Cerebral Palsy

Cerebral palsy is a group of permanent, non-progressive disorders of movement and posture. They result from a disturbance in the developing fetal or infant brain. It is the commonest cause of significant physical disability in childhood.

The underlying brain injury is non-progressive, but its clinical manifestations characteristically evolve as the child develops. The condition therefore needs ongoing, structured reassessment throughout childhood rather than a single, static diagnostic and management approach.

Full, coordinated multidisciplinary management of the wide range of associated motor and non-motor manifestations is the essential foundation of care.

Classification and pathophysiology

• Cerebral palsy is classified by the predominant motor pattern. Mixed patterns combine features of more than one category.
  • Spastic is the commonest type. It reflects upper motor neuron injury and produces increased muscle tone.
  • Dyskinetic reflects basal ganglia injury and produces involuntary, fluctuating movements.
  • Ataxic reflects cerebellar injury and produces incoordination and unsteady gait.
• Spastic cerebral palsy is further classified by the pattern and distribution of limb involvement.
  • Hemiplegia affects one side of the body.
  • Diplegia affects predominantly the lower limbs. It is classically associated with periventricular white matter injury of prematurity.
  • Quadriplegia affects all four limbs. It generally reflects more extensive brain injury and carries the strongest association with significant intellectual disability among the spastic subtypes.
• The causative brain injury may occur at three stages. Antenatal causes account for the majority of cases and include congenital infection, structural brain malformation and vascular events. Perinatal causes include hypoxic ischaemic encephalopathy and significant prematurity-related brain injury. Postnatal causes in early childhood include meningitis, significant head trauma and a hypoxic event.
• Periventricular leukomalacia is a pattern of white matter injury particularly associated with significant prematurity. It is an important, common pathological substrate. The periventricular white matter is particularly vulnerable to ischaemic injury during this developmental period.
• Periventricular leukomalacia characteristically produces the spastic diplegic pattern. The corticospinal tract fibres serving the lower limbs lie within this vulnerable region.

• The characteristic presenting features are delayed motor milestones, abnormal muscle tone (increased in spastic subtypes, fluctuating in dyskinetic subtypes), abnormal movement patterns, and persistence of primitive reflexes beyond the age at which they normally disappear. These typically become apparent over the first one to two years of life.
• Presentation and severity vary substantially. They range from mild, subtle motor impairment to severe, profound physical disability requiring full support for all activities of daily living.
• A substantial proportion of affected children have significant coexisting non-motor manifestations: intellectual disability, epilepsy, visual and hearing impairment, communication difficulty, and feeding and swallowing dysfunction. Assess these fully and proactively rather than assuming them from the motor presentation alone.
• Musculoskeletal complications develop over childhood as abnormal, sustained muscle tone acts on a growing skeleton. They include progressive contracture, hip subluxation or dislocation, and scoliosis.
• These warrant structured, regular orthopaedic surveillance throughout childhood. This includes hip surveillance radiography at defined intervals according to the child's Gross Motor Function Classification System level.
• Growth and nutritional difficulty is a further common, clinically significant manifestation. It reflects feeding and swallowing dysfunction, increased energy expenditure from abnormal movement patterns and, in some cases, reduced oral intake.

• Diagnosis is established clinically from the characteristic pattern of motor delay and abnormal tone, with a compatible relevant history.
• Structured, validated assessment tools support classification of motor subtype and functional severity. The most notable is the Gross Motor Function Classification System. It grades overall gross motor function from level one (walking without limitation) to level five (requiring full physical support, with limited head and trunk control).
• Magnetic resonance imaging of the brain is recommended in the great majority of confirmed cases. It characterises the underlying pathological substrate (periventricular leukomalacia, a structural malformation, or a focal vascular or hypoxic-ischaemic injury).
• Imaging also supports identification of any specific, potentially treatable or genetically relevant alternative diagnosis where the clinical or imaging picture is atypical.
• Full, proactive screening for common associated impairments is an essential, standard component of diagnostic evaluation once the core diagnosis is established. It includes formal ophthalmological and audiological assessment, cognitive and developmental assessment, and evaluation of swallowing function.
• Careful exclusion of a progressive, rather than static, neurological process is essential where the course suggests unexpected deterioration. The expected pattern in true cerebral palsy is evolving but fundamentally static. Unexpected deterioration directs consideration to an alternative progressive neurodegenerative or metabolic diagnosis.

• Full, coordinated multidisciplinary management is the essential, foundational component of care. It incorporates physiotherapy, occupational therapy, and speech and language therapy from an early age, addressing motor function, activities of daily living, and communication and swallowing needs respectively.
• Management of spasticity uses oral baclofen or diazepam.
  • Baclofen is typically commenced at a low paediatric dose, such as 0.75 to 2 mg per kg per day in divided doses. It is titrated gradually according to response and tolerability, with a maximum generally around 40 to 60 mg per day depending on age.
• For focal, troublesome spasticity affecting a specific muscle group, add botulinum toxin type A injection. It is a widely used, effective and generally well tolerated treatment for focal spasticity.
• Botulinum toxin is typically dosed according to total body weight, commonly up to 15 to 20 units per kg per treatment session, subject to defined maximum total doses. It is repeated at intervals of approximately three to six months as the effect wears off.
• Intrathecal baclofen via an implanted pump is an important option for children with severe, generalised spasticity not adequately controlled with oral medication or focal injection. It delivers drug directly to the cerebrospinal fluid at substantially lower total dose than oral treatment, reducing systemic adverse effects. It requires careful, specialist patient selection and ongoing device management.
• Selective dorsal rhizotomy is a neurosurgical procedure that selectively divides specific sensory nerve rootlets. It is a further option for carefully selected children, predominantly those with spastic diplegia and good underlying motor control potential. It aims to achieve a durable reduction in lower limb spasticity.
• Orthopaedic management is an essential, ongoing component throughout childhood and adolescence. It includes orthotic provision (ankle-foot orthoses and similar devices supporting functional positioning and gait). It also includes surgery for progressive contracture, hip subluxation or significant scoliosis: soft tissue releases, tendon transfers and, where indicated, bony procedures.
• Antiepileptic medication, where epilepsy coexists, follows the standard principles of seizure management. Individualise it according to seizure type and, where relevant, the child's broader cognitive and swallowing status.
• Full nutritional support addresses the substantial, common nutritional and growth challenges of this population. It includes specialist dietetic input and, where oral intake is significantly inadequate or unsafe given swallowing dysfunction, consideration of gastrostomy feeding.
• Structured long-term follow-up extends through childhood, adolescence and into adulthood. It is accompanied by full family support addressing the substantial, lifelong practical and emotional impact of the condition, and structured transition planning to adult services. Together these underpin holistic, individualised lifelong management.