

6.	Gross motor ‘180 degree manoeuvre’

a.	Lying supine: assess position adopted, e.g. ATNR.

b.	Pull to sit by hands: to assess head lag, and grasp.

c.	Sitting: assess sitting ability, then lateral propping.

d.	Hold up vertically, under axillae: to detect increased extensor tone, scissoring, automatic walking.

e.	Tilt side ways: to assess head righting.

f.	Ventral suspension: to detect excessive extensor tone.

g.	Parachute reflex: to detect asymmetry.

h.	Place prone: to detect back arching.

7.	Assess complications

a.	Neurological:

i.	vision: visual fields and extraocular movements.

ii.	hearing: for sensorineural deafness.

iii.	ears: for chronic serous otitis media.

iv.	gag reflex: for bulbar dysfunction.

b.	Dental problems:

i.	caries.

ii.	enamel hypoplasia.

iii.	peridontal disease.

iv.	malocclusion.

c.	Orthopaedic problems:

i.	back: kyphoscoliosis.

ii.	contractures and deformities.

iii.	hips: dislocation.

iv.	bed sores.

d.	Neglect

i.	nutrition screen for malnutrition.

ii.	auscultate chest for aspiration pneumonia.

iii.	palpate abdomen for constipation.

iv.	child abuse. 

v.	state of interventions, e.g. gastrostomy, NG tube.

e.	Functional assessment for activities of daily living.

8.	Assess possible etiology

a.	Spastic diplegia: prematurity.

b.	Spastic hemiplegia: CVA.

c.	Dyskinetic: kernicterus.

d.	Exclude myelomeningocoele / encephalocele.

e.	Exclude hepatosplenomegaly: metabolic causes.

f.	Exclude cardiomegaly: Pompe’s disease.

9.	Summary

a.	Diagnostic features suggestive of CP in the patient: this X year old boy has CP of X type as evident by the following features...

b.	Development assessment:

i.	this child is globally delayed because for his age.....

ii.	for a X years old child, I would expect.....

c.	Complications: in addition, his condition is complicated by....

d.	I would like to follow up with the following investigations:

i.	MRI / CT scan: may show the basis of CP or unexpected degenerative disorders.

ii.	TORCH screen in infants for intrauterine infection.

iii.	urinary metabolic screen for various degenerative conditions.

iv.	karyotyping for chromosomal anomalies. 

Management

1.	Overview

a.	A child with CP is a management problem because the diagnosis is easy to make.

b.	The aim in management is to ensure that the child develops into his / her full physical, mental and social capabilities.

c.	This is achieved by a total and multidisciplinary approach involving:

i.	minimizing disabilities and handicaps: physiotherapist, occupational therapist, orthopaedic surgeons, speech therapist.

ii.	education: parents, teachers, social worker.

iii.	treatment of concomitant medical complications: paediatricians, GP.

2.	Minimizing disabilities and handicaps

a.	In CP, 2 phases of motor disability are recognized:

i.	early phase: retardation / arrest of motor development.

ii.	static phase: abnormal pattern of posture and movements in which irreversible structural alterations have already occurred.

b.	According to which phase the patient presents in:

i.	early phase: employ neuro-developmental techniques.

ii.	static phase: treat along orthopaedic lines.

iii.	subsequently, give physiotherapy and speech therapy to both groups.

iv.	occupational therapy is given should any specific problems arise. 

3.	Neurodevelopmental techniques

a.	Preferred method for early phase before condition becomes static and irreversible.

b.	In the early phase the problem lies in the abnormal coordination of muscle action not related to any muscle weakness / paralysis.

c.	The principle in treatment is:

i.	inhibition of abnormal and

ii.	facilitation of normal patterns of posture and movements.

d.	Steps:

i.	inhibition of abnormal movements combined with facilitation of normal movements.

ii.	guide motor output to therapist sensory input.

iii.	gradually and systematically withdraw help so that the child learns to control his movements unaided. 

e.	At this stage, aim to prepare child for functional skills in ADL.

4.	Orthopaedic surgery

a.	Preferred method when condition has already reached the static and irreversible phase.

b.	Principles in treatment:

i.	decrease disability and complications.

ii.	increase residual motor functions.

c.	Correction of:

i.	contractures: tenotomy.

ii.	deformities from muscle imbalance: tendon transfer.

iii.	functional handicaps of hands and feet: arthrodesing operations.

5.	Physiotherapy

a.	Aim to remind patients of what they can do.

b.	Principles in treatment:

i.	reduce effect of abnormal muscle habits.

ii.	encourages fundamental motions and functions of the extremities.

c.	Consists of:

i.	specific exercise: feeding, grasping.

ii.	functional training in sitting, standing and walking.

iii.	gait training.

d.	Mechanical aids:

i.	to prevent and correct deformities for support.

ii.	to control involuntary movements to assist in wanted motions.

6.	Speech therapy

a.	Plays a role in:

i	education.

ii.	ocupation.

iii.	self – esteem and emotional well-being.

b.	Involves:

i.	stimulation of language development.

ii.	communication skills.

iii.	articulation.

7.	Occupational therapy

a.	Aim to improve muscle and joint function by training functional patterns and functional skills to ultimately help them gain independence.

b.	Helps to:

i.	improve muscular coordination.

ii.	hand-eye coordination.

iii.	develops concept of motion.

iv.	training in self-help skills.

8.	Nutritional needs

a.	High energy requirements for:

i.	accessory muscular movements.

ii.	tonic muscle contractions.

b.	Poor intake due to:

i.	dysfunctional swallowing.

ii.	inability to chew.

iii.	food texture.

c.	Needs caloric and vitamin supplements.

9.	Education

a.	Problems in CP:

i.	25 – 75% of CPs have mental retardation.

ii.	this is complicated by the motor abnormalities such as speech and vocalization making them all the more difficult to educate.

iii.	other problems: emotional lability, perceptual difficulties.

b.	Early aims are:

i.	to develop habits of self care of the body, cleanliness and safety.

ii.	to develop sense of physical normality and adequacy.

iii.	to be independent in ADL.

c.	Solutions

i.	supportive parents and family environment.

ii.	patient and understanding teachers.

iii.	smaller classes.

iv.	‘bring the world to child’ scenarios.

v.	stimulate physical coordination with language development. 

d.	Later on, the aim is to train patient to a suitable standard of education and ability so that they can find outside employment and contribute to society.

e.	Besides sheltered workshops, possible vocations include:

i.	life / cinema attendants.

ii.	office cleaners.

iii.	car washers.

10.	Treatment of medical complications

a.	Pneumonia: antibiotics and chest physio.

b.	Seizures: anti-convulsants.

c.	Malnutrition: supportive treatment and MSW.

d.	Contractures and bed sores: preventive approach.

e.	Dental problems.

f.	Child abuse: role of MSW.

11.	Psychological assessment

a.	This depends on:

i.	level and quality of mental functioning.

ii.	personality attributes.

iii.	areas of strength and weakness.

iv.	ability to benefit from therapy and stimulation.

v.	current performance level and maturity.

vi.	predict ultimate capacity in the future.

b.	Base on the evaluation, recommend:

i.	type of treatment and care most likely to be practical and rewarding.

ii.	educational placement and training in areas of special need.

iii.	guidance to parents and teachers in helping child to learn and adjust.

12.	Prognosis of CP depends on:

a.	Type of CP.

b.	Severity of CP.

c.	Degree of mental retardation.

d.	Extent of complications.

e.	Extent of support: parents, family and social background.