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CEREBRAL PALSY
Dr. Maninder S. Dhaliwal
Define
Is defined as a :
1) Persistent but not unchanging
2) Disorder of movement, tone and posture
3) Due to non-progressive defect/lesion
4) Of immature brain
( fetal life, infancy, childhood)
( immature brain cut off take as 5 yrs –AAP)
5) Commonly associated with a spectrum
of developmental disabilities such as –
I. Mental retardation (60%)
II. Epilepsy (33%)
III. Visual , hearing (deafness-10%) and
speech defects
IV. Strabismus(50%)
V. Cognitive dysfunction
VI. Sensory problems
VII. Emotional and behavioral problems.
• EXCLUDING progressive pathology and
lesion of spinal cord.
• INCLUDING non-progressive genetic d/s or
cong malformation
CLASSIFICATIONS
TOPOGRAPHIC
• MONO
• HEMI
• DIPLEGIA
• QUADRI
• DOUBLE
HEMIPLEGIA
• TRIPLEGIA
PHYSIOLOGY
• SPASTIC
• EXTRAPYRAMIDAL
• ATAXIC
• MIXED
• ATONIC
FUNCTIONAL
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•
CLASS 1 – NO limitation of activity
CLASS 2 – Slight limitation
CLASS 3 – Moderate limitation
CLASS 4 – No useful physical activity
Site of brain injury
Pathological
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• Periventricular leucomalacia
–spastic diplegia
• Stroke in utero - hemiplegia
• Multifocal encephalomalacia
-quadriplegia
• Cerebellar - ataxic
• Basal ganglia, thalmus,
putamen - dyskinetic
Cortical
Sub – cortical
Periventricular
Basal ganglia
Cerebellum
Brain stem
Severity
• Motor development quotient :
= motor age / chronological age × 100
>70 : minimal – deviancy without delay
50-70 : mild motor disorder
<50 : classical CP
Etiological
Prenatal
• I, iron def.,poor –nut.
• Inf, UTI, high fever
• Chorioamniotis
• HTN, DM
• Teratogens
• Poor ANC
• LOW SES
• Twins
• Fetal vasculopathy
Perinatal
• Birth asphyxia
• Premature / LBW
• IUGR
• Hyperbilirubenemia
• IVH
• Sepsis, pneumonia,
meningitis
• Develop.
malformation
Postnatal
•CNS infections
•Head injuries
•Seizures
•Hypoxic
damage
•Hyperpyrexia
damage
Early markers of CP
• SLOW head growth
• Poor head control
• Eye – roving eyes, poor hand
regard, persistent squint.
• Ear – lack of auditory
response
• Irritability, seizures, poor
suck, poor quality of sleep.
• Extreme sensitivity to light
• Cortical thumb beyond 8
weeks
• Handedness before 2 yrs
• Paucity of limb movements
• Scissoring of lower limbs
• Toe walking
• Abnormal tone
• Persistence of primitive
reflexes or failure to
acquire postural reflexes
• Stereotypic abnormal
movements
• Lack of alertness
Differential diagnosis
• In the early infancy when the child is in
hypotonic phase, neuromuscular conditions like
myopathies may cause diagnostic confusion.
• Children with mental retardation may have
hypotonia but do not have abnormal motor
patterns or postures
• Neurodegenerative conditions which have
onset in early infancy such as Tay-Sach
disease, Krabbe's disease, and Metachromatic
Leucodystrophy can mimic CP.
• The progressive course of these conditions can
be ascertained on the basis of history, and
relevant investigation can confirm the
diagnosis.
• Dopa-responsive dystonia and organic
aciduria like glutaric aciduria may look like
dystonic CP.
Assessment of General Health
• Growth, and nutritional disorders
• Frequent respiratory tract infections are
common because of ineffective cough reflex.
• Facial dysmorphism and other congenital
anomalies should be noted.
• Skin should be inspected for neurocutaneous
stigmata.
• Head circumference should be noted and
plotted .
• Reliable measures of length may be difficult to
obtain due to concomitant contractures or
scoliosis.
• Alternate measures to length such as
segmental measures of upper arm and lower
leg are sometimes used.
• Skinfold thickness is a useful and a less
cumbersome method of assessing nutritional
status.
Neurological Evaluation
The history of previous developmental
milestones should be obtained for all
domains of development i.e. gross & fine
motor, cognitive, speech and language and
socialization.
• A thorough neurological evaluation should be
performed which includes assessment of
cranial nerves, posture, muscle tone of
extremities, trunk and neck, deep tendon
reflexes; postural response and primitive
reflexes .
• Physical examination should include the
observation of child in prone, supine, sitting,
standing, and if appropriate in walking and
climbing positions.
• This is followed by assessment of the
current level of functioning in all these
domains and assessment of self help and
adaptive skills in daily activities such as
feeding, dressing, brushing teeth .
Disorder in Movement and
Posture
• Among the most clinically useful primitive
reflexes are Moro, Tonic labyrinthine and
ATNR
• Postural reactions are sought in each of 3
categories: righting, protection and
equilibrium.
Muscle Tone
• Abnormality of tone is an integral part of
CP.
• Hypertonia in CP may be purely due to
spasticity (pyramidal ) or else due to
dystonia (extrapyramidal).
Assessment of Cognition and
Behavior
• Metal retardation was found the commonest
associated problem in children with CP
• Conventional tests of intelligence may prove
erroneous in children with CP because of motor
and communication deficits.
• Age appropriate non-verbal intelligence tests
have to be administered for this purpose.
Assessment of Vision and
Hearing
• In children with hearing impairment with associated
microcephaly and congenital heart disease should be
looked for other stigmata of TORCH infection.
• In cases with dyskinetic CP, presence of hearing
impairment may point to kernicterus as a cause of CP.
• Sensorineural hearing loss is a prominent feature of
CP due to Iodine deficiency in endemic areas.
Assessment of Speech and
Language
• These may be due to hearing impairment,
cognitive deficits, or oromotor dysfunction
•
• Difficulty in communication by language or
gestures further compound behavior
problems.
Assessment of Feeding and
Nutrition
• Oromotor dysfunction, inability to self feed
, and inability to request for food due to
communication disorder result in feeding
problems and poor nutritional status in
children with CP .
• Gastro-esophageal reflux or
choking/coughing while feeding which may
further cause aversion to food .
• Child should also be assessed for tongue
thrust or tonic bite, hypersensitivity to
touch, drooling of saliva and dental
hygiene.
Orthopedic Problems
• Hip subluxation, scoliosis, equinus deformity, and
contractures of hamstring muscles and tendoachilles.
• Reduced bone density and propensity to fractures with
trivial trauma is common
• Equinus deformity is the most common
musculoskeletal abnormality in patients with CP.
• It is due to fixed or spastic contracture of
gastrocnemius and causes the typical tip toe or toe
heel gait in children with CP.
• Epilepsy: Epilepsy is more common in
children with CP. In a population based study
38% of children with CP had epilepsy
• Children with CP caused by CNS
malformations, CNS infection, and grey matter
damage have been reported to show a higher
frequency of epilepsy than children with CP of
other aetiology, and also had less chance of
becoming seizure-free
Role of Neuroimaging
• Neuroimaging (MRI preferred to CT) is
recommended in children with cerebral
palsy in order to establish structural brain
abnormality which may further help in
finding the etiology and giving prognosis
Disabling conditions be evaluated on multiple axes-
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Pathophysiology (underlying disease);
Impairment (clinically observable abnormality) ;
Functional limitations (effect on task performance);
Disability (effect on daily living) and
Societal limitations (effect on life time opportunities).
• Since CP is a changing disorder it is evident that
some limitations may not be evident early in life but
manifest in the school age or later.
• Assessment of Home Situations:
Evaluation is not complete without the
assessment of the home situation such as
family size, financial resources and family
support.
• Child rearing conditions that support and enrich
early development can compensate for many
biological deficits.
• On the other hand, poverty, illiteracy in parents,
large family size, frequent change in residence,
non-availability of special rehabilitation centers
may deprive the child of appropriate care.
• The hemiplegic type of CP has the best prognosis for
walking with 95% walking by 3 years compared to
40% of other groups.
• Age of sitting is a good guide to prognosticate about
walking. A child who is able to sit unsupported at 2
years will eventually be able to walk.
• On the contrary, a child whose sitting is delayed
beyond 3 years has remote prospects for functional
outdoor walking
• Management - The pediatrician's initial
role consists of making a correct
diagnosis,determining the etiology, and
identification of the type, extent and
severity of the neuromotor deficit as well
as of associated problems
(i) Breaking the news
ii) Comprehensive assessment
a multidisciplinary team comprising of a neuro-developmental
pediatrician as the team-leader,
• physiotherapist,
• occupational therapist,
• clinical psychologist,
• speech pathologist,
• orthopedic surgeon,
• otorhinolaryngologist,
• ophthalmologist,
• teacher,
• play therapist and
• social worker is required,
preferably under one roof.
Physiotherapy (P.T.) • P.T. especially when started early in life, is
helpful in promoting normal motor development,
and preventing deformity and contractures.
• In the young child it aims at reducing abnormal
patterns of movement and posture and
promoting the normal ones so as to enable the
child to gain maximal functional independence.
• A number of techniques have been used for this
purpose. The neurodevelopmental Bobath
technique is commonly used.
• It consists of guiding the child through
normal sequences of motor development,
• inhibition of primitive and abnormal
reflexes,
• re-inforcement of normal postural reflexes
and
• facilitation of normal movements.
Occupational Therapy
• The role of P.T. and O.T. are so closely linked that
they could infact be considered together.
• The occupational therapist is usually better trained
to advise on activites of daily living like feeding,
bathing, dressing, toilet training etc, and the
equipment needed to facilitate these.
• Co-ordination and sensory-perceptual integration
can be taught and multisensorial stimulation
provided through peg board, blocks and other toys
of different colours, textures, sizes and shapes, and
producing different sounds
Play Therapy: It is the use of a natural activity
with a young child, to help him consolidate the
levels of development that he has reached and
encourage him to move on, to the next level.
• Parents are taught to break down each activity
into its simplest components and make the child
practice it in a real life situation. It then is not
considered as an 'exercise' but becomes a way
of life.
Assistive and adaptive devices :
• Various simple modifications like angled
spoons, two handled cups etc can be
made to help the child.
• Old stools and boxes can be adapted to
provide support during sitting
• Parallel bars can be constructed with logs
of wood to help gait training.
• Standing frames and prone boards are a
useful intermediate stage in mobilization.
• A number of high technology devices like
programmed wheel chairs, electronic
feeding devices, various access systems,
computerized speech systems and
cochlear implants are available
• Splints, Casts and Calipers: Specially
designed shoes, ankle-foot orthoses
(AFO) and calipers
• AFOs, are particularly useful in children
with spastic diplegia who have dynamic
spasticity with tendo- achilles (TA)
tightening.
Management of Spasticity:
• Proper P.T. given regularly considerably reduces
spasticity and improves function.
• (i) Drugs:
• Baclofen - acts at the level of spinal cord
neurons and enhances GABA activity.
• It is commonly used in a starting dose of 1.25 2.5 mg BD orally and increased gradually upto a
maximum of 30 mgm/day, monitored by a clinical
response.
• It is not recommended in children with seizures
as it may provoke them. (
• ii) Diazepam- a small dose given half an hour
before PT is effective in some cases especially
where anxiety increases spasticity. Its
disadvantage is that it may cause unacceptable
drowsiness.
• (iii) Tizanidine an alpha 2 adrenergic agent and
• (iv) Dantrolene sodium which acts on calcium
channels have also been used, but experience
with them is limited.
• Baclofen has also been used intrathecally
using implantable infusion pumps.
• It may be helpful in cases of severe
spasticity or disabling total body dystonia
• Botulinum Toxin: (BTA): is derived from Claustridium
botulinum.
• It causes muscle relaxation by blocking the release of
acetylcholinesterase, with loss of motor end plates.
• As affected nerve roots sprout to form new junctions,
the relaxing effect reverses over 3-6 months.
• It is more often used in children with spastic diplegia.
• Surgery: Surgery is useful in some children with
spasticity, especially where mainly the lower limbs
are involved.
• Tendon lengthening and transfer and arthrodesis are
some of the procedures commonly performed.
• Generally multilevel surgery is required and is done
after 8 years of age.
• Simultaneous availability of intensive physiotherapy
is essential.
• Dorsal rhizotomy which involves selective
resection of posterior nerve roots from L 2_ to S 2
• It may be helpful in children with severe lower limb
spasticity, with sufficient trunk control and some
form of forward locomotion.
• Its advantage must be weighed carefully against
the sensory losses that may occur after the
procedure.
• Relief of athetosis and dystonia - is difficult
occasionally levo-dopa for severe
athetosis and carbamezepine for dystonia
may be helpful.
• Thalamotomy for athetoid CP, stereotactic
dentatomy and chronic cerebellar
stimulation via implanted electrode .
Management of Associated
Problems.
• Parent Counselling : This is one of the
most important aspects because parents
are pivotal in the management of their
child.
• It is an ongoing process, as the parents
need to be counseled periodically at
various stages of their child's
development.