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Spinal Cord Injuries: Non-traumatic

2.50 Contact Hours   -   1.00 CCU
FPTA Approval Number: CE18-588747. Accreditation of this course does not necessarily imply the FPTA supports the views of the presenter or the sponsors.

AOTA Classification Code: CAT 1: Domain of OT; CAT 2: OT Process; CAT 3: Contemporary Issues and Trends
Education Level: Intermediate
AOTA does not endorse specific course content, products, or clinical procedures. AOTA provider number 9575.

  • 0% complete
A score of 80% correct answers on a test is required to successfully complete any course and attain a certificate of completion.
Author:    Pamela Downey (MSN, ARNP)

Outcomes

This course includes the more common and important causes of nontraumatic spinal cord disorders including risk factors, clinical presentation, diagnosis, treatment and prognostic outcomes.

Objectives

Upon completion of this course, the participant will be able to:

  1. Describe the longitudinal organization of the spinal cord and spinal nerves in relation to the vertebrae.
  2. Describe the function of the spinal nerves in relation to their longitudinal organization.
  3. Describe three clinical presentations of a patient with a suspected spinal cord lesion. 
  4. Compare and contrast the seven spinal cord syndromes across manifestation and causation shown in Table 1: Spinal Cord Syndromes.
  5. Given a case scenario, the participant will be able to select the applicable differential diagnosis for myelopathy. 

Introduction

Spinal cord injury (SCI) occurs commonly. The incidence of traumatic SCI in the United States is about 40 million individuals per year, with approximately 250,000 living survivors of traumatic SCI as of July 2005.1 The prevalence of nontraumatic SCI is unknown, but it is estimated to be three to four times greater than traumatic SCI.2 SCI produces a wide variety of changes in systemic physiology that can lead to a number of complications, which rival the neurologic deficits in their impact on function and quality of life.

Medical complications after SCI are both common and severe. In the Model Spinal Cord Injury Systems Database, rehospitalizations occurred in 55% of patients in the first year after SCI and continued at a stable rate of about 37% per year over the next 20 years.3 Genitourinary and respiratory complications and pressure ulcers were the most common reasons for rehospitalization. Increased patient age and severity of the spinal cord lesion also impacted on the risk of complications requiring rehospitalization.

Pathologies that affect the spinal cord are diverse. In addition to trauma, nontraumatic common etiologies of myelopathy include inflammatory diseases, infections, vascular diseases, toxic-metabolic disorders, inherited-degenerative conditions, neoplasms and a plethora of others. The relative incidence of each of these entities depends in large part upon the clinical setting. In a regional neuroscience center in the United Kingdom, the most common cause of spastic paraparesis or quadriparesis among 585 patients was cervical spondylotic myelopathy (24%), followed by tumor (16%), multiple sclerosis (18%), and motor neuron disease (4%).4

This course will review some of the more common and important causes of nontraumatic spinal cord disorders including risk factors, clinical presentation, diagnosis, treatment and prognostic outcomes.

Life Expectancy

Life expectancy is reduced among survivors of SCI. Mortality rates are highest in the first year. For patients surviving at least one year after traumatic SCI, life expectancy is approximately 90% of normal.1,5,6 Variables which impact negatively on survival rate are higher neurologic level and severity of the injury as well as older age at the time of SCI.

The most common causes of death after traumatic SCI are diseases of the respiratory system followed by cardiovascular events.1,5 Prior to 1972, urinary complications were the leading cause of death. The risk of suicide is also increased among patients with SCI.6

Definitions to Remember

Amyotrophy: a painful condition with wasting and weakness of muscle.

Demyelination: destruction, removal, or loss of the myelin sheath of a nerve or nerves.

Myelopathy: any functional disturbance or pathological change in the spinal cord. When due to trauma, it is known as acute spinal cord injury. When inflammatory, it is known as myelitis. A disease that is vascular in nature is known as vascular myelopathy.

Myeloradiculopathy: a disease of the spinal cord and spinal nerve roots.

Radiculopathy: a disorder of the spinal nerve roots.

Vacuolar myelopathy: a heterogeneous group of conditions characterized by spinal cord degeneration.

Upper Motor Neuron Signs include:

  • Muscle Weakness
  • Spasticity
  • Clumsiness (loss of the ability to perform fine movements)
  • Clonus (involuntary, successive cycles of contraction/relaxation of a muscle)
  • Hyperreflexia (pathological reflexes, including Hoffman’s sign and positive Babinski sign)

Lower Motor Neuron Signs include:

  • Weakness
  • Clumsiness in the muscle group innervated at the level of spinal cord compromise
  • Muscle atrophy
  • Hyporeflexia
  • Muscle hypotonicity or flaccidity
  • Fasciculations
  • Sensory deficits
  • Bowel/bladder symptoms
  • Sexual dysfunction.

Paraparesis: a partial paralysis of the lower extremities.

Quadriparesis: weakness of both arms and both legs.

Ipsilateral: located on or affecting the same side of the body.

Contralateral: pertaining to, situated on, or affecting the opposite side.

Decussate: to cross.

Myotome versus dermatome: Like a dermatome, a myotome is a zone in the body that is served by one spinal nerve root. A dermatome is a zone in which sensory nerves travel on their way back to the central nervous system with sensory information. Myotomes are zones where motor nerves travel as they fulfill their responsibilities of signaling muscles to contract.

Transection: a cross section; division by cutting transversely.

Transverse: extending from side to side; situated at right angles to the long axis.

Spinal Cord Anatomy

There are 31 spinal cord segments, each with a pair of ventral (anterior) and dorsal (posterior) spinal nerve roots, which mediate motor and sensory function, respectively. The ventral and dorsal nerve roots combine on each side to form the spinal nerves as they exit from the vertebral column through the neuroforamina.

Figure 1
Cross-Sectional Anatomy of the Spinal Cord
Figure 1 - Cross-Sectional Anatomy of the Spinal Cord

Longitudinal Organization

The spinal cord is divided longitudinally into four regions:

  • Cervical
  • Thoracic
  • Lumbar
  • Sacral

The spinal cord extends from the base of the skull and terminates near the lower margin of the first lumbar vertebral body (L1). Below that level, the spinal canal contains the lumbar, sacral, and coccygeal spinal nerve roots that comprise the cauda equina.

Because the spinal cord is shorter than the vertebral column, vertebral and spinal cord segmental levels are not necessarily the same.

  • The C1 through C8 spinal cord segments lie between the C1 through C7 vertebral levels.
    • The C1 through C7 nerve roots emerge above their respective vertebrae.
    • The C8 nerve root emerges between the C7 and T1 vertebral bodies.
    • The remaining nerve roots emerge below their respective vertebrae.
  • The T1 through T12 cord segments lie between T1 through T8.
  • The five lumbar cord segments are situated at the T9 through T11 vertebral levels.
  • The S1 through S5 segments lie between T12 to L1.

Figure 2
Longitudinal Organization of Spinal Cord, Spinal Nerves, and Vertebrae

Figure 2 - Longitudinal Organization of Spinal Cord, Spinal Nerves, and Vertebrae

Cervical Cord

The first cervical vertebra (the atlas) and the second cervical vertebra (the axis), upon which the atlas pivots, support the head at the atlanto-occiput junction. The interface between the first and second vertebra is called the atlanto-axis junction.

Cervical spinal segments innervate the skin and musculature of the upper extremity and diaphragm:

  • C3 through C5 innervate the diaphragm, the chief muscle of inspiration, via the phrenic nerve
  • C4 through C7 innervate the shoulder and arm musculature
  • C6 through C8 innervate the forearm extensors and flexors
  • C8 through T1 innervate the hand musculature

Figure 3

Nerve Roots and Peripheral Nerves Corresponding to the Principle Movements of the Upper Extremity

Figure 3 - Nerve Roots and Peripheral Nerves Corresponding to the Principle Movements of the Upper Extremity

Figure 4

Figure 4 - Cervical Dermatomes

Thoracic Cord

The thoracic vertebral segments are defined by those that have an attached rib. The spinal roots form the intercostal nerves that run along the inferior rib margin and innervate the associated dermatomes, as well as, the intercostal abdominal wall musculature. These muscles are the primary muscles of expiration. The thoracic cord also contains the sympathetic nerves that innervate the heart and abdominal organs.

Lumbosacral Cord

The lumbosacral spinal cord contains the segments that innervate the muscles and dermatomes of the lower extremity, as well as, the buttocks and anal regions. Sacral nerve roots S3 through S5 originate in the narrow terminal part of the cord, called the conus medullaris.

  • L2 and L3 mediate hip flexion
  • L3 and L4 mediate knee extension
  • L4 and L5 mediate ankle dorsiflexion and hip extension
  • L5 and S1 mediate knee flexion
  • S1 and S2 mediate ankle plantar flexion

Sacral nerve roots also provide parasympathetic innervation of pelvic and abdominal organs. Lumbar nerve roots L1 and L2 contain sympathetic innervation of some pelvic and abdominal organs.

Figure 5
Nerve Roots and Peripheral Nerves Corresponding to the Principle Movements of the Lower Extremity
Figure 5 - Nerve Roots and Peripheral Nerves Corresponding to the Principle Movements of the Lower Extremity

Cauda Equina

In adults, the spinal cord ends at the level of the first or second lumbar vertebral bodies. The filum terminale, a thin connective tissue filament that descends from the conus medullaris with the spinal nerve roots, is connected to the third, fourth, and fifth sacral vertebrae. Its terminal part is fused to the periosteum at the base of the coccygeal bone.

Pathology at the T12 and L1 vertebral level affects the lumbar cord. Injuries to L2 frequently damage the conus medullaris. Injuries below L2 usually involve the cauda equina and represent injuries to spinal roots rather than to the spinal cord.

Cross-Sectional Anatomy

The spinal cord contains the gray matter, the butterfly-shaped central region, and the surrounding white matter tracts. The spinal cord gray matter, which contains the neuronal cell bodies, is made up of the dorsal and ventral horns, each divided into several laminae.7, 8

Dorsal Horn

The dorsal horn is the entry point of sensory information into the central nervous system (CNS). It is divided into six layers or laminae that process sensory information. More than a relay station for the transmission of sensory information, the dorsal horn also modulates pain transmission through spinal and supraspinal regulatory circuits.

Three major categories of sensory input that are important to the clinical examination of spinal cord pathology include:

  • Afferents from muscle spindles that participate in spinal cord reflexes.
  • Axons, mostly small and unmyelinated, mediating sensory modalities of pain and temperature. These can travel up and down a few segments before synapsing with the second order neurons, which then cross the midline of the cord in the anterior commissure, just anterior to the central canal, and then enter the contralateral anterior or lateral spinothalamic tract.
  • Axons mediating the sensory modalities of proprioception, vibration, and touch discrimination. These large myelinated fibers pass through the dorsal horn to enter the ipsilateral dorsal column.

Ventral Horn

The dorsal horn is the entry point of sensory information into the central nervous system (CNS). It is divided into six layers or laminae that process sensory information. More than a relay station for the transmission of sensory information, the dorsal horn also modulates pain transmission through spinal and supraspinal regulatory circuits.

Three major categories of sensory input that are important to the clinical examination of spinal cord pathology include:

  • Afferents from muscle spindles that participate in spinal cord reflexes.
  • Axons, mostly small and unmyelinated, mediating sensory modalities of pain and temperature. These can travel up and down a few segments before synapsing with the second order neurons, which then cross the midline of the cord in the anterior commissure, just anterior to the central canal, and then enter the contralateral anterior or lateral spinothalamic tract.
  • Axons mediating the sensory modalities of proprioception, vibration, and touch discrimination. These large myelinated fibers pass through the dorsal horn to enter the ipsilateral dorsal column.

White matter tracts

The major white matter tracts of clinical importance in the assessment of spinal cord disease include:

  • The dorsal, or posterior columns, the fasciculus gracilis, and the fasciculus cuneatus. These contain sensory information regarding joint position and vibration. They are organized anatomically such that cervical sections lie most laterally and sacral segments most medially. These pathways will cross in the medulla so that, in the spinal cord, these tracts contain ipsilateral sensory representation.
  • The anterior and lateral spinothalamic tracts contain sensory information regarding pain, temperature, and touch. These axons have crossed in the ventral commissure and, therefore, contain contralateral sensory representation. This tract is somatotopically organized with cervical inputs located most medially and sacral inputs most laterally.
  • The corticospinal tracts contain the upper motor neurons that originate in M1 of the primary motor cortex. These axons synapse either directly or indirectly on the anterior horn cells and, as such, have distinct sites of anatomic origin within M1.9 A single corticomotoneuronal axon synapses with many anterior horn cells of its own motor neuron pool and with those of agonists and antagonists, allowing for coordination of highly skilled movements.
  • The lateral corticospinal tract contains the majority (80 to 85%) of these fibers, which have previously decussated (crossed) at the cervicomedullary junction and therefore provide input to the ipsilateral musculature. Fibers are somatotopically organized within the tract such that fibers destined for upper extremity motor control lie most medially, while fibers controlling the lower extremity lie more laterally. The anterior corticospinal tract contains undecussated fibers, some of which will subsequently cross at the spinal level through the anterior commissure.

Other descending tracts include:

  • The tectospinal tract originates in the superior colliculus and mediates reflex postural movements of the head in response to visual and/or acoustic input.
  • The rubrospinal pathway originates from the magnocellular subdivision of the red nucleus in which there are direct connections with motoneurons innervating wrist muscles.
  • The vestibulospinal tracts arise from the vestibular nuclei and facilitate spinal cord reflexes and muscle tone to maintain posture.
  • Reticulospinal connections are widely assumed to be responsible for coordinated gross movements primarily of proximal muscles, whereas the corticospinal tract mediates fine movements, particularly of the hand.10 However, the reticulospinal system may form a parallel pathway to distal muscles, alongside the corticospinal tract. As a result, reticulospinal neurons may influence upper limb muscle activity after damage to the corticospinal system as may occur in stroke.11,12

Other ascending tracts include:

  • The dorsal and ventral spinocerebellar tracts carry inputs mediating unconscious proprioception directly to the cerebellum.
  • The spinoreticular tract carries deep pain input to the reticular formation of the brainstem.

Autonomic Fibers

Autonomic fibers of hypothalamic and brainstem origin descend in the lateral aspect of the spinal cord but not in a well-defined tract. These synapse with cell bodies in the intermediolateral columns of the central gray matter of the spinal cord. Sympathetic fibers exit between T1 and L2, and parasympathetic fibers exit between S2 and S4.

The sympathetic neurons lie in the lateral horn of the central gray matter at spinal levels T1-L3. The preganglionic fibers exit via the ventral root, spinal nerve, and ventral ramus to reach the paravertebral ganglion. Many will synapse at the paravertebral ganglion; others pass through it to terminate on postganglionic neurons (e.g., coeliac, superior mesenteric, and inferior mesenteric ganglia) more proximate to their end organ.

Parasympathetic neurons originate in the sacral spinal cord and exit the spinal cord with other afferents to the ventral ramus. After leaving the ventral ramus, they may subsequently join with sympathetic nerves to reach the viscera. These preganglionic fibers then synapse with a diffuse network of terminal ganglion cells that affect organs in the pelvis.

Autonomic dysfunction is an important determinant of site, extent, and severity of spinal cord pathology. Many autonomic functions can be affected by spinal cord pathology, but for clinical evaluation, the most useful symptoms relate to bladder control.

Autonomic bladder control is primarily parasympathetic and is unaffected by isolated injury to the sympathetic fibers. Voluntary bladder control is under somatomotor control, mediated by motor fibers originating from the anterior horn cells at levels S2-S4. A spinal cord lesion that interrupts descending motor and autonomic tracts above the S2 level produces an "automatic bladder" that cannot be emptied voluntarily, but empties reflexly when expanded to a certain degree, the so-called neurogenic bladder.13-16 Loss of descending inhibition of segmental reflex control leads to urinary urgency and incontinence. Injury to S2-S4 spinal levels interrupts the bladder reflex circuit. The bladder becomes flaccid and fills beyond capacity with overflow incontinence.

Other autonomic functions are disturbed by spinal cord pathology. The effects of spinal cord injury on the colon and rectum are similar to those of the bladder. Spinal cord transections interrupt voluntary control of the external sphincter and produce constipation. Sacral lesions cause a loss of the anal reflex and rectal incontinence. Impotence can result from spinal cord lesions at any level. Spinal cord injuries can also affect cardiovascular function, most dramatically with lesions above T6 which can produce a phenomenon of autonomic dysreflexia.

Blood Supply

A single anterior and two posterior spinal arteries supply the spinal cord (Figure 8). The anterior spinal artery supplies the anterior two-thirds of the cord.17-21 The posterior spinal arteries primarily supply the dorsal columns. The anterior and posterior spinal arteries arise from the vertebral arteries in the neck and descend from the base of the skull. Various radicular arteries branch off the thoracic and abdominal aorta to provide additional blood supply to the spinal arteries. The largest and most consistently present of these radicular branches is the great ventral radicular artery or the artery of Adamkiewicz, which supplies the anterior spinal artery.22 This artery enters the spinal cord anywhere between T5 and L1 (usually between T9 and T12).

In most individuals, the anterior spinal artery passes uninterrupted along the entire length of the spinal cord. In others, it is discontinuous, usually in its midthoracic segment, making these individuals more susceptible to vascular injury. The primary watershed area of the spinal cord in most individuals is in the midthoracic region.

Clinical Localization

A spinal cord lesion may be suspected when there are bilateral motor and sensory signs or symptoms that do not involve the head or face.

  • Motor deficits are manifest by weakness and long tract signs (spasticity, increased reflexes, Babinski sign).14,23-25
    • When the pathology is localized or segmental, these findings will be present in muscle groups innervated below that level and will be normal above.
  • A sensory level, with normal sensation above and reduced or absent below, can also often be defined and should be specifically sought.
  • Other so-called segmental signs include lower motor neuron findings (atrophy, flaccid weakness, loss of reflexes) in a myotomal distribution at the specific level of involvement. These, however, are usually not elicitable in thoracic lesions.

As well as longitudinal localization within the spinal cord, it can also be helpful to distinguish specific areas of functional loss with a spinal cord level (or across spinal cord levels for nonsegmental pathologies). Some disorders affecting the spinal cord preferentially affect different structures, and therefore careful testing of all spinal cord functions, including motor, reflex, and all sensory modalities, and sphincter function is important for clinical localization.

Several distinct spinal cord syndromes are recognized. These are useful for the clinical evaluation, as they often correspond to distinct pathologies. These are summarized in Table 1 and are further discussed below.

Table 1: Spinal Cord Syndromes
SyndromeClinical ManifestationsCauses
Segmental (Transection) SyndromeLoss of all sensory modalities, weakness below affected level; bladder dysfunction

Trauma, spinal cord hemorrhage, epidural or intramedullary abscess, transverse myelitis, epidural metastasis

Dorsal (Posterior) Cord Syndrome

Loss of proprioception, vibratory sensation, variable weakness; bladder dysfunction

Tabes dorsalis, Friedreich ataxia, subacute combined degeneration, AIDS myelopathy, epidural and extramedullary metastases, cervical spondylotic myelopathy, multiple sclerosis, atlantoaxial subluxation

Ventral Cord (Anterior Spinal Artery) Syndrome

Loss of pain and temperature sensation, weakness; bladder dysfunction

Spinal cord infarction, intervertebral disc herniation, radiation myelopathy, HTLV-1

Brown Sequard (Hemi-Cord) Syndrome

Ipsilateral weakness and loss of proprioception; contralateral loss of pain and temperature sensation

Knife or bullet injuries, multiple sclerosis, spinal cord tumors, disc herniation, infarction, infection

Pure Motor Syndrome

Weakness without sensory disturbance

Poliomyelitis, post-polio syndrome, primary lateral sclerosis, amyotrophic lateral sclerosis, HTLV-1, hereditary spastic paraplegia, lathyrism, progressive muscular atrophy, electric shock-induced myelopathy

Conus Medullaris Syndrome

Bladder and rectal dysfunction; saddle anesthesia

Disc herniation, spinal fractures, tumors

Cauda Equina Syndrome

Asymmetric multiradicular pain, leg weakness, and sensory loss; bladder dysfunction

Intervertebral disc herniation, epidural abscess, epidural tumor, intradural extramedullary tumor, lumbar spine spondylosis, spinal arachnoiditis, chronic inflammatory demyelinating polyneuropathy, sarcoidosis, carcinomatous meningitis, cytomegalovirus, herpes simplex virus, herpes zoster virus, Epstein Barr virus, Lyme disease, mycoplasma, and tuberculosis

Segmental (Transection) Syndrome

Pathologies that affect all functions of the spinal cord at one or more levels produce a segmental syndrome. Loss of function may be total or incomplete. A total cord transection syndrome results from the cessation of function in all ascending and descending spinal cord pathways and results in the loss of all types of sensation and loss of movement below the level of the lesion. Less profound injuries produce a similar pattern of deficits, which are less severe, i.e., weakness rather than paralysis and decreased sensation rather than anesthesia.

Acute transection can cause spinal shock, with a flaccid paralysis, urinary retention, and diminished tendon reflexes. Acute transection is usually temporary, and increased tone, spasticity, and hyperreflexia will usually supervene in days or weeks after the event.

Transverse injuries above C3 involve cessation of respiration and are often fatal if acute. Cervical cord lesions that spare the phrenic nerve but impair intercostal nerve function can produce respiratory insufficiency. Lesions above the L2 cord level will cause impotence and spastic paralysis of the bladder. There is loss of voluntary control of the bladder, which will empty automatically by reflex action.

Causes of segmental syndrome include acute myelopathies, such as traumatic injury and spinal cord hemorrhage. Epidural or intramedullary abscess, tumors, and transverse myelitis may have a more subacute presentation.

Dorsal (Posterior) Cord Syndrome

Dorsal cord syndrome results from the bilateral involvement of the dorsal columns, the corticospinal tracts, and descending central autonomic tracts to bladder control centers in the sacral cord (Figure 9). Dorsal column symptoms include gait ataxia and paresthesias. Corticospinal tract dysfunction produces weakness that, if acute, is accompanied by muscle flaccidity and hyporeflexia and, if chronic, by muscle hypertonia and hyperreflexia. Extensor plantar responses and urinary incontinence may be present.

Causes of a dorsal cord syndrome include multiple sclerosis (more typically the primary progressive form), AIDS myelopathy, tabes dorsalis, Friedreich ataxia, subacute combined degeneration, vascular malformations, epidural and intradural extramedullary tumors, cervical spondylotic myelopathy, and atlantoaxial subluxation.

Ventral (Anterior Spinal Artery) Cord Syndrome

Ventral cord or anterior spinal artery syndrome usually includes tracts in the anterior two-thirds of the spinal cord, which include the corticospinal tracts, the spinothalamic tracts, and descending autonomic tracts to the sacral centers for bladder control. Corticospinal tract involvements produce weakness and reflex changes. A spinothalamic tract deficit produces the bilateral loss of pain and temperature sensation. Tactile, position, and vibratory sensation are normal. Urinary incontinence is usually present.

The causes of a ventral cord syndrome include spinal cord infarction, intervertebral disc herniation, radiation myelopathy, and HTLV-1.

Brown-Sequard (Hemi-Cord) Syndrome

A lateral hemisection syndrome, also known as the Brown–Sequard syndrome, involves the dorsal column, corticospinal tract, and spinothalamic tract unilaterally. This condition produces weakness, loss of vibration and proprioception ipsilateral to the lesion, and loss of pain and temperature on the opposite side. The unilateral involvement of descending autonomic fibers does not produce bladder symptoms. While there are many causes of this syndrome, knife or bullet injuries and demyelination (such as occurs in multiple sclerosis) are the most common. Rarer causes include spinal cord tumors, disc herniation, infarction, and infections.

Central Cord Syndrome

The central cord syndrome is characterized by loss of pain and temperature sensation in the distribution of one or several adjacent dermatomes at the site of the spinal cord lesion caused by the disruption of crossing spinothalamic fibers in the ventral commissure. Dermatomes above and below the level of the lesion have normal pain and temperature sensation, creating the so-called "suspended sensory level." Vibration and proprioception are often spared.

As a central lesion enlarges, it may encroach on the medial aspect of the corticospinal tracts or on the anterior horn gray matter, producing weakness in the analgesic areas. Fibers mediating the deep tendon reflexes are interrupted as they pass from the dorsal to the ventral horn, thus causing tendon reflex loss in the analgesic areas. There are usually no bladder symptoms.

The classic causes of a central cord syndrome are slow-growing lesions such as syringomyelia or intramedullary tumor. However, central cord syndrome is most frequently the result of a hyperextension injury in individuals with long-standing cervical spondylosis. This form of central cord syndrome is characterized by disproportionately greater motor impairment in upper compared with lower extremities, bladder dysfunction, and a variable degree of sensory loss below the level of injury.26-28

Case Scenario

Clinicians frequently provide treatment to clients that have experienced a non-traumatic spinal cord injury. As Healthcare professionals understanding common, non-traumatic causes of myelopathy including inflammatory diseases, infections, vascular diseases, toxic-metabolic disorders, inherited-degenerative conditions, and neoplasms will positively affect the ability to proficiently assess, prepare a plan of care, implement the plan and evaluate its outcomes and explain deficits to other healthcare professionals. The below scenario demonstrates how increased awareness of non-traumatic spinal cord injury can improve outcomes for clients.

A 60-year-old female client is referred from an Urgent Care Center, Workman’s Compensation physician, for Outpatient Occupational Therapy to evaluate and treat the client with right shoulder pain, secondary to work-related upper extremity strain.

During the initial evaluation, the client indicates that she works for a local airline. She is the manager of the baggage claim department, specifically the area where lost baggage is stored until the owner is located and comes in for a pickup. She has been a manager for five years but has worked in the same department for 10 years. On this particular day, she was filling in for another agent, when she needed to place a small boarding bag on a top shelf. The client reports that when she raised the bag up over her head, she felt “electrical shock like” sensations down her back. Further investigation reveals that she experienced this same sensation about two weeks ago when washing her hair. Client states that she feels that her right arm is getting weak and impacting her ability to fasten a bra, wash her hair, as well as her ability to function optimally at her job.

Physical exam included ROM of both UE’s, which is WNLs. Strength assessment of BUE’s that indicates clients:

  • Strength in the RUE is 3+/5 as compared to the LUE which is 5/5.
  • Grasp strength assessment results are R-an average of 28#s, L-an average of 44# Norms are R 55#s L 45#s.
  • Arm Curl Test scores are R 12 reps in 30 secs with 5# dumbbell, and L 15 reps Norms are 13-19 reps in 30 secs with 5# dumbbell.

Each of these assessments indicates the client has a significant loss of strength in the RUE.

Sensory exam indicated the client presents with:

  • Numbness of the middle and index finger,
  • Observable atrophy in the intrinsic muscles of the hand and
  • Impaired pain and temperature sensation in the middle and index fingers.

Each of these assessments indicates the client has impaired motor and sensory function along the C 6-7 myotome/dermatome.

The client is scheduled for Occupational Therapy, 3x a week x 2 weeks. The treatment plan includes Therapeutic Ex-strengthening program. Self-Care Training-use of compensatory strategies to don/doff bra and wash hair and patient education. Therapeutic Activities- including functional task practice and task modification.

The above evaluation is faxed to the MD, with a follow-up phone call. During the phone call, the OT expressed concerns and advised the MD of findings of the exam with emphasis on the assessment results that are inconsistent with a strain, including; complaints of shock like sensations when extending head (+ Lhermitte’s sign), strength loss of RUE, and loss of sensation along the C6-7 myotome/dermatome.

The OT suspects the client has been misdiagnosed or presents with multiple diagnoses. Following the MD’s review, it is determined that the client has a probable cervical spondylosis, a form of central cord syndrome, as well as a shoulder strain.

Pure Motor Syndrome

A pure motor syndrome produces weakness without sensory loss or bladder involvement. This condition may involve only the upper motor neurons, producing hyperreflexia and extensor plantar responses, or only the lower motor neuron bilaterally, producing muscle atrophy and fasciculations. Other disorders involve both the upper and lower motor neurons and produce mixed signs.

The causes of a pure motor syndrome include chronic myelopathies such as HTLV-I myelopathy, hereditary spastic paraplegia, primary lateral sclerosis, amyotrophic lateral sclerosis, progressive muscular atrophy, post-polio syndrome, and electric shock-induced myelopathy.

Conus Medullaris Syndrome

Lesions at vertebral level L2 often affect the conus medullaris. There is early and prominent sphincter dysfunction with flaccid paralysis of the bladder and rectum, impotence, and saddle (S3-S5) anesthesia. Leg muscle weakness may be mild if the lesion is very restricted and spares both the lumbar cord and the adjacent sacral and lumbar nerve roots.

Causes include disc herniation, spinal fracture, and tumors.13,29

Cauda Equina Syndrome

Though not a spinal cord syndrome, cauda equina syndrome is considered here because its location within the spinal canal subjects it to many of the same disease processes that cause myelopathy. The syndrome is caused by the loss of functions of two or more of the 18 nerve roots constituting the cauda equina. Deficits usually affect both legs but are often asymmetric.

Symptoms include30-32:

  • Low back pain accompanied by pain radiating into one or both legs. Radicular pain reflects involvement of dorsal nerve roots and may have localizing value.30
  • Weakness of plantar flexion of the feet with loss of ankle jerks occurs with mid cauda equina lesions, involving S1, S2 roots. Involvement of progressively higher levels leads to corresponding weakness in other muscles.
  • Bladder and rectal sphincter paralysis usually reflect involvement of S3-S5 nerve roots.30,31
  • Sensory loss of all sensory modalities occurs in the dermatomal distribution of the affected nerve roots.

Many etiologies can cause a cauda equina syndrome, including intervertebral disc herniation, epidural abscess, epidural tumor, intradural extramedullary tumor, lumbar spine spondylosis, and a number of inflammatory conditions including spinal arachnoiditis, chronic inflammatory demyelinating polyneuropathy, and sarcoidosis.29,33-38 The cauda equina can also be the primary site of involvement in carcinomatous meningitis and a number of infections (e.g., cytomegalovirus, herpes simplex virus, herpes zoster virus, Epstein Barr virus, Lyme disease, mycoplasma, and tuberculosis).

Lhermitte's Sign

This well-described sign describes electric shock-like sensations that run down the back and/or limbs during flexion of the neck. This condition generally occurs with pathologies involving the cervical spinal cord but is not specific to etiology, occurring in patients with cervical spondylotic myelopathy,39 multiple sclerosis, radiation myelopathy, and vitamin B12 deficiency, among others. It can also occur with cervical nerve root pathology.

Diagnosis

The differential diagnosis of myelopathy is wide, but can be significantly narrowed by the clinical syndrome (Table 1 above). Other features in the examination and history also limit the differential diagnosis and tailor the diagnostic work-up. Clinical features of some of the more common causes of myelopathy are outlined in Table 2.

Table 2: Important Causes of Spinal Cord Dysfunction*
 AgeCourseClinical FeaturesDiagnosis

Cervical spondylotic myelopathy

Usually >60 yearsProgressive or stepwise courseModerate-severe cases demonstrate gait and leg spasticity and amyotrophy of hand or arms

MRI cervical spine

Transverse myelitis

Children, young adultsSubacuteSegmental cord syndromeMRI and CSF
Viral myelitisAny ageAcute-subacutePure motor syndrome or Segmental cord syndromeMRI and CSF
Epidural abscessAny ageSubacute; may worsen abruptlySegmental cord syndromeMRI
Infarction

Usually >60 years

Abrupt onsetAnterior cord syndrome

MRI with diffusion weighted sequences

Vascular malformation

>40 years (dural fistula)

20's (intramedullary AVM)
Acute and/or stepwiseRadicuomyelopathy

MRI, spinal angiography

Subacute combined degeneration

Any ageSlowly progressiveDorsal cord syndrome

Vitamin B12 levels

Radiation

Any ageSlowly progressive; beginning 6-12 months after radiation therapySegmental cord syndrome or Ventral cord syndrome

MRI, clinical history

Syringomyelia

Children, young adultsSlowly progressiveCentral cord syndrome

MRI

Epidural metastasis

Usually >50 yearsSubacute, may worsen abruptlySegmental cord syndrome

MRI

Intramedullary tumor

Young adultsSlowly progressiveCentral cord syndrome

MRI with gadolinium enhancement

ALS

Usually >60 yearsProgressivePure motor syndrome

Electromyography

*MRI: magnetic resonance imaging; CSF: cerebrospinal fluid; AVM: arteriovenous malformation; ALS: amyotrophic lateral sclerosis.

For patients with a clinical syndrome that suggests a localized process within the spinal cord (e.g., transection syndrome, central cord syndrome, ventral cord syndrome, etc.), an imaging study, usually magnetic resonance imaging (MRI), of the relevant section of the spinal cord is usually required.25,40 Administration of gadolinium contrast is often helpful. When an infectious or inflammatory disorder is suspected, CSF examination may be helpful. The role of positron emission tomography in evaluating patients with myelopathy is under investigation. It appears to be particularly sensitive to neoplastic disease.41 

In general, the pace at which spinal cord deficits appear dictates the urgency of the neurologic evaluation. Even when the deficits are not severe, acute myelopathic signs need to be evaluated urgently because neurologic deterioration can occur abruptly, and the clinical deficit at the time of intervention often dictates the chances of recovery. This is true particularly for compressive etiologies such as spinal cord metastases and epidural spinal abscess.

Specific Disorders Affecting the Spinal Cord

Inflammatory Diseases

Transverse Myelitis (TM)

TM is a segmental spinal cord injury caused by acute inflammation.42-44 The inflammation of TM is generally restricted to one or two segments, usually in the thoracic cord. TM is uncommon, with an approximate incidence of between one to five cases per million population annually.45

Most cases of TM are idiopathic and presumably result from an autoimmune process. Up to half of these patients have a preceding infection.46-48 TM can also occur in multiple sclerosis (MS) and can be the presenting demyelinating event.49 Neuromyelitis optica or Devic's disease is a disorder related to MS, in which demyelinating events are limited to the optic nerve and spinal cord.

  • TM is also associated with connective tissue diseases, including:
    • Systemic lupus erythematosus46,50-51
    • Mixed connective tissue disease52
    • Sjogren's syndrome46,53-54
    • Scleroderma55
    • Antiphospholipid antibody syndrome46
    • Ankylosing spondylitis56
    • Rheumatoid arthritis57

Clinical Presentation

  • Timeframe of Development
    • Typically develops rapidly over several hours.
    • Approximately 37% of patients worsen maximally within 24 hours.58
    • Occasionally patients worsen more slowly, over several weeks.
  • Characteristics
    • Typically the inflammation is bilateral, producing weakness and multimodality sensory disturbance below the level of the lesion.45,47
    • Unilateral syndromes (e.g., Brown Sequard) have also been described.
    • Almost all patients develop leg weakness of varying severity.
    • Arm weakness also occurs if the lesion is in the cervical cord.
    • Diminished sensation, pain, and tingling.
    • Tight banding or girdle-like sensation around the trunk, which may be very sensitive to touch.
    • Back and radicular pain
    • Bowel and bladder dysfunction, reflective of autonomic involvement, may also occur.

Diagnosis

  • MRI of the involved section of the spinal cord shows gadolinium-enhancing signal abnormality, usually extending over one or more cord segments.45,48,59-60 The cord often appears swollen at these levels.
  • CSF is abnormal in half of the patients, with elevated protein level (usually 100 to 120 mg/100 mL) and moderate lymphocytosis (usually <100 /mm3). Glucose levels are normal. Oligoclonal bands are usually not present in isolated TM, and when present suggest a higher risk of subsequent MS.45,61
  • Other studies can help delineate the underlying cause (Table 3).
Table 3: Potential Medical Work-up for Suspected Acute Transverse Myelitis*

Indicative Signs and Symptoms

Suggested Evaluation

Infectious Etiology

Fever

CSF Gram stain and bacterial culture

Meningismus

CSF PCR: HSV-1, HSV-2, HHV-6, VZV, CMV, EBV, enteroviruses

Rash

CSF viral culture

Concurrent systemic infection

CSF acid-fast bacilli smear and tuberculous culture

Recurrent genital infection

CSF anti-Borrelia burgdorferi antibodies

Symptoms of zoster radiculopathy

CSF VDRL

Adenopathy

CSF India ink and fungal culture

Residence in area endemic for parasitic infections

Chest radiograph

Lymphadenopathy

Serology for antibodies to HIV, HSV, VZV, HTLV-1, B. burgdorferi

Serology for hepatitis A, B, C, and Mycoplasma

Consider serology for parasites

Blood cultures

Systemic Inflammatory Disease (Vasculitis, Collagen Vascular Diseases, Mixed Connective Tissue Disease)

RashSerum ACE
Oral or genital ulcersAuto-antibodies: ANA, ds-DNA, Ro/SSA, La/SSB, Sm, RNP
AdenopathyComplement levels
Livedo reticularisUrinalysis with microscopic analysis for hematuria
SerositisLip/salivary gland biopsy
PhotosensitivityChest CT with intravenous contrast
Inflammatory arthritisSchirmer test
Erythema nodosumChest radiograph
XerostomiaGallium scan
KeratitisAntiphospholipid antiodies (anticardiolipin antibodies, Russel viper venom time, partial thromboplastin time)

Conjunctivitis

Contractures or thickening of skin

Anemia/leukopenia/thrombocytopenia

Raynaud phenomenon

History of arterial and venous thrombosis

Multiple Sclerosis

Previous demyelination event

Brain MRI

Incomplete deficit clinically with MRI abnormality ≤2 spinal segments and <50 percent of cord diameter

Evoked potentials

CSF oligoclonal bands and IgG index

Neuromyelitis Optica (Devic's Disease)

Optic neuritis

Evoked potentials

Clinical deficit with MRI abnormality ≥3 spinal segments

Brain MRI (usually negative)

NMO-IgG testing

Idiopathic Transverse Myelitis

No clinical or paraclinical features suggestive of another diagnostic category

Evoked potentials

Electromyography/nerve conduction velocity

*ACE: angiotensin-converting enzyme; ANA: anti-nuclear antibodies; CMV: cytomegalovirus; CSF: cerebrospinal fluid; EBV: Epstein-Barr virus; HHV: human herpes virus; HIV: human immunodeficiency virus; HSV: herpes simplex virus; HTLV-1: human T-cell lymphotropic virus 1; IgG: immunoglobulin G; NMO-IgG: neuromyelitis optica IgG autoantibody; VDRL: Venereal Disease Research Laboratory; VZV: varicella zoster virus.*ACE: angiotensin-converting enzyme; ANA: anti-nuclear antibodies; CMV: cytomegalovirus; CSF: cerebrospinal fluid; EBV: Epstein-Barr virus; HHV: human herpes virus; HIV: human immunodeficiency virus; HSV: herpes simplex virus; HTLV-1: human T-cell lymphotropic virus 1; IgG: immunoglobulin G; NMO-IgG: neuromyelitis optica IgG autoantibody; VDRL: Venereal Disease Research Laboratory; VZV: varicella zoster virus.

Modified with permission from: Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology 2002; 59:499. Copyright © 2002 Lippincott Williams & Wilkins.Modified with permission from: Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology 2002; 59:499. Copyright © 2002 Lippincott Williams & Wilkins.

Treatment

  • While patients are often treated with parenteral corticosteroid therapy, there is limited evidence that this approach alters outcomes.46,48,62
  • Patients with TM associated with systemic autoimmune disease may be more likely to receive treatment with corticosteroids and other immunosuppressive and immunomodulatory therapies.63

Prognosis

  • Most patients with idiopathic TM have at least a partial recovery, which usually begins within one to three months.62
  • Some degree of persistent disability is common, occurring in about 40% of patients.48,62
  • Significant recovery is unlikely if there is no improvement by three months. 
  • A very rapid onset with complete paraplegia and spinal shock have been associated with poorer outcomes.46,62,64
  • TM is generally a monophasic illness. However, a small percentage of patients may suffer a recurrence.65,66

Sarcoidosis

The granulomatous inflammation of sarcoidosis can affect the spinal cord and produce an acute or subacute segmental myelopathy.46,69-71 The lesions can be extramedullary or intramedullary and can involve the cauda equine, as well as, the cord.

Diagnosis

The granulomatous inflammation of sarcoidosis can affect the spinal cord and produce an acute or subacute segmental myelopathy.46,69-71 The lesions can be extramedullary or intramedullary and can involve the cauda equine, as well as, the cord.

Treatment

  • Patients with neurologic sarcoidosis are generally treated with corticosteroids and other immunomodulatory agents and can improve.

Paraneoplastic Syndromes

A number of distinct paraneoplastic syndromes involving the spinal cord has been described. These rare syndromes include:

  • Motor Neuron Syndrome:
    • Clinical Presentation:
      • Subacute, progressive, painless, and often asymmetric lower motor neuron weakness
      • Most often associated with lymphoma72
  • Acute Necrotizing Myelopathy:
    • Clinical Presentation:
      • Rapidly ascending syndrome of sensory deficits, sphincter dysfunction, and flaccid or spastic paraplegia or quadriplegia73
  • Subacute Sensory Neuronopathy:
    • Clinical Presentation:
      • Inflammatory disorder affecting the dorsal root ganglia, producing progressive loss of sensory modalities, leading to prominent ataxia74 
      • Most often associated with small cell lung cancer and anti-Hu antibodies.
  • Encephalomyelitis:
    • Diffuse involvement of the brain and spinal cord regions, in which cerebral manifestations frequently overshadow the myelopathy. 

Infections

Epidural Abscess

Spinal epidural abscess is a rare disease, occurring in only one patient per 10,000 requiring hospital admission.75 The most common pathogen is Staphylococcus aureus, which accounts for about two-thirds of cases.75 Damage to the spinal cord can be caused by direct compression of neural elements or arterial blood supply, thrombosis and thrombophlebitis of nearby veins, focal vasculitis, and/or bacterial toxins and mediators of inflammation.


Causes of epidural abscess include

  • Contiguous spread from infections of skin and soft tissues
  • Complications of spinal surgery and other invasive procedures, including indwelling epidural catheters
  • Other cases of epidural abscess arise from a remote site via the bloodstream

Risk factors include

  • Diabetes
  • Alcoholism
  • Human immunodeficiency virus (HIV) infection

Clinical Presentation

  • The classic clinical triad consists of fever, spinal pain, and neurologic deficits. However, only a few patients have all three components at presentation.76
  • The rate of neurologic progression is highly variable.

Diagnosis

  • MRI is the preferred test and is highly sensitive for this diagnosis.76
  • Blood cultures and/or aspirate of abscess contents are performed to identify the etiologic organism.

Treatment

  • Surgical decompression and drainage with systemic antibiotic therapy

Prognosis

  • Early diagnosis and treatment are imperative because the preoperative neurologic deficit is an important predictor of final neurologic outcome.

Acute Viral Myelitis

Viral invasion of the anterior horn cells occurs as part of an acute viral illness. Two distinct syndromes of spinal cord involvement are associated with acute viral disease.

First Syndrome

The virus targets the gray matter of the spinal cord, producing acute lower motor neuron disease.77

  • These viruses include:
    • Enteroviruses, such as poliovirus, coxsackie virus, and enterovirus.71,78
    • Flaviviruses, such as West Nile virus and Japanese encephalitis virus.79-81
  • Clinical Presentation:
    • Fever, headache, and meningismus
    • Asymmetrical flaccid weakness with reduced or absent reflexes
    • Few sensory symptoms or signs
  • Diagnosis
    • MRI often shows hyperintensities in the anterior horns of the spinal cord on T2-weighted imaging.78,82
    • CSF analysis demonstrates a moderate pleocytosis.
    • These features help to distinguish this form of viral myelitis from Guillain-Barré syndrome, which usually produces symmetric deficits, with no MRI abnormalities, and is associated with elevated CSF protein levels without pleocytosis.
  • Treatment:
    • Supportive in nature
  • Prognosis:
    • The prognosis for recovery is variable.

Second Syndrome

A second form of viral myelitis has clinical and diagnostic test features that are similar to transverse myelitis. The association between the myelitis and the virus is not always clear. In some cases, these may represent a post infectious transverse myelitis. In others, a positive polymerase chain reaction (PCR) test in the CSF suggests that the myelitis is directly related to the viral infection.

  • Associated viruses include:83-89
    • Cytomegalovirus
    • Varicella zoster
    • Herpes simplex virus
    • Hepatitis C
    • Epstein Barr virus
  • Treatment:
    • Antiviral agents
    • Corticosteroids

AIDS Myelopathy

HIV infection produces a vacuolar myelopathy, which is found in up to half of patients with AIDS at autopsy.76,90 The pathogenesis of vacuolar myelopathy is unknown but may be related to abnormal transmethylation mechanisms induced by the virus and/or cytokines. Pathological descriptions include demyelination of the dorsal columns and the dorsal half of the lateral columns, with prominent vacuoles within the myelin sheaths. This pathologic appearance is similar to the changes seen in the subacute combined degeneration of the cord.

Clinical manifestations occur when the pathology is advanced, and only about one-fourth of patients demonstrating vacuolar myelopathy at autopsy have symptoms during life.91

Clinical Presentation

  • In typical cases, a slowly progressive spastic paraparesis is accompanied by loss of vibration and position sense and urinary frequency, urgency, and incontinence.68
  • Upper-extremity

Diagnosis

  • MRI of the spine is usually normal.
  • CSF examination may show nonspecific abnormalities, such as protein elevation.
  • Abnormal sensory evoked potentials may precede clinical symptoms of myelopathy.

Treatment

  • Aggressive antiretroviral therapy can lead to improvement of symptoms.92
  • In one case series, the use of intravenous immunoglobulin appeared to be associated with neurologic improvement.93

HTLV-1 Myelopathy

Human T-cell lymphotropic virus type I (HTLV-1) causes a progressive neurologic disease, which is called either HTLV-1-associated myelopathy (HAM) or tropical spastic paraparesis (TSP).94,95 Pathologic studies demonstrate inflammation of the lateral corticospinal, spinocerebellar, and spinothalamic tracts, with relative sparing of the posterior columns.96

This disorder is endemic in southern Japan, the Caribbean, South America, the Melanesian islands, Papua New Guinea, the Middle East, and central and southern Africa, with seroprevalences as high as 30% in southern Japan.97-99 In contrast, it is quite rare in the United States and Europe. It is more common in women than men.

Clinical Presentation

  • Primarily involves the thoracic cord
  • HAM/TSP is characterized by a slowly progressive spastic paraparesis and urinary disturbance.
  • HAM/TSP has also been associated with other nervous system pathology that results in less frequent symptoms, suggesting cerebral, cerebellar, cranial nerve, and peripheral nerve involvement.94,99

Diagnosis

  • MRI of the spinal cord may show spinal atrophy, particularly of the thoracic cord.95,100
  • Brain MRI often shows subcortical, periventricular white matter lesions.
  • CSF examination reveals a mild lymphocytosis and/or elevated protein concentration in some patients. Anti-HTLV-I antibodies are detected in the CSF with a high CSF/serum ratio. The virus can be cultured from CSF lymphocytes and proviral DNA detected by PCR.

Treatment

  • Steroids and other immunomodulatory treatment may slow progression, but this has not been studied.

Prognosis

  • In general, neurologic deficits continue to progress slowly.

Syphilis

Syphilitic meningomyelitis and meningovascular myelitis represent an earlier form of syphilis infection in which focal inflammation of the meninges secondarily affects the adjacent spinal cord thus presenting as a subacute progressive myelopathy101 and/or affects the anterior spinal artery where the clinical presentation may be one of a spinal cord infarction. Tabes dorsalis is a form of tertiary neurosyphilis in which the dorsal or posterior columns of the spinal cord are primarily affected.

Clinical Presentation

  • Sensory ataxia and lancinating pains reflecting dorsal column and dorsal nerve root involvement.

Diagnosis

  • CSF examination may be normal or demonstrate elevated protein level, lymphocytosis, and/or a reactive VDRL.

Treatment

  • Antibiotic treatment may reverse symptoms.

Tuberculosis

Tuberculosis can produce a myelopathy by different mechanisms. Infection of the vertebral body leads to tuberculous spondylitis or Pott's disease, which can lead to secondary cord compression. Tuberculomas within the intramedullary, intradural, and extradural space can also produce myelopathy. 102,103

Clinical Presentation

  • Back pain over the affected vertebra
  • Low-grade fever
  • Weight loss

Parasite Infection

The parasites Schistosoma mansoni and Schistosoma haematobium typically infect the spinal cord. The lower thoracic region of the spinal cord is most frequently involved, followed by the lumbar and sacral regions. Spinal cord involvement can lead to permanent paralysis. Cysticercosis (i.e., infection caused by eggs of the pork tapeworm) has been reported to cause a cyst within the spinal cord.104

Clinical Presentation

  • Rapidly progressing symptoms of transverse myelitis, including:
    • Lower limb pain
    • Weakness
    • Bowel and bladder dysfunction105,106

Diagnosis

  • CSF evaluation reveals pleocytosis and elevated protein. Eosinophilia occurs in almost half of patients.
  • MRI demonstrates signal change and swelling within the cord.

Treatment

  • Glucocorticoids
  • Praziquantel

Prognosis

  • At least partial recovery.

Others

Bacterial meningitis may be complicated by a myelopathy due to formation of an epidural abscess, myelitis, or vasculitic infarction.107-109
Lyme disease rarely affects the spinal cord. However, cases have been described in which clinical and MRI features resembling acute transverse myelitis have been attributed to Lyme disease. 110,111 CSF in these cases typically demonstrates a lymphocytic pleocytosis and elevated protein.

Vascular Diseases

Spinal Cord Infarction

Infarction of the spinal cord is rare compared with cerebral infarction.

Causes of spinal cord infarction include

  • Surgical procedures
  • Pathologies affecting the aorta112
  • Etiologies that also produces brain infarction (e.g., atherosclerosis, embolism, and hypercoagulable and vasculitic disorders).112-115
  • Severe systemic hypotension or cardiac arrest

Clinical Presentation

Clinical Presentation is consistent with the functional loss within the anterior spinal artery territory and include:

  • Sudden onset of symptoms
  • Paralysis
  • Loss of bladder function
  • Loss of pain and temperature sensation below the level of the lesion 
  • Position and vibratory sensation are spared
  • Frequently associated with back pain

Diagnosis

  • MRI will demonstrate a T2-signal change consistent with cord ischemia but may be normal in the first 24 hours.116,117 Diffusion-weighted imaging (DWI) is more sensitive.118-120 
  • CSF protein level may be elevated, but pleocytosis is rare.

Treatment

  • Treatment is generally supportive and focused on the underlying aortic pathology and/or secondary stroke prevention.

Prognosis

Less than half of patients show substantial motor recovery following spinal cord infarction.112,121

Vascular Malformations

Vascular malformations of the spinal cord are classified into types according to their location and vascular pathology. 122,123

Dural arteriovenous fistula

Dural arteriovenous fistula is the most common type, making up about 70% of all lesions.124 These exist on the dural surface, and drain intradurally by retrograde flow through a single medullary vein to the anterior or posterior median vein, resulting in engorgement of the coronal venous plexus.

  • Risk Factors:
    • 50 years of age or older
    • More common in men than women
  • Clinical Presentation:
    • Progressive, often step-wise myeloradiculopathy, probably related to venous hypertension.
    • Some patients present with neurogenic claudication.125
    • Symptoms may initially fluctuate, but eventually a permanent and progressive paraparesis with sensory disturbances and sphincter dysfunction occur.
  • Diagnosis:
    • MRI with contrast-enhanced MR angiography (MRA) can identify a dural arteriovenous fistula but has imperfect sensitivity.125-130 The most common finding is a nonspecific hyperintense signal lesion on T2-weighted images. The more specific findings of intradural flow voids on T2-weighted images, and/or serpentine enhancement on MRA and T1 images, are seen in 85 to 100% or more of patients.
    • If MRI/MRA cannot be performed, myelography with supine and prone images may demonstrate the serpentine vessels within the intradural space.
    • When positive, MRA and/or myelography can help guide the performance of the spinal angiogram, which remains the gold standard test, and is required prior to therapeutic intervention. Spinal angiography can be a difficult diagnostic procedure in this setting, often requiring multiple injections of segmental arteries in order to identify the feeding artery, especially when MRA does not provide specific guiding information. However, at least one observational study found that in experienced hands, the complication rate is low (1 to 2%) and did not (at least in this series) involve any neurologic morbidity.131
  • Treatment:
    • Occlusion of the fistula by surgery or endovascular embolization can be helpful in stabilizing, even ameliorating, the neurologic deficits.129,132-134

Intramedullary spinal arteriovenous malformations

Intramedullary spinal arteriovenous malformations (AVMs)135,136 are supplied by medullary arteries and drain through medullary veins.

  • Risk Factors:
    • The mean age at clinical presentation is the mid-20s, but close to 20% of the lesions are diagnosed in children under 16 years of age.135
  • Clinical Presentation:
    • A myelopathy is produced by the mass effect of the lesion or by ischemia or hemorrhage into the cord.
    • Some patients present instead with subarachnoid hemorrhage.
  • Diagnosis:
    • MRI is sensitive for intramedullary AVM, showing a cluster of low-intensity signal foci.123
    • Contrast-enhanced MRA also helps localize the nidus and identify arterial supply and venous drainage.
  • Treatment:
    • These lesions are treated by endovascular occlusion, surgical resection, or both.137

Spinal Epidural Hematoma

Spinal epidural hematoma can complicate procedures that involve a spinal dural puncture, usually in patients with thrombocytopenia or bleeding diathesis, including anticoagulant therapy.138 This rarely occurs spontaneously.

Risk Factors

  • A predisposition to bleeding139-141
  • Some occur in the setting of minor trauma

Clinical Presentation

  • Patients typically present with local and/or radicular pain, followed by loss of sensory, motor, and bladder and bowel function.139-141
  • The source of bleeding is usually venous rather than arterial, and symptoms typically present over days.
    • More abrupt presentations have also occurred.

Diagnosis

  • MRI is a sensitive imaging modality for these lesions.140,142 MRI findings vary according to the age of the clot. In the first 24 hours the hematoma is usually isointense on T1- and hyperintense on T2-weighted images. After 24 hours, it becomes mostly hyperintense on T1 and T2.

Treatment

  • For patients with significant and/or progressing neurologic deficits prompt surgical intervention, usually a laminectomy, and evacuation of the blood.
    • Timely decompression of the hematoma is essential to avoid permanent loss of neurologic function.143,144
  • Many individuals with minor, stable neurologic deficits can be managed by observation and have a good prognosis for complete recovery.139,141

Toxic, Metabolic Disorders

Subacute Combined Degeneration

Deficiency in vitamin B12 (cyanocobalamin) leads to degeneration of the dorsal and lateral white matter of the spinal cord.

Clinical Presentation

  • Slowly progressive weakness, sensory ataxia, paresthesias and, ultimately, spasticity, paraplegia, and incontinence.145
  • Not all patients with neurologic abnormalities will have anemia or macrocytosis.146 

Nitrous oxide abuse can also lead to subacute combined degeneration, by inactivation of vitamin B12.148-151

Treatment

  • Supplemental treatment with vitamin B12 can stop progression and will produce neurologic improvement in most patients.147

Copper Deficiency Myeloneuropathy

A syndrome similar to the subacute combined degeneration of vitamin B12 deficiency can occur with acquired copper deficiency.

Risk Factors

Result of gastrointestinal surgery
Excessive zinc ingestion (e.g., overuse of denture cream).152-154

Clinical Presentation

  • As above under subacute combined degeneration
  • Hematologic abnormalities

Treatment

Treatment can prevent progression

Prognosis

  • Patients with significant neurologic deficits at presentation often remain disabled.

Radiation Myelopathy

Myelopathy is a serious complication of radiation therapy to the spinal cord.155,156 White matter tracts in the lateral aspects of the cord are preferentially affected.157,158

Risk Factors159

  • A high radiation dose
  • Fractionation size
  • Concomitant chemotherapy

Clinical Presentation

Two distinct Clinical Presentations are described:

  • A transient myelopathy typically occurs two to six months after irradiation. It is usually mild and resolves spontaneously over several months.
  • A late progressive myelopathy begins 6 to 12 months after irradiation. Presentation begins insidiously and generally progresses inexorably, although some cases stabilize.

Diagnosis

  • MRI is important to exclude other etiologies and will typically show hyperintensity on T2 and FLAIR sequences.

Treatment

  • There is no effective treatment.

Electrical Injury

High voltage electrical injury can be associated with a variety of neurologic complications, including spinal cord injury. Direct heat and electrical injury to neural elements, as well as, a delayed microvascular disease are proposed mechanisms.

  • Different syndromes have been described:
    • A transient flaccid paralysis, called keraunoparalysis, is apparent immediately following the electrical injury.
      • Clinical Presentation:160,161
        • Affects the legs more than the arms
        • Typically resolves within the first 24 hours
        • Often associated with lightning strike
        • Peripheral vasoconstriction apparent
        • Sensory disturbances
  • Other patients with electrical injury develop a more enduring and sometimes permanent spinal cord injury.162-165
    • Clinical Presentation:
      • Clinical signs of myelopathy may be present at the time of injury or may develop after several days or weeks.
      • Motor deficits are more prominent than sensory findings.
      • Bladder and other sphincter dysfunction can occur.
    • Diagnosis:
      • Spine MRI is typically normal.
    • Treatment:
      • Supportive in nature.

Hepatic Myelopathy

Progressive myelopathy is a rare neurologic complication of chronic liver disease with portal hypertension.166-169 Neuropathological studies have demonstrated demyelination of the lateral corticospinal tracts with various degrees of axonal loss.168

Clinical Presentation

  • The myelopathy is predominantly or entirely motor in manifestation, reflected as a spastic paraparesis that progresses over months to paraplegia.170
  • Deficits are limited to the lower extremities.
  • Sensory and bladder function are often unaffected

Diagnosis

  • In contrast to hepatic encephalopathy, ammonia-lowering treatments have little or no effect on the myelopathy.

Treatment

  • In contrast to hepatic encephalopathy, ammonia-lowering treatments have little or no effect on the myelopathy.

Prognosis

  • Patients with early manifestations of spinal cord impairment may improve with liver transplantation.170

Decompression Sickness Myelopathy

Impairment of spinal cord function can be a manifestation of decompression sickness, a complication of deep sea diving.171-175 Gaseous occlusion of venous plexus within the spinal cord is one postulated mechanism of injury.

Clinical Presentation

  • Symptoms usually develop during or immediately after ascent but may be delayed for several hours or a few days.
  • The thoracic cord is most commonly involved, producing paraparesis of varying severity and a sensory level in the mid or low thoracic region.
  • Lesions at higher spinal cord levels produce quadriplegia.174

Diagnosis

  • Both MRI and pathologic studies have shown multifocal white matter degeneration in the posterior and lateral columns of the spinal cord with secondary ascending and descending tract degeneration.169,174

Treatment

  • Early therapeutic recompression frequently reverses symptoms and signs.172

Prognosis

  • Residual corticospinal and minor sensory signs may remain for months or indefinitely.175

Lathyrism and Konzo

Two disorders of spastic paraparesis have been described, which occur in association with increased dietary intake of food plants with neurotoxic potential, as occurs in certain geographic regions during times of famine.176,177 Pathologic studies have demonstrated a loss of myelinated fibers in the corticospinal and spinocerebellar tracts. The toxin appears to be the neuroexcitatory amino acid, beta-N-oxalylaminoalanine.

Neurolathyrism

Neurolathyrism is associated with prolonged consumption of the grass or chickling pea (Lathyrus sativus).178

Clinical Presentation

  • Exposed individuals develop a slowly developing spastic paraparesis with cramps, paresthesias, and numbness, accompanied by bladder symptoms and impotence.
  • Some patients have tremors and other involuntary movements in their arms.

Treatment

  • There is no treatment

Konzo

Konzo is a disorder characterized by acute spastic paraparesis or quadriparesis. It is linked to high exposure to cyanogenic compounds in diets containing insufficiently processed bitter cassava (Manihot esculenta).179,180 This disorder is less well characterized than lathyrism, and it may reflect a disorder of intracranial rather than intraspinal motor pathways.

Neoplasm

Both benign and malignant tumors can produce a myelopathy as a result of external compression or intramedullary growth.

Extradural spinal cord compression

The most common syndrome is that of extradural spinal cord compression, as produced by metastases to the extradural space.

Clinical Presentation

  • Progressive weakness below the level of the lesion with accompanying sensory loss and bladder dysfunction.181
  • Typically there is pain at the site of involvement.
  • Progression to paraplegia can occur abruptly, as a result of vascular compression.

Diagnosis

  • Gadolinium-enhanced spinal MRI182

Treatment

  • High-dose corticosteroids with radiation therapy and/or surgical decompression

Prognosis

  • The prognosis for neurologic recovery depends on the severity of the deficit at the time of intervention.

Intramedullary spinal cord tumors

Intramedullary spinal cord tumors are typically primary CNS tumors (ependymoma, astrocytoma). Metastases are less likely.183,184

Clinical Presentation

  • These produce a progressive myelopathy, often with central cord features.

Diagnosis

  • MRI with gadolinium will show the tumor.185
  • Biopsy with histologic examination is usually required for diagnosis.

Treatment

  • Because of their intramedullary location, management of these lesions is difficult. 

Myelopathy can also occur as a complication of radiation therapy, as a complication of intrathecal chemotherapy, particularly methotrexate and cytarabine and as a paraneoplastic syndrome.

Inherited and Degenerative Conditions

Amyotrophic Lateral Sclerosis (ALS)

ALS is a neurodegenerative disorder that produces progressive weakness, usually with mixed upper and motor neuron signs.186,187

Risk Factors

  • Begins insidiously in older adults (usually >60 years) and progresses steadily.

Clinical Presentation

  • Typically, there is asymmetric limb weakness with a mixture of upper and lower motor neuron features.
  • Sensory and sphincter disturbances are usually absent.

Diagnosis

  • MRI is normal.
  • Electromyography typically shows denervation in clinically affected muscles.

Unusual variants of ALS

Unusual variants of ALS with atypical symptoms can present a diagnostic challenge. Primary lateral sclerosis is a rare variant of ALS with primarily upper motor neuron features.188

Clinical Presentation

  • Muscle stiffness leading to overt and progressive spasticity without associated muscle weakness or atrophy typifies the disorder.
  • In about two-thirds of patients, there is an ascending pattern with spasticity spreading in a rather stereotyped fashion from the legs to the arms and finally to involve the bulbar musculature.189

Diagnosis

  • without positive test findings, this is a diagnosis of exclusion.

Treatment

  • There is no treatment.

Familial form of ALS

Clinical Presentation

  • A long pre-paretic phase of lower extremity discomfort, followed by a slowly ascending asymmetric paresis that is often accompanied by bladder symptoms.190-193

Hereditary Spastic Paraplegias (HSP)

HSP is a large group of inherited neurologic disorders, in which the prominent feature is a progressive spastic paraparesis.194 HSP is classified according to the mode of inheritance, the genetic locus when known, and whether the spastic paraplegia syndrome occurs alone or is accompanied by additional neurologic or systemic abnormalities ("pure" versus "complicated").195-199 There have been no recent epidemiological studies, but previously the incidence has been reported to be between 1 in 10,000 and 1 in 100,000.

Genetically diverse, with at least 28 genetic loci for HSP identified, the final common pathway for these disorders is a degeneration of the corticospinal tracts.195 Inheritance is usually autosomal dominant, but recessive and X-linked variants (e.g., Pelizaeus-Merzbacher disease) are known.

Clinical Presentation for the typical patient with pure HSP195

  • HSP is a clinical diagnosis, based in large part on the family history.
  • MRI may be normal or may show atrophy in the spinal cord.
  • Thinning of the corpus callosum on brain MRI is seen in half of patients with the autosomal recessive form of HSP.200
  • The diagnosis is based on careful exclusion of other etiologies.

Diagnosis

  • HSP is a clinical diagnosis, based in large part on the family history.
  • MRI may be normal or may show atrophy in the spinal cord.
  • Thinning of the corpus callosum on brain MRI is seen in half of patients with the autosomal recessive form of HSP.200
  • The diagnosis is based on careful exclusion of other etiologies.

Treatment

  • Symptomatic management195

Adrenoleukodystrophy

Adrenomyeloneuropathy, a variant of Adrenoleukodystrophy, an x-linked recessive disorder.

Risk Factors

  • Generally presents in adult men
  • Female carriers of the mutation

Clinical Presentation

  • A slowly progressive spastic paraparesis and mild polyneuropathy.201,202
  • Sensory and sphincter disturbances are typically absent.
  • There may be mild adrenal insufficiency

Diagnosis

  • MRI is typically normal.
  • In the absence of a family history, the finding of a neuropathy on electrophysiologic testing may be a clue to the disorder.
  • The diagnosis is made by the finding of increased very long chain fatty acids in plasma, red blood cells, or cultured skin fibroblasts.

Friedreich Ataxia

Friedreich ataxia is an autosomal recessive degenerative disorder of uncertain pathogenesis that typically presents in adolescence.203 The neuropathologic changes in Friedreich ataxia include degeneration of the posterior columns and the spinocerebellar tracts of the spinal cord and loss of the larger sensory cells of the dorsal root ganglia.

Clinical Presentation

  • Progressive ataxia of all four limbs and gait
  • Weakness
  • Absent reflexes with extensor plantar responses
  • Loss of position and vibration sense
  • Pain and temperature are intact
  • Cardiomyopathy and diabetes mellitus are part of the syndrome
  • Patients with late-onset disease are more likely to have retained reflexes, spasticity, and no cardiomyopathy.204

Diagnosis

  • MRI may show atrophy of the cervical cord.

Treatment

  • There is no treatment.

Prognosis

  • Disease severity and rate of progression are highly variable.

Others

Syringomyelia

Syringomyelia is a fluid-filled, gliosis-lined cavity within the spinal cord. Most lesions are between C2 and T9. They can, however, descend further down or extend upward into the brainstem (syringobulbia). A syrinx can represent a focal dilation of the central canal, or it may lie separately, within the spinal cord parenchyma.205

Causation

  • Syringomyelia most commonly occurs in the setting of the Chiari malformation Type 1.206
  • Congenital malformations (e.g., Klippel-Feil syndrome, and tethered spinal cord)
  • Post infectious
  • Post inflammatory (transverse myelitis and multiple sclerosis)
  • Spinal neoplasms (especially ependymoma and hemangioblastoma)
  • Posttraumatic205-209

Clinical Presentation

  • A syrinx can be asymptomatic and discovered incidentally on spinal cord imaging.
  • Other patients present with progressive central cord deficits that can include a prominent central pain syndrome in a segmental distribution.210

Diagnosis

  • MRI will typically identify the intramedullary cavity.
  • Gadolinium administration will increase the sensitivity of finding an associated tumor.

Treatment

  • Surgical decompression with fenestration and/or shunt placement is recommended for patients with neurologic deterioration or intractable central pain.208-210

Prognosis

  • Neurologic deficits usually stabilize after intervention and sometimes improve.

Cervical Spondylotic Myelopathy

In many case series, cervical spondylotic myelopathy is the most common cause of myelopathy, particularly in older adults.4 Degenerative changes in the vertebral bodies, discs, and connecting ligaments encroach on the cervical canal, producing a progressive myelopathy.211

Clinical Presentation

  • The diagnosis is made by correlating clinical features with evidence of cervical spondylosis and cord compression on MRI.212

Diagnosis

  • The diagnosis is made by correlating clinical features with evidence of cervical spondylosis and cord compression on MRI.212

Treatment

  • Cervical immobilization
  • Surgical decompression (when and if to operate remains controversial)

Ossification of the Posterior Longitudinal Ligament (OPLL)

OPLL is a condition of abnormal calcification of the posterior longitudinal ligament, usually in the cervical spine.213-218 Its pathogenesis is not known.

Risk Factors

  • More common in Asians than non-Asians (2.4 versus 0.16%)
  • Male/female ratio 2:1
  • Idiopathic skeletal hyperostosis
  • Ankylosing spondylitis
  • Spondyloarthropathies

Clinical Presentation

  • Typical presentation is in the fifth to sixth decades of life with progressive myelopathic symptoms, but they are also at risk for and can present with acute spinal cord injury.219

Diagnosis

  • It is diagnosed by characteristic findings on CT or MRI.

Treatment

  • Surgical decompression for significant compressive symptoms

Surfers' Myelopathy

A syndrome of acute low back pain followed by progressive lower extremity numbness and weakness without apparent trauma has been described in at least 26 patients while surfing.220-225

Risk Factors

  • Generally young (ages 15 to 46 years)
  • Mostly male
  • Often surfing for the first time

Clinical Presentation

  • Acute low back pain
  • Progressive lower extremity numbness and weakness

Diagnosis

  • MRI shows restricted diffusion in the lower thoracic spinal cord to the conus medullaris.
  • The pathogenesis is unclear. However, the MRI findings along with reports of individuals lying prone on the surfboard with possible lumbar hyperextension for prolonged periods of time, suggest that vascular compression may play a role.

Treatment

  • Supportive in nature.

Prognosis

  • The severity of the neurologic deficits and degree of recovery is variable.

Conclusion

Disorders that affect the spinal cord often target specific structural and functional anatomic regions, producing distinct clinical syndromes. The spinal cord syndromes are summarized in the table (Table 1 above). The clinical syndrome along with other features in the examination and history usually significantly limits the differential diagnosis and tailors the diagnostic work-up (Table 2 above).

Pathologies that affect the spinal cord are diverse. In addition to trauma, common nontraumatic etiologies of myelopathy include inflammatory diseases, infections, vascular diseases, toxic-metabolic disorders, inherited-degenerative conditions, neoplasms and a plethora of others. The neurologic examination will characterize the deficits as to the spinal cord syndrome. The spinal cord syndrome along with the clinical setting and course of presentation with the findings of a neuroimaging study (usually magnetic resonance imaging) will identify the likely pathogenesis in most cases.

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This course is applicable for the following professions:

Advanced Registered Nurse Practitioner (ARNP), Athletic Trainer (AT/AL), Certified Registered Nurse Anesthetist (CRNA), Clinical Nurse Specialist (CNS), Licensed Practical Nurse (LPN), Licensed Vocational Nurses (LVN), Occupational Therapist (OT), Occupational Therapist Assistant (OTA), Physical Therapist (PT), Physical Therapist Assistant (PTA), Registered Nurse (RN)

Topics:

CPD: Practice Effectively, Medical Surgical, Neurology


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