Central Cord Syndrome (CCS)

Central cord syndrome (CCS) is a neurological syndrome caused by an injury to the center of the spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Variable Variables represent information about something that can change. The design of the measurement scales, or of the methods for obtaining information, will determine the data gathered and the characteristics of that data. As a result, a variable can be qualitative or quantitative, and may be further classified into subgroups. Types of Variables 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 Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). Spinal Cord injury
  • Prevalence: approximately 9% of those with traumatic spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Osteoarthritis Osteoarthritis (OA) is the most common form of arthritis, and is due to cartilage destruction and changes of the subchondral bone. The risk of developing this disorder increases with age, obesity, and repetitive joint use or trauma. Patients develop gradual joint pain, stiffness lasting < 30 minutes, and decreased range of motion. 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 Multiple Sclerosis Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis (MS)
  • Medullary tumor → progressive cervical myelopathy with central cord features
    • Ependymoma Ependymoma Ependymomas are glial cell tumors arising from CSF-producing ependymal cells lining the ventricular system. Ependymomas most commonly occur within the posterior fossa in contact with the 4th ventricle, or within the intramedullary spinal cord. Ependymoma
    • Astrocytoma Astrocytoma Astrocytomas are neuroepithelial tumors that arise from astrocytes, which are star-shaped glial cells (supporting tissues of the CNS). Astrocytomas are a type of glioma. There are 4 grades of astrocytomas. Astrocytoma
    • Rarely metastatic disease
  • Spondylosis
    • Atlantoaxial (C1–C2) instability (e.g., due to rheumatoid arthritis Rheumatoid arthritis Rheumatoid arthritis (RA) is a symmetric, inflammatory polyarthritis and chronic, progressive, autoimmune disorder. Presentation occurs most commonly in middle-aged women with joint swelling, pain, and morning stiffness (often in the hands). Rheumatoid Arthritis (RA))
    • Spinal arthropathies
  • Osteoporosis Osteoporosis Osteoporosis refers to a decrease in bone mass and density leading to an increased number of fractures. There are 2 forms of osteoporosis: primary, which is commonly postmenopausal or senile; and secondary, which is a manifestation of immobilization, underlying medical disorders, or long-term use of certain medications. Osteoporosis (compression fractures)

Pathophysiology

Injury to the vertebral column Vertebral column The human spine, or vertebral column, is the most important anatomical and functional axis of the human body. It consists of 7 cervical vertebrae, 12 thoracic vertebrae, and 5 lumbar vertebrae and is limited cranially by the skull and caudally by the sacrum. Vertebral Column causes a spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). Spinal Cord injury involve 1 or more of the following:

  • Vertebral fracture Fracture A fracture is a disruption of the cortex of any bone and periosteum and is commonly due to mechanical stress after an injury or accident. Open fractures due to trauma can be a medical emergency. Fractures are frequently associated with automobile accidents, workplace injuries, and trauma. Overview of Bone Fractures
  • 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 Fracture A fracture is a disruption of the cortex of any bone and periosteum and is commonly due to mechanical stress after an injury or accident. Open fractures due to trauma can be a medical emergency. Fractures are frequently associated with automobile accidents, workplace injuries, and trauma. Overview of Bone Fractures
  • Contusion/ edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema at the site of injury →
  • Simultaneous compression of the spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). Spinal Cord anteriorly (by osteophytes or disc) and posteriorly (by the ligamentum flavum) 

Affected areas of the spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. 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 Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). Spinal Cord lesion

Diagnosis

  • Physical exam as above
  • Imaging
    • X-rays X-rays X-rays are high-energy particles of electromagnetic radiation used in the medical field for the generation of anatomical images. X-rays are projected through the body of a patient and onto a film, and this technique is called conventional or projectional radiography. 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 Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Hypotension Hypotension is defined as low blood pressure, specifically < 90/60 mm Hg, and is most commonly a physiologic response. Hypotension may be mild, serious, or life threatening, depending on the cause. Hypotension due to neurogenic shock Shock Shock is a life-threatening condition associated with impaired circulation that results in tissue hypoxia. The different types of shock are based on the underlying cause: distributive (↑ cardiac output (CO), ↓ systemic vascular resistance (SVR)), cardiogenic (↓ CO, ↑ SVR), hypovolemic (↓ CO, ↑ SVR), obstructive (↓ CO), and mixed. Types of Shock.
    • High-dose steroids (methylprednisolone) to suppress edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). 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 Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of 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 Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones fragments that disrupt the spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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 Posterior cord syndrome Posterior cord syndrome (PCS) is an incomplete spinal cord syndrome affecting the dorsal columns, the corticospinal tracts (CSTs), and descending autonomic tracts to the bladder. Posterior cord syndrome is rare but has a diverse range of etiologies, including demyelinating disorders, degenerative spinal conditions, neoplastic causes, vascular abnormalities, and hereditary neurodegenerative disorders. Posterior Cord Syndrome (PCS): an incomplete cord syndrome that affects the posterior aspect of the spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). 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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