Chapter 17 - Diagnosis and management of spinal cord emergencies

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Abstract

Most spinal cord injury is seen with trauma. Nontraumatic spinal cord emergencies are discussed in this chapter. These myelopathies are rare but potentially devastating neurologic disorders. In some situations prior comorbidity (e.g., advanced cancer) provides a clue, but in others (e.g., autoimmune myelopathies) it may come with little warning. Neurologic examination helps distinguish spinal cord emergencies from peripheral nervous system emergencies (e.g., Guillain–Barré), although some features overlap. Neurologic deficits are often severe and may quickly become irreversible, highlighting the importance of early diagnosis and treatment. Emergent magnetic resonance imaging (MRI) of the entire spine is the imaging modality of choice for nontraumatic spinal cord emergencies and helps differentiate extramedullary compressive causes (e.g., epidural abscess, metastatic compression, epidural hematoma) from intramedullary etiologies (e.g., transverse myelitis, infectious myelitis, or spinal cord infarct). The MRI characteristics may give a clue to the diagnosis (e.g., flow voids dorsal to the cord in dural arteriovenous fistula). However, additional investigations (e.g., aquaporin-4-IgG) are often necessary to diagnose intramedullary etiologies and guide treatment. Emergency decompressive surgery is necessary for many extramedullary compressive causes, either alone or in combination with other treatments (e.g., radiation) and preoperative neurologic deficit is the best predictor of outcome.

Introduction

Myelopathies are rare but potentially devastating neurologic disorders. In some situations prior comorbidity (e.g., advanced cancer) provides a clue but in others (e.g., autoimmune myelopathies) it may come with little warning. Most acute spinal cord injuries in the neurosciences unit are traumatic and are discussed in Chapter 15 of this volume.

In this chapter we will discuss the rare acute myelopathies which will need immediate medical or neurosurgical management. Many patients present with puzzling symptomatology and may quickly worsen in the intensive care unit, even requiring mechanical ventilation when cervical segments get involved.

Section snippets

Epidemiology

Spinal cord emergencies are rare but devastating neurologic disorders with a variety of causes. Age, sex, and race are important factors to take into account when considering the likely cause of the spinal cord emergency. Spinal epidural abscesses have an incidence of 0.88/100 000, are commonest in those aged 50–70 and males predominate (Ptaszynski et al., 2007, Pradilla et al., 2009). Autopsy studies report approximately 5% of cancer patients have epidural spinal cord compression (Barron et

Neuropathology

Knowledge of the etiologies of extrinsic compression of the spinal cord requires an understanding of spinal anatomy. The spinal cord is encased within the thecal sac, bordered anteriorly by the vertebral body and intervertebral discs (nucleus pulposus and annulus fibrosis), posteriorly by the posterior spinal processes, and laterally by the pedicles and lamina. Between the bony thecal sac and outer layer of the cord (the dura) lies the epidural space, which contains fat and venous plexuses. The

Clinical presentation

It is essential that one consider the setting when evaluating spinal cord emergencies, as there will often be clues to guide the clinician to the correct diagnosis. For example, a patient with widely metastatic lung cancer presenting with acute paraplegia is likely to have epidural metastatic spinal cord compression; an anticoagulated patient with rapidly progressive paraplegia may have an epidural hematoma; fever, back pain, and neurologic deficits raise the possibility of spinal epidural

Extramedullary lesions

Magnetic resonance imaging (MRI) of the entire spine is the imaging modality of choice for acute nontraumatic spinal cord emergencies. Its excellent visualization of soft tissues and the intramedullary spinal cord and its safety in those with a coagulopathy make it preferable to computed tomography (CT) myelogram, which also risks introduction of infection to the subarachnoid space in lumbosacral spinal epidural abscess. Imaging of the entire spine is important as patients with metastatic or

Respiratory failure and spinal shock

In cases of quadriparesis assessing and protecting the airway are the first steps. While respiratory failure is a rare complication of spinal cord injury, initial assessment of the need for intubation and mechanical ventilation should not be overlooked, particularly in high cervical myelopathies.

Indications for intubation are pooling secretions, periods of deoxygenation, and need for a facemask to correct hypoxemia. In some patients hypercarbia emerges quickly and can only be recognized with a

Outcome prediction

The best predictor of neurologic outcome for cord compression from epidural compression is the neurologic status at the time of diagnosis (further emphasizing the importance of early diagnosis). The prognosis for metastatic epidural spinal cord compression is poor, with a median survival of between 3 and 6 months. The risk of mortality with spinal epidural abscess ranges from 6 to 32%, while with spinal cord infarct it is 10–25%. Despite the severity of initial disability with spinal cord

Neurorehabilitation

Early involvement of physical and occupational therapy is important for patients with spinal cord disorders to help manage complications of neurogenic bowel and bladder, gait impairment, and spasticity. Many of these patients require antispasticity drugs (e.g., baclofen) and prolonged inpatient rehabilitation may be helpful for recovery in many such patients.

Disclosures

Dr. Flanagan has no disclosures to report.

Dr. Pittock is a named inventor on patents (#12/678,350 filed 2010 and #12/573,942 filed 2008) that relate to functional AQP4/NMO-IgG assays and NMO-IgG as a cancer marker, and receives research support from Alexion Pharmaceuticals, the Guthy-Jackson Charitable Foundation, and the National Institutes of Health (NS065829). Dr. Pittock has provided consultation to Alexion Pharmaceuticals, MedImmune, and Chugai Pharma, but has received no personal fees or

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