Cerebral Palsy

Dr Prateek Kumar Panda

MD, DNB, MNAMS, DM (Pediatric Neurology), FEBN
EULAR course in pediatric rheumatology
Certificate course in genetic counselling
Co-convenor, DM Pediatric Neurology Program
Co-chair, Pediatric Neurology division
Assistant Professor
Department of Pediatrics
AIIMS, Rishikesh
Outline
• Definition
• Epidemiology
• Classification
• Etiopathogenesis and Risk factors
• Clinical features
• Comorbidities
• Diagnosis
• Treatment
• Prognosis
Introduction
• Neurodevelopmental disorder
• Abnormalities of muscle tone, movement and motor skills
• Attributed to injury to the developing brain
• Non progressive disorder
Definition (International consensus 2005)

• 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 behavior, by epilepsy
and by secondary musculoskeletal problems.
The Definition and Classification of Cerebral Palsy. Dev Med Child Neurol. 2007;49:1–44.
Epidemiology
• Overall global prevalence: 2/1000 live births (1)
•  India: Estimated incidence: 3/1000 live births.(2)
• Developed world
• Prematurity
• Extremely low birth weight related morbidities
• Developing countries:
• Intranatal asphyxia
• Postnatal hyperbilirubinemia
• Prenatal rubella

1. Pediatrics. 2016;137.
2. BMJ 2017;356:j462
Classification (Physiological)

Spastic
Choreo-
Cerebral athetoid
Dyskinetic
Palsy
Dystonic
Ataxic
Classification (Topographical)

Spastic CP

Quadriplegic Diplegic Monoplegic

 Topographical Classification

Graham, H., Rosenbaum, P., Paneth, N. et al. Cerebral palsy. Nat Rev Dis Primers 2, 15082 (2016) doi:10.1038/nrdp.2015.82
Functional Classification
Gross Motor Function Classification System (GMFCS)

Children (Basel). 2017 Apr; 4(4): 30.

Functional Classification

Paulson A and Adams JV. Overview of Four Functional Classification Systems Commonly Used in Cerebral Palsy. Children
(Basel). 2017 Apr; 4(4): 30.
Etiology
Prenatal/Neonatal causes
• Prematurity
a) PVL
b) IVH
c) BPD
• HIE
• Neonatal Hypoglycemia
• Congenital Structural abnormalities
a) Inherited abnormalities
b) Secondary to
I. Infections
II. Radiation
III. Toxins (AED/Alcohol/Nicotine)
Sewell MD, Eastwood DM, Wimalasundera N. Managing common symptoms of cerebral palsy in
children. BMJ. 2014;349:g5474
Prenatal/Neonatal causes
• Multiple births
• Stroke
• Intracranial Hemorrhage
• Intrauterine infections
• Neonatal Infections
• BIND
• Genetic susceptibility

BMJ. 2014;349:g5474
Post-neonatal Period Causes
• Stroke
• Head trauma
• Hypoxic events like near-drowning
• Febrile encephalopathy
• Status epilepticus sequelae

BMJ. 2014;349:g5474
Causal factors
Risk factors
• Congenital abnormalities
• Children with CP have more congenital abnormalities (cleft lip and palate,
hypospadias, gut atresias) than other children
• Infections:
• TORCH (CMV)
• Congenital enterovirus
• Arenavirus
• Lymphocytic choriomeningitis virus
• Toxic factors
• Alcohol: Severe malformations like lissencephaly
• Valproate: Interfere with transport of monocarboxylic acids, and carbohydrate
and lipid metabolisms
• Cocaine
• Multiple gestation
• High rate of preterm birth
• Monozygotic twins: Encephalomalacia or porencephaly and secondary CP
• Vascular disease of pregnancy
• Pre-eclampsia and IUGR
• Preterm birth
• Maternal or placental factors such as vasointestinal peptide act early on the
fetal neural axis to stimulate their development
• Post-term birth: Placental involution
• Maternal factors: Maternal thyroid disease
• Gender: M>F
• SES
• Parental education
Genetics
ApoE
• ApoE4 allele associated with worse clinical phenotype (1)
• An additional study found evidence of risk associated with ApoE
alleles E2 and E3. (2)
• Mechanism: (3)
• Less efficient transport of lipids important for repair and subsequent
synaptogenesis
• Worse protection against oxidative injury
• Brain inflammation

1. Eur J Paediatr Neurol 2015; 19: 286–91

2. Dev Med Child Neurol 2010; 52: 666–71.
3. Pediatr Res. 2014 Mar; 75(3): 424-430
Monogenic CP
• Ataxic CP genes
• KCNC3 (severe dominant negative loss of function of potassium channels)
• ITPR1 (Inositol triphosphate-gated calcium channel)
• SPTBN2 (Beta III Spectrin, is a part of the dynamic cytoskeleton)
• KANK1 (Spastic Quadriplegia)
• Uncontrolled actin polymerization
• Adaptor protein 4 complex subunits
• Intracellular trafficking
• alpha-Amino- 3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor-
mediated glutamate excitotoxicity
• ADD3: actin filament accumulation
Dev Med Child Neurol. 2017 May;59(5):462-469.
 Pathogenesis
Hypoxic ischemic/
Inflammatory conditions

Excessive
extracellular excessive release
production of Oxidative Deprivation of matrix of glutamate
proinflammatory stress growth factors modification
cytokines

Primary defect in myelination, gliosis,

and thalamic degeneration with Primary cortical and basal
secondary cortical and thalamic ganglia neuronal injury (Term)
maldevelopment (preterm)

Handbook of Clinical Neurology, Vol. 111 (3rd series)

Pediatric Neurology Part I
 Pathogenesis

• Influx of Na/Ca with

• Cellular energy failure
Reperfusion Cytotoxic
water into cells • Apoptosis
Neuronal
•
phaseFailure of Na/K Edema
•Depolarization of • Cell death
Injury
ATPase pump
neurons

Handbook of Clinical Neurology, Vol. 111 (3rd series)

Pediatric Neurology Part I
 Pathogenesis
Activation of Neuronal
Nitric oxide synthase

NMDA Receptors Calcium influx Free radical production

Increase
Excitatory
Neurotransmitter
extracellularly
AMPA Receptors Na/K influx Cell membrane and
mitochondrial damage

Activation of apoptotic
Handbook of Clinical Neurology, Vol. 111 (3rd series)
genes
Pediatric Neurology Part I
Clinical features
Early signs
Neurobehavioural signs
• Excessive Irritability
• Lethargy
• Sleeps poorly
• Frequent vomiting
• Difficulty in handling and cuddling
• Poor visual attention

Indian J Pediatr. 2018 Nov;85(11)

Developmental reflexes

NeoReviews Plus 2005

Developmental Maneuvers
Developmental Maneuvers Significant findings
Supine • Head persistently in midline
• Limbs unduly extended with persisting fisting
• Reduced or asymmetrical movements
• Not tracking till 180 by 12 weeks
• Not turning head to sounds
Prone • Head to side, not in midline by 12 weeks
• Child not raised off plane of surface by 6 weeks
• Face not at 45-90 degree & no weight bearing on forearms by 12
weeks
• Pelvis still not flat by 12 weeks
• Knees drawn under abdomen >6 weeks
Pull to sit • Pronounced head lag
• Feeling of resistance with flexion of knees
• Entire body rising spontaneously to feet
Developmental Maneuvers

Developmental Maneuvers Significant findings

Held sitting • Inability to lift chin intermittently
• Rounding of back pronounced

Held standing • Unable to lift chin momentarily beyond 8 weeks

• Unable to bear weight at all
• Abnormal degree of plantar flexion
Ventral Suspension • Excessive head lag with limbs hanging limply
• Advanced head control
Motor tone and posture
• Tone: Normal/Increased/Decreased
• Poor head control
• Persistent or asymmetric fisting
• Abnormal oromotor patterns (tongue thrusting/grimacing)
Motor milestones
• Serial examination of motor milestones
• AAP recommendation:
• Complete developmental screening at 9, 18, 30 and 48 months
Clinical patterns
Spastic syndromes
• Upper motor neuron signs
• Spasticity
• Hyperreflexia (± clonus)
• Extensor plantar response.
• Mass movements (instead of fine and individual movements)
• Slow and effortful voluntary movements

Indian J Pediatr. 2018 Nov;85(11)

• Spastic diplegia :
• Gross motor problems particularly in lower limbs
• Retained fine motor functions in upper limbs

• Spastic quadriplegia:
• Severe motor impairments
• Both upper and lower limbs are affected nearly equally
• Speech and language development
• Visual impairment, Epilepsy, Feeding difficulty
• Spastic hemiplegia
• Arm is typically affected more than leg
• Athetotic posturing
• Sensory deficits
• Intellectual impairment, hemianopia, and other visual problems
• Behavioural problems including anxiety, oppositional defiance, and specific
phobias.
Dyskinetic syndromes
• Involuntary movements
• Stiff limbs during attempted movement or with emotion
• DTR: Normal/Difficult to elicit
• Athetoid movements of toes
• Dystonia: striatal toe
• No contractures
• Choreo-athetoid CP
• Rapid disorganized unpredictable contractions of individual muscles/ muscle
groups
• Involves face, bulbar muscles, proximal extremities and digits
• Slow writhing movements involving distal muscles
• Oropharyngeal difficulties
• Primitive reflexes often persists
• Dystonic CP
• Co-contraction of agonist and antagonist muscles.
• Co- existent pyramidal signs and dysarthria.

• Ataxic CP
• Delayed Motor and language milestones
• Ataxia usually improves with time.
Clinical features
• “Positive” features
• Spasticity
• Hyper-reflexia
• Co- contraction
• “Negative" features
• Weakness
• Loss of selective motor control
• Sensory deficits
• Poor balance

Graham HK, Selber P. Musculoskeletal aspects of cerebral palsy. ]

BoneJointSurg (Br)2003;85-B:157-166.
Co-morbidities
• Intellectual disability
• Occurs in about 50% patients
• Spastic Quadriplegic: most severely affected
• Language development in hemiplegic CP

• Neurobehavioural/Neurodevelopmental disorders
• Occurs in nearly 25% cases
• Behavioural , emotional, and/or psychiatric disorders
• Emotional lability, poor attention and vigilance, OCD traits

Pediatrics. 2012;130:e1285–312.
• Epilepsy
• Occurs in 25-45% patients
• Most common in spastic Quadriplegic CP

• Visual disorders
• Occurs in nearly 30% cases
• Most common in CP following immaturity
• Low visual acuity; strabismus; Amblyopia; Visual field defects

Novak I, Hines M, Goldsmith S, Barclay R. Clinical prognostic

messages from a systematic review on cerebral palsy. Pediatrics.
2012;130:e1285–312.
• Hearing/Speech impairment
• Observed in 30-40%
• Aphasia/dysarthria/mutism

• Growth failure
• Poor nutrition
• Inadequate intake and gastrointestinal problems

Pediatrics. 2012;130:e1285–312.
• Gastrointestinal disorders
• Occurs in nearly 90% patients
• Constipation/GERD/Vomiting/swallowing disorders/pain

• Pulmonary disorders
• Secondary to recurrent aspirations/scoliosis/respiratory muscle
incoordination

Pediatrics. 2012;130:e1285–312.
• Orthopedic disorders
• Subluxation/dislocation
• Progressive dysplasia of hip joints/ foot deformities/scoliosis
• Torsional changes in long bones

• Urinary disorders
• Occurs in 30-60% children
• Dysfunctional voiding syndrome
• Enuresis/ frequency/ urgency/ stress incontinence

Pediatrics. 2012;130:e1285–312.
• Pain
• Reported by 50-75% patients
• 25% experience pain that limits activities
• Causes: Dystonia/ hip subluxation/ constipation
• May go unrecognized due to communication difficulties

• Sleep
• Sleep wake transition
• Excessive daytime sleepiness and difficulty in arousal

Pediatrics. 2012;130:e1285–312.
How to evaluate??
• Functional evaluation
• Tone evaluation
• Investigations

Indian J Pediatr. 2018 Nov;85(11)

Investigations
• Neuroimaging
• Hearing assessment
• Visual assessment
• EEG
• Skeletal x ray
• DEXA/viamin D
• Sleep study
• GERD screening
• Gait analysis
Diagnosis of CP is essentially clinical.
INCLEN tool (INDT-NMI)
• Three sections:
• Section-I: Four questions to elicit information from the parents/primary caregiver
of the child regarding attainment of selected motor developmental milestones.
• Section-II (Observations): Three observations for assessing hand function, gait
and muscle weakness.
• Section-III: Six questions, and the operator (graduate physician) does the
neurological examination necessary for confirmation of NMI.
• Other Information on age at onset of symptoms, course of the illness and
obvious clinical evidence of involvement of spinal cord (i.e. pilonidal
sinus, tuft of hair).
• Sensitivity: 75%; Specificity: 87%
Role of Neuroimaging
Neurosonogram:
• Ventriculomegaly in IVH and PVL
• Proxy marker for loss of cortical white matter or gray matter 
• Cerebellar haemorrhage
• Can be serially performed in high risk newborns

The Journal of Pediatrics, 2004; 145(2), S19–

S27.doi:10.1016/j.jpeds.2004.05.018 
Role of CT scan:
• CT detects abnormality in upto 70-80% cases
• Findings:
• Diffuse brain atrophy
• White matter atrophy and hypodensities with or without ventriculomegaly
• Malformations including pachygyria, and Dandy-Walker malformation (Spastic
CP)
• Cystic encephalomalacia
• Focal vascular insult
• Correlates with topographic distribution of motor deficit.

J Afr Imag Méd 2013; (5), 3: 134-142

MRI Brain
• Should be done in all patients (AAP recommendations 2004)
• Helps in
• Broader appreciation of timing of lesions
• Extent of white matter involvement
• Complexity of the motor spectrum of disability
• In case of any discrepancy between clinical findings and
Neuroimaging:
• Rule out underlying metabolic disorder

Neuroimaging and cerebral palsy in children. Minerva Pediatr. 2009 Aug;61(4):415-24.

MRI:
• White matter damage
• Grey matter damage:
• Basal ganglia, cortical defects, thalamic abnormalities, and diencephalic
lesions.
• Cortical malformations
• Stroke
• Neonatal MRI: Birth association of perinatal medicine; 2016)
• Should be obtained between 5 and 14 days of age
• Sensitivity: 98% (80-100%); Specificity: 89% (62-98%)

The Journal of Pediatrics, 2004; 145(2), S19–

S27.doi:10.1016/j.jpeds.2004.05.018 
Periventricular hyperintensities and undulating ventricular
margins
Multi cystic encephalomalacia
T2 hyper intensities in posterior putamen (open arrow) and thalami bilaterally (dotted line with closed arrow).
Hyper intensities in occipital lobe; characteristic of neonatal hypoglycemic insult.
T2 hyper intensity involving bilateral globus pallidi, a feature of kernicterus sequelae
• Hearing assessment:
• OAE
• BERA is must for all patients
• Vision assessment:
• Fundus examination
• Strabismus
• VEP
• EEG
• Mandatory if the child has a h/o seizures
• Should be performed in a child who is not gaining milestones (after an initial static
insult) to r/o hypsarhhythmia.
Skeletal x ray
• Skeletal maturation
• Osteoporosis
• Spine: scoliosis; spondylolysis; spondylolisthesis
• Hip
• windswept deformity, increased femoral anteversion, apparent coxa valga,
subluxation, deformity of the femoral head, hip dislocation, and formation of a
pseudoacetabulum.
• Knee: flexion contracture, patella alta, and patellar fragmentation
• Ankle: Progressive equinovalgus and equinovarus; subluxation of the
talonavicular joint
https://doi.org/10.1148/radiographics.22.2.g02mr19257
DEXA scan: Osteoporosis

PSG:
• Multiple issues related to muscle tone, neuromuscular regulation of
the airway, posture and chest deformity.
• Most common indication: Chronic snoring and apnea

Videofluoroscopic swallow study (VFSS): For oromotor inccordination

Turk Pediatri Ars. 2017 Mar; 52(1): 23–29.

GERD screening:
• 24 hour ambulatory esophageal pH monitoring
• Abnormal GERD: Reflux index >5%
• Multiple intraluminal impedance (MII) monitoring
• Indication:
• Severe feeding difficulties
• FTT
• Anemia
• Recurrent chest infections

Child Care Health Dev. 1993 Mar-Apr;19(2):109-18

Int J Mol Sci. 2017 Aug; 18(8): 1671
Clinical Gait analysis
• Aim: To determine what is causing a patient to walk in the way he/she
does.
• Four main types of data recorded simultaneously: spatiotemporal,
kinematics, kinetics and electromyography data
• Standardised clinical videos are recorded with numerical video
cameras generally synchronised with the opto-electronic system

EFFORT Open Rev. 2016 Dec; 1(12): 448–460.

• Spatiotemporal data:
• walking speed, cadence, stride length, step length, step width, duration of the
stance and swing phases 
• Kinematic data: Movement of the body described in CGA by the
angular variations of the different joints/segments: ankle, knee, hip,
pelvis and trunk.
• Dynamic or kinetic data: It describe the forces applied by the patient
during his/her gait
• Force-plate(s) embedded in the ground of the gait laboratory are used.
• Electromyography (EMG) data show the timing and intensity of the
muscle activity 
• Timing of the activity (such as lacking, delayed or permanent activity)
• Co-contraction periods
• Muscle spasticity during gait.
Management
Principles of treatment
• CP cannot be cured
• Goals
• Promote function
• Prevent secondary impairments
• Increase the child's developmental capabilities.
• Functional priorities
• Communication
• Activities of daily living
• Mobility in the environment
• Walking
• Primary abnormalities
• spasticity, hyper-reflexia, co- contraction, weakness, loss of selective motor
control, sensory deficits and poor balance
• Secondary abnormalities: Growth disorders
• Joint reaction forces determine the ultimate shape of bones
• Maldirected or bent levers reduce the effectiveness of muscle action
• Normal muscle growth
• Tertiary abnormalities
• Compensations that the individual uses to circumvent the primary and
secondary abnormalities of gait
Gage JR, Novacheck TF. An update on the treatment of gait
problems in cerebral palsy. JPediatrOrthop2001;10:265-274
Grade Modified Tardieu Scale Modified Modified Ashworth Scale
0 No resistance throughout the course of the No increase in muscle tone
passive movement

1 Slight resistance throughout the course of the Slight increase in muscle tone, manifested by a catch and
passive movement, with no clear catch at release or by minimal resistance at the end of the range of
precise angle motion when the affected part(s) is moved in flexion or
extension

2 Clear catch at precise angle, interrupting the Marked increase in muscle tone, manifested by a catch in
passive movement, followed by release the middle range and resistance throughout the remainder
of the range of motion, but affected part (s) easily moved

3 Fatigable clonus (<10 s when maintaining Considerable increase in muscle tone, passive movement
pressure) occurring at precise angle difficult

4 Infatigable clonus (>10 s when maintaining Affected part(s) rigid in flexion or extension
pressure) occurring at precise angle

Ansari  NN et al.  Physiother Theory Pract 2006;22:119–25

Boyd  RN et al. Eur J Neurol 1999;6 (Suppl 4):S23–35
Modalities for spasticity management
• Pharmacologic Modalities
• Baclofen

• Benzodiazepines: Clonazepam Dantrolene

• Alpha2-adrenergic agonists: tizanidine, clonidine

• Local injections

• Phenol injections

• Botulinum toxin injections

• Intrathecal baclofen
Indian J Pediatr. 2005 Oct;72(10):869-72
Modalities for spasticity management
• Non-pharmacologic Modalities
• Physiotherapy
• Occupational therapy
• Use of adaptive equipment
• Use of Orthoses
• Various orthopaedic surgical procedures
• Selective Dorsal rhizotomy

Indian J Pediatr. 2005 Oct;72(10):869-72

Generalized Spasticity:
Benzodiazepines (1st line)
• MOA:
• Increase the affinity of GABA to the GABA A receptor complex pre and post-synaptically
• Reduce generalized spasticity, hyperreflexia, and painful muscle spasms.
• Improve sleep and reduce anxiety
• Dosages:
• Diazepam: 1 to 10 mg per dose administered 3 to 4 times per day
• Clonazepam: 0.01-0.3 mg/ kg/day given 2 to 3 times per day
• Side effects:
• Sedation
• Drooling, ataxia, and cognitive dullness

] Neurol Neurosurg Psychiatry 2005;76(4):459-463.

Baclofen (Yellow measure)
• MOA:
• Gamma-amino butyric acid (GABA) agonist.
• Reduces the release of excitatory neurotransmitters and substance P by binding to
the GABAB receptors
• Dose: Starting dose is 2.5 mg daily, maximum of 20-60mg/day
• Side effects
• Weakness, sedation, ataxia, nausea, impaired cognition, orthostatic hypotension,
dizziness, depression
• Withdrawal syndrome:
• seizures, hallucinations, hyperthermia, dysesthesia, pruritis, and rebound
spasticity.
J Child Neuro12001; 16(1):31-36.
Dantrolene Sodium (Yellow measure)
• MOA:
• Inhibiting calcium release from the sarcoplasmic reticulum, thus uncoupling
electrical excitation from contraction
• Dosage:
• started at a low dose, typically 0.5 mg/kg given twice daily,
• Increasing by 0.5 mg/kg every 5-7 days to a maximum dose of 12 mg/kg/day or
400 mg/day.
• Side effects:
• Skeletal muscle weakness
• Fatigue, drowsiness and diarrhea
• Hepatotoxicity

Indian J Pediatr. 2005 Oct;72(10):869-72

Alpha2 Adrenergic Agonists (Yellow
measure)
• MOA:
• Hyperpolarization of motoneurons
• Prevention of release of excitatory amino acids from the presynaptic terminal
of spinal interneurons
• Facilitation of the action of inhibitory neurotransmitter glycine
• Antinociceptive effects mediated by release of substance P in the spinal cord

c
Alpha2 Adrenergic Agonists
• Dosages:
• Tizanidine: 1 mg given at bedtime for children under 10 yrs and 2 mg for
children of 10 yrs or more; maintenance dose is 0.3-0.5 mg/kg/day QID.
• Clonidine: Starting dose of 0.05 mg per day and increased by 0.05 mg every
week to a maximum of 0.3 mg per day TDS
• Side effect:
• Sedation
• Hypotension especially with clonidine
• Depression, dry mouth, dizziness, and hepatotoxicity.

Indian J Pediatr. 2005 Oct;72(10):869-72

Focal Spasticity: Local drug Injections
Botulinum Toxin
• Exotoxin produced by the bacterium Clostridium botulinum.
• MOA:
• Inhibits presynaptic acetylcholine release at the neuromuscular junction
• Causes reversible partial flaccid paralysis of the muscle in which it is injected
• Effect starts within 12-72 hours and lasts upto 3-6 months
• Reversal of effect: Proximal nerve sprouting followed by recovery of
vesicle turnover and gradual regression of sprouts.
• Repeat after every 3-6 months

Cochrane Database Syst Rev 2004; 18(4) : CD003469.

• Formation of antibodies
• Side effects:
• Local pain and bruising
• Excessive weakness of muscles, constipation, fever, and incontinence
• Uses:
• Focal spasticity
• Reduces spasticity in lower extremity
• Improve joint range of motion and gait
• Improve equinus deformity
• Improve adductor tone
• Strongly recommended for limb spasticity
Cochrane Database Syst Rev 2004; 18(4) : CD003469
Alcohol and Phenol Injections (Yellow
measure)
• Injected in the specific nerve or muscle after identifying the nerve and
muscle affected by electrical stimulation.
• MOA
• local neuronal necrosis secondary to denaturation of proteins
• Onset occurs within hours, lasts upto 2-36 weeks.
• Concentration of the medication, duration of exposure, method of
delivery, and history of prior injection influences the duration
• Disadvantages:
• Need for special skills, Electrical stimulation procedure
• Sedation, and Anaesthesia.

Indian J Pediatr. 2005 Oct;72(10):869-72

State of Evidence

Novak I, McIntyre S, Morgan C, et al. A systematic review of interventions for children with cerebral palsy: state of the
evidence. Dev Med child Neurol. 2013 Oct;55(10):885-910.
Dystonic CP:
Trihexylphenidyl
• MOA
• Anticholinergic action
• Reduces striated muscle contraction and parasympathetic activity
• Dosages: 0.5mg (infant); 1 mg (Child); 2mg (adolescent) thrice a day
• Adverse effects:
• Dry mouth and eyes
• GI disturbances; Urinary retention
• Behavioral disturbances
• Levodopa: Inconclusive evidence
J Child Neurol. 2014 Apr;29(4):534-7.
AACPDM Recommendations for Dystonic
CP
• Indications for treatment:
• Pain or difficulty sleeping associated with dystonia in CP
• Oral baclofen is considered a first line medication for the management of
dystonia in CP
• Trihexyphenidyl can be used as a second line medication.
•  Benzodiazepines: Intermittently for dystonic storms or disturbed sleep
• Gabapentin: Dystonia associated with pain.
• Clonidine: Disturbed sleep associated with dystonia.
•  Intrathecal baclofen: Severe generalized hypertonia with a combination of
dystonia and spasticity.
Choreoathetoid CP
Benzodiazepins
Tetrabenazine :
• MOA:
• Benzoquinolone that depletes synaptic dopamine transmission
• Reducing vesicular monoamine uptake
• Post synaptic dopaminergic receptor antagonist
• Adverse effects:
• Sedation; Bradykinesias
• Hypertonia; Muscle rigidity
• Behavioral issues
Antipsychotic medications: Fluphenazine and haloperidol
• MOA: Blocks the effect of dopamine on basal ganglia
•  Adverse effects:
• Weight gain
• Endocrine disturbances
• Sedation
• Anticholinergic effects
• Hypotension
• Seizures and extrapyramidal symptoms.
Management of Drooling (AACPDM
Recommendations)
• Optimize conditions - Optimize positioning and medical management
of factors that affect drooling.
• Consider whether medications being used for other conditions, such
as epilepsy, are increasing drooling.
• Oral motor treatment (Yellow measure)
• Anti-cholinergic agents:  
• Glycopyrrolate, scopolamine, benzhexol and benztropin are the most
commonly used agents.
• Adverse side effects such as excessive thickening of secretions, urinary
retention, constipation, headache, blurred vision and behavioral disturbance.
• Intraglandular Botulinum toxin injections to the submandibular +/-
parotid glands (Strongly recommended)
• Consider after inadequate response to anti-cholinergic treatment.
• Repetitive dosing at 6 month intervals
• Side effects: Irritation at the injection site, pain, hematoma, dry mouth,
thickened secretions, or problems with chewing and swallowing from
diffusion to the surrounding muscles thus, increasing aspiration risk.
Epilepsy
• Special consideration while choosing drug:
• Earlier age of onset
• Poorer seizure control
• Increased risks of status epilepticus
• Need for more than one antiepileptic drug for seizure control in children with
CP and seizures.
• The principles of drug therapy in children with cerebral palsy and
epilepsy are the same as those for children with epilepsy in general.
• In general, carbamazepine or lamotrigine should be the first-line AEDs
for focal-onset seizures, and valproate for generalized seizures

 Indian J Cereb Palsy 2016;2:3-21

GERD
Nonpharmacological measures:
• Raising the head of the bed
• Reducing weight
• Limiting secondary exposure to smoke
• Avoiding caffeine, spicy foods, fatty foods, and chocolate
Pharmacological:
• Acid suppressants: 4- to 8-week course of either H2RAs or PPIs and
regularly assessing the need for long-term acid suppression therapy
(NASPGHAN and ESPGHAN 2018 guidelines)

Canadian Family Physician November 2019, 65 (11) 796-798

• Histamine-2 receptor antagonists:
• Increasingly ineffective within 6 weeks of initiating treatment (tachyphylaxis)
• Due to changes in the sensitivity of H2 receptors, changes in H2 receptor turnover,
and an increase in receptors for other mediators of acid secretion (eg, gastrin).
• Prokinetic agents (Mosapride) (1):
• Lower the rate of reflux by promoting an increased rate of gastric emptying,
improving esophageal peristalsis, and increasing LES pressure
• Baclofen: a γ-aminobutyric acid receptor agonist, reduces TLESR episodes (2)
• Side effects: Dizziness, fatigue, and a lower threshold for seizures
• 2018 guidelines by NASPGHAN and ESPGHAN suggest that baclofen should only be
considered in children if other pharmacologic treatments have failed

1. Pediatr Int 2017;59(3):347-51. 
2. J Pediatr Gastroenterol Nutr 2004;38(3):317-23.
Constipation:
Dietary modification:
• More fluids, absorbable and non-absorbable carbohydrate
• Non-absorbable carbohydrate (sorbitol) is found in some fruit juices like apple, pear and
prune juices.
• Balanced diet including whole grains, fruits and vegetables.
• The recommended daily fiber intake is age (in years) + 5 in g/day
Toilet training:
• After 2 to 3 years of age
• The child is encouraged to sit on the toilet for 5 to 10 minutes, 3 to 4 times a day immediately
after major meals for initial months.
• Daily record (stool diary) of bowel movements, fecal soiling, pain or discomfort, consistency
of stool and the laxative dose.

Indian Pediatrics;Vol 53;April,2016

Drugs:
• PEG: 0.5-1 gm/kg/day.
• Less side effects as compared with lactulose.
• Lactulose: Distention, bloating and excess flatus.
• Glycerin: Suppository to draw water into the rectum to produce a bowel
movement.
• MgSO4:
• MOA: Intraluminal accumulation of hyperosmolar particles.
•  Intraluminal secretion of cholecystokinin and increases nitric oxide (NO) release.
• Adverse effects: Reducing the intestinal absorption of fat, protein and carbohydrates
following solid meal ingestion.
Osteoporosis:
• Bisphosphonates:
• Suppress bone resorption
• Improves bone density
• Strongly recommended
• Calcium and Vitamin D supplementation (Yellow measure)

Dysphagia: (Yellow measures)

• Promoting safe swallowing by changing food textures, sitting position, oral
motor skills and using oral appliances and equipment
Rehabilitation
Early Interventions (Yellow measures)
• Principle: Therapy and early education to promote acquisition of
milestones, via group or individual stimulus
• Get care for your child as early as possible.
• Get your child assessed at a health centre.
• Ask about special places and community projects that help children
with disabilities.
• Ask where to find people with special training in helping children with
disabilities.
• Treatments happen in the home, not the hospital.
Communication
• Look, talk, touch, and sing to your child often.
• Tell your child what you are doing.
• Give your child choices: Yes/no, gestures, point, use communication
boards, smile.
• Use single words and simple gestures.
• Give time for your child to respond.
• Do not force a child to speak
• Alternative and augmentative
communication (Yellow measure)
Feeding
• Positioning:
• Hold your child in an upright position.
• Turn your child’s body towards you as far as possible.
• Stablise the child’s head in a neutral position.
• Support the jaw.
• • Introduce food of different textures:
• Children with swallowing difficulties may need thicker food.
• Give small bites of food with a high nutritional value.
• Encourage your child to feed him/herself.
Positioning (Yellow measure)
• Carrying your child
• Carry her in an upright position.
• Bend his hips and knees.
• Helping your child to lie down
• On her stomach: Place a roll, wedge or cushion under your child.
• On his side: Keep both arms forward. Bend one hip and knee.
• Sitting: Using a special chair
• Keep your child’s feet flat on the floor.
Keep the bottom level against the back of the chair.
Standing and walking
Daily Living activities
• Stimulate your child to do the activity by herself
• Eating and drinking:
• Let your child try to feed himself.
• She should sit in a good position alone in a chair or stool.
• He may need to use a spoon with a thick handle to make it easier to hold.
• Using the toilet:
• When going to the toilet, encourage your child to help as much as possible
during undressing and dressing.
• Make something, like a bar, for the child to hold onto. – Use a box or special
chair for her to sit on.
Behavioural therapy (Yellow measure)

Note: No evidence of CBT in CP patients.

Physical Therapy
• Integral part of CP management
• Range from regular exercise program to modalities like electrical
stimulation
• Helps in:
• Encourages caretakers to learn how best to handle, toilet, wash, and feed
their children with CP
• Promotes posture, mobility, and transfer.
Physiotherapy
• Improves
• Muscle strength,
• Local muscular endurance
• Overall joint range of motion.
• Appropriate posture
• Progressive resistive exercises: Improves muscle strength (Yellow measure)
• Program using low resistance and more repetitions: Enhances local muscular
endurance
• Exercises to improve balance, posture control, gait, mobility, and ability to
transfer.
• Stretching: For contracture prevention (Yellow measure)
 Indian J Pediatr. 2005 Nov;72(11):979-83.
Occupational therapy
• It seek to improve function
• Aim: Increasing functional skills and independence
• Focus:
• Maximizing a child’s ability to accomplish activities of daily living, education,
and work
• Improving the fine motor activities
• Organizing play areas
• Providing adaptive equipment for self care and learning
• Modify the learning environment to facilitate attention and information
processing.

A systematic review of interventions for children with cerebral palsy

state of the evidence. Dev Med Child Neurol. 2013;55:885–910.
Yellow measures
Occupational therapy after BoNT
• Improved hand use via CIMT
• Goal‐directed training,
• Strength training
• Functional hand splints
• Improved symptom management via casting and immobilisation splint
• Strongly recommended
Electrical Stimulation (Yellow measures)
• Goal: To increase muscle strength and motor function.
• Neuromuscular electrical stimulation (NMES)
• Application of transcutaneous electrical current that results in muscle
contraction
• Mechanism:
• Increases muscle strength by increasing the cross-sectional area of the muscle
• Increased recruitment of type 2 muscle fibres
• Increasing the muscle blood flow and bulk (TES)

 Indian J Pediatr. 2005 Nov;72(11):979-83.

• Body Weight Support Treadmill Training: (Yellow measure)
• Child is supported in a harness on the treadmill in an upright posture limiting
weight bearing
• Attempts to walk on the slowly moving treadmill, eliciting the stepping
movements

Fitness training: (Strongly recommended)

• Planned structured activities involving repeated movement of skeletal
muscles that result in energy expenditure to improve or maintain levels of
physical fitness
• Effective only in those that can take aerobic training.
• Patterning
• Concept: Typical motor development can be facilitated in the brain injured
children by passively repeating the sequential steps of typical development.

• Constraint-induced therapy (Strongly recommended)

• Normally functioning or stronger upper extremity is immobilized for a
variable duration in order to force the use of the affected or weaker upper
extremity over time.

Dev Med Child Neurolo Supp12002; 91:44.

Goal‐directed training/functional training:
• Task specific practice of child‐set goal‐based activities using a motor learning
approach
• Improves hand function (Green measure)
• Also improves gross motor functions but evidence is weak

Context-focused therapy:
• Changing the task or the environment (but not the child) to promote
successful task performance
• Strongly recommended to improve functionality
Hydrotherapy: (Yellow measures)
• Aquatic‐based exercises
• Improved vitals and gross motor function

Massage: (Yellow measure)

• Therapeutic stroking and circular motions applied by a massage
therapist to muscles to relieve pain and tension
• Reduces pain and spasticity
Play therapy (Yellow measure)
• Improves play skills
• Improved child coping and reduced stress
Bimanual training: (Strongly recommended)
• Repetitive task training in the use of two hands together
• Aim to improve hand function in hemiplegic CP

• Acupuncture (Yellow measure)

• Electro‐stimulation to scalp and body via needles and manual pressure
• Reduces painful muscle spasm and overall motor function
Vojta: (Yellow measure)
• Therapist applied pressure to defined zones on the body whilst
positioned in prone, supine or side lying, where the stimulus leads to
automatically and involuntarily complex movement
• Improves strength and movement
Whole‐body vibration: (Yellow measure)
• Assistive technology that transmits low‐frequency vibration to the
body through a broad contact area of a vibrating surface, e.g. feet in
standing, buttocks in sitting, or whole body
• Improves strength and gait
Pressure care (Strongly recommended)
• Prevention of pressure ulcers via good positioning, repositioning, and
suitable support surfaces
• Foam mattresses; wheelchair seat cushions

Sensory processing: (Yellow measure)

• Therapeutic activities to organize more appropriate responsiveness
(i.e. not hyper‐responsive and not hyporesponsive) to task and
environmental demands, including self‐regulation
• Hippotherapy (Yellow measure)
• Therapeutic horse-back riding has been shown to improve muscle
tone, balance, and postural control in children with CP
• Also helps to enhance social integration
• Improves hip and trunk symmetry and stability 
• Improved gross motor function 

Biofeedback: (Yellow measure)

• Electronic feedback about muscle activity to teach voluntary control
• Improves muscle activation and active range of motion
• Effective in combination with other treatments
Oral motor treatment: (Yellow measures)
• Sensory stimulation to lips, jaw, tongue, soft palate, larynx, and
respiratory muscles to influence the oropharyngeal mechanism
• Improves verbal speech as a result of non‐speech oral motor
exercises 
• Improved safety of swallowing and reduced drooling
Virtual Reality (Yellow measures)
• Use of interactive simulations created with computer hardware and
software to present users with opportunities to engage in environments
that appear to be and feel similar to real-world objects and events
• VR applications use interactive simulations that respond to a user’s
movement such that a child can interact within a virtual environment
while performing functional activities
• In the virtual environment, task difficulty can be easily adjusted to
provide sufficient challenge for a child while playing.
• It can also provide immediate visual and auditory feedback that is related
to task performance or results.
Therasuits: (Yellow measure)
• Breathable soft dynamic orthotic full body suit, designed to
improve proprioception, reduce reflexes, restore synergies and
provide resistance
• Conductive Education (Yellow measure)
• Hungarian educational classroom‐based approach to teaching movement using
rhythmic intention, routines and groups
• Improved ‘orthofunction’ (response to biological and social demands)
• Also improves cognition

Home programmes: (Strongly recommended)

• Therapeutic practice of goal‐based tasks by the child, led by the parent
and supported by the therapist, in the home environment
Solution‐focused brief therapy: (Yellow measure)
• Resource orientated and goal focused approach to generating
solutions to life challenges
• Reduces parental depression, improved coping and improved
parenting skills
NEURODEVELOPMENTAL TREATMENT
(NDT)
Red Measure
• Conceptual basis:
• Motor abnormalities in children with CP are due to failure of normal development
of postural control and reflexes because of the underlying dysfunction of the
central nervous system
• Aim:
• To facilitate normal motor development and function (Red STOP)
• To prevent development of secondary impairments due to muscle contractures
and joint and limb deformities (Red STOP)
• Various techniques to inhibit and control abnormal tone, reflexes and
movement patterns have been described.
• Direct, passive handling and guidance to optimise function
Effects of neurodevelopmental treatment (NDT) for cerebral palsy: an AACPDM evidence
report. Dev Med Child Neurol 2001; 43 : 778-790.
SENSORY INTEGRATION (SI) (Red
Measure)
• Conceptual basis: In order to develop and execute a normal adaptive
behavioural response, the child must be able to optimally receive,
modulate, integrate, and process the sensory information.

• It facilitates the normal development and improve the child's ability

to process and integrate sensory information (visual, perceptual,
proprioceptive, auditory, etc)
Mental Retardation and Developmental Disabilities Research
Revzezos 2005; 11 : 143-148.
Hyperbaric Oxygen therapy
• Mechanism
• Increase the oxygen available to the neurons surrounding the injured area of the brain and
revive these dormant neurons
• Decrease brain edema by inducing cerebral vasoconstriction
• Pressure: 1.75 atmospheric pressure
• Complications:
• Ear pain,
• Bleeding from ears, tympanic membrane perforation
• Myopia
• Pneumothorax
• Seizures
• Red STOP as per the current evidences

Evid Rep Technol Assess (Summ). 2003; 85 : 1-6.

Orthopedic Interventions
• To treat correct deformities induced by muscle overactivity
• Serial casting :
• To stretch shortened muscles
• Improves passive range of motion of lower limbs (Green measure)
• Reduces spasticity (Yellow)
• Use to augment the effect of Botulinum toxin also (Green)
• Multiple tenotomies and transfers of the thigh adductors
• Femoral osteotomy
• Arthrodesis
• Muscle lengthening (for CTEV): Yellow measure
Orthosis

Ankle-foot Orthosis Knee Orthosis Hip-Knee Orthosis

Removable external devices designed to support weak or ineffective joints or muscle

• Hip surveillance: active surveillance and treatment for hip joint
integrity to prevent hip dislocation (Green measure)
• Reduced hip dislocation and need for orthopaedic surgery 
• Hand surgery: (yellow measure)
• Surgery to improve hand function and alignment
• Improve thumb in palm posture
• Hip surgery: (yellow measure)
• Orthopaedic surgery to improve musculoskeletal alignment of the hip
• Reduced hip subluxation via soft tissue surgery (adductor release)
• Reduced hip subluxation via bony surgery 
Surgical Interventions
Selective Dorsal Rhizotomy (SDR)

• Selectively divides parts of the dorsal lumbosacral roots of the spinal

cord
• Interrupts the afferent limb of the reflex arc on the sensory side
• Reduces spasticity without causing paralysis (Green measure)
• Improves gait kinematics
• Improves functionality

Long-term functional benefits of selective dorsal rhizotomy for spastic cerebral

palsy. J Neurosurg Pediatr. 2013;12:142–50.
• Indications:
• Typically ambulatory or nearly ambulatory (GMFCS levels II and III, sometimes
GMFCS level I)
• Some degree of preserved selective motor control in the lower extremities
• Have adequate strength
• 4 to 7 yrs of age
• Spared cognitive skills
• Motivated to walk
• Have minimal, if any, dystonia
• Commitment to a rigorous daily physical therapy program for 6 to 12 months
INTRATHECAL BACLOFEN (Yellow
measure)
• Advantages
• Higher cerebrospinal fluid (CSF) concentration
• Less chances of generalized side effects that are associated with oral
administration.
• Also helps in dystonia
• Adverse effects:
• Weakness, fatigue, confusion, hypotonia and lethargy
• Respiratory depression and coma
• CSF leakage, catheter infections and meningitis.

J Child Neuro12001; 16(1):31-36.

Indian J Pediatr. 2005 Oct;72(10):869-72
Deep Brain Stimulation
• Indication: Severe generalized dystonia associated with significant
impact on care/comfort.
• Effective in Dyskinetic CP (Yellow measure)
• MOA:
• Precise mechanism of action not known
• Stimulation should create a signal modification in the target nuclei or replace
a physiological signal into an aberrant output from the target nuclei.
• DBS parameters: Variable amplitude (range, 1-6.5 V), pulse width
(range, 60-240 μs) and frequency (range, 30–185 Hz)
• Effects of deep brain stimu- lation in dyskinetic cerebral palsy: a meta-
analysis. Mov Disord. 2013;28:647–54.
• Eur J Pediatr Neurol. 2018 Mar;22(2):308-315. 
• Also improves some aspects of psychological distress,
• Interpersonal sensitivity, depression, paranoid ideation, and psychotic
symptoms
• Adverse effects:
• Infection
• Worsening dystonia
• Dysarthria/Hemiparesis
• Seizures
• Spasm of pharynx

• Eur J Pediatr Neurol. 2018 Mar;22(2):308-315. 

SELMS (Single Event Multilevel Surgery)
Yellow measure
• Definition: At least one surgical procedure, performed on two
different anatomic levels (hip, knee or ankle) on both sides of the
body
• Principle: Identification and correction of all contractures and lever
arm deformities, deemed to be interfering with dynamic gait function.
• Neither uniform nor symmetrical

Gait Posture. 2013 Jan;37(1):23-8.

• Individually tailored to the child’s needs as determined by
• Standardized physical examination
• Radiological evaluation
• Functional assessment
• Includes:
• Muscle tendon lengthening
• Tendon transfer
• Rotational osteotomy
• Stabilization of the hip and foot

Gait Posture. 2013 Jan;37(1):23-8.

Surgical intervention for drooling
• Indications:
• Profuse, persistent anterior drooling
• Continued symptoms despite maximal conservative or pharmacological
treatment
• Patients with posterior drooling who have chronic aspiration and/or recurrent
respiratory infections
• Surgical procedures may include duct ligation or rerouting, sublingual
or submandibular gland excision, and varying combinations of these
procedures.
• Side effects: Xerostomia; Wound infection; Duct recanalization
Surgical Intervention for epilepsy
• Focal Resection: A portion of the brain is removed and subdural
electrodes are implanted to help control the seizures.
• Hemispherectomy: Almost an entire side of the brain is removed in
order to disable the portion of the brain that’s causing the seizures.
This surgery is used only in rare cases.
• Corpus Callosotomy: The corpus callosum in the brain is cut in order
to reduce generalized seizures. No portion of the brain is removed
with this type of surgery
Surgical Intervention for GERD
• Nissen fundoplication: Wrapping the stomach fundus around the
lower end of the esophagus to increase pressure on the LES.
• Indication: When pharmacologic therapy fails.
• Complication rate in children with neurologic impairments (12.8%) is
higher than in the general population (4.2%)
• Yellow measure

 Pediatr Heal Med Ther 2015;6:1-8

Feeding issues: Gastrostomy
• Surgical placement of a non‐oral feeding tube to prevent or reverse
growth failure, or prevent aspiration pneumonia, e.g. percutaneous
endoscopic gastrostomy (PEG), jejunostomy
• Yellow measure
Dev Med child Neurol. 2013 Oct;55(10):885-910.
Dev Med child Neurol. 2013 Oct;55(10):885-910.
CP Mimics
Type of CP Differential Diagnosis
Spastic quadriplegic CP Posttraumatic atlanto-axial instability
Neural tube defects
Pelizaeus-Merzbacher disease
Peroxisomal disorders
Sandifer syndrome
Spastic Diplegic CP Arginase deficiency
Hereditary spastic familial paraparesis
Juvenile metachromatic leukodystrophy
Extra-pyramidal CP Primary Neurotransmitter deficiencies
Dopa Responsive Dystonia
Glutaric aciduria type I
Ataxic CP Neurodegenerative causes of ataxia
Complementary treatments
 Prognosis

• Good Prognostic signs: • Poor prognostic signs:

• Hemiplegic CP • No head balance by 20 mo
• CP with athetosis or ataxia. • Retained primitive reflexes
• Sitting by 2 y • No postural reactions by 24 mo
• Crawling before 30 mo of age • No crawling by approximately 5 y
of age
• Spastic CP
• No walking by 9 yr of age
Thank you