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CEREBRAL PALSY
REVIEW OF LITERATURE
1
INTRODUCTION
2
World Cerebral Palsy Day is a social movement and a day to celebrate
and affirm the lives of the 17 million people living with cerebral palsy
(CP). It was launched in 2012 by Cerebral Palsy Alliance (Australia) and
United Cerebral Palsy (USA). It is supported by over 350 CP service
organisations, universities, parent groups, research institutions, student
groups, schools and children's hospitals from 52 countries.
World Cerebral Palsy Day is celebrated every year on 5 Oct to raise
awareness as well as to ensure that persons with cerebral palsy have
access to their human rights and opportunities, just like everyone else.
The theme for world cerebral palsy day 2016 is - 'We will tell the world …
I am here, we are here'.
HISTORY
Even though history lacks written proof, there is no doubt that cerebral
palsy has existed as long as women have giving birth. But it was not until
1861 that an English surgeon, named Dr. William John Little wrote the
first medical description.
The term cerebral palsy was first used by Sir William Osler, a British
medical doctor in the late 1800s. It became widely known and soon
replaced the earlier name of Little’s disease. No great studies were made
in the field of CP until the 1980s when a government funded study
combined information about 35,000 cerebral palsy births. The study
found relatively few cases of cerebral palsy were actually accompanied
by trauma at birth. This revelation sparked interest in the medical
community about cerebral palsy.
Beginning at the end of the 19th century Sigmund Freud and Sir William
Osler both contributed important perspectives on the condition. From
the mid-1940s the founding fathers of the American Academy for
Cerebral Palsy and Developmental Medicine in the USA, and Mac Keith,
Polani, Bax, and Ingram of the Little Club in the UK, were among leaders
who moved the concepts and descriptions of CP forward, and caused
this condition to become the focus of treatment services, advocacy, and
research efforts.
3
The 1990s brought advances in the early diagnosis of CP in infants. It was
determined early identification of the disorder helps prevent permanent
contracture of limbs. Progress was made in diagnostic techniques for
brain imaging and analysis.
Therapies for CP were developed to help increase physical movement.
Braces and assistive technologies helped facilitate mobility and increase
quality of life. Changes in the perception of counselling and
psychological services helped people with CP deal with the emotional
effects of the disorder.
There are many people live successful life with cerebral palsy, spreading
awareness about living well with CP, like Christopher Nolan, Stephen
Hopkins, Christy Brown, RJ Mitte, Jhamak Ghimire, Josh Blue, Paul Blake
(won gold in 2016 Paralympics) and many more are there…..
DEFINITION
4
d) The age before which cerebral palsy cannot be reliably recognized
These additional criteria need not be uniformly provided, they are
precisely specified, but even this poses problems. So, To label a Child
having cerebral palsy there must be:-
i. Some form of brain damage involving motor pathway
ii. Must be non-progressive, non-hereditary.
iii. There may be dynamic clinical pattern despite a static pathology.
iv. Non-curable but amenable to training.
The term CP has, however, been variably used, with poor comparability
across different places and times, indicating need for a consensual
definition. Epidemiologists, in particular, require consistent terminology
and concepts across time and space in order to identify changing
patterns of diseases and disorders. So, one must have a clear idea of
several aspects of the definition:-
o “A group”: There is general agreement that CP is a heterogeneous
condition in terms of etiology as well as in types and severity of
impairments. Several groupings are possible and warranted to serve
different purposes. These groupings may show overlap. Therefore, the
singular form CP is used (as opposed to cerebral palsies) as an umbrella
term.
o “Disorders”: This refers to conditions in which there is disruption of the
usual orderly processes of child bio-psychosocial development. The
disorders are persistent.
o “Development”: The notion of alteration in development is essential to
the CP concept. It distinguishes CP from phenotypically similar disorders
in children or adults due to late-acquired lesions, at a time when motor
development is relatively well-developed.
5
The development aspect of CP is also important with regard to
management strategies that may include interventions that address the
developmental consequences of the functional limitations associated
with CP and interventions that are directed at the underlying
neurobiological processes.
The motor impairments of CP manifest very early in child development,
usually before 18 months of age, with delayed or aberrant motor
progress. The clinical picture of CP evolves with time, development,
learning, training, therapies, and other factors.
o “Movement and posture”: Abnormal motor behaviour is the core
feature of CP. It is characterized by various abnormal patterns of
movement and posture related to defective coordination of movements
and/or regulation of muscle tone. However, patients with
neurodevelopmental disabilities that do not primarily affect movement
and posture are not considered to have CP.
o “Causing”: Activity limitations are presumed to be a consequence of
motor disorder. Thus disorders of movement and posture that are not
associated with activity limitations are not considered part the CP group.
o “Activity limitation”: The World Health Organization’s international
classification of functioning, disability and health speaks of activity as the
execution of a task or action by an individual, and identifies activity
limitation as difficulties an individual may have in executing activities.
This term amplifies the previous of disability to recognize changing
international concepts and terminology.
o “Attributed to”: Understanding of developmental neurobiology
(including the effects of genetic, chemical, and other influences on brain
development) is increasing rapidly, such that it is becoming possible to
identify structural and other evidence of brain mal development in
people with CP.
As a consequence, structural-functional connections and correlations are
becoming more clearly delineated than has previously been possible. It
must, however, be acknowledged that at the present time a full
understanding of causal pathways and mechanisms leading to CP
remains elusive in many cases.
6
o “Disturbances”: This term refers to processes or events that in some
way interrupt, damage, or otherwise influence the expected patterns of
brain maturation, and result in permanent (but non-progressive)
impairment of the brain. In a proportion of cases it is currently not
possible to identify a specific disturbance or a specific timing of the
events that appear to impact on maturation. These disturbances may
include cerebral dysplasia.
o “Fetal or infant”: The specification fetal or infant reflects the idea that
disturbances that occur very early in human biological development
impact differently on motor function than disturbances that occur later,
even those that occur in early childhood. There is no explicit upper age
limit as, depending on aspects of motor functioning, the first two or
three years of life may be concerned. Therefore, the notion of early
lesion would appear more useful clinically than arbitrarily specified time
limits. In practical terms, disturbance resulting in CP is presumed to
occur before the affected function has developed (e.g. walking,
manipulation etc.).
o “Brain”: The term brain includes the cerebrum, the cerebellum, and the
brainstem. It excludes motor disorders of spinal, peripheral nerve,
muscular or mechanical origin (note, however, that alterations in the
neuromuscular and musculoskeletal systems may occur in CP as a
consequence of the chronic motor impairment. These alterations may
restrict further motor function or patients with CP, and be associated
with secondary changes in skeletal alignment and/or functional
capacity).
o “Accompanied by”: In addition to the disorder of movement and
posture, people with CP often show other disorders or impairments.
These may be caused by the same disturbances as those that caused CP
and/or represent indirect consequences of the motor impairment
and/or be caused by independent factors (hence the term accompanied
by “as opposed to associated with”).
o “Sensation”: Vision, hearing, and other sensory modalities may be
affected.
o “Cognition”: Both global and specific cognitive processes may be
affected, including attention. Note, however, that when a child has
7
severely delayed cognition and no motor signs (except perhaps for some
degree of hypertonicity or hypotonicity) it is not usual to include them
within the concept of CP.
o “Communication”: Expressive and/or receptive communication and/or
social interaction skills may be affected.
o “Perception”: The capacity to incorporate and interpret sensory and/or
cognitive information may be impaired both as a function of the
‘primary’ disturbance(s) to which CP is attributed, and as a secondary
consequence of activity limitations that restrict learning and perceptual
development experiences.
o “Behavior”: This also includes behavioural problems in the context of
psychiatric disorders, such as features of autism, ADHD, mood disorders
and anxiety disorders.
o “Seizure disorder”: Virtually every seizure type and many epileptic
syndromes may be seen in patients with CP. Rarely, the seizure disorder
may be cause of CP (e.g. as consequence of prolonged infantile status
epilepticus), or it may result in further motor impairment.
It is hoped that this definition will clarify the CP concept and allow
unified use of the term both within and across the concerned fields. As it
relies essentially on clinical aspects and does not require sophisticated
technology, it should be possible to apply this definition very widely.
In other way, ‘Cerebral palsy is a disorder of posture, movement and
tone due to a static encephalopathy acquired during brain growth in
fetal life, infancy or early childhood. Though the brain disorder is
unchanging, the effects are dynamic, as the brain matures, and child’s
developmental capabilities extend.’
After having idea of what is CP, one must know what NOT CP is;
– Neuro-developmental disabilities that do not primarily affect
movement and posture.
– Motor dysfunctions from recognized progressive brain disorders.
– Severely impaired cognition and no motor signs (except for some
degree of hypotonicity).
– Primary disorders of spinal cord (neural tube defects),
neuropathies, and myopathies.
8
EPIDEMIOLOGY
Cerebral palsy results from an injury in the developing CNS, which can
occur in utero, during delivery, or during the first 5 years of life.
Improved care in neonatal intensive care units and higher survival for
very low-birth weight infants increases the risk of CP.
The natural course of CP has changed greatly during the past 50 years. If
appropriate health care is available, affected children without significant
comorbidities have actuarial survival approaching that of the general
population. However, mortality is higher and lifespan shorter in children
with severe quadriparesis, hydrocephalus, lack of basic functional skills,
refractory seizures, and profound mental retardation.
More than 50% of patients with the disorder can walk without arm
assistance; 25% cannot walk, and 30% are mentally-impaired.
Neurological problems are common and include seizures (35%), sensory
impairment of the arms (97%), hydrocephalus (9%), autonomic
dysfunction, impairment of visual perception (20–40%), and learning
disabilities.
Hemiplegic patients experience abnormal stereognosis (97%),
diminished two-point discrimination (90%), and diminished
proprioception (46%).
Urinary incontinence is common (23.5%), especially in children and
adults with quadriparesis and mental retardation.
There are two approaches to estimating the frequency cerebral palsy.
Prevalence is the proportion of a population with the condition at a
given point in time and is used to plan services.
Incidence is the proportion of new cases with the condition arising in a
population. It is used in etiological studies seeking to understand the
causal pathways.
The number of children born in a region that survive to be described as
having CP, divided by the number of neonatal survivors (NNS) born in
that region, known as NNS prevalence.
Most of the recently published studies and Centres for disease control
and prevention, estimates overall prevalence of CP is 2 to 2.5 per 1000
9
live births, and the incidence is 3.6 per 1000 children with male to
female ratio are 1.4:1.0.
The prevalence of cerebral palsy reported from developing countries
tends to be in a similar range with that of developed countries.
However, given the likelihood of higher rates of both mortality and post-
neonatally acquired cerebral palsy. With extremely few survivors of very
preterm birth in the absence of sophisticated neonatal intensive care,
developing courtiers have virtually no very preterm CP.
The Collaborative Perinatal Project, in which approximately 45,000
children were regularly monitored from in utero to the age of 7 year,
found that most children with CP had been born at term with
uncomplicated labour and deliveries. In 80% of cases, features were
identified pointing to antenatal factors causing abnormal brain
development. A substantial number of children with CP had congenital
anomalies external to the central nervous system (CNS). Fewer than 10%
of children with CP had evidence of intra-partum asphyxia.
The frequency of CP increases with decreasing duration of gestation. The
prevalence of CP has increased somewhat as a result of the enhanced
survival of very premature infants weighing <1,000gm, who go on
develop CP at a rate of approximately 15 per 100 survived very
premature neonates.
The prevalence ranged from 146 per 1000 children born at 22 to 27
weeks of gestation, steadily declining thereafter to an estimated 62 per
1000 children born at 28 to 31 weeks, 7per 1000 at 32 to 36 weeks’
gestation, and 1.13per 1000 in term-born infants.
The prevalence is highest in children weighing 1000 to 1499g (59.18 per
1000 live births), and lowest in children weighing over 2500g (1.33 per
1000 live births).
It is 8.33 per 1000 live births in children weighing between 1500gm and
2499gm. The prevalence among children weighing under 1000g was not
significantly different from the prevalence among those weighing 1000
to 1499g, but in both cases was significantly higher than among children
weighing 1500 to 2499g.
10
In 2006, the European Cerebral Palsy Study examined prenatal and
perinatal factors as well as clinical findings and results of MRI in a
contemporary cohort of more than 400 children with CP.
In agreement with the Collaborative Perinatal Project study, more than
half the children with CP in this study were born at term, and less than
20% had clinical or brain imaging indicators of possible intra-partum
factors such as asphyxia.
The contribution of intra-partum factors to CP is higher in some
underdeveloped regions of the world. Also in agreement with earlier
data, antenatal infection was strongly associated with CP and 39.5% of
mothers of children with CP reported having an infection during the
pregnancy, with 19% having evidence of a urinary tract infection and
11.5% reporting taking antibiotics.
The risk of CP increases with increasing plurality as anticipated given the
tendency to shorter gestation and slower intrauterine growth with
increasing plurality.
A collaborative population-based study combined data from 1.14 million
births, including 24,115 twin births. It observed that, compared with
singletons, gestation-specific CP rates in 1-year survivors, were higher in
both very preterm and term-born twins, but not in moderately preterm-
born twins.
The rate being 1.6/1000 NNS for term singletons compared with 6.1 for
term multiples (5-8 times greater in twins). The contribution of multiple
births to frequency of CP is increasing with the increasing rate of
multiple births, and this has been attributed to increasing maternal age
and use Infertility treatments (assisted reproduction therapies).
The use of infertility treatment is likely to be responsible for the
increased rates of triplet births with their alarming 20-47 times greater
risk of CP per infant, an approximately 50-fold increase in risk per
pregnancy.
Among children from multiple pregnancies, 24% were from pregnancies
after infertility treatment compared with 3.4% of the singleton
pregnancies.
11
Death of a twin in utero carries an even greater risk of CP that is 8 times
that of a pregnancy in which both twin survive and approximately 60
times the risk in a singleton pregnancy.
Analysis of types of CP showed a greater reduction in some syndromes
than others, being most marked in diplegia and dyskinetic syndromes,
while hemiplegia contributed are relatively higher proportion of the
total.
Prevalence of Spastic Diplegia is 35%, Hemiplegia 25%, Spastic
Quadriplegia 20%, and Extra-pyramidal (athetoid, dyskinetic) is 15%. In
recent times due to better obstetric care and decrease in incidence of
bilirubin encephalopathy, incidence of choreoethetoid CP has
significantly become low.
There is a small but growing proportion of post neonatally acquired
cerebral palsy is acquired as a result of early surgery to correct
congenital heart defects and of spontaneous cerebrovascular accidents.
“CP is more common and more severe in boys compared to girls and this
effect is enhanced at the extremes of body weight. Male infants with
intrauterine growth retardation and a birth-weight less than the 3rd
percentile are 16 times more likely to have CP than males with optimal
growth, and infants with weights above the 97th percentile are 4 times
more likely to have CP.”
12
weighing less than 1500 gm and 250 –fold in normal birth weight
neonates who had sustained severe asphyxia ( APGAR score <3 at 20
minutes).
Risk factors for cerebral palsy can be broadly divided in three
categories:-
1. Prenatal factors: 75%
2. Perinatal factors: 10-15%
3. Post natal factors: 10%
a) Maternal factors:-
o Bad obstetric history, very short (<3 months) or very long (>3 years)
interval between two pregnancies, positive family history of CP.
o Iodine deficiency, Iron deficiency, poor nutrition.
o High grade fever, Urinary tract infections.
o Diseases e. g. diabetes, hypertension, hyperthyroidism
o Intrauterine infections {rubella, toxoplasmosis, cyatomegalovirus
infection, HIV infection, herpes simplex infection}.
o Teratogens e. g. Drugs, Radiations, Smoking, Alcohol, environmental
toxins, cocaine and lead.
o Fertility problems e.g. advanced age at conception, history of infertility,
recurrent fetal wastage.
o Poor ante natal care, low socio economic status.
b) Fetal factors:-
• Twins {vanishing twin}/ abnormal fetal presentation.
• Fetal thrombotic vasculopathy.
• Intra Uterine Growth Retardation
c) Placental factors:-
o Chorioamnionitis, chorionic plate thrombi, histological lesions
13
• Prematurity or VLBW (very low birth weight) is the single most
important determinant of CP
• Prenatal Asphyxia or birth asphyxia
• Ischemic perinatal stroke
• Hypoglycemia, dyselectrolytemias
• Hyperbilirubenemia
• Intra vascular and Intra cerebral bleeds
• Sepsis, pneumonia, meningitis
• Premature separation of placenta
3. Post natal factors; First two years of life has utmost significance in post natal
period for developing CP. These contribute up to 10% of total cases,
Asphyxia
• Recent studies suggests that with improved obstetric and newborn care,
with advanced investigation technologies like electronic fetal
monitoring, fetal blood sampling, The frequency of birth asphyxia as
14
measured by Apgar score along with maternal and fetal mortality
markedly decreased, without a net decrease in the prevalence of CP.
• Estimation of the role of birth asphyxia in neurologic impairment is
difficult because there is no direct means for measurement of birth
asphyxia, which is commonly inferred from clinical signs such as
abnormalities on electronic fetal monitoring, low-Apgar scores, need for
respiratory support, and neonatal encephalopathy.
• None of these signs is specific to asphyxia states. Of the potentially
asphyxiating conditions examined in delivery records, only tight nuchal
cord (Nelson et al) was associated with spastic CP and then only with
spastic quadriparesis, not hemiparesis or diplegia.
• NCPP (national collaborative perinatal project) states that there is a
strong relationship between spastic diplegia and HIE (hypoxic ischemic
encephalopathy) induced periventricular leukomalacia.
• It suggests that infant of normal birth weight and 5-minute APGAR score
of 3 or less had 5% probability of developing CP. Apgar score of 3 or less
at 10 minutes increased the risk to 17% and same score at 20 minutes
was associated with 57% risk. HIE grade 2 and 3 have been found to be
associated with CP. Risk of developing CP in a neonate increases if 2nd
stage of labour prolonged by >4 hrs.
Genetical factors
15
• Grether and Nelson in a case control study on children with spastic CP
and birth weight of more than 2500gm found that maternal fever above
38 degree C in labour room and chorioamnionitis was associated with
increase in the risk of unexplained CP.
Biochemical abnormalities
16
• low-APGAR scores or asphyxia and acidosis,
• Intracranial haemorrhage and Hypotension,
• Patent ductus arteriosus (PDA),
• Sepsis, Necrotizing enterocolitis or surgery.
17
Clinicopathologic correlation of cerebral palsy
CLASSIFICATION
• It is understandable that in such a diverse collection of disorders many
attempts at classification should be of limited value. The history of these
attempts has been well reviewed by Ingram.
• Krageloh-Mann and her Swedish and German colleagues, in a
comparative study of bilateral spastic CP in south west Germany and
western Sweden, have compared the Swedish system of classification,
based on defined clinical syndromes and similar to that of Ingram, with
the German system based on limb distribution of motor function (leg-
dominated, three- or four-limb-dominated and dyskinetic-spastic).
• Traditional classification schemes have focused principally on the
distributional pattern of affected limbs with an added modifier
describing the predominant type of tone or movement abnormality, but
it has become apparent that additional characteristics must be taken
18
into account for a classification scheme to contribute substantively to
the understanding and management of this disorder.
• No classification system is useful unless it is reliable. It is therefore not
enough to specify the characteristics to be used in classification; they
must be operationally defined so that, in general, competent examiners
will classify the same individual in the same way given identical
information.
• It is important to remember that the purpose of accurate classification
of children with CP is not as an academic exercise, but to achieve a full
assessment and understanding of their problems in order to provide the
most appropriate help. The purpose of classification includes:
I. Description: providing a level of detail about an individual with CP
that will clearly delineate the nature of the problem and its severity.
II. Prediction: providing information that can inform healthcare
professionals of the current and future service needs of individuals
with CP.
III. Comparison: providing sufficient information to permit reasonable
comparison of series of cases of CP assembled in different places.
IV. Evaluation of change: providing information that will allow
comparison of the same individual with CP at different points in time.
1. INFORMATION REQUIRED FOR
Information required for classification
All classification documentation should include the age of the child, the
nature of the information available from clinical history (e.g. whether
from clinical notes, maternal recall or period of observation of the child),
and the extent to which metabolic and neuroimaging investigation has
been performed.
19
characteristics may be more useful than others. Thus a standardized
classification should be operationally defined and reliable.
1. Motor abnormalities
• Nature and typology of the motor disorder
• Functional motor abilities
2. Accompanying impairments
3. Anatomical and Neuroimaging findings
• Anatomic distribution
• Neuroimaging findings
4. Causation and timing
1. Motor abnormalities
A. Nature and type of the motor disorder: The type of abnormal
muscle tone or involuntary movement disorder observed or
elicited is usually assumed to be related to the underlying
pathophysiology of the disorder, and may also reflect etiologic
circumstances, as in kernicterus.
The classification system described in the Reference and Training
Manual of the Surveillance of Cerebral Palsy in Europe (SCPE),
which divides CP into three groupings based on the predominant
neuro-motor abnormality-
i. Spasticity
20
ii. Dyskinetic: differentiated into dystonia and choreoathetosis
iii. Ataxic
21
children with CP. Bulbar and oro-motor difficulties are common in
cerebral palsy and can produce important activity limitation, but
there is as yet no activity limitation scale for such functions.
22
• General heading for each level:-
– Level I - Walks without Limitations
– Level II - Walks with Limitations
– Level III - Walks Using a Hand-Held Mobility Device
– Level IV -Self-Mobility with Limitations, May Use Powered Mobility
– Level V - Transported in a Manual Wheelchair
• Before 2 years age
Level I - Infants move in and out of sitting and floor sit with both hands
free to manipulate objects. Infants crawl on hands and knees, pull to
stand and take steps holding on to furniture. Infants walk between 18
months and 2 years of age without the need for any assistive mobility
device.
Level II -Infants maintain floor sitting but may need to use their hands
for support to maintain balance. Infants creep on their stomach or crawl
on hands and knees. Infants may pull to stand and take steps holding on
to furniture.
Level III -Infants maintain floor sitting when the low back is supported.
Infants roll and creep forward on their stomachs.
Level IV -Infants have head control but trunk support is required for floor
sitting. Infants can roll to supine and may roll to prone.
Level V -Physical impairments limit voluntary control of movement.
Infants are unable to maintain antigravity head and trunk postures in
prone and sitting. Infants require adult assistance to roll.
• Between 2-4 years age
Level I- Children floor sit with both hands free to manipulate objects.
Movements in and out of floor sitting and standing are performed
without adult assistance. Children walk as the preferred method of
mobility without the need for any assistive mobility device.
Level II- Children floor sit but may have difficulty with balance when both
hands are free to manipulate objects. Movements in and out of sitting
are performed without adult assistance. Children pull to stand on a
stable surface. Children crawl on hands and knees with a reciprocal
pattern, cruise holding onto furniture and walk using an assistive
mobility device as preferred methods of mobility.
23
Level III- Children maintain floor sitting often by “W-sitting” (sitting
between flexed and internally rotated hips and knees) and may require
adult assistance to assume sitting. Children creep on their stomach or
crawl on hands and knees (often without reciprocal leg movements) as
their primary methods of self-mobility. Children may pull to stand on a
stable surface and cruise short distances. Children may walk short
distances indoors using an assistive mobility device and adult assistance
for steering and turning.
Level IV- Children sit on a chair but need adaptive seating for trunk
control and to maximize hand function. Children move in and out of
chair sitting with assistance from an adult or a stable surface to push or
pull up on with their arms. Children may at best walk short distances
with a walker and adult supervision but have difficulty turning and
maintaining balance on uneven surfaces. Children are transported in the
community. Children may achieve self-mobility using a power
wheelchair.
Level V - Physical impairments restrict voluntary control of movement
and the ability to maintain antigravity head and trunk postures. All areas
of motor function are limited. Functional limitations in sitting and
standing are not fully compensated for through the use of adaptive
equipment and assistive technology. At Level V, children have no means
of independent mobility and are transported. Some children achieve
self-mobility using a power wheelchair with extensive adaptations.
• Between age 4-6 years
Level I- Children get into and out of, and sit in, a chair without the need
for hand support. Children move from the floor and from chair sitting to
standing without the need for objects for support. Children walk indoors
and outdoors, and climb stairs. Emerging ability to run and jump.
Level II- Children sit in a chair with both hands free to manipulate
objects. Children move from the floor to standing and from chair sitting
to standing but often require a stable surface to push or pull up on with
their arms. Children walk without the need for any assistive mobility
device indoors and for short distances on level surfaces outdoors.
Children climb stairs holding onto a railing but are unable to run or jump.
24
Level III- Children sit on a regular chair but may require pelvic or trunk
support to maximize hand function. Children move in and out of chair
sitting using a stable surface to push on or pull up with their arms.
Children walk with an assistive mobility device on level surfaces and
climb stairs with assistance from an adult. Children frequently are
transported when travelling for long distances or outdoors on uneven
terrain.
Level IV- Children sit on a chair but need adaptive seating for trunk
control and to maximize hand function. Children move in and out of
chair sitting with assistance from an adult or a stable surface to push or
pull up on with their arms. Children may at best walk short distances
with a walker and adult supervision but have difficulty turning and
maintaining balance on uneven surfaces. Children are transported in the
community. Children may achieve self-mobility using a power
wheelchair.
Level V- Physical impairments restrict voluntary control of movement
and the ability to maintain antigravity head and trunk postures. All areas
of motor function are limited. Functional limitations in sitting and
standing are not fully compensated for through the use of adaptive
equipment and assistive technology. At Level V, children have no means
of independent mobility and are transported. Some children achieve
self-mobility using a power wheelchair with extensive adaptations.
• Between age 6-12 years
Level I - Walks without limitation/ restriction, Ambulatory in all
settings, Can walk indoors and outdoors and climb stairs without using
hands for support, Can perform usual activities such as running and
jumping, Has decreased speed, balance and coordination.
Level II - Walks without assistive devices but limitation in community
settings, Can climb stairs with a railing, Has difficulty with uneven
surfaces, inclines or in crowds, Has only minimal ability to run or jump.
Level III - Walks with hand held mobility device/ assistive devises
indoors and outdoors on level surfaces, May be able to climb stairs using
a railing, May propel a manual wheelchair and need assistance for long
distances or uneven surfaces.
25
Level IV - Walking ability severely limited even with assistive devices,
may use powered mobility, Standing transfers, with or without
assistance.
Level V - Has physical impairments that restrict voluntary control of
movement, Ability to maintain head and neck position against gravity
restricted, Impaired in all areas of motor function, Cannot sit or stand
independently, even with adaptive equipment, Cannot independently
walk but may be able to use powered mobility.
• It was developed to measure the need for assistance and the severity of
disability in children between the ages of 6 months and 7 years. It may
be used with children above the age of 7 years as long as their functional
abilities are below those expected of children aged 7 who do not have
disabilities.
• It measures level of independence in self-care, sphincter control,
mobility, locomotion, communication and social function. It is a data set
of 18 items that measure functional performance in 3 domains:- Self
care, Mobility, And Cognitive.
26
Manual ability classification system (MACS)
• MACS was devised for description of arm and hand function. It describes
limitation in hand use in five grades.
• Eliasson et al. (2006) developed the MACS.
• based on the perspective of the child’s typical manual performance in
daily life.
• It focuses on everyday performance, is not designed to classify the best
capacity, and does not intend to distinguish between hands in terms of
capacities.
(MACS)
Yes No
Does the child perform even Does the child perform a number of manual tasks which
difficult manual tasks with fair commonly need to be adapted or prepared, and help is
speed and accuracy and does only needed occasionally ?
not need to use alternative
ways of performance ? NO
Yes No
LEVEL 2 LEVEL 3
LEVEL 1 LEVEL 4
Handles LEVEL 5
Handles most
Handles objects Handles a
objects but with objects
easily and limited Does not handles
somewhat reduced with
successfully. At selection of objects and has
quality and/or difficulty,
most limitations easily manage severely limited
speed of needs
in the ease of objects in ability to perform
achievement. May help to
performing adapted even simple
avoid some tasks prepare
manual tasks situations, actions. Requires
or use alternative and/ or
requiring speed requires total assis
ways of modify
and accuracy. continuous
performance. activities.
support
27
Gross motor function measure 66 (GMFM-66)
ii. Sitting
28
2. Accompanying impairments
In many individuals with cerebral palsy, other impairments interfere
with the ability to function in daily life, and may at times produce even
greater activity limitation than the motor impairments that are the
hallmark of cerebral palsy.
These impairments should be classified as present or absent, and if
present, the extent to which they interfere with the individual’s ability to
function or participate in desired activities and roles should be
described.
In concurrence with the SCPE recommendation, the presence or absence
of epilepsy (defined as two or more afebrile, non-neonatal seizures) be
recorded, and IQ, hearing and vision be assessed. While SCPE provides
terminology for describing different degrees of cognitive, hearing and
visual impairment, the IQ score, corrected vision in each eye, and decibel
loss (if any) in each ear be recorded.
29
differentiation of unilateral versus bilateral motor involvement.
This distinction should be considered as part of a multiaxial
classification scheme, thus it should be coupled with a description
of the motor disorder and functional motor classification in both
upper and lower extremities.
30
A normal examination is generally associated with a normal outcome but
a single examination can never predict outcome. Importance of serial
examinations to follow persistence or recovery of abnormal signs should
be emphasized.
Repeatedly normal neurological examination and resolution of signs
carries a good prognosis. Persistence of abnormal state, persistence of
deviant signs, deterioration suggests bad prognosis.
Neurological Examination
There are certain signs and symptoms which are labelled as RED FLAG
signs for developing CP, by careful observation of those one can make an
early diagnosis of CP. They are listed as:-
– Lake of alertness, decreased spontaneous mobility
– Increased Stereotyped abnormal movements, seizures
– Feeding problems, drooling
– Poor quality of sleep
– Constant fisting after 2 months of Age
– Reduced head circumference or fall in its growth
– Delayed social smile
– Poor head control present at 3 months of age
– Excessive extensor tone or dystonia
– Hypo tonicity with Frog posture or Hyper tonicity
– Deviant Development e.g. Hand Preference before the age of 12
months, sitting in “W” posture
31
– Ramped Synchronised movements
– Persistent asymmetry in posture, Movement and Reflexes
– Delayed appearance of postural reflexes and developmental
milestones
– Persistent primitive reflexes
– Persistent asymmetric tonic neck response
– Scissoring or assumption of equinus position of feet
– Visual problems: roving eyes, no visual following, poor hand
regard after 6 months, persistent squint
– Lack of auditory response.
32
• Persistence of obligatory primitive reflexes >12 months indicator of poor
loco-motor prognosis
• “Primitive reflex profile (PRP)” was developed as a research tool-grading
system for quantitating primitive reflexes, helps in early diagnosis.
General Movements
• These are series of gross movements with variable speed and amplitude
which involve all parts of body but lack a distinctive sequencing of the
participating body parts.
• These are the first movements in human fetus to develop, emerge
before isolated limb movements.
• These are based on careful observation of the maturation of series
general movements (SGM) from soon after birth even in preterm infant,
to 16–20 week’s term age equivalent.
• These are the best expression of functional motor development at this
early age, analogous to later more familiar functional motor milestones.
• At any general movement age the basic characteristics of normal GMs
are participation of all body parts and movement complexity and
variation.
• Heinz Prechtl said, quality of general movements (GMs), accurately
reflects the condition of the nervous system of the fetus and young
infant.
• Age-specific characteristics of normal general movements
GM type Period of presence in weeks’ Description
PMA (post menstrual age)
Preterm From 28 week to 36-38 week Extremely variable movements, including many pelvic tilts
GMs and trunk movements
Writhing Term and during first 2 months Small to moderate amplitude, slow to moderate speed;
GMs writhing quality
Fidgety 6-9 weeks post-term Continuous flow of small and elegant movements occurring
GMs irregularly all over the body, i.e., head, trunk, and limbs, Small
movements superimposed on larger movements
• Keywords to describe the quality of GMs are variation and complexity.
• The majority of infants show normal suboptimal movements, which are
sufficiently variable and complex but not fluent.
33
• Mildly abnormal GMs are insufficiently variable and complex and not
fluent, and definitely abnormal GMs are virtually devoid of complexity,
variation, and fluency.
• cramped synchronized general movements, characterized by a suddenly
occurring en bloc movement, in which trunk and flexed or extended
limbs stiffly move in utter synchrony. These are pathological GM.
• Persistent abnormal GMs, even while passing the transformational
phases at 36 to 38 weeks’ PMA and 6 to 8 weeks post-term high-risk
(70%–85%) for CP.
• Persistent cramped-synchronized GM, invariably develop CP; PPV=100%.
• Prediction best at the age of fidgety GM that is, at 2 to 4 months post-
term.
• Total absence of fidgety movements predicts CP with an accuracy of 85%
to 98%.
34
• Symptomatic hypoglycaemia with seizures
• Septicaemia
35
Evolution of clinical signs in spastic CP
Age/ Stage Signs
Birth Non-specific (features of HIE)
Birth–12 weeks (hypotonic stage) Easily provoked Moro’s/ATNR/ automatic walking
4 - 6 months (dystonic stage) Extensor hypertonia- max in supine and vertical
suspension, Persistent ATNR, Moro, Walking reflexes
7–8 months to 2–3 years (spastic Scissoring, diplegic gait
stage)
SPASTIC DIPLEGIA
36
necrosis of descending fibers of the motor cortex, optic and acoustic
radiations occurs. MRI typically shows scarring and shrinkage in the
periventricular white matter with enlargement of cerebral ventricles.
Locomotor development is more impaired than manipulative skills.
Mental subnormality may coexist, but is less common and less severe
than in spastic tetraplegia. Diplegic children may have normal mental
function and can communicate without difficulty.
Their oromotor and gastrointestinal functions are normal. They often
have learning disabilities, visual perceptual deficits and strabismus.
There is a tendency to fall backwards because of poorly developed
balance reactions.
The first clinical indication of spastic diplegia is often noted when an
affected infant begins to crawl. The child uses the arms in a normal
reciprocal fashion but tends to drag the legs behind more as a rudder
(commando crawl) rather than using the normal 4-limbed crawling
movement.
If the spasticity is severe, application of a diaper is difficult because of
the excessive adduction of the hips. If there is paraspinal muscle
involvement, the child may be unable to sit.
Examination of the child reveals spasticity in the legs with brisk reflexes,
ankle clonus, and a bilateral Babinski sign. When the child is suspended
by the axillae, a scissoring posture of the lower extremities is
maintained.
Walking is significantly delayed, the feet are held in a position of
equinovarus, and the child walks on tiptoe.
Severe spastic diplegia is characterized by disuse atrophy and impaired
growth of the lower extremities and by disproportionate growth with
normal development of the upper torso.
Epilepsy is relatively uncommon and if present is easy to control with
anti-epileptic drugs.
Acquired cryptorchidism is although a rare condition but may occur with
spastic CP, therefore it should be actively checked.
37
SPASTIC HEMIPLEGIA
Age Signs
Birth Rarely diagnosed at birth–asymmetric tone abnormalities or primitive
reflexes may be transient
At 3 months Decreased hand regard
At 4 months Persistence of obligatory grasp
At 5 months Early hand preference
At 6months Tone abnormalities, Asymmetric movements reduced kicking, asymmetric
persistent primitive reflexes (obligatory grasp)/postural reaction
At 12 months Clear signs of hemiplegia
38
walks on tiptoe because of the increased tone in the antigravity
gastrocnemius muscles, and the affected upper extremity assumes a
flexed posture when the child runs.
Ankle clonus and a Babinski sign may be present, the deep tendon
reflexes are increased, and weakness of the hand and foot dorsi-flexors
is evident.
Children with hemiplegia commonly have weakness of the ipsilateral
trapezius muscle, but a normally functioning sternomastoid muscle.
Since both muscles are supplied by the spinal accessory nerve, this may
be a phylogenetic difference, the trapezius behaving like a limb muscle,
and the sternomastoid as an axial muscle.
About one-third of patients with spastic hemiplegia have a seizure
disorder that usually develops in the 1st yr or 2; approximately 25% have
cognitive abnormalities including perceptual and learning problems and
mental retardation. Dyspraxia on the affected side is a late feature.
Sensory defect of cortical type – two point discrimination, and
astereognosis on the affected side are observed in 51% of cases.
Homonymus hemianopia is also frequently present.
Speech and language disorder are seen with both left as well as right
hemiplegia. Mental retardation is less common in hemiplegia.
Pre natal factors are more important in hemiplegic CP. Improved neuro-
imaging technologies have shown that a vascular lesion mostly in the
middle cerebral artery occurring due to hypo perfusion in the pre natal
period is probably the most common association.
MRI is far more sensitive than CT for most lesions seen with CP, although
a CT scan may be useful for detecting calcifications associated with
congenital infections.
In the European CP study, 34% of children with hemiplegia had injury to
the white matter that probably dated to the in utero period and 27%
had a focal lesion that may have resulted from a stroke.
Other children with hemiplegic CP had had malformations from multiple
causes including infections (e.g., cytomegalovirus), lissencephaly,
polymicrogyria, schizencephaly, or cortical dysplasia.
39
Focal cerebral infarction (stroke) secondary to intrauterine or perinatal
thromboembolism related to thrombophilic disorders, like the presence
of anticardiolipin antibodies, is an important cause of hemiplegic CP.
Family histories suggestive of thrombosis and inherited clotting
disorders, such as factor V Leiden mutation, may be present and
evaluation of the mother may provide information valuable for future
pregnancies and other family members.
Cranial nerve involvement is common in hemiplegic children. The facial
nerve is most commonly involved with ipsilateral weakness of mild
degree followed by 6th 12th and 2nd cranial nerve.
Vasomotor changes are also common in the affected limbs, which are
colder than normal and may show cyanosis distally, sometimes with
oedema.
40
– Dysphasia is commoner in acquired than in congenital hemiplegia and
increases in frequency the later the age at which the hemiplegia
develops.
– Less-specific speech defects are also seen such as simple retardation of
speech development, due probably to mental subnormality.
– In congenital hemiplegia the lower limbs have internal rotation, i.e.
(equino-varus deformity) while in acquired hemiplegia, there is
superimposed external rotation (equino-valgus deformities)
41
– windswept deformities of the hips with related pelvic obliquity
leading to scoliosis or kyphoscoliosis
– dislocation of hip, knee-flexion deformities, equinovarus or valgus
at the ankles.
As in diplegia, associated disorders are common, with an increased
incidence of intellectual deficit in approximately 80% of cases, and a high
incidence of visual problems and epilepsy. Speech rarely develops and
when it does it is usually Dysarthric.
In severe cases the ability to move against the gravity is very slight,
Spasticity, hyperreflexia, cranial nerve involvement with pseudo bulbar
palsy, optic atrophy and mental retardation present.
It is observed that 15% of all cases with spastic quadriplegia have
prenatal cause, 27% have combined pre and perinatal factors, and 27%
have post natal cause.
Perinatal insults are observed in a significant proportion of children with
spastic quadriplegia, such as:-
– Prolonged, difficult or precipitate delivery
– fetal distress
– antepartum haemorrhage Intra-ventricular haemorrhage
– Apnoea and convulsions in the newborn period
Intra uterine infections (TORCH) and congenital malformations of brain
are also associated with this type of CP.
The most common lesions seen on pathologic examination or on MRI
scanning are severe PVL and multicystic cortical encephalomalacia.
Holoprosencephaly, schizencephaly, polygyria, agyria or macrogyria may
be found on imaging.
Poly-porencephaly nay be seen, probably indicating an intra-uterine
ischemic episode.
Prognosis for improvement and for any degree of quality of life is very
poor.
42
It is also known as “Extra Pyramidal CP”, and it is less common than
spastic CP and makes up approximately 15-20% of patients with CP.
Unlike spastic CP, upper extremities are more affected than lower
extremities in extra pyramidal CP. Feeding may be difficult, tongue
thrust and drooling may be prominent. Speech is typically affected and
voice modulation is impaired because the oropharyngeal muscles are
involved.
Muscle tone is variable and purposeful movements are poorly executed
and coordinated. Tonic reflexes dominate the posture.
Involuntary movements, which are exaggerated by voluntary
movements, changes in emotion and anxiety or speech are the hallmark
of this type. Impaired volitional activity manifested as uncontrolled and
purposeless movements that disappears during sleep.
Dyskinetic CP has two distinct subtypes:- a). choreoathetoide, b).
dystonic type
Evolution of clinical signs in dyskinetic CP:-
Age Signs
Newborn May have features of early bilirubin encephalopathy
Hypotonic stage (up to 4months) Poor head control, poor feeding
Dystonic stage Primitive obligatory extensor posture, persistent primitive
reflexes
Stage of involuntary movement Choreoathetoid movements/ dyskinesia, deafness, enamel
dysplasia
While involuntary movements dominate the choreoathetoide subgroup,
the dystonic type has more abnormalities in tone and posture and
tendency to retain stereo typed distorted posture.
Speech defects are common, intelligence with in normal range and
seizures are uncommon in choreo-athetoide.
The dystonic type is associated with hypoxia and the neuropathologic
correlate is status marmoratus-marbled appearance of basal ganglia,
which results from diffuse gliosis.
These children have a characteristic appearance with open mouth,
grimacing face and bizarre contractions of limb at every attempted
movement.
The term dystonia refers to the abnormality in tone in which muscles are
rigid throughout their range of motion and involuntary contractions can
43
occur in both flexors and extensors leading to limb positioning in fixed
postures.
Extrapyramidal CP can also be associated with lesions in the basal
ganglia and thalamus caused by metabolic genetic disorders such as
mitochondrial disorders and glutaric aciduria.
Infants withstands 10 minutes of total intrapartum hupoxia-ischemia
without long term consequences, but are very unlikely to survive more
than 20 minutes of this. Of those who have suffered an intervening
period of total hypoxia-ischemia, The typical outcome is one of
dyskinetic cerebral palsy, with or without seizures but with relative
intellectual sparing or normal intelligence.
Extrapyramidal CP secondary to acute intra-partum near total asphyxia is
associated with bilaterally symmetric lesions in posterior putamen and
ventro-lateral thalamus. These lesions appear to be the correlate of the
neuropathologic lesion called “status marmoratus” in the basal ganglia.
In patients with dystonia who have a normal MRI, it is important to have
a high level of suspicion for “segawa disease” (dihydroxy
phenylalanine=DOPA, responsive dystonia), which causes prominent
dystonia that can resemble CP. These patients typically have diurnal
variation in their signs with worsening dystonia in the legs during the
day.
If the part of the body affected predominantly is bulbar muscles, this is
described as congenital suprabulbar paresis or “Worster-Drought
syndrome”.
Kernicterus/ Bilirubin Encephalopathy is associated with neonatal hyper
bilirubinemia, lead to choreoathetoide type of CP and the pathologic
correlate is selective involvement of central grey nuclei. It has two
forms:-
i. Acute Form
– Phase 1 (1st 1–2 days): Poor sucking, stupor, hypotonia, seizures.
– Phase 2 (middle of 1st week): Hypertonia of extensor muscles,
opisthotonos, retrocollis, fever.
– Phase 3 (after the 1st week): Hypertonia.
ii. Chronic Form
44
– First year: Hypotonia, active deep tendon reflexes, obligatory
tonic neck reflexes, delayed motor skills.
– After first year: Movement disorders (choreoathetosis, ballismus,
tremor), upward gaze, sensorineural hearing loss.
Differences between spasticity & dystonia:
Features Spasticity Dystonia
On examination You feel You see
Tendon reflexes Exaggerated Generally normal
Clonus Present Absent
Pathological reflexes Present rare
45
Prenatal factors were thought to be involved in 25% and no cause could
be found in 41%, so that the opportunities for prevention are less in this
group than in ataxic diplegia.
Genetic factors in children with congenital ataxia associated with mental
retardation, suggesting that autosomal recessive inheritance is common.
Prematurity, low birth weight for gestation and perinatal complications
are associated with ataxic CP.
Hydrocephalus in early life is often associated with ataxia or ataxic
diplegia.
Post-natally acquired ataxia and ataxic diplegia are much less common.
Causes include meningitis, hydrocephalus complicating this, viral
encephalitis (especially varicella), trauma (accidental and non-
accidental), surgical lesions of the cerebellum and the effects of their
surgical treatment.
A careful scrutiny of the family history may indicate a familial and
genetic condition such as metachromatic leucodystrophy, ataxia-
telangiectasia or Friedeich's ataxia, should be recognised and ruled out
while making diagnosis of Ataxic CP for prognostic and genetic reasons.
The disequilibrium syndrome; A case has been made for separating off
from the main body of children with ataxic-CP a subgroup in which the
condition seems sometimes to be inherited as an autosomal recessive
character. These children show severe retardation in motor
development, seldom walking independently under 9 years of age.
Spasticity, mental retardation and various sensory deficits may coexist.
HYPOTONIC or ATONIC CP
46
Foster’s sign: Child with atonic CP, when suspended while held under
the arms, flexion of both legs at the hip occurs usually positive in atonic
CP.
MIXED TYPE CP
TONE
47
Passive tone is range of motion around a joint; active tone is
physiological resistance to movement.
Scarf Sign: Baby is supine and head is maintained in the midline. Arm is
held at the wrist and pulled across the chest towards the opposite
shoulder. In a normal-term infant the elbow does not quite reach the
mid-sternum, whereas the elbow of a hypotonic infant extends beyond
the midline with ease.
Popliteal Angle: With infant in a supine position, the thigh is held in the
knee chest position by supporting the thigh with examiner’s left hand.
The leg is then extended by gentle pressure with examiner’s right hand
index finger placed behind the ankle and popliteal angle is measured.
Normal-term infants allow extension of the knee to approximately 80
degrees.
Abductor Angle; Infant lies supine with legs extended and head in the
midline. Both hips are abducted maximally by holding the knee with
index finger cutting over the front of thighs. The angle between thighs is
the abductor angle.
Hypotonia refers to abnormally diminished tone and is the most
common abnormality of tone in neurologically compromised neonates.
A hypotonic infant is floppy and often assumes a frog-leg posture at rest.
Hypotonia can reflect pathology of the cerebral hemispheres,
cerebellum, spinal cord, anterior horn cell, peripheral nerve,
neuromuscular junction, or muscle.
Spasticity is characterized by an initial resistance to passive movement,
followed by a sudden release, referred to as the clasp-knife
phenomenon.
Rigidity, seen with lesions of the basal ganglia, is characterized by
resistance to passive movement that is equal in the flexors and
extensors regardless of the velocity of movement (lead pipe).
Strength while examination of muscle power, one should include all
muscle groups. It is important not only to assess individual muscle
groups, but also to determine the pattern of weakness (i.e., proximal vs
distal; segmental vs regional).
Testing for pronator drift can be helpful in localizing the lesion in a
patient with weakness. This test is accomplished by having the patient
48
extend his or her arms away from the body with the palms facing
upward and the eyes closed. Together, pronation and downward drift of
an arm indicate a lesion of the contralateral corticospinal tract.
Muscle tone norms:-
ASSESSMENT OF SPASTICITY
49
Grade 5—Affected part is rigid in flexion or extension.
Modified Tardieu Scale
Quantify spasticity by assessing muscle response to stretch at
given velocity
Measures velocity-dependent nature of spasticity
It is a 6-point rating scale for describing the quality of muscle
reaction
Inter rater reliability good
Considered superior to MAS for spasticity measurement
Scoring:-
0 --No resistance throughout the course of the passive movement
1 --Slight resistance throughout the course of passive movement,
no clear catch at a precise angle
2--Clear catch at a precise angle, interrupting the passive
movement, followed by release
3-- Fatigable clonus with less than 10 seconds when maintaining
the pressure and appearing at the precise angle
4--Unfatigable clonus with more than 10 seconds when
maintaining the pressure and appearing at a precise angle
5--Joint is immovable
Velocities:-
V1: As slow as possible, slower than the natural drop of the limb
segment under gravity
V2: Speed of limb segment falling under gravity
V3: As fast as possible, faster than the rate of the natural drop of
the limb segment under gravity
Interpretation:
The point at which a ‘‘catch’’ or clonus is felt during a quick stretch
of the muscle at a velocity V3 determined the angle of muscle
response (R1)
Angle of full range of motion (R2) is equivalent to the passive
range of motion when the muscle is stretched at V1
Difference between (R1) and (R2) indicates the relative
contribution of spasticity versus contracture
50
A large difference between (R1) and (R2) indicates more spasticity
whereas a small difference indicates more contracture
Other objective spasticity rating scale
Percent of function Global pain scale
scale Adductor tone rating
Disability scale scale
Spasm frequency scale
Developmental Tests: These tests describe the development of the child
in various functional stages.
Denver Development Screening Test: evaluates the developmental
deficits in infants and young children from age 1 month to 6 years
in the areas of global motor function, language, fi ne-motor
adaptation and social contact.
It is not an IQ test nor will it predict what the level of the child’s
future intelligence and ability will be.
Bayley Scales of Infant Development: evaluates cognition,
language, social behaviour and motor functions in children from 1
to 42 months old. The purpose is to diagnose developmental
delay.
51
developmental evaluation are mandatory for accurate diagnosis and
follow up.
The essential Clinical findings include:-1. Motor delay, 2. Abnormalities
of tone and reflexes, 3. Absence of regression, must be present in a
patient before labelling as CP.
A careful family history is essential. A history of motor deficit in a sibling
or parent or of parental consanguinity will raise the suspicion of a
genetically determined disorder and the relatives concerned should be
examined.
Algorithm for the evaluation of the child with cerebral palsy (CP)
1. Confirm that History does not suggest a progressive or degenerative disorder of CNS
2. Assure that Clinical features suggestive of progressive or degenerative disorder of CNS are
not present on examination
3. Classify the Type of CP as per recent guidelines
4. Screen for associated comorbid conditions:-
– Developmental delay/ mental retardation
– Ophthalmologic/ hearing problems
– Speech and language delay
– Feeding and swallowing difficulties
– If history of seizure is present than obtain an EEG
Did the child have undergone previous neuroimaging study or any other lab. Study ? (e.g. in
neonatal period) that determined the aetiology of CP ?
Yes No
No need for further Obtain neuroimaging study (MRI
diagnostic tests preferred over CT-scan)
52
By accurate family history cases of Friedreich’s ataxia, ataxia-
telangiectasia, Lesch-Nyhan syndrome, myotonic dystrophy and other
diseases may be recognized.
The outcome of other pregnancies is often relevant to the diagnosis of
CP; prematurity, neonatal jaundice and births are common occurrences
in the family history.
Accurate determination of the aetiology of CP has specific implications
regarding treatment, prognosis, and on-going medical management of
associated conditions.
The importance of determining whether there is a malformation, genetic
aetiology, or injury and whether the injury is due to an acquired pre-,
peri-, or postnatal process has obvious significance from the point of
view of assessment of recurrence risk, counselling of families, and
implementation of prevention programs, and when medico-legal issues
arise. Determining causality also helps limit further unnecessary testing.
Finally, understanding the aetiology of CP has implications for
prevention and intervention strategies.
Injury to the brain as a potential cause of cerebral palsy in the postnatal
period can be demonstrated by performing fetal MRI.
Fetal MRI is very recent, emerging and promising technology for the
evaluation of the fetal CNS.
The most common reason for performing fetal MRI-CNS has been
ventriculomegaly. The aetiology of ventriculomegaly:- hydrocephalus
from obstruction (e.g. aqueductal stenosis), malformation (e.g.
holoprosencephaly), or destruction of brain tissue (e.g. periventricular
leukomalacia, infarction).
Recent studies encompassing a total of 1,426 children who underwent
either CT or MRI scans indicated an abnormality in 62% to 100% of
individuals (mean for CT, 77%; for MRI, 89%). For the combined CT and
MRI class I studies (n = 238), 88% of children had abnormal scans.
MRI is the modality of choice and provides definitive information in the
postnatal evaluation of the term infant in whom hypoxic ischemic brain
injury has occurred and in whom CP will eventually be diagnosed.
– Many recent studies for e.g. European cerebral palsy study
involving 644 children with CP who had MRI scans found
53
abnormalities in 89% (range 68% to 100%). The yield on MRI
depended on the type of CP that was present
(mixed>quadriplegic>hemiplegic>diplegic>ataxic>dyskinetic>hypo
tonic)
– It suggests, MRI is helpful in determining whether the injury was
prenatal, perinatal, or postnatal in onset. It states that with
different types of CP, onset was prenatal in 37%, perinatal in 35%,
and postnatal in 4%.
– MRI scans showed that white-matter damage of immaturity,
including periventricular leukomalacia (PVL), was the most
common finding (42.5%), followed by basal ganglia lesions
(12.8%), cortical/subcortical lesions (9.4%), malformations (9.1%),
focal infarcts (7.4%), and miscellaneous lesions (7.1%). Only 11.7%
of these children had normal MRI findings.
– Recently SCPE has acknowledged a MRI-Based radiologic scoring
system for extent of brain injury in children with Hemiplegia,
which covered 7 aspects across 4 domains: 1) number of affected
lobes, 2) volume and type of white matter injury, 3) extent of gray
matter damage, and 4) major white matter tract injury, which
demonstrated high inter- and intrarater reliability and was
significantly associated with MACS classification and motor
evaluations.
CT scan in a child with CP may on occasion [0%-22.5%] detect conditions
that are surgically treatable that might not be detected by neurologic
examination, like hydrocephalus, porencephaly, arteriovenous
malformation, subdural hematomas and hygromas, and vermian tumor.
CT (as well as MRI) may detect abnormalities that suggest a potentially
treatable inborn error of metabolism.
– The yield of finding an abnormal CT scan in a child with CP is good
(average 77%) and depending on the type of CP
(hemiplegic>ataxic>mixed>diplegic>quadriplegic>hypotonic>dyski
netic) with the percent abnormal in those with dyskinetic CP being
much lower than in other forms of CP.
– When abnormal, CT scans are helpful in delineating the timing of
the aetiology of CP in most children. Timing of injury based on CT
54
scan findings are 32% in prenatal, 50% perinatal, 12% postnatal
and in 6% unclassifiable.
Recommendations for Neuroimaging
– Neuroimaging is recommended in the evaluation of a child with CP
if the etiology has not been established.
– MRI, when available, is preferred to CT scanning because of the
higher yield of suggesting an etiology and timing of insult leading
to CP.
Metabolic and Genetic work-up Metabolic or genetic causes for CP
occur infrequently (i.e. 0–4%). Neuroimaging studies have shown that 7
to 11% of children with CP will have a brain malformation suggesting
additional risk for genetic and possibly a metabolic etiology.
– Metabolic and genetic studies should not be routinely obtained in
the evaluation of the child with CP.
– If the clinical history or findings on neuroimaging do not
determine a specific structural abnormality or if there are
additional and atypical features in the history or clinical
examination, metabolic and genetic testing should be considered.
– Detection of a brain malformation in child with CP warrants
consideration of an underlying genetic or metabolic etiology.
Evidence indicates that cerebral infarction due to pre- or perinatal
cerebrovascular occlusion occurs in 13 to 37% of children with
hemiplegic CP, most likely aetiology of cerebral infract to be due to
coagulopathies. So, diagnostic work-up for coagulation disorder should
be considered.
DIFFERENTIAL DIAGNOSIS
The basic question to be asked at the outset, and repeatedly later on, is
whether the condition is indeed non-progressive, conforming to the
definition of CP, or whether it may be due to a progressive, conforming
to the definition of CP, or whether it may be due to a progressive
disorders such as a degenerative CNS or neuromuscular disease.
CP is neither a specific disease nor a pathological or etiological entity.
Common neuro-degenerative disorders and neuro-metabolic disorders
which are mimics of CP should be ruled out.
55
A. Conditions Presenting with True Muscle Weakness, Which is Not Seen in
CP Although Hypotonia and Poor Motor Planning Often Give the
Impression of Apparent Weakness:-
– Muscular dystrophies: Duchenne and Becker types.
– Infantile neuro-axonal dystrophy.
– Mitochondrial cytopathies.
– Few Cerebral white matter diseases
B. Conditions with Significant Dystonia/ Involuntary Movements:-
– DOPA responsive dystonia.
– PKAN—pantothenase kinase associated neurodegeneration.
– Pyruvate dehydrogenase deficiency.
– Glutaric aciduria type-I.
– Leigh syndrome.
– Juvenile neuronal ceroid lipofuscinoses.
– Rett syndrome.
– Pelizaeus-Merzbacher disease.
– 3-methyl-glutaconic aciduria.
– 3-methylcrotonyl co-enzyme A carboxylase deficiency.
– Lesch Nyhan syndrome.
C. Conditions with Predominant Diplegia or Quadriplegia:-
– Adrenoleukodystrophy.
– Adrenomyeloneuropathy.
– Arginase deficiency.
– Metachromatc leukodystophy.
– Hereditary progressive spastic paraplegia.
– Holocarboxylase synthetase deficiency.
– Prenatal iodine deficiency (“neurological cretinism”).
D. Conditions with Ataxia (Ataxic CP is Rare)
– Angelman syndrome.
– Niemmann-pick disease type C.
– Ataxia-telengiectasia.
– Ponto-cerebellar hypoplasia or atrophy.
– Chronic/adult GM2 gangliosidosis.
– Mitochondrial cytopathy (NARP mutation).
– Posterior fossa tumors.
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– X-linked spino-cerebellar ataxia
E. Conditions with Significant Bulbar and Oral Motor Dysfunction
Worster-drought syndrome/bilateral perisylvian/ opercular
syndrome.
F. Slowly Progressive Disorders Sometimes Misdiagnosed as Cerebral Palsy
Polyneuropathy
• GM1 gangliosidosis type-II
• Hereditary motor and sensory neuropathy
• Infantile neuroaxonal dystrophy
• Metachromatc leukodystrophy
Ataxia
• Abetalipoproteinemia
• Ataxia telangiectasia
• Friedreich ataxia
Spasticity—Chorea
• Familial spastic paraplegia
• Glutaric aciduria type-I
• Lesch-Nyhan Syndrome
• Niemann-Pick disease type C
• Pelizaeus-Merzbacher disease
• Rett syndrome
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v. To assess for the other co morbidities
vi. To assess general health
vii. To assess the family functioning
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– Physical medicine and – Anaesthetist
rehabilitation specialist – Dietician
– Child psychologist – Radiologist
– Ophthalmologist – Social worker
– Otolaryngologist – Counsellor.
– Orthopedician
Neurological Gastrointestinal
Miscellaneous • Equinus
• Hip subluxation/ dislocation
• Sleep problems
• Scoliosis
• Lower urinary tract dysfunction
• Hamstring contracture
• Emotional problems
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– Motor deficits in children with spastic CP appear to correlate with
the severity of cognitive deficits, in contrast with those children
with dyskinetic CP, in whom this relationship is lacking.
– Laterality of hemiplegia may also be a contributing factor; those
children with right hemiplegia may be more likely to have
impaired language function due to left hemisphere injury,
although this remains controversial.
– There is also a strong association between greater intellectual
impairment in children with CP and the presence of epilepsy, an
abnormal EEG, or an abnormal neuroimaging study.
– Quadriplegic CP has highest risk for learning disability while
hemiplegic CP has lower risk, and other types of CP have
intermediate risk.
Psychological/ behavioural problems
– A diagnosis of CP is extremely stressful for the family and the child
when he grows up. This causes various reactions ranging from
denial to anger, guilt and depression.
– Other features include attention n deficit hyperactivity disorder,
tantrums, social naivety, Passivity and autistic spectrum disorders.
– Behavioural problems found in 30-50% of CP patients.
– Coping with the emotional burden of disability is easier if the
family has strong relationships, financial security, and supportive
members of the community.
Ophthalmological Impairments
– Visual impairments and disorders of ocular motility are common
20-30% (avg.28%) in children with CP.
– There is an increased risk of strabismus, amblyopia, nystagmus,
retinal abnormalities, cortical visual impairment, Optic nerve
hypoplasia, severe myopia, optic atrophy, and refractive errors.
– The condition causing CP such as congenital cytomegalo-virus,
toxoplasmosis or rubella may also have caused a cataract,
colobomas or retinopathies.
– Retinopathy of prematurity is amenable to control by laser
therapy or cryo-therapy.
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– Other problems like micropthalmia, bupthalamos or congenital
glaucoma.
– In severe cases, the optic nerves may also be damaged. Loss of
coordination of the muscles controlling eye movements is very
common. The child cannot fix his gaze on an object. In half of the
cases, binocular vision does not develop. Myopia is a concomitant
problem.
– If these ophthalmological problems not diagnosed and managed
early, visual deficits can interfere with developmental progress
and rehabilitation.
– Strabismus can lead to permanent mononuclear vision loss or
amblyopia.
Hearing impairment
– Hearing impairment occurs in approximately 12% of children with
CP.
– This is more common if the etiology of CP is related to very low
birth weight, kernicterus, Intra Uterine infections, neonatal
meningitis, or severe hypoxic-ischemic insults, or use of ECMO.
– Children with CP who have mental retardation or abnormal
neuroimaging studies are at greater risk for hearing impairment.
– Either from associated sensory-neural loss or from secretory otitis
media, these are very common in children with Eustachian tube
dysfunction in bulbar palsy.
– Of concern are recent studies from the centers for disease control
that almost 50% of the children found to have severe congenital
hearing loss (with or without CP) in the greater Atlanta area were
not recognized until almost age 3 years.
– In Neonate hearing assessment can be done through:-
• Brainstem evoked auditory response (BERA)
• Auditory response cradle (ARC)
• Post-auricular myogenic response (PAM)
• Oto-acoustic emissions (OAE).
– In older children:-
• Behaviour observation audiometry
• Distraction test
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• Conditioning audiometry
• Pure tone audiometry.
Speech And Language Disorders
– Because of bilateral cortico-bulbar dysfunction in many CP
syndromes, anarthric or dysarthric speech and other impairments
related to oro-motor dysfunction are common.
– For example, articulation disorders and impaired speech
intelligibility are present in 38% of children with CP.
– Jaw stability, lip closure and spontaneous swallowing are
considered prerequisites for the normal development of speech,
feeding and drooling control. The rhythmicity of oral feeding
movement and of speech production is related.
– Because their impaired mobility can cause limited interaction with
individuals in the environment, children with CP might not be able
to develop the linguistic skills needed to develop more complex
speech patterns.
– Language (as opposed to speech) deficits in CP go hand-in-hand
with verbal intellectual limitations associated with mental
retardation.
– The Cochrane data base has determined the effectiveness of
speech and language therapy by improving communication skills
in children with CP.
Epilepsy
– Data from recent studies suggests that 43% (range 35 to 62%) of
children with CP develop epilepsy.
– The prevalence of epilepsy also varies depending on the type of
CP that is present. Children with spastic quadriplegia (50–94%) or
hemiplegia (30%) have a higher incidence of epilepsy than
patients with diplegia or ataxic CP (16 to 27%).
– Children with CP who have abnormal neuroimaging studies are
more likely to have epilepsy. 54% of children with CP and an
abnormal CT had epilepsy in contrast 27% of those who had a
normal scan.
– Children with CP had a higher incidence of epilepsy with onset
within the first year of age (47% versus 10%), history of neonatal
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seizures (19% versus 3%), status epilepticus (16% versus 1.7%),
need for poly therapy (25% versus 3%), and treatment with
second-line antiepileptic drugs (31% versus 6.7%).
– They also had a lower incidence of generalized seizure (28%
versus 59%) and of remaining seizure-free (37% versus 90%).
Factors associated with a seizure-free period of 1 year or more in
epileptic children with CP include normal intelligence, single
seizure type, monotherapy, and spastic diplegia.
– Recommendations derived from many studies, An EEG should not
be obtained for the purpose of determining the etiology of CP.
– An EEG should be considered when a child with CP has a history or
examination features suggesting the presence of epilepsy or an
epileptic syndrome.
Feeding problem
– 30 to 80% of children with CP feed with difficulty.
– Feeding problem due to bulbar palsy, handling and seating
difficulties, involuntary movements or posture and tone problems
are a common first presentation.
– Lip closure and jaw stability develop early in infancy. By 6 months
of age, the child can produce bilateral vocalization (p, b and m). By
36 months the child’s jaw stability is nearly fully-developed.
Infantile pattern of swallowing persists until about 18 months of
age.
– The children are prone to oral hypersensitivity and to bite
whatever is put in their mouths. Parents are taught to desensitize
the child’s mouth before feeds. The reverse may be the case and
the child’s mouth may need stimulation during feed.
– When this is chronic, the chest develops an increased antero-
posterior diameter. Gastro-esophageal regurgitation may occur
leading to postprandial dyspepsia or heartburn and vomiting.
These symptoms may provoke crying or reflex syncope. The
bizarre neck and trunk movements of Sandifer's syndrome are
often noted.
– They are especially at risk because of oral, pharyngeal or
esophageal Dysphagia and due to oral motor dysfunction.
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– Furthermore because of communication difficulties many of them
are unable to request food and drink.
– Presence of seizures worsens the feeding intake. As a result they
do not receive adequate nutrition resulting in growth retardation.
– Due to in-coordination of the swallowing reflex these patients
have tendency for aspiration and they suffer from recurrent
respiratory illness.
– Feeding may take the parents 4 to 6 hours a day, and it is
punctuated by repeated spillage of food, coughing, choking or
regurgitation.
– Hypotonia, stiffness of temporo-mandibular joint during after an
episode of seizures and post ictal drowsiness or parental fear to
feed them also contributes to reduction in total caloric intake of
these children.
– Feeding problems are further compounded by unawareness of
parents about the disease and associated problems. The main
reasons for the lack of awareness are illiteracy, misconception
about disease and associated problems.
– Gastro-esophageal reflux occurs in up to 75% of children with CP
because of abnormal peristalsis and lower esophageal sphincter
function. It can occur without over vomiting. Reflux can cause
chronic esophagitis with painful swallowing, anemia, and stricture
formation.
Drooling or Sialorrhea
– Drooling or sialorrhea, defined as the anterior spillage of saliva
from the mouth, an estimated 10% of children with CP have
severe drooling.
– Drooling is a normal finding in infancy but usually resolves by age
15 to 18 months as oral motor and sensory functions improve.
Beyond age 4 years, drooling while awake is considered abnormal.
– Drooling can be Result of:-
• Poor jaw or body stability that results in insufficient
swallowing movements and poor lip closure
• Poor sensory feedback
• Teething (considered normal)
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– Drooling severity:-
• Dry = never drools
• Mild = wet lips only
• Moderate = wet lips and chin
• Severe = clothing becoming damp
• Profuse = clothing, hands, tray, objects become wet
– Drooling problem frequently contributes to social isolation and
loss of self-esteem.
– Additional concerns are in areas of hygiene and spread of
secretion-born pathogens, particularly as the affected child moves
into school and community settings.
– Drooling can cause skin irritation about the face and has been
reported to contribute to dehydration.
– Finally, drooling may contribute to malfunction or damage of
assistive technology or equipment needed to promote
independence and social inclusion.
– Drooling should be viewed as one aspect of a spectrum of
impairments related to oral motor and oral sensory function that
also includes speech (i.e. articulation) problems, feeding
difficulties, and swallowing dysfunction, including aspiration.
Incontinence
– It is persistent for children with severe cerebral palsy. It is also a
problem, alongside enuresis, for many less severely affected
children for longer than is seen in most children.
– Day time urinary incontinence the commonest presenting
symptom. Hyper-reflexic detrusor contractions with reduced
bladder capacity also considerable feature observed along with
detrusor-sphincter dyssynergia (DSD).
– Toileting routines should be attempted when the child is ready
developmentally.
Constipation
– Constipation is a risk in children who are inactive and if this is
allowed to persist there is a risk of acquired mega colon, a lot of
colicky pain after meals and soiling with overflow. Sometimes
constipation can build-up when the child is unwell.
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Sleep problems
– It is very common for children with CP to be difficult to settle at
night, or to wake up unduly frequently during the night.
– For those with four limb involvement or with severe spasticity in
the legs the lack of activity in the day does not leave them tired at
night.
– Seen in about a quarter of cases
– Difficulty falling asleep
– Frequent night awakening
– Disturbed sleep schedule
– Causes: Behavioral, neurologic, and physical
– Biologic clock abnormal
– Physical discomfort.
Respiratory Problems
– Ineffective cough reflex, weak respiratory muscles, spasticity of
the chest wall.
– Increased risk of upper airway obstruction
– Chronic obstructive sleep apnea
– Chronic aspiration or reflux
– Severe scoliosis and obesity can both cause restrictive lung
disease
– Intervention
– Early Assessment with Polysomnography (PSG) and other tests
– Early management of Gastroesophageal reflux (GER) and scoliosis
– Prevention of obesity.
Pain Problems
– Significant proportion (75%), Mild –Severe variety
– Pain is potential risk factors for many psychological problems like:-
• Stubbornness, aggressiveness,
• self-abusive behavior as biting, head banging, or scratching.
Malnutrition
– Children with CP frequently grow poorly and have high
prevalence of poor growth, because of both neurological and
nutritional causative mechanisms.
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– Nutritional factors, primarily protein energy malnutrition, have
been postulated as the major determinant of poor growth due to
inadequate intake, excess losses, and possibly altered energy
requirements (required extra calories due to spasticity/athetosis).
– Retrospective clinical studies of enteral feeding in children with
CP suggest that aggressive nutritional supplementation may
improve linear growth in some instances, especially when
instituted early in life.
– The non-nutritional factors include seizures, non-ambulation, and
spastic quadriparesis.
– Nutritional rehabilitation is also reported to improve the
impairment of immune system, cognitive ability and
neuromuscular function associated with malnutrition.
– An index of linear growth is essential in the assessment of
children, but is difficult to obtain in children with CP, mainly
because of fixed contractures.
– Errors in conventional measures of body size (e.g. height or
recumbent length) arise from the spasticity and joint contractures
in these children, making reliable evaluation of body size difficult.
– Recent work has documented the utility and accuracy of upper-
arm or lower-leg lengths as replacements for unreliable
measures of height.
– Upperarm length is preferred to the lower-arm length because
the acromion is easier to palpate than the ulnar stylion and
Lower-leg length is the only measure of growth of the lower body
segment for which suitable standards are available which do not
encompass a joint.
– Use of triceps skin-fold thickness, using cut-off value of <10th
centile for age and sex, is recommended to screen for suboptimal
fat stores in children with CP.
– Bone age delay is common in children with CP (68% with a delay
of more than 1 year). The linear growth of children with CP is
similar to that of children without CP when bone age is used
instead of chronological age.
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– Anthropometric measurements must be recorded once in 3
months in children less than 5 years and once in 6 months in
children more than 5 years to document the growth patterns and
nutritional status.
Orthopaedic problems
– To assess Probability and severity of developing orthopaedic
problems in a child with CP, GMFCS is very essential.
– GMFCS level 1,2 and 3 more likely to learn stand and walking and
may have milder form of deformity like equinus at ankle, while
GMFCS level 4 &5 are more likely to have severe deformity,
contractures, scoliosis or hip subluxation.
– A contracture is a deformity around a joint that cannot be
corrected by simple stretching.
– Viewing deformity retrospectively, it is seen that most start off as
postural deformities-tendencies to spend most time in certain
positions.
– They can go on to become fixed deformities, where the non-bony
structures become shortened or lengthened and then structural
deformities, where there are actual bony changes.
– Their lack of postural ability makes people with CP prime
candidates for deformity in two ways:
• There is paucity of movement, with a few preferred
positions and a lack of variety of positions.
• There is excess activity in specific muscle groups in an effort
to make up for lack of ability.
– Asymmetrical complex of deformity:-
• Scoliosis
• Preferred head turning, possibly with related gaze defects.
• Preferred hand use, due to more weight being taken on one
side of the body, with the contralateral hand relatively
freed for use.
• Wind-swept hips
• Ankle and foot deformity
– The clinical features of a child have been recorded as primary
impairment (type of motor disorder, associated co-morbidity and
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limbs distribution) and secondary impairment (deformity level),
functional ability (gross motor abilities, hand function abilities, self
care abilities) and social consequence (schooling).
– Presence of hypo- or hyper-mobility in a joint was the criteria for
considering the joint as deformed.
– Based on total no. of deformed joints, each child has been
classified in four categories:-
1) NIL; all joints have full passive range of motion
2) Mildly deformed; 15% joints have limitation
3) Moderately deformed; 45% joints have limitation
4) Severely deformed; 75% joints have limitation
5) Profoundly deformed; >75%joints have limitation
– Hip sub-luxation and dislocation is caused by muscle imbalance
around the joint. Mean age at first registration of hip
displacement was 4 years, but some hips showed migration
percentage (MP) >40% already at two years of age. The hip is
particularly at risk of dislocation when the range of passive hip
abduction (in flexion) reduces below 45°.
– Risk of hip displacement (MP >40%) was directly related to the
level of gross motor function. Zero percent in children in GMFCS
level I to 64% in GMFCS level V.
– Osteopenia is a risk in immobile children with cerebral palsy, with
consequent increased risk of fractures. While enabling children to
spend part of each day in a standing frame and promoting physical
activity, e.g. with hydrotherapy, is likely to promote improve bone
density, use of bisphosphonates for 12-18 months has been
shown to increase bone density by 20-40 %, with no apparent
adverse effects.
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Many neuro-protective strategies once considered for infants have been
tried in adult stroke, and most have failed.
In premature infants, an increase in survival without a decrease in
prevalence added more healthy citizens but also more disabled children
to the population.
Now, however, drop in prevalence of CP in one sector of preterm may
be occurring. Until recently, strategies for primary or secondary
prevention of CP were apparently ineffective, but of late there is
evidence that some proportion of CP may be preventable.
Randomized clinical studies have demonstrated no decrease in CP due to
birth asphyxia by fetal monitoring in labour (Primary prevention).
Secondary prevention of cerebral palsy due to birth asphyxia by
hypothermia Cochrane review (2009) shows there is evidence from the
eight RCTs included in this systematic review that therapeutic
hypothermia is beneficial to term newborns with hypoxic ischemic
encephalopathy. Cooling reduces mortality without increasing major
disability in survivors.
Despite efforts to prevent prematurity, available strategies has not yet
been consistently successful at decreasing the frequency of preterm
birth in the US.
To date, there is no evidence-based approach to prevent intrauterine
exposure to infection and inflammation.
Identification of factors leading to thrombosis of fetal or neonatal stroke
is still a work in progress.
Prevention of congenital maldevelopment, except in folate deficiency or
with use of acne or antiepileptic medications, is in general not yet
feasible.
Prevention of neurologic disability due to complications of multiple
gestations is possible through lowering the rate of multiple gestations by
avoidance of pregnancy in older women and by avoidance of
implantation of multiple foetuses in IVF.
Genetics and cerebral palsy: Familial aggregation of CP is observed in
groups with high consanguinity, and a Swedish national database
indicates increased familial risk for CP. Inherited thrombophilia’s occur in
greater numbers of children with perinatal stroke than in the general
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population, in very preterm infants with CP, and in CP overall. These can
be prevented by avoiding consanguineous marriages.
There are also genetic components of a number of risk factors for CP,
including preterm birth, abruption of the placenta, chorioamnionitis, and
pre eclampsia.
LEVELS OF PREVENTION OF CEREBRAL PALSY/MENTAL RETARDATION
– It is estimated that around 25% to 30% of mental retardation (MR)
is potentially preventable by public health measures such as
improving the nutritional status, access to basic medical facilities,
and good pre and perinatal care.
A. Primary Prevention—preventing the occurrence of retardation:-
– Health Promotion-
• Health education, especially for adolescent girls.
• Improvement of nutritional status in community.
• Optimum health care facilities.
• Improvement in pre, peri and postnatal care.
– Specific protection-
• Universal iodization of salt.
• Rubella immunization for women before pregnancy.
• Folic acid administration in early pregnancy.
• Prevention of teratogen exposure (e.g. drug, radiations)
• Prenatal/ ante-natal Aneuploidy scan
• Genetic counselling and prenatal diagnosis.
• Detection and care for high-risk pregnancies.
• Prevention of Rh-isoimmunization.
• Universal immunization for children.
B. Secondary Prevention— halting disease progression by Early Diagnosis
and Treatment:
– Neonatal screening for treatable disorders--hypothyroidism,
phenylketonuria, galactosemia, homocysteinuria, congenital
hydrocephalus.
– Intervention with ‘at risk’ babies.
– Early detection and intervention of developmental delay.
C. Tertiary prevention—preventing complications and maximization of
functions, by Disability Limitation and Rehabilitation:-
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– Stimulation, training, and education, and vocational opportunities.
– Mainstreaming/integration.
– Support for families.
– Parental self-help groups.
ANTENATAL APPROACHES TO PREVENTION
– Adequate antenatal check-up (iodized salt, folic acid, etc.)
– Reduce the rate of preterm birth
– Limiting the number of embryos transferred with IVF
– Screening and treatment of asymptomatic bacteriuria during
pregnancy
– Erythromycin for women with premature rupture of the
membranes
– Anti-platelet drugs to prevent pre-eclampsia
– Calcium channel-blockers and an oxytocin antagonist (atosiban)
for women with preterm labour
– 17a progesterone caproate and cervical cerclage for women with
previous preterm birth and short cervix (i.e. <2.5 cm)
– Treatment of mothers expected to deliver prematurely
glucocorticoids (e.g. B-methasone)
– Prevention of hyper-bilirubinemia (RH incompatibility).
POSTNATAL APPROACHES TO PREVENTION
– Hospital-based safe and aseptic delivery.
– Aggressive phototherapy for hyper-bilirubinemia.
– Postnatal steroids given to premature infants to decrease the risk
BPD increase the risk CP: limiting the use of this treatment.
– There are many interventions which could prevent cerebral palsy.
These may be considered as follows:-
– PROVEN
• MMR vaccination.
• Iodine supplementation in iodine-deficient areas.
• Anti-D for Rh-negative women.
• Reducing methyl mercury exposure.
• Transfer of the very preterm infant in-utero to tertiary
centre.
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• Reducing embryos transferred in in fertility treatments to
three or less.
• Fencing swimming pools.
• Home support for socially-disadvantaged parents.
– PROBABLE
• Early routine ultrasound.
• Avoiding excessive alcohol intake in pregnancy.
• Phototherapy for neonatal jaundice.
• Vitamin K at birth to prevent brain haemorrhage.
• Infant restraints in cars.
• Child abuse prevention strategies.
• Sudden infant death syndrome prevention strategies.
– POSSIBLE
• Mode of delivery for very preterm births including breech
presentation.
• Zinc folate or fish oil to prevent intrauterine growth
retardation (IUGR).
• Methods of detecting IUGR.
• Operative delivery for fetal distress.
• Amnioinfusion for umbilical cord compression.
• Rescue therapy for birth hypoxia-ischemia.
– DOUBTFUL
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MANAGEMENT OF CEREBRAL PALSY AND COMORBIDITIES
Multidisciplinary management under one roof with various specialists
and a Family-Centered care is important. Paediatric neurologist
involvement for detailed clinical neurological examination, diagnosis and
treatment forms core of multidisciplinary management.
The successful treatment of cerebral palsy obviously relies on accurate
assessment but assessment of what? This will depend to a great extent
on the child's age but will always cover certain areas:-
the residual neurological ability (and its likely development)
the anatomical restrictions (and the propensity to deformity), And
Physical Ability
the child's overall developmental level and other disabilities (e g
vision, epilepsy)
The child's social context (Oral Competence and Communication
Behaviour)
Aims of therapy
– To relieve pain & discomfort – Contain contractures
– Improve function & mobility – Communication & education
– Facilitate care – Socio-emotional development
Components of therapy
1. Physiotherapy 3. Speech stimulation and
2. Occupational therapy therapy
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4. Multisensory stimulation 7. Aids and appliances
5. Behaviour modification 8. Surgery
6. Medication and vaccination
9. Management of associated problems
10.Other therapies
11.Counselling and social support groups
Benefits of Early Intervention
– Anatomical: Maintains soft tissue extensibility optimizes musculoskeletal
growth and development.
– Physiological: Early visual and sensory inputs optimize movement,
posture and balance.
– Developmental: Reinforces positive developmental patterns.
– Reduces cost of treatment, minimizes complications and mitigates need
for adaptive equipment.
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since she can usually manage at best to treat the child for some hours
each week, and her aim must largely be to teach the parents to be their
own child's therapists.
Rationale for early physiotherapy in CP is based on Plasticity of brain.
The intact neurons in the brain may substitute for lost function, new
synapses may form and re-organisation of neurons take place so that the
child gains function as he grows. This process is termed neuronal
plasticity.
Aims of physiotherapy:-
– Reduction of spasticity
– Prevention of abnormal posture
– Prevention of contractures and deformities
– Inhibition of primitive reflexes
– Facilitation of normal patterns of movement
– Stimulation of sensory, cognitive and perceptual functions.
There are various methods and techniques used, enlisted below:-
i. Neurodevelopmental technique (Baboth)
ii. Kabot and Knott method
iii. Philps and Denver method
iv. Fay’s method
v. Pohl’s method
vi. Vojta method.
Neurodevelopmental technique (NDT- Baboth): It is most popular and
most widely used and aim is to first normalize the muscle tone and
stimulate normal movement by:-
– Inhibiting the pathological tonic reflex activity by use of reflex
inhibiting posture and patterns of movement.
– Facilitation of higher centers to control the tone and activation of
postural reactions (righting and equilibrium reaction).
NDT methods are for early treatment of common manifestation of
cerebral palsy.
Vojta method of therapy Vojta established 18 points in the body for
stimulation and used the positions of reflex crawling and reflex rolling.
He proposed that placing the child in these positions and stimulation of
the key points in the body would enhance CNS development. In this way
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the child is presumed to learn normal movement patterns in place of
abnormal motion.
Neuro-facilitation techniques: these methods stimulate the CNS
(Sensory input) and accelerate neuromotor maturation (reflex motor
output) through the process of neuronal plasticity.
Conventional exercises Conventional exercises consist:-
– active and passive range of motion exercises
– stretching
– strengthening
– fitness to improve the cardiovascular condition
– balance training
Sports activities: Always include sports activities in the rehabilitation
program. Play improves mental capacity and provides psychological
satisfaction. Sports are helpful in decreasing stiffness and contractures in
adolescents using the wheelchair.
Sports scientifically shown to have significant therapeutic effects in CP
are swimming and horseback riding. They help to increase muscle
strength and range of motion in the joints, improve sitting balance and
body control and provide fun.
Do not prevent sitting in the W-position [G] for fear of hip subluxation.
W-sitting does not increase femoral ante-version or cause hip
subluxation. Children with femoral ante-version sit in a W-shaped
position because it is comfortable.
Children with spastic diplegic CP, short duration of dynamic sitting
balance training results in improvement in upper extremity function
with improvement in dynamic sitting balance.
Benefits and limitations: Physiotherapy cannot correct the movement
problem in CP. The effect of physiotherapy in preventing contractures
and deformities or improving balance and coordination is also limited.
Physiotherapy is beneficial in promoting the neurological development
of the child and teaching the child to use his existing potential in the
best possible way. By improving mobility, physiotherapy may also
prevent secondary mental and psychosocial retardation.
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2. OCCUPATIONAL THERAPY
It assess and treat problem related to activities of daily living(ADL):-
– Fine motor development
– Cognition
– Perceptual maturation
– Functional skills needed for play and self-care
The occupational therapist advises the parents on ADL: bathing, feeding
and dressing.
Ayres sensory integration therapy aims to enhance the child’s ability to
organize and integrate sensory information. In response to sensory
feedback, CNS perception and execution functions may improve and the
motor planning capacity of the child may increase.
Various adaptive equipment’s like bucket seat, feeder chair, hammock
are advised for positioning of child.
Proper Holding position is by maintaining head in midline, shoulder in
forward semi flexed position, elbow extended, legs abducted at hip,
flexed at hip and knee.
Tendency for scissoring can be prevented by keeping a rolled towel or
pillow between the legs. Babies should be made to lie in side-lying
position as it helps inhibit TLR (Tonic Labyrinthine Reflex) and
encourages midline position of hands.
Posture during feeding: Head in midline with chin tucked down,
shoulder forward. Arms internally rotated with hands in lap. Straight
trunk, hip and knees flexed, face to face position also facilitates eye-to-
eye contact and communication. Small amounts of feed and firm in
consistency should be given.
To improve visual fixation: eye to eye contact and baby rattles.
To promote motor development chairs, standing frames, bolster, Lycra
suits therapy ball, rollers and special types of walkers used.
To guide auditory and visual stimulation various types of colourful toys
with soft sound are used.
Light tactile brushing over joints is recommended for tactile stimulation.
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3. SPEECH THERAPY
Helps in overcoming communication problems
Appropriate positioning of head and jaw is basic to both feeding and
speech.
Speech therapist employ auditory stimulation and work for
improvement of muscle control required for feeding and articulation.
Speech therapist also helps in developing an effective feeding
programme along with occupational therapist.
4. MULTI-SENSORY STIMULATION
It covers all sensory modalities:-
Cutaneous–Touching, Tickling, stroking, gentle rubbing, gentle bouncing,
gentle to and fro rocking with a tune, making purring sound on
abdomen, gentle massaging.
Visual–showing colourful cloths pieces, ribbons, balloons, toys.
Auditory–swinging, playing tunes, hums, parallel vocalization, gentle
clapping/sounds of bangles, bells, animals, talking, building simple
conversation around daily routines.
Taste and Smell–Getting the child to experience different tastes and
odours.
5. BEHAVIOUR MODIFICATION
Goal specification-Specified description of desired behaviour to be
learnt, based on current skills level and needs.
Task analysis-Breaking activity into sequential steps: number of steps
depends on child’s learning capacity.
Rewarding-Pleasant event following a given behaviour; can be material
(food) or social (praise, attention); should be immediate, consistent,
appropriate and contingent.
Modelling-Showing how, or demonstrating, so that the child imitate and
learn.
Shaping-Successive approximation to final task; teaching the simplified
version of the total task and gradually increasing the complexity.
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Chaining-Breaking the task into small steps and teaching one after
another.
Back chaining-Teaching the last step first and then going backwards.
Forward chaining-Teaching the first step first.
Prompting-Assisting the child verbally or physically (hand over hand,
gesturing, pointing) and gradually fading the assistance.
6. ORTHOTIC MANAGEMENT
Orthotic interventions like orthoses & orthotic surgeries play a crucial
part in the management of children with CP.
According to The International Society of Prosthetics and Orthotics
(ISPO) Aims of orthotic management are:-
– To correct and/or prevent deformity,
– To provide a base of support,
– To facilitate training in skills, and
– To improve the efficiency of gait.
A degree of compromise may be necessary in planning orthotic
intervention, as orthoses prescribed to prevent or correct deformities or
improve gait efficiency may restrict movements and limit other
activities.
Physiotherapy for contracture, few essential basics must be followed:-
– At least three times a day
– At least one joint above and below
– Minimum 10 minutes and complete range of movement
Recommendations for orthotic intervention to improve gait efficiency
are based on the integrity of the plantar flexion-knee extension couple
Soft polyurethane foam splints have proved to be very effective in
reducing severe knee-flexion contractures in children with CP when
applied nightly.
There are various effective Orthoses available:-
– AFO (ankle foot orthoses)
o Hinge AFO
o Posterior Leaf Sparing (PLS) AFO
o Solid/rigid type
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– WHO (wrist hand orthoses)
– KAPO’s (knee-ankle-foot orthoses)
– Twister orthoses
– Supra Malleolar orthoses
– Rigid plastic TLSO’s ( thoraco-lumbar-sacral orthoses)
– HpO (hip orthoses)
– SWASH (standing-walking-and sitting hip abduction orthoses)
– HASSO’s(hip abduction spinal sitting orthoses)
Various orthotic surgeries like for Hip dislocation:-
– Prevention:- adductor-tenotomy/obturator-neurectomy,
derotation-osteotomy
– Correction:- open reduction, resection of head of femur (Girdle-
stone procedure)
Release of contractures, e.g. Tendo – Achilles release
Upper limb hand surgery
Recent Advances in the Orthopedic Management:-
– The use of instrumented gait analysis to aid decision-making
regarding single event multilevel surgery (SEML) and to study its
outcome.
– Orthopaedic selective spasticity control surgery (OSSCS) devised by
a Japanese Orthopedic surgeon, Takashi Matsuo.
7. MEDICAL TREATMENT
Anti-Epileptic Drugs are used keeping in view the type of seizures and
side effects (Vigabatrin causes visual impairment and Valproate leads to
weight gain). A very high incidence of WEST syndrome has been
associated with patients of spastic quadriplegia.
The guidelines of stopping the therapy are similar to the general
population, but only a 1/5th of the patients may achieve remission and
withdrawal of medication.
Tigabine- a newer anti-epileptic drug has been shown to bring about
seizure control as well as reduction in spasticity with improvement in
function.
Sizure control is poor specially in children with Mental Retardation.
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Polytherapy required in 42% of cildren with cp.
Anti-Reflux medication: Gatroesophageal regurgitation may occur
leading to postprandial dyspepsia (heart burn) and vomiting. These
symptoms may provoke crying or reflex syncope. Food thickeners and
barrier treatment such as Gaviscon can be used as well as an upright
position after meals.
Other drugs like Metoclopramide (0.1mg/kg/dose at 6-8hourly),
Cisapride (10-20mg Tds), H2 antagonists and Proton Pump Inhibitors
may be used for GERD.
Medication for Drooling & excessive Salivation: there are variety of
treatment options to help decrease severe drooling. These include
behavioral and oral motor therapies, pharmacologic management, and
surgical interventions.
– Behavioural interventions and oral motor therapy are more likely
to be effective for children with better cognitive functioning and
milder drooling.
– Excessive drooling, If this is persistent, they may benefit from
Hyoscine skin patches behind the ear or between the scapula.
– Atropine sulphate: [for 3-7 kgs=0.1 mg, 7-10 kgs=0.15 mg, for 10-
18 kg=0.2 mg, 18-30 kgs=0.3 mg, for >30 kgs=0.4 mg. at Every 4-6
hours as needed].
– Glyco-pyrrolate: [ 0.04–0.4 mg/kg/day, in 2 or 3 divided doses]
– Trihexyphenidyl: Starting dose 0.02-0.04mg in 2 or 3 divided
doses, increasing to 0.1-0.3/kg/day
– Benztropine: Starting dose, 0.5-1mg given once a day; maximum
dose, 6 mg/day
– The surgical approaches that have been tried include gland
excision, duct ligation, duct rerouting, and interruption of the
parasympathetic nerve supply to the glands.
– Novel treatments for drooling:-The use of an intraoral appliance
for drooling, first advocated by Castillo-Morales in Europe.
– The use of intraglandular botulinum toxin injections to reduce
saliva production is also receiving attention.
Medication for constipation:-
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– Adequate, frequent fluid intake including fresh fruit juice
especially during fever
– Varied, stimulating diet
– Communicative meal times
– Some pureed fruit and fibers in diet
– Exercise including Hydrotherapy
– Relaxed and consistent toileting
– Lactulose as stool softener
– Senokot (calcium sennoside) syrup in the morning
– Prune juice and syrups of figs
– Osmotic luxatives e.g. magnesium sulphate can be used
Medication for Dystonia:-
– Unexplained dystonia—Trial of Levodopa (50mg qid P/O)
– Dystonia in CP (without hyperkinetic movement)- Trihexyphenidyl
(0.25 mg/kg/day, divided in bid or qid)
– Dystonia + hyperkinetic movement=Tetrabenazine (12.5-25
mg/kg/day, divided bid or qid)
– Spasticity + Dystonia= Baclofen, diazepam
– Myoclonus + Dystonia=Clonazepam (0.01-0.03 mg/kg/day in bid
doses p/o. increase every 3rd day by 0.25-0.50 mg till a maximum
dose of 0.2 mg/kg/day is reached)
Osteopenia is a risk in immobile children with CP, with consequent
increased risk of fractures. Use of bisphosphonates for 12–18 months
has been shown to increase bone density by 20–40%, with no apparent
adverse effects.
Vaccination: with uncontrolled progressive Epilepsy in CP, indicates DT
rather than DPT vaccine. Annual influenza vaccination should be
provided for those with recurrent or chronic respiratory illnesses.
Pneumococcal immunisation is recommended for those with chronic or
recurrent pulmonary illnesses.
MANGEMENT OF SPASTICITY
In spastic CP, the lesion in the brain results in loss of inhibition of the
lower motor neurones, and the effect of this is hypertonia and spasticity,
which can produce a significant participation limitation in children.
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Spasticity Is usually manifest in the flexor muscle groups. Spasticity leads
to:-
i. Decrease in excursion of muscle
ii. Increased muscle stiffness
iii. Dynamic muscle contractures
iv. Fixed muscle contractures
v. Torsional bony deformities and joint instabilities
Various grades of spasticity, assessment and management:-
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Dantrolene Start with 1mg/kg with Vomiting, dizziness, Not widely accepted due
sodium gradual diarrhoea to serious side effects like
increases until good hepatotoxicity. May acute liver dysfunction
results are obtained exacerbate and severe paralysis
Max dose: 100mg/day seizures in cerebral
palsy patients
Tizanidine Dose not well- Hypotension, day Better tolerated than
established in children time sleepiness, baclofen
dry mouth and in both cerebral and spinal
weakness forms of spasticity
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There are seven serotypes of botulinum toxin, A through G. All are
polypeptide neurotoxin proteases that cleaves one or more vesicle
fusion proteins at the neuromuscular junction, thereby preventing
release of acetylcholine into neuromuscular junction, thus preventing
contraction of muscle and causing its relaxation.
Effect usually seen within 2-3 days, and peak effect occurs at 4 weeks.
However the result is not permanent because with the time (usually 3
months) the end plate region expands and collateral sprouts of axons
start growing, and establish new N-M junctions, a with a return of
spasticity.
Two serotypes, A and B, are approved by the United States Food and
Drug Administration, as well as by European regulatory agencies, for use
in humans.
Two commercial forms of type A are available worldwide: BOTOX from
Allegran Inc. and Dysport from Ipsen Ltd. One form of type B is available,
known as MyoBloc in the USA and NeuroBloc in Europe.
The measure of potency of BTX is the “mouse unit” defined as the
quantity of toxin that causes death in 50% of mice of a defined weight
and strain.
Botox and Dysport are supplied as freeze-dried powders, which are
reconstituted in sterile saline before use. MyoBloc is supplied as a
solution, which may be diluted or used as supplied.
Botulinum therapy must always be complemented by continuing
physiotherapy and effective containment of contractions by orthoses.
Favourable factors for patient selection
– Focal goals with specific anticipated functional benefits.
– Increased Dynamic muscle stiffness.
– Muscular hypertonia with a functional goal.
Negative factors for therapy
– Severe fixed contractures (mild contractures may respond to
treatment combined with casting).
– Bony torsion and joint instability.
– Bleeding disorders.
– Too many target muscles—consider other treatment options, or
prioritize.
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Target muscles for Botulinum toxin in various types of CP:-
Type of CP Primary target Secondary target
Quadriplegia Adductors Calf & hamstrings
Hemiplegia Calf Hamstrings
Diplegia Hamstrings Calf
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Baclofen is delivered to the intrathecal space with the synchronized
infusion system, consisting of pump-and-drug reservoir implanted
subcutaneously in the abdomen and a catheter inserted most commonly
at the T11–T12 level. The pump is remotely programmable via
computer. The reservoir, which contains an alarm indicating low-drug
level, is refilled per-cutaneously, usually every 9–12 weeks but up to 24
weeks under normal conditions.
Selection criteria:-
– Severe spasticity of spinal or cerebral origin.
– Age more than 4 years
– Spasticity unresponsive to oral baclofen or patient has
unacceptable side effects to oral baclofen.
– lower extremity spasticity of 3 or greater on the Ashworth scale.
Contraindication: Allergy or hypersensitivity to oral baclofen.
A bolus infusion is used as a screening tool, with a decrease in one unit
on the Ashworth scale being the standard for proceeding in most
centers.
Dosage—25–100 microgram
Advantages: reversibility, titratable and bypasses BBB
Adverse effects: CSF leaks, seromas, catheter related wound infections
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necrosis, vascular complications, and dysesthesias. Pain is more likely
after treatment of a mixed nerve.
Advantage:- it has immediate action which renders it possible for
physician to determine the results of block immediately; it has a low cost
and can circumvent or delay the need for surgical procedure.
VI. Neurosurgery
Brain surgery in children with CP has at present very limited
justification. Thalamotomy by various methods has been tried in some
children with athetosis and rigidity, but improvement in surgical
techniques and safety are needed before this can be happily
recommended. A cerebellar approach with stereotactic dentatotomy
(stereotactic electrocoagulation of dentate nucleus) and chronic
cerebellar stimulation via implanted electrodes is reported, which has
given encouraging results in relieving spasticity in some patients with
CP, including some children.
Selective dorsal rhizotomy (SDR): cuts overactive dorsal nerve rootles,
reducing aberrant activity from muscle spindles and reducing spasticity.
A lumbar laminectomy or laminoplasty is performed. Individual nerve
rootlets from L4 or L5 to S1 are stimulated. Those giving rise to
excessive muscle activity are severed. Typically 25 to 60% of nerve
rootlets are cut.
Multiple controlled trails have demonstrated the ability of SDR to
reduce lower-limb spasticity and increase range of motion.
Improvements have been seen in gross motor function, self-care, and
gait.
SDR plus physiotherapy (PT) has been shown in two trails to be superior
to PT alone for improvement of gross motor function and range of
motion.
The best candidates for surgery are children ages 3–7 years, with spastic
diplegia, good trunk control, good leg strength, and isolated leg
movements (i.e. leg movements without involuntary involvement of
other limbs).
The presence of concomitant dystonia, severe fixed joint deformities,
scoliosis and previous orthopaedic operations are contraindications to
surgery.
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Hypotonia, sensory loss, bladder dysfunction and spinal deformity have
been reported following the surgery but these are rare events.
Selective motor fasciculotomy/ selective neurotomy
The SMF of musculo-cutaneous, median and ulnar nerves significantly
reduces spasticity in the affected muscle groups and thereby improves
the self-care (motor) function in selected people with cerebral palsy
who have harmful resistant spasticity without any organic shortening of
the muscle. This procedure is safe and the spasticity does not recur.
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COMPLEMENTARY OR ALTERNATIVE TREATMENT METHODS (CAM)
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There has been a marked improvement in survival for two groups with
the most severe disabilities, namely children who are largely immobile
and fed by others, and adults who are dependent on gastrostomy
feeding.
In these groups mortality fell by some 3.4% annually. For the other
groups, there may have been a modest trend, though the effect is only
marginally significant statistically. If so, the improvement is roughly
comparable to that in the general population, i.e. an annual decline of
approximately 1%.
Young children with severe CP and mental subnormality, who are often
also subject to epilepsy, have a high mortality rate from respiratory
infections, status epilepticus and other problems. Severe CP also carries
a poor prognosis for mortality in older patients. The yearly mortality rate
was approximately 5/1000, and for severely handicapped patients it was
about 29/1000.
Twenty-year survival is 89.3% for females and 86.9% for males. For those
with no severe functional dis-abilities the 20-year survival was 99%,
while those severely disabled in all three functional groups had a 20-year
survival of 50%. Severe epilepsy, particularly frequent episodes of status,
probably plays a role in shortening life expectancy in the severely
handicapped.
It is sometimes asserted that quality of care is the most important
determinant of life expectancy.
Quality of care is a rather vague term that may refer to any or all of the
following:-
– The expertize of the caregivers, ranging from highly qualified
professionals to relatively unskilled (and low paid) staff.
– The accessibility of physicians and emergency services.
– The quantity of care and equipment provided, which is often a
reflection of the funds available.
QOL (quality of life) has defined by WHO as an individual’s perception of
their position in life in the context of the culture and value system in
which they live, and in relationship to their goals, expectation, standards
and concerns. More specific concept is HRQOL (health related QOL) has
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been defined to differentiate health from more general social and
environmental issues.
LAQ-CP (Lifestyle Assessment Questionnaire – Cerebral Palsy
questionnaire evaluates the impact of disability in children with CP and
their families. It has 46 items, organized into six dimensions: physical
independence, mobility, clinical burden, schooling, economic burden,
and social integration [6].
Based on scores in each item, dimensional scores and a final standard
score, known as Lifestyle Assessment Score (LAS) is obtained.
These are expressed as a percentage score. The classification of the
HRQOL according to LAS is as follows: Good (<30%); mildly-affected (30-
50%); moderately-affected (51-70%); and severely-affected (>70%).
EARLY STIMULATION
By early ‘infant stimulation’ programs we mean early interventional
therapy for babies at risk for developmental delay and periodic
developmental assessment, in motor development, cognitive
functioning, language or adaptive functioning.
Aims of Early Stimulation
i. Stimulating the child through the normal developmental channels.
ii. Prevention of developmental delay.
iii. Prevention of asymmetries and abnormalities
– To prevent wastage of muscles
– To prevent fixity of joints
– To prevent contractures of the joint
– To decrease and prevent the tightness of the muscles and tendons
– Detection of transient abnormalities and minimization of
persistent abnormalities.
The process and principles
i. Every child has certain potentials which have to be explored. Start
the training with what the child knows, so that your baby has the
feeling of success.
ii. Proceed to the skills in which the child needs to be trained.
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iii. Appreciate the child for even little efforts.
iv. Reward is a must once the child masters the skill.
v. Involve normal children of the same mental age while training the
child.
vi. Anything appropriate and attractive available at home can be
used as training material.
vii. Though a child can be trained anytime and every time but a well
fed baby in right mood would respond better.
viii. Exercise patience while dealing with a slow child and do not get
disheartened by child’s failure.
The greatest single contributor in early stimulation is the MOTHER (the
key stimulator). The real foundations regarding stimulation are building
upon her mind and her love.
Her enthusiasm and the thrill of discovery of new skills of her baby are
the basic building blocks.
The parental attitudes especially that of the mother has the greatest
influence on the development of personality of the child during the
early.
Early stimulation is important both for the growing brain and body.
Adequate nutrition and the presence of both parents during the early
years are also crucial to a child’s being. All these factors contribute
towards a normal healthy adult. The stimulation the child receives
depends on life at home the family structure.
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