Transverse Myelitis
Transverse Myelitis: Excerpt from The 5-Minute Pediatric Consult
Yang Mao-Draayer, MD, PhD
Transverse Myelitis - BASICS
Transverse Myelitis - description
Demyelination in the spinal cord causing acute or subacute loss of motor, sensory, and autonomic function, often preceded by midback pain; may occur after infection or vaccination, or be associated with multiple sclerosis (MS). The term acute transverse myelitis (ATM) implies an inflammatory disease of the spinal cord; acute transverse myelopathy is a broader term that refers to any process that suddenly impairs spinal cord function.
Transverse Myelitis - epidemiology
No gender predilection
Transverse Myelitis - incidence
1–4/1,000,000 per year, affecting all ages with peaks between the ages of 10 and 19 years and between 30 and 39 years
Transverse Myelitis - pathophysiology
A presumed autoimmune-mediated inflammation and demyelination of the spinal cord. Postinfectious etiology predominates in children.
Transverse Myelitis - associated conditions
- Most commonly associated with infection
- May be part of demyelinating disorders:
- May either occur as 1st episode of MS or in the setting of that disease
- Occurs with demyelination of optic nerves (i.e., optic neuritis), also called Devic disease or neuromyelitis optica (NMO)
- Monophasic demyelinating disease: Acute disseminated encephalomyelitis (ADEM): Multiple demyelinating lesions in the brain as well as in the spinal cord
- Systemic inflammatory diseases (e.g., systemic lupus erythematosus, primary antiphospholipid syndrome, juvenile rheumatoid arthritis, sarcoidosis, connective tissue disease, vasculitis) may present with acute or recurrent transverse myelitis.
- ATM is rarely seen in association with metabolic causes of myelopathy (e.g., vitamin BDoes not usually occur in association with inherited demyelinating diseases (e.g., adrenomyeloneuropathy/leukodystrophy, Pelizaeus–Merzbacher, globoid cell leukodystrophy, and metachromatic leukodystrophy); these usually present with chronic myelopathy.
Transverse Myelitis - DIAGNOSIS
Transverse Myelitis - signs & symptoms
Transverse Myelitis - history
- Most prominent is neurologic dysfunction consistent with a spinal cord injury at a specific level. Bilateral, but not necessarily symmetric, symptoms are usually present.
- ATM may be suggested by history of back pain, sensory level, and urinary/fecal incontinence or retention. The patient often has lower extremity weakness or inability to bear weight, possibly with decreased spontaneous use of hands.
- Details of the temporal course of the symptoms are important, because sudden onset of weakness raises the possibility of acute structural or vascular causes of myelopathy. In ATM, onset of spinal cord dysfunction usually progress in 4 hours to 21 days, the patient’s signs usually plateau and evolve toward spasticity/hyperreflexia.
- ATM is often preceded by respiratory illness, vaccination, or systemic illness.
- Determine history of infection or systemic inflammatory disease.
- Other important findings in history include vascular/ischemia, neoplasia, MS, radiation exposure, trauma, or immunodeficiency, to rule out other causes of acute myelopathy.
Transverse Myelitis - physical exam
- Extreme irritability: Extent of weakness may be assessed by how vigorously the child resists examination.
- Fever, hypertension, tachycardia, meningeal signs may be present; in such cases, CNS infection needs to be ruled out; point tenderness over the spine may suggest trauma or infection.
- Neurologic: Check visual acuity and color vision; funduscopic examination for optic nerve head pallor (optic neuritis)
- Increased tone: Spastic weakness is usually symmetric, legs more than arms
- Reflexes are usually brisk, with positive Babinski sign.
- A sensory level (a partial level is commonly seen) that may spare joint position and vibration may be present.
- Urgent complication: Sphincter dysfunction, urinary retention or incontinence; check for loss of anal wink, bladder dilatation, and large postvoid residual urine (>100 mL)
Transverse Myelitis - tests
Transverse Myelitis - lab
The 1st priority in acute myelopathy is to rule out structural cause: Compressive myelopathy:
- Gadolinium-enhanced MRI of spine (above the level that could explain weakness or sensory deficit) will exclude other/structural causes of myelopathy and can be diagnostic for transverse myelitis.
- The 2nd priority is to define the presence/absence of spinal cord inflammation and to rule out other CNS infection.
- Lumbar puncture is usually done after imaging; often shows normal or slightly increased protein levels and mild pleocytosis with lymphocyte predominance. Elevation of IgG index and presence of oligoclonal bands suggest MS or other systemic inflammatory disease. Results of CSF Gram stain; bacterial, viral, and fungal culture; Venereal Disease Research Laboratories test; Lyme antibodies; and polymerase chain reaction of specific viruses should all be negative in ATM.
- 3rd priority is to define extent of demyelination. Gadolinium-enhanced MRI of the brain and the orbit and evoked potential studies (e.g., visual evoked potential, somatosensory evoked potential) may identify other sites.
- Investigation for underlying systemic inflammatory disorder including ESR and ANA is appropriate. Studies for granulomatous disease or spirochetal infection: Purified protein derivative/anergy panel, serum angiotensin-converting enzyme, elevated in sarcoidosis, rapid protein reagin, Lyme titer. For underlying metabolic disorder: Very-long-chain fatty acid (VLCFA; looking for subacute combined degeneration).
- Viruses associated with ATM include the herpes viruses (Epstein–Barr virus, varicella-zoster virus, herpes simplex virus), cytomegalovirus, mumps; rubella; influenza; hepatitis A, B, and C; and HIV. Positive IgM or >4-fold increase in IgG levels on 2 successive tests to a specific infectious agent suggests diagnosis of parainfectious ATM.
Diagnosis requires evidence of inflammation of the spinal cord. MRI and cerebrospinal fluid analysis are often key in patients with suspected ATM. Enhancing spinal cord lesion or pleocytosis or IgG index is required for the diagnosis. If both test results are negative, consider repeating tests in 2–7 days.
Transverse Myelitis - differencial diagnosis
- Presentation of ATM in a toddler may resemble osteomyelitis, arthritis, toxic synovitis, or even a sudden abdominal problem: Extreme irritability, unwillingness to bear weight
- Extremity weakness seen in ATM may resemble an acute neuromuscular disorder (e.g., Guillain–Barré syndrome or polymyositis), in which cases the definitive myelopathic signs (e.g., spastic tone, hyperreflexia, upturned toes, sensory level) are not present. Guillain–Barré syndrome is frequently confused with ATM, particularly when the latter does not show typical upper motor neuron findings; normal MRI and the albuminocytologic dissociation (high-protein, low cell count) in cerebrospinal fluid from patients with Guillain–Barré syndrome helps distinguish the 2 conditions.
- Must exclude other causes of myelopathy that require different treatment. For example, urgent neurosurgical consultation should be obtained if imaging reveals a surgically remediable cause of myelopathy (e.g., epidural abscess, tumor, arteriovenous malformation); emergency basis radiation, and/or high-dose IV corticosteroid therapy for neoplastic cord compression.
- ATM may be the presenting feature of MS, especially in patients with partial ATM and abnormal initial brain MRI; in such cases, consider follow-up MRIs.
- NMO (or Devic disease) is a rare (even rarer in children than adults) and aggressive demyelinating disease. When children present with recurrent transverse myelitis with an elongated (greater than three vertebral segments) intramedullary spinal cord lesion, serum NMO antibody testing should be obtained to facilitate the diagnosis and treatment.
- Compressive myelopathies:
- Vertebral osteomyelitis/discitis
- Intrinsic or extrinsic tumor
- Spine trauma
- Epidural abscess
- Infectious causes of myelopathy:
- Poliomyelitis: Concern about underlying immunodeficiency
- Lyme disease: In children with possible exposure, serology is nonspecific but sensitive unless antibiotics given early in the course, which ablates immune response.
- Syphilis: Usually chronic, tertiary form (tabes dorsalis), although meningovascular lues may cause acute myelopathy.
- Vascular: Cord ischemia (postcardiac surgery), cord arteriovenous malformation
Transverse Myelitis - TREATMENT
Transverse Myelitis - initial stabilization
Unlike acute polyneuropathy (i.e., Guillain–Barré syndrome), ATM rarely causes respiratory insufficiency unless patients have cervical lesions. In such cases, an intensive care setting to anticipate respiratory failure or autonomic instability, mechanical ventilation, cautious use of antihypertensive agents may be necessary.
Transverse Myelitis - special therapy
Symptomatic management: Anticipate urinary retention to prevent perforated bladder. Autonomic deficits may require bowel/bladder regimen, catheterization, prophylactic antibiotics, and stool softeners.
Transverse Myelitis - phys therapy
Physical and occupational therapy may help promote functional recovery and prevent contractures.
Transverse Myelitis - medication
- IV methylprednisolone may be useful in ATM or other acute demyelinating diseases based on a few observational studies. IV immunoglobulin or plasmapheresis may be a safe and effective therapeutic alternative in patients who do not respond to or are intolerant of IV methylprednisolone.
- Cyclophosphamide has been reported to be useful in myelitis associated with systemic inflammatory diseases.
Transverse Myelitis - FOLLOW UP
Transverse Myelitis - prognosis
- 1/3 of patients with ATM recover completely; symptoms generally resolve (gradually) in 3–6 months; 1/3 of patients are left with moderate disability, and 1/3 suffer severe disability. Residual neurologic deficits include fixed weakness and sensory or autonomic deficits. Sphincter dysfunction improves more slowly than do other deficits. Treatment is largely based on symptoms, and long-term physical or occupational therapy may be beneficial.
- Prognostic factors: Older age, increased deep tendon reflexes, and positive Babinski sign may indicate better course. Rapid progression, back pain, and spinal shock predict poor recovery.
Transverse Myelitis - bibliography
- Defresne P, Hollenberg H, Husson B, et al. Acute transverse myelitis in children: Clinical course and prognostic factors. J Child Neurol. 2003;18:401–406.
- Defresne P, Meyer L, Tardieu M, et al. Efficacy of high dose steroid therapy in children with severe acute transverse myelitis. J Neurol Neurosurg Psychiatry. 2001;71:272–274.
- Miyazawa R, Ikeuchi Y, Tomomasa T, et al. Determinants of prognosis of acute transverse myelitis in children. Pediatr Int. 2003;45:512–516.
Patient information on multiple sclerosis. Available at: http://www.nmss.org.- Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002;59:499–505.
- Weinshenker BG, O’Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999;46:878–886.
Transverse Myelitis - CODES
Transverse Myelitis - icd9
323.9 Transverse myelitis
Transverse Myelitis - FAQ
- Q: What features of transverse myelitis could lead you to diagnose MS?
- A: History indicating other, resolved, neurologic symptoms (e.g., internuclear ophthalmoplegia, optic neuritis, focal weakness, and numbness that lasted at least 24 hours to several days), other lesions on brain/spine MRI at the time of presentation, or subsequent new MRI lesions
- Q: What is the usual clinical course of ATM?
- A: The course of ATM in children proceeds through three stages:
Initial motor loss precedes sphincter dysfunction in most patients; there is often a sensory loss following dermatomal levels, usually over 2–3 days
Plateau: Mean duration of plateau is 1 week.
Recovery phase - Q: What causes the pain and irritability commonly seen in children with transverse myelitis?
- A: Pain in transverse myelitis may be due to neuropathic pain from nerve root inflammation, nociceptive pain from dural inflammation, muscle spasm from motor dyscontrol, bladder distention from dysautonomia, psychologic distress from loss of motor control, or dysesthesia from demyelination of spinothalamic tract.
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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