Cerebral Palsy

Definition

• Definition: A group of permanent disorders of the development of movement and posture, causing activity limitation, attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.
• The "CP Plus" Concept: The motor disorders are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems.

Classification and Types of Cerebral Palsy (CP)

I. Physiological Classification (Based on Motor Abnormality)

Classified by the type of movement disorder and the site of brain injury.

1. Spastic CP (Most Common: 70–80%)

• Site of Lesion: Pyramidal tract / Motor Cortex.
• Clinical Features:
  • Hypertonia: "Clasp-knife" spasticity.
  • Reflexes: Hyperreflexia, Clonus, Extensor Plantar Response (Babinski +).
  • Posturing: Scissoring of lower limbs (adductor spasm), fisting of hands.
  • Gait: Toe-walking (Equinus), Crouch gait, or Stiff-knee gait.

2. Dyskinetic CP (10–15%)

• Site of Lesion: Basal Ganglia (Extrapyramidal system).
• Etiology: Strongly associated with Kernicterus and Hypoxic-Ischemic Encephalopathy (HIE) in term infants.
• Subtypes:
  • Choreoathetoid: Rapid, jerky, irregular movements (chorea) + slow, writhing movements (athetosis). Worse with stress/voluntary movement.
  • Dystonic: Sustained muscle contractions causing twisting and repetitive movements or abnormal postures. Tone fluctuates ("Lead-pipe" or "Cogwheel" rigidity may be felt).
• Key Feature: Primitive reflexes persist longer; intelligence is often preserved despite severe physical disability.

3. Ataxic CP (< 5%)

• Site of Lesion: Cerebellum.
• Clinical Features:
  • Hypotonia: "Floppy infant" presentation initially.
  • Incoordination: Intention tremor, dysmetria (past-pointing).
  • Gait: Wide-based, unsteady, "drunken" gait.
  • Speech: Scanning speech (Dysarthria).

4. Mixed CP

• Combination of types. Most common is Spastic-Dyskinetic (seen in severe HIE).

II. Topographical Classification (Based on Limb Distribution)

Used primarily for Spastic CP.

III. Functional Classification - Gross Motor Function Classification System (GMFCS)

1. Introduction

• Definition: The GMFCS is a standardized, 5-level clinical classification system that describes the gross motor function of children and youth with Cerebral Palsy (CP) on the basis of their self-initiated movement with particular emphasis on sitting, walking, and wheeled mobility.
• Purpose:
  • To classify the severity of motor disability.
  • To predict future motor potential (prognosis).
  • To facilitate communication between professionals and families.
  • To guide management planning (e.g., surgery vs. wheelchair).
• Key Concept: Distinctions between levels are based on functional limitations, the need for assistive technology (walkers/crutches), and wheeled mobility, much less on the quality of movement.

2. General Description of Levels (The 5-Level System)

The classification is age-dependent (stratified for <2yrs, 2-4yrs, 4-6yrs, 6-12yrs, 12-18yrs). The core distinction remains consistent across ages:

3. Detailed Age-Specific Descriptors (6–12 Years)

This age group is most commonly assessed in exams.

• Level I: Children walk at home, school, outdoors, and in the community. They can climb stairs without the use of a railing. They perform gross motor skills such as running and jumping, but speed, balance, and coordination are limited.
• Level II: Children walk in most settings and climb stairs holding onto a railing. They may experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowded areas or confined spaces. They may walk with physical assistance, a hand-held mobility device, or use wheeled mobility over long distances.
• Level III: Children walk using a hand-held mobility device (walker/crutches) in most indoor settings. They may climb stairs holding onto a railing with supervision or assistance. They use wheeled mobility when traveling long distances and may self-propel for shorter distances.
• Level IV: Children use methods of mobility that require physical assistance or powered mobility in most settings. They may walk for short distances at home with physical assistance or use powered mobility or a body support walker at school. At school, outdoors, and in the community, they are transported in a manual wheelchair or use powered mobility.
• Level V: Children are transported in a manual wheelchair in all settings. Children are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements.

4. Utility and Prognostic Value

• Stability: GMFCS levels are generally stable after age 2. A child classified at Level IV at age 3 is unlikely to improve to Level II.
• Walking Prediction:
  • Level I-II: Will achieve independent walking.
  • Level III: Will walk with aids.
  • Level IV-V: Unlikely to achieve functional community ambulation.
• Hip Surveillance: The frequency of hip X-rays is determined by GMFCS level (e.g., Level V requires X-rays every 6 months due to 90% risk of displacement; Level I requires rare monitoring).

5. Other Classification Systems (The "F-words" Companions)

While GMFCS covers Motor function, comprehensive CP assessment uses analogous scales:

• MACS: Manual Ability Classification System (Hand function).
• CFCS: Communication Function Classification System.
• EDACS: Eating and Drinking Ability Classification System.

6. Summary Table for Quick Recall

Evaluation of a Child with Cerebral Palsy (CP)

Step I: History and Risk Factor Assessment

• Prenatal: TORCH infections, maternal toxins, brain malformations, intrauterine stroke.
• Natal: Perinatal Asphyxia (HIE), prolonged labor, instrumental delivery.
• Postnatal: Kernicterus, meningitis, head trauma, hypoglycemia.
• Developmental Red Flags:
  • Hand Preference < 1 year: Indicates hemiplegia on the contralateral side.
  • "Commando Crawl" (Bunny hopping): Dragging legs (Diplegia).
  • Delay in milestones: Head holding >4mo, Sitting >8mo.
  • Feeding difficulties: Choking, dribbling (Bulbar involvement).

Step II: Clinical Examination

A. General Physical Examination

• Microcephaly: Common in severe quadriparetic CP.
• Neurocutaneous markers: To rule out phakomatoses.
• Dysmorphism: Suggests genetic/syndromic etiology.

B. Neurological Examination (The 3 Pillars)

1. Tone and Posture:
   • Spastic CP: Hypertonia, clasp-knife spasticity, scissoring of lower limbs.
   • Dyskinetic CP: Fluctuating tone, dystonia, choreoathetosis.
   • Hypotonic/Ataxic: "Floppy infant" initially, evolving into ataxia.
   • Cortical Thumb: Thumb adducted into the palm (fisting) beyond neonatal period.
2. Primitive Reflexes (Persistence is Pathognomonic):
   • Persistence of Moro, ATNR (Asymmetric Tonic Neck Reflex), Palmar Grasp beyond 6 months prevents voluntary motor development.
   • Obligatory ATNR: Child cannot turn head without extending arm (prevents rolling over).
3. Postural Reactions (Delayed/Absent):
   • Parachute Reflex: Absent or asymmetric (in hemiplegia).
   • Landau Reflex: Abnormal.

C. Musculoskeletal Assessment (Secondary Complications)

• Hip Surveillance: Check for hip subluxation (Galeazzi sign, limited abduction).
• Contractures: Tendo-Achilles (equinus), Hamstrings (crouch gait).
• Spine: Scoliosis.

Step III: Classification (Diagnostic Formulation)

• Physiologic: Spastic (Cortical), Dyskinetic (Basal Ganglia), Ataxic (Cerebellar).
• Topographic:
  • Hemiplegia: Arm > Leg (Unilateral).
  • Diplegia: Leg > Arm (Periventricular Leukomalacia / Prematurity).
  • Quadriplegia: All 4 limbs (Severe HIE).

Step IV: Functional Classification (Mandatory for Prognosis)

• GMFCS (Gross Motor Function Classification System):
  • Level I: Walks without limitations.
  • Level II: Walks with limitations.
  • Level III: Walks using hand-held mobility device.
  • Level IV: Self-mobility with limitations (powered wheelchair).
  • Level V: Transported in a manual wheelchair (Head control poor).
• MACS: Manual Ability Classification System (Hand function).
• CFCS: Communication Function Classification System.

Step V: Investigations

1. Neuroimaging (The Gold Standard)

Neuroimaging is recommended for all children with cerebral palsy to establish an etiology and timing of the insult.

• MRI Brain (Modality of Choice):

  • Diagnostic Yield: Identifies a cause in 85–90% of cases.
  • Timing: Preferable after 2 years of age (when myelination is complete) for better visualization, but can be done earlier if indicated.
  • Classic Findings & Clinical Correlations:
    • Periventricular Leukomalacia (PVL): Reduction in white matter volume around ventricles (squaring of ventricles). Highly specific for Prematurity and Spastic Diplegia.
    • Multicystic Encephalomalacia: Extensive cystic degeneration of cortex/subcortex. Associated with severe Hypoxic-Ischemic Encephalopathy (HIE) and Spastic Quadriplegia.
    • Basal Ganglia/Thalamic Lesions: Hyperintensity in globus pallidus/putamen. Associated with Kernicterus or Hypoxic-Ischemic injury in term infants (Dyskinetic CP).
    • Focal Cortical Infarction/Porencephaly: Wedge-shaped defects in specific vascular territories (e.g., Middle Cerebral Artery). Associated with Spastic Hemiplegia.
    • Malformations: Lissencephaly, Schizencephaly (suggests prenatal genetic/developmental etiology).

• CT Scan:

  • Less sensitive than MRI.
  • Indication: Primarily used to detect Calcifications (e.g., TORCH infections like CMV/Toxoplasmosis) or intracranial hemorrhage if MRI is unavailable.

2. Metabolic and Genetic Testing

Performed if neuroimaging is normal (approx. 10–15% cases) or if there are "Red Flags" suggesting a mimic (progressive course, family history, loss of milestones).

• Metabolic Screen:
  • Serum: Ammonia, Lactate (Mitochondrial disorders), Amino acids (PKU, Homocystinuria).
  • Urine: Organic acids (Glutaric Aciduria Type 1 – a treatable mimic of Dyskinetic CP).
  • Thyroid Profile: Hypothyroidism causes hypotonia/delay.
• Genetic Testing:
  • Karyotype / Microarray: If dysmorphism or multiple congenital anomalies are present.
  • Specific Gene Panels: For hereditary spastic paraplegias (HSP) or ataxia syndromes.

3. Neurophysiological Studies

• Electroencephalogram (EEG):
  • Indication: Not routine. Done only if there is a history of seizures or paroxysmal events.
  • Findings: Focal spikes (hemiplegia), Hypsarrhythmia (West syndrome complication), or generalized discharges.
• Evoked Potentials:
  • BERA (BAER): Mandatory screening for Hearing Loss, especially in Dyskinetic CP (Kernicterus) and severe HIE.
  • VEP (Visual Evoked Potentials): To assess Cortical Visual Impairment (CVI).

4. Sensory Evaluation (Vision and Hearing)

• Ophthalmology Assessment:
  • Check for Strabismus (Squint), Refractive errors, and Cortical Visual Impairment (common in HIE).
  • Fundoscopy for Chorioretinitis (TORCH) or Optic Atrophy.
• Audiometry:
  • Sensory-neural hearing loss is common in post-meningitic and dyskinetic CP.

5. Coagulation Profile

• Thrombophilia Screen: (Protein C, S, Factor V Leiden).
• Indication: Specifically for children with Spastic Hemiplegia (perinatal stroke) to rule out hypercoagulable states.

6. Functional Assessment (for Management Planning)

• Gait Analysis: 3D Gait analysis in ambulant children to plan orthopedic surgery.
• Hip Surveillance (X-ray Pelvis): Annual/Bi-annual X-rays to monitor "Reimer’s Migration Index" (Risk of hip dislocation).

Management of Cerebral Palsy (CP)

1. Goals and Principles

• Goal: To maximize functional independence, prevent secondary deformities, and improve Quality of Life (QoL), rather than "curing" the neurological deficit.
• Approach: Multidisciplinary Team (MDT) involving Pediatrician, Neurologist, Orthopedician, Physiotherapist, Occupational Therapist, and Speech Therapist.
• The "F-Words" in Childhood Disability: Focus on Function, Family, Fitness, Fun, Friends, and Future.

2. Rehabilitative Management (The Foundation)

• Physiotherapy (PT):
  • Prevention of contractures (passive stretching).
  • Strengthening of antigravity muscles.
  • Gait training and postural control.
  • CIMT (Constraint-Induced Movement Therapy): For hemiplegic CP (restraining the good hand to force use of the affected hand).
• Occupational Therapy (OT):
  • Training for Activities of Daily Living (ADL): Feeding, dressing, hygiene.
  • Fine motor coordination.
• Orthotics and Assistive Devices:
  • AFO (Ankle Foot Orthosis): To correct dynamic equinus (toe-walking) and improve stability.
  • Mobility Aids: Walkers, Crutches, Wheelchairs (manual/powered) based on GMFCS level.

3. Pharmacological Management of Tone

Target is to reduce spasticity/dystonia to facilitate hygiene or function.

A. Spasticity Management

B. Dystonia Management

• First Line: Trihexyphenidyl (Anticholinergic). High doses often required.
• Levodopa Challenge: Mandatory in all dystonic CP to rule out Dopa-Responsive Dystonia.
• Others: Baclofen, Gabapentin.

4. Surgical Management

• Orthopedic Surgery:
  • SEMLS (Single Event Multi-Level Surgery): Correction of all deformities (soft tissue releases + osteotomies) in one session to reduce rehab time. Ideally done at age 7–10 years.
  • Tendon Lengthening: Tendo-Achilles lengthening (TAL) for fixed equinus.
  • Hip Surveillance: Routine X-rays to detect hip subluxation. Surgical release of adductors or femoral osteotomy if Migration Percentage >30–40%.
• Neurosurgery:
  • Selective Dorsal Rhizotomy (SDR): Permanent reduction of spasticity by cutting sensory nerve rootlets in the spinal cord.
  • Ideal Candidate: Pure spastic diplegia, good strength, cognitive ability to participate in rehab, no dystonia.

5. Management of Comorbidities ("CP Plus")

• Epilepsy (40%): Treat based on seizure type. Avoid Phenobarbital/Benzodiazepines if sedation worsens motor function.
• Gastrointestinal:
  • Feeding: Gastrostomy (PEG) for severe dysphagia/aspiration risk (GMFCS V).
  • GERD: PPIs, Fundoplication.
  • Constipation: Laxatives, high fiber.
• Sialorrhea (Drooling):
  • Socially stigmatizing.
  • Tx: Glycopyrrolate, Scopolamine patches, Botox to salivary glands.
• Communication: Augmentative and Alternative Communication (AAC) devices for non-verbal children.
• Bone Health: Calcium/Vit D supplementation; Bisphosphonates for fragility fractures (osteopenia common due to immobility and antiepileptics).

6. Summary of Interventions by GMFCS Level

• Level I-III (Ambulant): Focus on gait improvement, Botox, Orthopedic surgery (SEMLS).
• Level IV-V (Non-Ambulant): Focus on comfort, hygiene, preventing hip dislocation, spinal alignment, and technology for communication.

Early Diagnosis and Intervention in Cerebral Palsy

1. Rationale (The "Why")

• Neuroplasticity: The infant brain has the highest potential for reorganization and recovery in the first 2 years of life ("Critical Period").
• "Wait and Watch" is obsolete: Delaying diagnosis until milestones are missed loses the window of opportunity for neuroprotection and effective rehabilitation.

2. Early Diagnosis (The "How")

Current international guidelines (Novak et al., 2017) state CP can be accurately predicted before 5 months corrected age.
A. Diagnostic Tools (< 5 Months Corrected Age)
The "Three Pillars" for high-risk infants (e.g., Preterm, HIE):

1. MRI Brain (Gold Standard): Identifies structural correlates (e.g., Periventricular Leukomalacia in preterms, Basal Ganglia lesions in term asphyxia).
2. Prechtl’s General Movements Assessment (GMA):
   • Method: Observation of spontaneous infant movements.
   • Abnormality: Absence of "Fidgety Movements" (small, elegant, dancing movements) at 3–4 months is >95% predictive of CP.
   • Cramped Synchronized Movements: Predicts severe spastic CP.
3. Hammersmith Infant Neurological Examination (HINE): A quantifiable clinical score (Scores < 57 at 3 months predict CP).
   B. Clinical "Red Flags" (for Low-Risk / Term Infants)

• Hand Preference: Any hand preference before 1 year indicates hemiparesis.
• Tone: Persistent fisting (Cortical thumb), scissoring, or head lag beyond 4 months.
• Reflexes: Obligatory ATNR, persistence of Moro/Palmar grasp.

3. Early Intervention (The "What")

Once "High Risk of CP" is identified, intervention starts immediately (even before a definitive label is given).
A. Principles of Early Intervention (EI)

• Family-Centered Care: Parents are the primary therapists; training them is key.
• Task-Specific Training: Active motor learning (e.g., encouraging reaching) is superior to passive stretching.
• Enriched Environment: Sensory-motor stimulation to promote dendritic branching.
  B. Specific Interventions

1. Motor:
   • Physiotherapy: Facilitation of head control, rolling, and sitting.
   • CIMT (Baby-CIMT): Constraint-Induced Movement Therapy for infants with asymmetric hand use (hemiplegia).
2. Feeding and Nutrition: Management of dysphagia (oro-motor therapy) and ensuring adequate caloric intake (gastrostomy if needed).
3. Sensory/Communication: Screening for Vision (CVI) and Hearing impairment early to provide aids.
4. Surveillance: Monitoring hip migration (X-rays) to prevent dislocation.

4. Goals of Early Intervention

• Prevent secondary complications (contractures, deformities).
• Maximize functional independence (improve GMFCS trajectory).
• Support parent-infant bonding and mental health.