Indian J Pediatr (November 2018) 85(11):1006–1016
DOI 10.1007/s12098-017-2475-1
REVIEW ARTICLE
Guest Editor: Bhim S. Pandhi
Cerebral Palsy: An Overview
Sheffali Gulati 1 & Vishal Sondhi 1
Received: 25 April 2017 / Accepted: 5 September 2017 / Published online: 20 November 2017
# Dr. K C Chaudhuri Foundation 2017
Abstract Cerebral palsy (CP) is a neurodevelopmental disor-
der characterized by abnormalities of muscle tone, movement
and motor skills, and is attributed to injury to the developing
brain. The clinical features of this entity evolve over time and
the specific CP syndrome may be recognizable only after 3–5 y
of age; although suggestive signs and symptoms may be
present at an earlier age. The management involves neurolog-
ical rehabilitation (addressing muscle tonal abnormalities, and
devising physical and occupational therapies) and diagnosis
and management of co-morbidities (including epilepsy, im-
pairment of cognition, vision, hearing, and disturbances of
growth and gastrointestinal function). The management,
therefore, is multidisciplinary involving the treating physician
working with a team of rehabilitation-, orthopedic-, psycho-
logic-, and social care- providers.
Keywords Spasticity . Birth asphyxia . Prematurity .
Botulinum toxin . Rehabilitation
Introduction
Cerebral palsy (CP) is a neurodevelopmental condition that
affects muscle tone, movement and motor skills. This is not a
single disease but rather a heterogeneous clinical syndrome
resulting from injury to the developing brain [1]. Although
the disorder itself is non-progressive, the clinical expression
* Sheffali Gulati
sheffaligulati@gmail.com
1
Child Neurology Division, Department of Pediatrics, All India
Institute of Medical Sciences, New Delhi, India
changes over time as the brain matures. The current definition
of CP, as adopted by the International consensus in 2005 is [2]:
‘Cerebral palsy describes a group of permanent disorders of
the development of movement and posture, causing activity
limitation, that are attributed to non-progressive disturbances
that occurred in the developing fetal or infant brain’. The
motor disorders of CP are often accompanied by disturbances
of sensation, perception, cognition, communication, and be-
havior, by epilepsy and by secondary musculoskeletal
problems.
In the recent decades, there has been recognition for the
need of effective rehabilitation and management strategies
for these children. Here, the authors review the diverse clinical
features of CP and provide a perspective of management
methods and developments in rehabilitation for these children.
Epidemiology
There is paucity of robust data from Indian subcontinent,
nonetheless, the overall global prevalence of CP is estimated
to be 2 per 1000 live births [3]. The reported incidence, prev-
alence, and most common causes of CP have varied over time
and varied over geographical locations based on the develop-
ment of prenatal, intranatal and postnatal pediatric care. Thus,
while the developed world deals with predominantly prema-
turity and extremely low birth weight related morbidities, the
pockets of developing countries are still coping with prenatal
rubella, intranatal asphyxia and postnatal hyperbilirubinemia.
The etiology of CP is diverse and multifactorial. It is usually
caused by injury to the brain before or at birth. This is the
major causative factor. It can also happen between the ages
of 3–5 y. The common causes of CP have been illustrated in
Table 1 [4].
Indian J Pediatr (November 2018) 85(11):1006–1016 1007

Table 1 Etiological factors associated with CP [4] mo visit [5].

Prenatal/ Neonatal Causes Post-neonatal period causes Similarly, Rasthriya Bal Swasthya Karyakaram
(RBSK) of Government of India attempts to screen chil-
• Prematurity • Stroke dren from birth to 18 y of age for selected health condi-
a. Periventricular leukomalacia • Head trauma tions including defects at birth, deficiencies and develop-
b. Intraventricular hemorrhage • Hypoxic events like
c. Bronchopulmonary dysplasia near drowning
mental delays. Also, RBSK has a concept of district early
• Hypoxic ischemic encephalopathy • Febrile encephalopathy intervention center to provide referral support for those
• Neonatal hypoglycemia a. Sepsis detected with health conditions during screening. In ad-
• Congenital structural abnormalities b. Meningitis/ dition, a tool developed by INCLEN and improvised by
a. Inherited malformations meningoencephalitis
b. Secondary to • Status epilepticus
AIIMS (AIIMS Modified INDT-NMI Tool) can be used
i. Infections sequelae for screening for neuromuscular impairments including
ii. Radiation cerebral palsy.
iii. Toxins like anti-epileptic
drugs, alcohol, nicotine
• Multiple births
• Stroke
• Intracranial hemorrhage
• Intrauterine infections Clinical Features
• Neonatal infections
• Bilirubin induced neurological
dysfunction The clinical features of specific CP syndromes have been
• Genetic susceptibility highlighted in Table 2. There are three predominant CP syn-
dromes (spastic, dyskinetic and ataxic). Hypotonic CP, though
described earlier, is absent from the contemporary classifica-
Early Signs and Classification tions. Majority of patients with “hypotonic CP” in early infancy
later develop spastic, dyskinetic or ataxic CP. However, some
The classification of CP is based on the type and distribution children may continue to be hypotonic due to involvement of
of motor abnormalities (Table 2). The specific CP syndromes cerebro-cerebellar circuits or the extrapyramidal circuity.
are best recognized after 3–5 y of age, although suggestive
signs and symptoms may be present in infancy. The early a) Spastic syndromes: Patients with spastic syndromes
signs of CP include: have upper motor neuron signs including spasticity,
hyperreflexia (± clonus), and extensor plantar response.
a) Neurobehavioral signs: Neurobehavioral signs suspicious In addition, these children have mass movements (instead
for CP are excessive irritability, lethargy, sleeps poorly, of fine and individual movements), and slow and effortful
vomits frequently, is difficult to handle and cuddle, and voluntary movements. Spastic syndromes include:
has poor visual attention.
b) Developmental reflexes: Delay in disappearance or exag- i. Spastic diplegia: Briefly, this is a child with gross mo-
geration of a developmental reflex may be an early indi- tor problems particularly in lower limbs, with usually
cator of motor disability. Infants with an exaggerated ton- retained fine motor functions in upper limbs. It is usu-
ic labyrinthine reflex may have ophistotonic posturing or ally associated with periventricular leukomalacia
may roll over at an age earlier than appropriate. Similarly, (PVL) and periventricular hemorrhagic infarction
the child may have abnormal response on vertical suspen- (PVHI). Some of these children may have associated
sion, wherein instead of assuming a sitting position, they visual difficulty.
have persistent extension of legs. ii. Spastic quadriplegia: Children with spastic quadriple-
c) Motor tone and posture: Tone in extremities/ trunk may gia have severe motor impairments. Both upper and
be normal, increased or decreased. Poor head control, lower limbs are affected nearly equally, and majority
persistent or asymmetric hand fisting, and abnormal have very little speech and language development,
oromotor patterns (tongue thrusting/ grimacing) may be visual impairment, epilepsy, and feeding difficulty.
the early motor signs. Sometimes, however, an increased MRI in these children may show multicystic
neck extensor and axial tone may make head control seem encephalomalacia.
better than it actually is. iii. Spastic hemiplegia: In spastic hemiplegia, arm is typ-
d) Motor milestones: Serial examination of motor mile- ically affected more than leg. Athetotic posturing
stones is an effective screening process for picking CP may accompany spasticity. Most children with spas-
early. The American Academy of Pediatrics recommends tic hemiplegia have associated sensory deficits.
complete developmental screening at 9, 18, 30, and 48 These sensory deficits are reflected as poor muscle
Table 2 Clinical features of cerebral palsy
1008

Proportion of Chief causes Affected infants Clinical features Clinical features

CP patients among infants among older children

Spastic CP • Clasp knife spasticity • Clasp knife spasticity

• Brisk deep tendon reflexes • Brisk deep tendon reflexes
• Extensor plantar responses • Extensor plantar responses
• Contractures are common • Contractures are common
Spastic diplegia 15–25% PVL Prematurity • < 6 mo • Lower limbs are affected
Hypotonia of lower limbs more than upper limbs
• ≥ 6 mo • Toe walking
Commando crawl • Relatively preserved
W-Sitting function of upper limbs,
Tendoachilles tightening cognition and speech
• May have visual impairment
Spastic hemiplegia 20–40% • Neonatal stroke Term infants usually • Asymmetry while natural hand • Arms affected more than legs
• Prenatal stroke movements/ bringing hands in • Sensory deficits that manifest
• Cortical malformations midline or during neonatal as reduced bulk on affected
reflxes like Moro’s side may be present
• Early hand dominance (before 12 mo)
• In sitting position, affected leg tends
to extend
Spastic quadriplegia 20–40% • Perinatal asphyxia Preterm/ term infants • Severe motor and cognitive delay • All 4 limbs are affected, with
• Congenital infection • Poor head control upper limbs affection equal
• Cerebral dysgenesis • May have hypotonia for intial 4–6 mo, to or more than lower limbs
which evolves to spasticity by 9–12 mo • Associated with severe cognitive,
• By 12 mo, infants when pulled to sitting speech and visual impairement
are unable to flex the legs and have • Feeding difficulties, chronic
poor truncal balance respiratory insufficiency, and
seizure disorder are common
Dyskinetic CP 10–15% • Hypotonia at rest, with variable tone • Characteristic involuntary
with movement or emotion movements
• Reduced spontaneous movements • Dysarthria
• Head persistently turned to a side • Intellectual disability
• Drooling • Dystonia
• Involuntary grimace • Contractures are less common
Choreo-athetoid CP • Severe hyperbilirubinemia Preterm and term • Striatal toe • Chorea ± athetosis
(Rh/ ABO incompatibility/ • Athetosis is most apparent
G6PD deficiency) while reaching for an object
• Oropharyngeal difficulties
are common
Dystonic CP • Perinatal asphyxia Term infants • Repetitive, patterned, twisting
& posturing of limbs and trunk
• Sudden involuntary increase in
tone affecting both flexor and
Indian J Pediatr (November 2018) 85(11):1006–1016
Indian J Pediatr (November 2018) 85(11):1006–1016 1009

Hypotonic CP, that was described earlier, has been removed from the contemporary classification. The majority of patients with “hypotonic CP” in infancy, later develop other forms of CP with variable/
during attempted movement or
mass on the affected side and do not correlate to

• Tendon reflexes are normal or

extensor muscles, may occur
motor deficits [6]. In addition, they may have asso-

• Speech is slow, jerky and

may be difficult to elicit
ciated intellectual impairment, hemianopia, and other
visual problems. Also, behavioral problems are com-
among older children

mon among children with hemiplegic CP including

Clinical features

with emotion anxiety, oppositional defiance, and specific phobias.

explosive
b) Dyskinetic syndromes: Patients with dyskinetic CP often

• Ataxia
have more than one form of involuntary movement. The
limbs often become stiff during attempted movement or
with emotion. Tendon reflexes may be normal or may be
difficult to elicit. Athetoid movements of toes especially
great toe (striatal toe) is a significant indicator of extrapy-
ramidal dysfunction. Dyskinesia may also be seen in
some spastic syndromes. However, unlike patients with
spastic CP, children with purely dyskinetic syndromes do
• Delayed motor milestones
and language milestones

not develop contractures. The dyskinetic CP can be fur-

ther sub-classified into:
Clinical features
among infants

i. Choreo-athetoid CP: It is characterized by rapid disor-

• Hypotonia

ganized unpredictable contractions of individual

muscles/ muscle groups involving face, bulbar mus-
cles, proximal extremities and digits. In addition, they
have slow writhing movements involving distal mus-
cles. Oropharyngeal difficulties may result from facial
grimacing. Primitive reflexes often persist into
No specific predilection

childhood.
ii. Dystonic CP: It is characterized by co-contraction of
Affected infants

agonist and antagonist muscles. Often, they have co-

existent pyramidal signs and dysarthria.
c) Ataxic CP: Ataxic CP is rare and should be distinguished
from progressive neurodegenerative disorders. Motor and
language milestones are delayed. Ataxia usually improves
with time.
•Etiology is frequently not

Cerebellar hypoplasia
•? Early prenatal events

Joubert syndrome
•? Genetic causes

Associated Co-morbidities
Chief causes

known

While the emphasis in CP is on motor disabilities,

CP Cerebral palsy; PVL Periventricular leukomalacia

there are significant accompanying disorders of cere-

bral function. Some children have epilepsy, impairment
of cognition, vision, hearing, and many have disturbed
Proportion of
CP patients

gastrointestinal function and growth. Broadly, children

5–10%

with more severe motor disabilities are more likely to

have co-morbidities. These co-morbidities have been
illustrated in Table 3 [7].
Table 2 (continued)

Management
increased tone

The management of a child with CP involves baseline

Ataxic

investigations (Table 4) and a multidisciplinary team to ad-

dress medical, social, psychological, educational and
1010 Indian J Pediatr (November 2018) 85(11):1006–1016

Table 3 Conditions associated with cerebral palsy [7]

Co-morbidities Remarks

Intellectual disability • Occurs in approximately 50% patients with CP

• Children with spastic quadriplegia are most severely affected
• Language development in hemiplegic CP is related to cognitive ability rather than side of lesion
Neurobehavioral/ • Occurs in nearly 25% patients with CP
neurodevelopmental disorders • Commonly have behavioral, emotional, and/or psychiatric disorders, including emotional lability,
poor attention and vigilance, and obsessive-compulsive traits
• Autistic traits may be observed in upto 7% children with CP, higher rates in those with non-spastic CP
Epilepsy • Occurs in 25 to 45% of patients with CP
• Most common in patients with spastic quadriplegia and acquired hemiplegia, and less common in
spastic diplegia and CP that is mainly athetoid
Visual disorders • Observed in nearly 30% children with CP
• More common in children with CP following prematurity
• Low visual acuity may be due to cortical impairment/strabismus, amblyopia, refractive errors and visual
field defects
Hearing/ Speech impairment • Observed in 30–40%
• Impairments include aphasia, dysarthria or mutism
Growth failure • Growth failure is usually due to poor nutrition caused both due to inadequate intake and by gastrointestinal
abnormalities
Gastrointestinal disorders • Nearly 90% children with CP may have concurrent constipation, gastro-esophageal reflux ± vomiting,
swallowing disorders or abdominal pain
Pulmonary disorders • Chronic pulmonary disease occurs in children with CP secondary to recurrent aspiration, scoliosis and
respiratory muscle incoordination
Orthopedic disorders • Common orthopedic problems in children with CP include subluxation, dislocation, and progressive
dysplasia of the hip, foot deformities, and scoliosis
Urinary disorders • Thirty to 60% of children with CP have dysfunctional voiding symptoms, including enuresis, frequency,
urgency, and stress incontinence
Pain • Pain is reported by 50 to 75% of children with CP, and approximately 25% experience pain that limits
activities
• Pain may occur secondary to dystonia, hip subluxation or constipation
• Pain in children with CP may go unrecognized due to communication difficulties
Sleep • Exact prevalence is not known
• Typically characterized by disorders involving sleep-wake transition, excessive daytime sleepiness,
and arousal

therapeutic goals. The salient neuroimaging findings have Functional Evaluation

been depicted in Fig. 1. The management should be directed
at stimulating the child’s development with the aim to obtain The management depends upon patient’s limitation of func-
maximal independence in activities of daily living. tion. The most commonly used functional classification is

Table 4 Investigations in a child

with cerebral palsy Investigations Remarks

MRI Brain • Should be done in all infants

• Correlate MRI finding with history and clinical findings;
if there is a discrepancy, rule out underlying metabolic disorders
Hearing assessment • BERA and hearing assessment is a must for all infants
Vision assessment • Visual assessment specifically for fundus assessment, strabismus
with/without VEP should be performed
EEG • Mandatory if the child has history of seizures
• Should be performed in a child who is not gaining milestones
(after an initial static insult) though the parents are denying any
history of seizures

BERA Brainstem evoked response audiometry; EEG Electroencephalogram; MRI Magnetic resonance imaging;
VEP Visual evoked potential
Indian J Pediatr (November 2018) 85(11):1006–1016
Fig. 1 Salient MRI changes in cerebral palsy. Panel A shows a T2
weighted image with periventricular hyperintensities and undulating
ventricular margins (solid arrow). This is typically seen in prematurity
associated insult and commonly manifests as spastic diplegia. Panel B
shows multi cystic encephalomalacia (dotted line with arrow). This
pattern of watershed lesions is seen commonly in term infants with
ischemia/ asphyxia and manifests clinically with spastic quadriplegia.
Gross Motor Function Classification System (GMFCS). This
system categorizes a patient’s motor function based on his age
and performance in various settings. The discussion about
GMFCS is beyond the preview of this article [8]. Manual
Ability Classification System (MACS) and Communication
Function Classification System (CFCS) are other widely used
tools for evaluation of functional status.
Tone Evaluation
Tone evaluation is important for assessment of specific muscle
groups before and after an intervention, such as botulinum
toxin (BTX) or baclofen. Two most commonly used scales
are the Modified Ashworth scale and the Modified Tardieu
scale. While the Modified Ashworth scale is a measure of
resistance to passive range of motion; the Modified Tardieu
1011
Panel C illustrates T2 hyper intensities in posterior putamen (open
arrow) and thalami bilaterally (dotted line with closed arrow). This is
typically seen in infants with term hypoxic ischemic encephalopathy
(HIE) and manifests as dyskinetic cerebral palsy. Panel D highlights T2
hyper intensities in occipital lobe (solid arrow); characteristic of neonatal
hypoglycemic insult. Panel E shows T2 hyper intensity involving
bilateral globuspallidi (open arrow), a feature of kernicterus sequelae
scale defines the spasticity angle, and quality of move-
ment at multiple velocities. Though these are common-
ly used during the course of management, the reliabil-
ity characteristics of these tests are not ideal.
Management of Motor Impairment
Physical Therapy
Physical therapy (PT) is an integral part of CP
management and may range from a regular exercise
program to variety of modalities including electrical
stimulation. Although its effectiveness in promoting
physical function is uncertain, PT aids in encouraging
caretakers to learn how best to handle, toilet, wash,
and feed their children with CP and to promote posture,
1012
mobility, and transfer. Occupational therapy (OT) tech-
niques seek to improve function, but focus on maximiz-
ing a child’s ability to accomplish activities of daily
living, education, and work. These are particularly help-
ful in maximizing available hand function.The PT ap-
proaches which are not recommended due to lack of
efficacy include neurodevelopmental therapy and senso-
ry integration therapy. In addition, electrical stimulation,
hydrotherapy, massage and whole-body vibration have
failed to show clinically significant improvement [9].
The PT and OT based approaches which have been
recommended in literature include [9]:
1. Bimanual training for hemiplegic CP where the child is
trained to use both hands together through repetitive tasks.
2. Constraint induced movement therapy involves restraint
of the unaffected limb to encourage the use of affected
limb during the therapeutic tasks. The restraint may be
by the use of casting or physically restraining by holding
the normal hand.
3. Context focused therapy involves changing the environ-
ment rather than the child’s approach.
4. Goal directed functional training lays emphasis on activ-
ities based on goals set by the child, using motor learning
approach.
Management of Spasticity
Tone reduction may be considered if hypertonia or spasticity
interferes with function, comfort, or care. But before venturing
for tone reduction, it should be confirmed that the “increased
tone” is not beneficial to the child; for example, some patients
with severe lower extremity hypertonia use their heightened
tone to “stand up” on otherwise weak limbs and may lose their
ability to stand and bear weight through their legs after treat-
ment. Hence a team approach with pre-decided goals is
essential.
Oral Medications
Oral antispasticity agents including benzodiazepines, baclofen
and tizanidine have the advantage of ease of administration
but the disadvantages of systemic effect and significant side-
effects [10, 11]. Thus, they are most appropriate for children
with generalized spasticity, and/or in children in whom only
mild tone reduction is needed. Unfortunately, the evidence on
the efficacy of these agents is less rigorous. Hence, the choice
of the agent is more a matter of personal experience rather than
evidence-based medicine. The commonly used drugs have
been highlighted in Table 5.
Indian J Pediatr (November 2018) 85(11):1006–1016
Botulinum Toxin
Botulinum toxin type A (BTX-A) when injected into the affect-
ed muscles, blocks the presynaptic release of acetylcholine
from motor endplates of the lower motor neuron and decreases
tone by limiting muscle contraction. Hence it produces
chemodenervation. The best evidence for use of BTX-A is for
equines varus (treated by injections into the gastrocnemius-
soleus muscles), but BTX-A is now used for a variety of focal
indications including knee and hip flexion spasticity, and spas-
ticity of the upper extremity. Patients younger than 4 y old and
without fixed contractures are expected to have the most favor-
able response because the musculoskeletal and nervous system
is most plastic in growing children [12]. Nonetheless, BTX-A
also may be useful to treat focal dystonias in older children and
adults. The total dosage ceiling used by experienced injectors
has risen from the initial recommendation of 4 U/kg to greater
than 16 U/kg in specific specialist cases; but the dosing has to
be individualized. Most reports suggest that the drug is gener-
ally safe, but there have been case reports of life-threatening
systemic toxicity. The severe adverse events that have been
reported include generalized muscle weakness, vision difficul-
ties, ptosis, swallowing difficulties, urinary incontinence, and
respiratory compromise [10].
Phenol and Alcohol
Phenol and alcohol are the other agents associated with
chemodenervation, and have been used in children with CP,
although neither has been rigorously tested. Phenol is typically
injected at a concentration of 3%–6% aqueous solution, whereas
absolute alcohol is diluted to 30%–50%. The target nerve is
identified with electrical stimulation, a procedure that may be
poorly tolerated in children, making sedation or anesthesia nec-
essary. The agent is injected perineurally, where it promotes
denervation via axonal degeneration. The effect is not perma-
nent, with functional re-innervation occurring over months to
years. Adverse effects include pain and paresthesia if a mixed
motor nerve and sensory nerve is targeted. Nonetheless, the
absence of immunogenicity and the lower cost compared to
BTX-A make these agents more attractive in some settings [10].
Intrathecal Baclofen
Intrathecal baclofen (ITB), administered via a catheter at-
tached to a programmable pump implanted subcutaneously,
achieves higher cerebrospinal fluid drug levels as com-
pared with oral administration, but may also be associated
with substantial complications. Overdose from a program-
ming error is possible, and may lead to respiratory depres-
sion and coma, while more commonly abrupt withdrawal
may occur due to emptying of the reservoir, pump failure,
or catheter withdrawal.
Table 5 Oral medications for spasticity/ dystonia [10, 11]

Drug Mechanism of action Dose Side effects

Muscle relaxants
Baclofen • Bind to GABAB receptors of laminae I − IV
of the spinal cord, where primary sensory
fibers end, thereby inhibiting the release of
excitatory neurotransmitters and causing
presynaptic inhibition of mono- and polysynaptic
reflexes
Initial doses, titrated to effect • Worsening of axial hypotonia
(review treatment if no benefit within 6 wk): • Constipation
• 1–6 y: 0.3 mg/kg/d in 3–4 divided doses, increased • Respiratory depression
to 0.75–2 mg/kg/d (or 10–30 mg/d) in 3–4 divided doses • Increased risk of seizures for
• 6–8 y: 0.3 mg/kg/d in 3–4 divided doses, increased children aged ≤2 y
to 0.75–2 mg/kg/d (or 30–40 mg/d) in 3–4 divided doses
• 8–10 y: 0.3 mg/kg/d in four divided doses, increased
to 0.75–2 mg/kg/d (or 60 mg/d) in 3–4 divided doses
Indian J Pediatr (November 2018) 85(11):1006–1016

• 10–18 y: initially 5 mg three times daily increased

gradually, up to 60 mg/d (max. 100 mg/d)
in 3–4 divided doses
Tizanidine Acts as a central α-2 receptor agonist at supraspinal • Initial dose • Sedation
and spinal levels to reduce spasticity by inhibition < 10 y: 1 mg twice daily • Hypotension
of spinal polysynaptic reflex activity. > 10 y: 2 mg OD • Agitation
• Maximum • Depression
0.05 mg/kg/d • GI problems
> 12 y: 2 mg TDS
Diazepam Acts post-synaptically by binding to a specific site of Initial doses titrated to effect: • Drowsiness/ sedation
the gamma-aminobutyric acid A receptor (GABAA), • 1–12 mo: 0.25 mg/kg twice daily (which can be useful to aid sleep)
enhancing the endogenous effects of GABA as • 1–5 y: 2.5 mg twice daily • Hypersalivation
an inhibitory neuro-transmitter • 5–12 y: 5 mg twice daily • Respiratory depression should not normally
• 12–18 y: 10 mg twice daily occur at the doses used to treat spasticity if
(maximum 40 mg/d) low starting doses are used
• Tolerance and dependence limit the
long-term use of diazepam

Antidystonic medications
Trihexyphenidyl Anticholinergic agent works at the • Start with initial low dose (1–2 mg/d); increase slowly • Dry mouth
level of the basal ganglia (by 2 mg/wk); higher doses (>50 mg/d) might be needed • Blurred vision
• Usual dose: 2–60 mg/d in 2–3 divided doses • Urinary retention
• Reduced gut motility
• Constipation, and ileus
Tetrabenazine Dopamine depletors, prevent • Initial dose: 12.5 mg/d •Akathisia
neurotransmitter degradation • Titrate to maximum dose 150 •Depression and other mood disorders
mg/d in 2 divided doses •Agitation
1013
1014
Surgical Therapy
Selective Dorsal Rhizotomy (SDR)
SDR is a surgical procedure that selectively divides parts of the
dorsal lumbosacral roots of the spinal cord. This interrupts the
afferent limb of the reflex arc on the sensory side, thus reducing
spasticity without causing paralysis. This technique may pro-
vide a small gain in function. Candidates for SDR are typically
ambulatory or nearly ambulatory (GMFCS levels II and III,
sometimes GMFCS level I), are between 4 and 7 y of age,
have spared cognitive skills and are motivated to walk, have
some degree of preserved selective motor control in the lower
extremities, have adequate strength, and have minimal, if any,
dystonia. In addition, the family must be able to commit to a
rigorous daily physical therapy program for 6 to 12 mo [13].
Deep Brain Stimulation
Deep brain stimulation (DBS) is primarily used for dystonias.
A meta-analysis of DBS in adults with dyskinetic CP suggests
that it may be effective [14].
Orthopedic Interventions
Despite best medical and rehabilitation management, children
with spastic CP will often eventually require orthopedic sur-
gery to correct deformities induced by muscle overactivity.
Correction typically involves serial casting (to stretch short-
ened muscles; no evidence of clinical benefit), multiple
tenotomies and transfers of the thigh adductors (limited evi-
dence; small improvement in gait function) and, in severe
cases, femoral osteotomy [15].
Dystonia and Mixed Movement Disorders
Motor dysfunction in CP is not simply attributable to spastic-
ity or corticospinal tract dysfunction; instead, it results from a
disruption of the complex integration of multiple areas of the
brain, including the cortex, cerebellum, basal ganglia, and
brainstem. As a result, a child with CP may manifest other
kinds of hypertonia and movement disorders. Oral medica-
tions frequently used to manage dystonia/ dyskinesia have
been illustrated in Table 5. BTX-A is an effective therapy
for focal dystonia.
Feeding and Nutrition
Feeding problems and oromotor dysfunction, secondary to
dyskinesia, bulbar palsy and/or pseudobulbar palsy, are com-
mon in children with CP. These children have sucking/ chewing
and swallowing difficulties manifesting as prolonged feeding
times (> 3 hours per day), choking and frequent vomiting
Indian J Pediatr (November 2018) 85(11):1006–1016
[16]. Thus, these children should have 3–4 monthly assessment
of feeding skills and nutritional status (including weight and
stature). Among infants with growth failure and/ or chronic
aspiration, alternative feeding techniques (including
gastrostomy feeding) should be considered.
Drooling
Oromotor dysfunction in children with CP often leads to
drooling, which is a significant impediment to social accep-
tance [17]. Approaches to improve drooling include pharmaco-
therapy, behavior-therapy, and surgery. A trial of behavioral
therapy is appropriate for children who are able to understand
commands and cooperate with training. This consists of oral
awareness and oral motor skills training, designed to improve
lip and jaw closure and tongue movements with positive rein-
forcement for swallowing. Pharmacotherapy for drooling in-
cludes anticholinergic drugs (trihexphenidyl or glycopyrrolate;
act by decreasing flow of saliva), and BTX-A injection into
salivary glands [18].
Surgical techniques include repositioning of the submandib-
ular ducts and unilateral ligation of a parotid duct, and/or exci-
sion of the sublingual gland. Although surgery reduces
drooling frequency and severity in 75 to 90% of patients, com-
plications such as ranula formation, dry mouth, difficulty with
swallowing, and changes in the consistency of oral secretions
are common, limiting the acceptance of this intervention [19].
Social Support
Social and psychological support is essential, as it is for any
family with a child with chronic medical condition. The doc-
tor should be prepared to answer all the parent’s questions like
“How did it happen?” and “why to my child?”. Parents of a
child with CP may experience chronic grief, as well as guilt,
frustration, denial, resentment, and embarrassment.
Information about their child’s condition should be provided
to parents with honesty and sensitivity. The process of evalu-
ation and management should be described, with the under-
standing that repetition may be necessary because the parents
may not assimilate all the information during the first visit
[20]. A second opinion should be offered if appropriate.
Future Developments
Robot-assisted activity can improve upper-arm function in
adults with impaired mobility after stroke [21]. Preliminary
data suggests that this type of therapy also improves motor
function in the upper and lower extremities that are caused by
CP or other acquired brain injuries. These data suggest that
use of such interventions in children with CP can improve
movement and function as they do in adults with stroke
Indian J Pediatr (November 2018) 85(11):1006–1016
[1].Virtual reality has been incorporated into physical inter-
ventions to increase motivation and perhaps could take advan-
tage of neuroplasticity. Transcranial magnetic stimulation and
functional MRI that probe motor cortex function, can poten-
tially provide insights into typical neuromotor maturation and
guide potential therapeutic endpoints [22]. These and many
other advances at the interface of technology and rehabilita-
tion can pave the way for future therapeutic programs.
Prognosis
A majority of children with CP survive to adulthood. In a
regional analysis of children with CP who were born in vari-
ous regions of the United Kingdom between 1980 and 1996,
20-y survival ranged from 87 to 94% [23]. In multivariate
analysis, survival was related to severity of impairment, birth
weight, and socioeconomic status, with the number of severe
impairments having the greatest effect.
The prognosis for walking is poor in children who do not
achieve head balance by 20 mo, retain primitive reflexes, or
have no postural reactions by 24 mo, or do not crawl by
approximately 5 y of age. Usually, all children with hemiple-
gic CP will walk, as will many with athetosis or ataxia. The
prognosis for walking is good in children who sit by 2 y and
crawl before 30 mo of age. Some children who sit between 3
and 4 y of age eventually walk, but most require aids or braces
or have restricted functional ambulation. In general, children
who walk independently do so by approximately 3 y of age;
those who walk only with support may take up to 9 y. A child
who does not walk by 9 y of age is unlikely to ever walk, even
with support [24].
In addition, functional outcome in CP depends on multiple
factors besides motor function. These include intelligence,
physical function, ability to communicate, and personality at-
tributes. Social (e.g., family support) and environmental fac-
tors and the availability of specialist medical care play an
important role.
Conclusions
A coordinated effort on the part of general practitioner,
specialist, therapists and the parents is necessary to pro-
vide optimal care. The highest standard of care for chil-
dren with CP requires a team of experts working togeth-
er, who understand the importance of a completely thor-
ough approach in order to systematically identify and
treat the motor disorders, other neurological disorders,
and a myriad of associated impairments in affected
children.
1015
Contributions Both the authors were involved in literature review and
preparing of manuscript. SG will act as guarantor for this paper.
Compliance with Ethical Standards
Conflict of Interest None.
Source of Funding None.
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