Central Cord Syndrome (CCS)

Central cord syndrome (CCS) is a neurological syndrome caused by an injury to the center of the spinal cord, affecting the spinothalamic tracts ((STTs) sensory) and medial aspect of the corticospinal tracts ((CSTs) motor), most often due to trauma in patients with cervical spondylosis. A less frequent but classic cause of CCS is syringomyelia. Clinical manifestations are motor deficits in the arms more so than the legs and variable sensory deficits below the level of injury. Diagnosis is made clinically and is supported with neuroimaging. Definitive management can be medical or surgical, depending on the severity of the injury. Rehabilitation is the key to maintaining functionality and improving chances of recovery.

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Overview

Definition

Central cord syndrome (CCS) is a neurological syndrome caused by an injury to the center of the spinal cord, affecting the spinothalamic tracts ((STTs) sensory) and medial aspect of the corticospinal tracts ((CSTs) motor).

Epidemiology 

  • Most common form of incomplete spinal cord injury
  • Prevalence: approximately 9% of those with traumatic spinal cord injury
  • Men are more commonly affected.
  • Young patients: usually due to trauma (e.g., automobile accidents) 
  • Older patients (> 65 years): usually caused by neck hyperextension combined with underlying spinal disease (e.g., osteoarthritis)

Etiology

  • Blunt trauma (most common)
    • Motor vehicle accidents
    • Falls from a significant height
    • Penetrating trauma due to gunshot or knife injuries
  • Syringomyelia (cystic lesion between C2 and T9)
    • Congenital
      • Chiari malformation type I
      • Klippel-Feil syndrome
    • Postinfectious: transverse myelitis
    • Inflammatory: multiple sclerosis (MS)
  • Medullary tumor → progressive cervical myelopathy with central cord features
    • Ependymoma
    • Astrocytoma
    • Rarely metastatic disease
  • Spondylosis
    • Atlantoaxial (C1–C2) instability (e.g., due to rheumatoid arthritis (RA))
    • Spinal arthropathies
  • Osteoporosis (compression fractures)

Pathophysiology

Injury to the vertebral column causes a spinal cord injury related to the force and direction of the traumatic event and the anatomic vulnerability of individual spinal elements.

Causes of spinal cord injury involve 1 or more of the following:

  • Vertebral fracture
  • Dislocation at 1 or more joints
  • Ligamentous tears
  • Herniation of the intervertebral disc

A cervical spine hyperextension injury can be related to: 

  • Fall with neck hyperextended (especially from a significant height, such as a roof)
  • High-velocity trauma (e.g., motor vehicle accident)
  • Penetrating injuries (e.g., knife or gunshot injuries)
  • Cervical spondylosis 

Mechanism of injury:

  • Spine subluxation and/or fracture →
  • Contusion/edema at the site of injury →
  • Simultaneous compression of the spinal cord anteriorly (by osteophytes or disc) and posteriorly (by the ligamentum flavum) 

Affected areas of the spinal cord by central cord syndrome:

  • Medial aspect of the CSTs/ anterior horn gray matter → weakness in the arms > legs 
  • Axonal disruption in the white matter as the fibers pass from the dorsal to the ventral horn → loss of deep tendon reflexes
  • Compression of the STT → sensory deficits
  • Both somatic and visceral motor nuclei are affected.
Location of lesion in central cord syndrome

Location of lesion in central cord syndrome (CSS):
Rough depiction of the area affected by CSS (affects lateral spinothalamic tracts (STTs) and lateral corticospinal tracts (CSTs) to varying degrees)

Image by Lecturio.

Clinical Presentation and Diagnosis

Understanding the structures affected by a central cord lesion is key to their correlation with clinical signs and symptoms. Diagnosis of CCS is by clinical exam and diagnostic imaging. 

Clinical presentation

History:

  • Site of injury: impact with the neck hyperextended
  • Types of injuries that cause cervical fractures:
    • Blunt trauma from a motor vehicle accident
    • Penetrating trauma from gunshot or knife
    • Falling trauma: downstairs or from a significant height (e.g., off a roof or a ladder)
  • No trauma: 
    • Syringomyelia or tumor
    • Inflammatory/demyelinating (MS)
    • Postinfectious inflammation (transverse myelitis)

Physical exam:

  • Motor deficit: weakness in upper limbs > lower limbs (CST)
  • Sensory deficits: Pain and temperature sensation are absent in the distribution of 1 or several adjacent dermatomes but intact further above and below the lesion (spinothalamic fibers disrupted).
  • Preserved vibration and position sensation (intact posterior columns)
  • No usual bladder symptoms but urinary retention may occur
  • Spasticity 
  • Loss of deep tendon reflexes at the level of the spinal cord lesion

Diagnosis

  • Physical exam as above
  • Imaging
    • X-rays of the cervical spine may show:
      • Vertebral fractures
      • Spinal subluxation 
    • CT: shows impingement of the spinal canal
    • MRI: gold standard for evaluating the spinal cord and surrounding soft tissues

Management and Prognosis

Central cord syndrome has a good prognosis, although factors such as older age and more-severe neurologic injury at presentation are associated with a lower likelihood of neurologic recovery. 

Management

Medical management:

  • Conservative treatment is the most common. 
  • With acute severe trauma: 
    • Treat hypotension due to neurogenic shock.
    • High-dose steroids (methylprednisolone) to suppress edema
  • Baclofen (muscle relaxant) for spasticity

Rehabilitation:

  • PT: for improving strength and range of motion (ROM) of lower extremities
  • Occupational therapy: upper limb training
    • Improves ability to perform daily activities
    • May improve neuropathic pain 
  • External fixation of the spine: 4–6 weeks 
  • Up to 75% of patients show some neurological improvement in functionality.

Surgical management:

  • Considered early on for:
    • Spinal instability 
    • Ongoing spinal cord compression with progressive neurologic deterioration
  • Involves procedures such as decompression laminectomy 

Complications of CCS

  • Autonomic dysreflexia: lack of a coordinated autonomic response with HR and blood pressure in spinal injuries above T6 
  • Neurogenic bladder

Prognosis

  • Variable:
    • Functionality depends on the extent of the injury and rehabilitation.
    • Most patients recover the ability to walk.
  • Cervical fractures prolong recovery time.
  • Bladder function usually returns 6–8 months after injury.

Differential Diagnosis

  • Ventral (anterior) cord syndrome (ACS): an injury to the anterior, or ventral, ⅔ of the spinal cord (or incomplete cord syndrome) that spares the dorsal columns. The syndrome is caused by occlusion of the anterior spinal artery or trauma causing disc herniation and bone fragments that disrupt the spinal cord. Clinical manifestations are loss of motor and sensory function below the level of injury. Diagnosis of ACS is by clinical exam and neuroimaging with MRI. Management is directed at resolving the underlying cause.
  • Posterior cord syndrome (PCS): an incomplete cord syndrome that affects the posterior aspect of the spinal cord and is characterized by loss of vibration and position senses below the level of injury. As a very rare condition, the status of PCS as a separate clinical entity is still under debate in the literature and can overlap with CCS. Diagnosis is made clinically and supported by neuroimaging. Management can be medical/rehabilitative or surgical if indicated.
  • Cruciate paralysis: a rare neurological condition that affects the cervicomedullary junction. The condition presents with bilateral upper limb paresis without the involvement of the lower limbs. The etiologies of cruciate paralysis include traumatic injuries, postsurgical complications, and metabolic disorders. Diagnosis and management are similar to other incomplete cord syndromes.

References

  1. Ameer, MA, Tessler, J, & Gillis, CC. (2021). Central cord syndrome. In StatPearls. StatPearls Publishing. Retrieved September 1, 2021, from http://www.ncbi.nlm.nih.gov/books/NBK441932/ 
  2. Go, S. (2020). Spine trauma. In J. E. Tintinalli, et al. (Ed.), Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, Chapter 258 (9th ed.). http://accessmedicine.mhmedical.com/content.aspx?aid=1167028097 
  3. Kim, E, et al. (2021). Disorders, diseases, and injuries of the spine. McMahon, PJ, & Skinner, HB (Eds.). Current Diagnosis & Treatment in Orthopedics (6th ed.). http://accessmedicine.mhmedical.com/content.aspx?aid=1181616865
  4. Ropper, AH, Samuels, MA, Klein, JP, & Prasad, S. (2019). Diseases of the spinal cord. Ropper, AH, et al. (Eds.). Adams and Victor’s Principles of Neurology (11th ed.). McGraw-Hill Education. Retrieved September 1, 2021, from http://accessmedicine.mhmedical.com/content.aspx?aid=1162599484 
  5. Hansebout, RR, & Kachur, E. (2018). Acute traumatic spinal cord injury. UpToDate. Retrieved September 1, 2021, from https://www.uptodate.com/contents/acute-traumatic-spinal-cord-injury
  6. Eisen, A. (2020). Anatomy and localization of spinal cord disorders. UpToDate. Retrieved September 1, 2021, from https://www.uptodate.com/contents/anatomy-and-localization-of-spinal-cord-disorders
  7. Brooks, NP. (2017). Central cord syndrome. Neurosurg Clin N Am. (28)41–47. http://dx.doi.org/10.1016/j.nec.2016.08.002

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