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Cerebral Palsy
Cerebral Palsy
1. Pediatrics. 2016;137.
2. BMJ 2017;356:j462
Classification (Physiological)
Spastic
Choreo-
Cerebral athetoid
Dyskinetic
Palsy
Dystonic
Ataxic
Classification (Topographical)
Spastic CP
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)
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
Excessive
extracellular excessive release
production of Oxidative Deprivation of matrix of glutamate
proinflammatory stress growth factors modification
cytokines
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
• 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
• 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
• 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
PSG:
• Multiple issues related to muscle tone, neuromuscular regulation of
the airway, posture and chest deformity.
• Most common indication: Chronic snoring and apnea
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
• Local injections
• Phenol 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
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.
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
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.
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