What are the definition, causes, symptoms, and treatment options for quadriplegia?

Quadriplegia: Definition, Causes, Symptoms, and Treatment

Quadriplegia is a form of spastic cerebral palsy affecting all four limbs and trunk (24% of spastic CP cases), or alternatively, paralysis of all four extremities resulting from cervical spinal cord injury—two distinct conditions requiring different diagnostic and management approaches. 1, 2

Definition and Classification

Cerebral Palsy-Related Quadriplegia

• Quadriplegia in cerebral palsy represents bilateral spastic involvement where all four limbs and trunk are affected, distinguished from diplegia (lower limbs more affected) and hemiplegia (unilateral involvement). 1, 3
• This is a permanent, non-progressive disorder attributed to disturbances in the developing fetal or infant brain, with spasticity being the predominant motor type (85-91% of cases). 1, 3
• Clinical features include velocity-dependent increased muscle tone, brisk deep tendon reflexes, and upper motor neuron dysfunction. 4

Traumatic Spinal Cord Injury Quadriplegia (Tetraplegia)

• Tetraplegia is profound paralysis of all four extremities due to cervical spinal cord injury, representing the most devastating type of spinal cord trauma. 5, 2
• The prototypical modern SCI is cervical incomplete injury without fracture/dislocation (central cord syndrome) in older adults following falls or low-energy trauma, expected to become the most common form. 1
• Central cord syndrome presents with disproportionately greater motor impairment in upper versus lower extremities, bladder dysfunction (urinary retention), and variable sensory loss below the lesion level. 1

Causes and Risk Factors

Cerebral Palsy Quadriplegia

• The complete causal pathway is unclear in approximately 80% of cases, though risk factors across conception, pregnancy, birth, and postneonatal periods are identifiable. 1
• 14% of cases have a genetic component, with advances in genetics likely to modify future diagnostic processes. 1
• Risk factors include prematurity, birth asphyxia, neonatal encephalopathy, intrauterine growth restriction, and pregnancy complications. 4

Traumatic Quadriplegia

• Motor vehicle accidents cause approximately 50% of spinal injuries, with falls (especially from height or diving), sports, and assaults accounting for most remaining cases. 1
• Most victims are males between ages 10-30 years, though the demographic is shifting toward older adults with central cord syndrome. 1
• Cervical spine injury risk is approximately 2% after blunt trauma requiring emergency imaging, tripling with craniofacial injury. 1
• Hyperextension mechanism in spondylotic or congenitally narrow canal causes compression between hypertrophic disc-osteophyte complex and buckled ligamentum flavum. 1

Clinical Presentation and Symptoms

Cerebral Palsy Quadriplegia

• Truncal hypotonia combined with limb spasticity is characteristic, where different body regions show varying tone abnormalities. 4
• Brisk lower limb reflexes indicate upper motor neuron dysfunction, the hallmark of spastic cerebral palsy. 4
• Inability to sit by 9 months or early hand asymmetry are critical red flags. 4

Associated Comorbidities (Cerebral Palsy)

• Chronic pain affects 75% of cases. 1, 3
• Epilepsy occurs in 35% of patients. 1, 3, 4
• Intellectual disability affects 49% of individuals. 1, 3
• Musculoskeletal problems (hip displacement) occur in 28%. 1, 3
• Additional complications include behavioral disorders (26%), sleep disorders (23%), functional blindness (11%), and hearing impairment (4%). 1

Traumatic Quadriplegia Presentation

• Cervical and high thoracic injuries produce respiratory failure and profound hypotension from loss of cardiovascular sympathetic innervation. 1
• Loss of sympathetic innervation to lymphatic organs induces immune paralysis, increasing infection susceptibility. 1
• Normal bowel and bladder function with otherwise complete motor/sensory paralysis, shifting sensory findings, and normal deep tendon reflexes early after injury suggest functional (non-organic) paralysis requiring psychiatric evaluation. 6

Diagnostic Approach

Cerebral Palsy Quadriplegia

• MRI of the brain is mandatory to identify white matter injury, cortical/deep gray matter lesions, or brain maldevelopments, achieving >95% diagnostic accuracy when combined with clinical examination. 4
• Perform Hammersmith Infant Neurological Examination (HINE), with scores <57 at 3 months having 96% predictive value for cerebral palsy. 4
• Document developmental milestones and pregnancy/birth complications systematically. 4
• Measure serum creatine phosphokinase (CK) to exclude Duchenne muscular dystrophy, which shows diminished (not brisk) reflexes. 4
• Any acute neurological deterioration requires urgent neuroimaging to exclude stroke, hemorrhage, or spinal cord compression, as cerebral palsy is non-progressive by definition. 4

Traumatic Quadriplegia

• MRI showing loss of cervical lordosis, canal narrowing, and spinal cord signal change confirms structural injury. 1
• Baseline MRI plays a critical role in clinical decision-making and outcome prediction per AOSpine guidelines. 1

Treatment and Management

Acute Management of Traumatic SCI

• Early surgical decompression within 24 hours is recommended based on AOSpine North America/International guidelines, though only 20-50% of patients reach specialized centers within this window. 1
• Surgery <96 hours shows benefit, with standardized mean difference favoring early intervention. 1
• Maintain spinal motion restriction by manually stabilizing the head to minimize secondary spinal cord injury risk (Class IIb recommendation). 1
• Do not use immobilization devices unless properly trained, as their benefit in first aid is unproven and potentially harmful (Class III). 1

Respiratory Management

• Upper cervical injuries reduce vital capacity by >50%, often requiring prolonged airway support. 1
• Early tracheostomy (<7 days) may reduce ICU hospitalization and laryngeal complications from prolonged intubation, with earlier timing possible after posterior surgical approaches. 1

Cerebral Palsy Management

• Immediate referral to cerebral palsy-specific early intervention before 6 months corrected age optimizes neuroplasticity and functional outcomes. 4
• Focus on postural control, movement training, and parent education as core intervention components. 4
• Serial monitoring every 4-6 months is essential to identify emerging contractures and guide therapeutic interventions. 7
• Standardized goniometry measuring hip, knee, and ankle joint angles should assess both lower extremities including iliotibial band, hamstrings, and gastrocnemius. 7

Functional Rehabilitation (Traumatic Quadriplegia)

• Surgical restoration of elbow/wrist extension or handgrip improves autonomy in at least 70% of tetraplegic patients, enabling eating, personal care, self-catheterization, and productive work. 2
• One-stage combined procedures with immediate activation of transferred muscles reduce adhesion risk, facilitate relearning, avoid immobilization effects, and enhance functional recovery compared to traditional multistage approaches. 2
• Transfer of axillary, musculocutaneous, and radial nerve fascicles from above the injury level are effective options to enhance motor outcome and sensory protection. 2

Bladder Management

• Intermittent urinary catheterization is the reference method, reducing long-term urinary tract infection and urolithiasis risk while increasing continence probability. 1
• Remove indwelling catheters as soon as medically stable to minimize urological risks. 1

Pain Management

• Introduce multimodal analgesia combining non-opioid analgesics, ketamine, and opioids during surgical management to prevent prolonged pain (GRADE 2+). 1
• Oral gabapentinoid treatment for >6 months controls neuropathic pain, combined with tricyclic antidepressants or serotonin reuptake inhibitors when monotherapy fails (GRADE 1+). 1

Pressure Ulcer Prevention

• Early mobilization once spine is stabilized, visual/tactile checks of at-risk areas daily, repositioning every 2-4 hours, discharge tools (cushions, foam, pillows) to avoid interosseous contact, and high-level prevention supports (air-loss/dynamic mattress) are essential measures (GRADE 2+). 1

Positioning and Stretching

• Stretching for at least 20 minutes per zone, completed by simple posture orthoses (elbow extension, metacarpophalangeal flexion-torsion, thumb-index commissure opening) and bed/chair positioning to prevent predictable deformities. 1

Prognosis

Cerebral Palsy Quadriplegia

• In high-income countries, 2 in 3 individuals with cerebral palsy will walk, 3 in 4 will talk, and 1 in 2 will have normal intelligence. 1, 3
• Motor function classification may change during the first 2 years, with approximately half of infants having GMFCS level reclassified. 3

Traumatic Quadriplegia

• Mean disease duration for central cord syndrome is variable, with recovery potential depending on injury completeness and timing of intervention. 1
• Full recovery is possible in incomplete injuries, as demonstrated by patients regaining full sensorimotor function after initial quadriplegia. 1

Critical Pitfalls to Avoid

• Do not assume a single muscle group is responsible for limited motion—combined contractures frequently coexist in cerebral palsy. 7
• Do not rely on single assessment timepoints—contractures evolve and require serial monitoring every 4-6 months. 7
• Do not delay transfer to specialized SCI centers—only 20-50% of patients arrive within the critical 24-hour therapeutic window. 1
• Do not touch electrocution victims while power is on—turn off power at source before approaching. 1