Spastic Quadriplegia

Spastic quadriplegia is a bilateral spastic cerebral palsy with severe involvement of all four limbs, trunk and often the bulbar muscles. Most children are at gross motor function levels IV and V, and are unable to walk.

It makes up about 15 to 20% of cerebral palsy. Common causes are severe hypoxic ischaemic encephalopathy at term, extensive periventricular or cystic white matter injury, brain malformations, neonatal meningitis, kernicterus, infection and metabolic disorders.

Most affected children have additional disabilities: intellectual disability, epilepsy (about half), visual impairment, dysphagia, and communication difficulty. Life expectancy is reduced in those with the most severe disability.

Pathophysiology.

Widespread cortical, subcortical or white matter injury disrupts corticospinal and corticobulbar tracts bilaterally. This causes spasticity, weakness, abnormal posture and primitive reflexes, with dysphagia and poor airway protection.

Muscle shortening, bone deformity and poor weight bearing cause hip dislocation, scoliosis and osteopenia.

• Hypotonia in infancy evolving to marked spasticity, with persistent primitive reflexes and no head control or sitting.
• Contractures, scissoring, windswept hips, hip subluxation or dislocation, and scoliosis.
• Feeding difficulty, drooling, aspiration, gastro oesophageal reflux and malnutrition, constipation, and recurrent chest infection.
• Epilepsy, visual impairment (cortical), hearing impairment, sleep disorder, pain and irritability.

Red flags.

• Recurrent chest infections, weight loss or dysphagia.
• Unexplained distress or pain (hip dislocation, reflux, constipation, fractures, dental disease).
• Rapid worsening of tone, new neurological signs or regression.

• Clinical diagnosis, supported by MRI brain to define the pattern of injury and to exclude progressive causes.
• Hip surveillance by pelvic radiography from age 1 to 2 years, spine radiography, and swallow assessment (videofluoroscopy).
• Nutrition assessment, bone density in those with fractures, vision and hearing assessment, and EEG for seizures.
• Metabolic and genetic testing when MRI is atypical or the course is progressive.

Differential diagnosis. Neurodegenerative and metabolic disorders, genetic encephalopathies, leukodystrophy, spinal muscular atrophy (hypotonia), and congenital myopathies.

• Multidisciplinary care: neurology, orthopaedics, gastroenterology, respiratory, nutrition, physiotherapy, occupational therapy, speech therapy, palliative care and social support.
• Spasticity and dystonia: positioning, seating and orthoses, botulinum toxin, oral baclofen 0.3 mg/kg/day in 3 divided doses (maximum 2 mg/kg/day), and intrathecal baclofen for severe disease.
• Nutrition and swallow: thickened feeds, gastrostomy (with antireflux procedure if severe reflux or aspiration), and energy supplementation.
• Drooling: glycopyrronium oral 20 micrograms/kg 3 times daily, increased to a maximum of 100 micrograms/kg for each dose (maximum 3 mg), or botulinum toxin to salivary glands.
• Orthopaedics: hip surveillance, soft tissue release and reconstruction for hip displacement, and spinal fusion for progressive scoliosis.
• Respiratory: airway clearance, vaccination, and treatment of aspiration. Treat constipation (macrogol), epilepsy and pain, and screen for bone fragility with vitamin D and calcium, and bisphosphonate under specialist direction for low trauma fractures.
• Communication aids, adapted seating, equipment and educational support. Palliative care input and advance care planning.

Monitoring. Growth, nutrition, hip migration, scoliosis, seizure control, respiratory health and carer wellbeing.

Referral and escalation. Refer to a paediatric neurodisability and orthopaedic service. Seek urgent review for aspiration pneumonia, unexplained distress or hip dislocation.