Overview
Definition:
Cerebral palsy (CP) is a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances in the developing fetal or infant brain
The motor disorder of CP is often accompanied by other problems that affect sensation, perception, cognition, communication, and behavior, and by the occasional seizure disorder.
Epidemiology:
The prevalence of CP varies globally, estimated between 1.5 to 4 per 1,000 live births
Risk factors include prematurity, low birth weight, multiple births, maternal infections, and complications during pregnancy or delivery
Postnatal causes include meningitis, encephalitis, head trauma, and hypoxic-ischemic events.
Clinical Significance:
CP is the most common motor disability in childhood, profoundly impacting a child's development, functional independence, and quality of life
Accurate classification and timely, multidisciplinary management are crucial for optimizing outcomes and supporting affected individuals and their families.
Clinical Presentation
Symptoms:
Delayed motor milestones, such as sitting, crawling, or walking
Muscle stiffness or floppiness
Poor coordination or balance
Abnormal reflexes or gait patterns
Speech and swallowing difficulties
Vision and hearing impairments
Seizures.
Signs:
Spasticity (increased muscle tone), hyperreflexia, clonus, scissoring gait (in spastic diplegia/hemiplegia)
Dystonia (involuntary muscle contractions), fluctuating tone
Ataxia (unsteady gait, tremor) in ataxic CP
Mixed patterns of tone abnormality.
Diagnostic Criteria:
Diagnosis is primarily clinical, based on observing persistent motor abnormalities in a child younger than 2-3 years old, with a history of factors known to increase CP risk
Neuroimaging (MRI brain) is often used to identify underlying brain lesions, supporting the diagnosis and identifying etiology.
Diagnostic Approach
History Taking:
Detailed birth history (gestational age, birth weight, delivery complications, Apgar scores)
Neonatal course (hypoxia, jaundice, infections, seizures)
Developmental milestones achieved and delays
Family history of neurological or developmental disorders
Presence of associated conditions like epilepsy, visual or hearing impairment.
Physical Examination:
Comprehensive neurological examination: assessment of muscle tone (passive range of motion, resistance to passive movement), reflexes (deep tendon reflexes, superficial reflexes), primitive reflexes (persistence or absence), voluntary movements, coordination, gait, and posture
Screening for associated sensory, visual, auditory, and cognitive impairments.
Investigations:
Magnetic Resonance Imaging (MRI) of the brain: most sensitive imaging modality for detecting structural brain abnormalities
typically performed between 6 months and 2 years of age
Electroencephalogram (EEG): if seizures are suspected or present
Genetic testing: may be considered if a specific genetic syndrome is suspected
Metabolic screening: for inherited metabolic disorders that can mimic CP.
Differential Diagnosis:
Genetic neurometabolic disorders (e.g., PKU, Tay-Sachs)
Spinal muscular atrophy
Muscular dystrophies
Congenital myopathies
Friedreich's ataxia
Hereditary spastic paraplegia
Transient ischemic attacks
Benign congenital hypotonia.
Gmfcs Classification
Introduction:
The Gross Motor Function Classification System (GMFCS) is a standardized, age-based observational tool used to describe the gross motor abilities of children with CP
It classifies children into five levels based on their functional mobility and need for assistive devices.
Levels:
GMFCS Level I: Walks without assistance
GMFCS Level II: Walks with assistive devices (walkers, crutches)
GMFCS Level III: Walks with a hand-held mobility device (walker, cane)
GMFCS Level IV: Requires physical assistance or uses power-assisted mobility devices
GMFCS Level V: Severe limitations in head control and trunk support
requires extensive manual assistance.
Age Bands:
GMFCS is applied to three age bands: 0-2 years, 2-4 years, and 4-6 years, with subsequent bands for older children, to account for developmental changes
The classification provides a framework for prognosis, goal setting, and intervention planning.
Clinical Relevance:
GMFCS is a critical tool for understanding functional limitations, predicting future mobility, guiding therapeutic interventions, and evaluating treatment outcomes
It helps standardize communication among healthcare professionals and researchers.
Spasticity Management
Goals Of Management:
Reduce pain and discomfort
Improve range of motion and prevent contractures
Enhance functional abilities (sitting, walking, self-care)
Facilitate participation in daily activities
Improve hygiene and cosmetic appearance.
Pharmacological Management:
Oral medications: Baclofen (10-60 mg/day divided doses
start low, titrate slowly)
Diazepam (0.1-0.3 mg/kg/day divided doses
used short-term due to sedation and dependence)
Tizanidine (0.5-2 mg/kg/day divided doses
less sedating than diazepam, monitor liver function)
Dantrolene sodium (0.5-2 mg/kg/dose
for severe spasticity, monitor liver function).
Botulinum Toxin Injections:
Botulinum toxin type A (Botox, Dysport): injected into spastic muscles (e.g., adductors, hamstrings, calf muscles)
Provides temporary relief of spasticity (3-6 months)
Used in conjunction with physical and occupational therapy to improve function and prevent contractures.
Intrathecal Baclofen Pump:
For severe, generalized spasticity unresponsive to oral medications or botulinum toxin
A surgically implanted pump delivers baclofen directly into the cerebrospinal fluid
Requires careful patient selection, programming, and maintenance.
Orthopedic Surgical Interventions:
Selective dorsal rhizotomy (SDR): selectively cuts sensory nerve roots in the spinal cord to reduce spasticity, primarily in the lower limbs
Tendon transfers and lengthening: correct fixed deformities and improve muscle balance
Osteotomies: correct bone deformities (e.g., hip dislocations, foot deformities).
Therapies And Supportive Measures:
Physical therapy: stretching, strengthening, gait training, balance exercises
Occupational therapy: fine motor skills, activities of daily living
Orthotics and bracing: to support posture, improve alignment, and prevent contractures
Assistive technology: wheelchairs, walkers, adaptive equipment
Multidisciplinary team approach involving physiatrists, neurologists, orthopedic surgeons, therapists, and social workers.
Complications
Early Complications:
Contractures and joint deformities
Pain
Sleep disturbances
Feeding difficulties and aspiration
Respiratory infections.
Late Complications:
Hip dislocation (especially in GMFCS IV-V)
Scoliosis
Chronic pain
Pressure sores
Osteoporosis
Gastroesophageal reflux disease
Cognitive and behavioral issues.
Prevention Strategies:
Regular stretching and range of motion exercises
Appropriate use of orthotics
Timely orthopedic surgical interventions
Good nutritional support
Seizure control
Comprehensive developmental and sensory assessments.
Prognosis
Factors Affecting Prognosis:
Severity of motor impairment (GMFCS level)
Presence and severity of associated conditions (epilepsy, cognitive impairment)
Early diagnosis and intervention
Quality of rehabilitation services
Family support and resources.
Outcomes:
Prognosis is highly variable and depends on the factors above
Many individuals can achieve functional independence with appropriate support and interventions
Lifelong management is often required.
Follow Up:
Lifelong follow-up is essential, with regular assessments by a multidisciplinary team to monitor motor development, manage spasticity, address associated conditions, and adapt interventions as the child grows and their needs change
Transition planning for adult care is also crucial.
Key Points
Exam Focus:
GMFCS levels I-V and their implications for mobility
Primary management strategies for spasticity: oral medications, botulinum toxin, intrathecal baclofen pump, SDR
Recognizing and managing common complications like contractures and hip dislocations
The multidisciplinary team approach is central to CP care.
Clinical Pearls:
Always assess tone and reflexes systematically
Early identification of motor delays is key
Involve parents in goal setting and therapy planning
Consider the impact of CP on other systems (vision, hearing, cognition)
Spasticity management is about function and quality of life, not just muscle tone reduction.
Common Mistakes:
Underestimating the impact of associated conditions on overall function
Delaying referral to specialists for spasticity or orthopedic concerns
Inadequate follow-up leading to preventable complications
Focusing solely on motor deficits without addressing psychosocial aspects
Prescribing oral antispastics without considering side effects or alternative treatments.