• acute transverse myelitis (ATM) is a relatively rare inflammatory disease of the spinal cord (incidence approximately 1–4 cases/million/year [Transverse Myelitis Consortium Working Group, 2002]
    • peaks at ages 10-19 and then 30-39 years
  • a pathobiologically heterogeneous syndrome characterized by acute or subacute spinal cord dysfunction with a severe prognosis – many patients end up with severe residual deficits
  • onset acute (hours) or subacute (up to 3 weeks)
  • most common causes:
    • infectious, para/post-infectious
    • demyelinating diseases (MS/CIS, NMOSD)
    • idiopathic – without any clear etiology despite a thorough investigation
  • diagnosis is often challenging in the initial phase and includes 2 steps:
    • confirmation of myelitis (differentiation from tumor, external compression, spinal ischemia/hematoma)
    • determination of the cause of myelitis


  • myelitis is associated with multiple conditions
  • spinal involvement is a common manifestation of MS, not rarely presenting as the first symptom
  • lesions typically span 1–2 segments, are asymmetrical, and often located in the dorsolateral region, particularly in the cervical and thoracic cord
  • in the acute phase, they show postcontrast enhancement
  • clinically, they usually present as incomplete spinal lesions
  • diagnosis is often supported by a positive brain MRI finding and evidence of intrathecal IgG synthesis with oligoclonal bands on isoelectric focusing
  • an autoimmune disease affecting the cells of salivary, lacrimal, and other glands (pancreas, vaginal glands, parotid gland)
  • clinically presents as dry mouth (xerostomia), swallowing disorders, bad breath, increased tooth decay, dry eyes (xerophthalmia) with burning sensations, and intolerance to sunlight
  • typically manifests in the 5-6th decade of life
  • CNS involvement occurs in 25-30% of patients; myelitis is rare (only about 1% of affected individuals) and is usually described as long, expansively behaving central T2 hyperintense lesions
  • Sjögren’s syndrome is often associated with other autoimmune diseases, such as RA, SLE, or NMO
  • CNS involvement in SLE is relatively common (up to 50%)
  • typically, it presents as aseptic meningitis or vascular involvement (vasculitis, ischemic cerebrovascular stroke)
  • myelitis is rare (2-3%)
    • many cases described as myelitis due to SLE were, in fact, SLE-associated NMO
    • NMO usually presents as LETM, often with subsequent development of optic neuritis
    • AQP4-IgG antibodies are found in the serum – test all patients with SLE who develop optic neuritis (ON) or myelitis
  • direct infectious involvement
    • history of infectious disease in the last 4 weeks combined with laboratory evidence of the infectious agent (cultural, serological, or PCR)
  • post-infectious / post-vaccination complications
  • MRI findings are non-specific, mostly long T2 hyperintense lesions with saturation after contrast agent administration

Clinical presentation

  • back pain
  • fast, usually gradual, development of motor, sensory, and autonomic dysfunction (typically within > 4 hours and < 3-4 weeks)
    • an apoplectic onset with deficits reaching the nadir in < 4 hours indicates a vascular event
    • motor and sensory deficits correspond to the level of the lesion and are usually bilateral, characterized by para- or quadriparesis/plegia
    • autonomic dysfunction is almost always present in the form of impairment of bladder, sexual, gastrointestinal, cardiovascular, and thermoregulatory functions
    • the brain is not affected in cases of idiopathic ATM
  • fever (infectious etiology)

Diagnostic evaluation

Diagnostic criteria for myelitis
(Transverse Myelitis Consortium Working Group, 2002)

The diagnostic evaluation of ATM involves a combination of clinical assessment, imaging studies, and laboratory tests, including CSF analysis. The goal is to confirm the diagnosis, identify the underlying cause, and rule out other conditions

  • typical clinical presentation – progressive bilateral (may be asymmetric) motor deficit with sensory level and development within 4 hours to 21 days
    • the 4-hour interval is proposed to distinguish acute spinal infarction with sudden onset. However, it is not a reliable diagnostic feature as inflammatory myelitis can present with very rapid onset < 4 hours, whereas ischemia in AVM can be gradual (> 4 hours) [Nohejlová, 2018]
    • the motor and sensory deficit is accompanied by autonomic dysfunction
  • lesion on MRI
    • Longitudinally Extensive Transverse Myelitis (LETM) – lesion ≥ 3 segments, usually affecting the entire cross-section of the spinal cord (> 2/3) [DeSanto, 2011]
    • Acute Partial Transverse Myelitis (APTM) with lesions < 2 segments and usually eccentric asymmetric involvement
    • the lesion is not bright on DWI
    • initially, MRI findings may be negative
  • evidence of inflammation
    • CSF: pleocytosis, increased IgG index
    • presence of enhancement on MRI after gadolinium administration (baseline imaging may show no enhancement)
    • both infectious and autoimmune spinal cord inflammation have similar clinical and laboratory manifestations
    • infectious etiology is indicated by the presence of the pathogen in CSF or a positive antibody index in serum and CSF, systemic signs of infection, and epidemiological history
  • exclusion of non-inflammatory etiology of spinal lesion (MRI, CT myelography if MRI is contraindicated)
    • extramedullary compression
    • intramedullary tumor
    • spinal ischemia (occlusion of the anterior spinal artery with typical lesion localization, spinal AV malformation, aortic dissection, etc.) – onset usually < 4 hours
  • determine the etiological diagnosis of myelitis (see table above)
    • idiopathic – extensive diagnostics do not reveal any other disease
    • secondary (infection, demyelination disease, systemic disease-related ATM, etc.)
  • T2 – hyperintense
  • T1 – isointense or hypointense
  • T1C+ – usually postcontrast enhancement is present  (diffuse, focal, peripheral) but may be absent initially
  • DWI – negative
  • the spinal cord is enlarged, lesion occupies > 2/3 of the cross-sectional area of the cord
  • assessment of the longitudinal extent of the lesion on MRI
    • longitudinally extensive transverse myelitis (LETM) – isolated lesion that extends across ≥ 3 vertebral segments, usually affecting the entire cross-section of the spinal cord
    • partial transverse myelitis (APTM) with lesions that extend across ≤ 2 segments and often with eccentric partial involvement on axial scans
  • a brain MRI should be obtained in all patients
    • look for evidence of concomitant or prior lesions that may provide clues about the etiology
    • up to 40% of patients have asymptomatic brain lesions at the time of the first ATM attack (⇒ CIS/MS)
    • the presence of MS-like brain lesions in patients with partial TM portends a high risk of transition to clinically definite MS in 3-5 years
  • idiopathic ATM may have normal baseline MRI findings in up to 40% of cases, or the lesion may not enhance after contrast administration ⇒ DDx of ischemia is challenging in the acute stage   [Scotti, 2001]
  • follow-up MRI is recommended after 2-7 days
Acute transverse myelitis on MRI
Longitudinally Extensive Transverse Myelitis (LETM)
  • spinal cord enlargement
  • various degrees of post-contrast enhancement, including its absence
  • if MRI is contraindicated, perform myelo-CT to exclude spinal cord compression
  • look for signs of infection, inflammation, or malignancy
  • typical findings in ATM include pleocytosis, increased protein concentration, and the presence of oligoclonal bands
  • CSF culture may be performed if an infectious cause is suspected
  • initially, normal CSF findings are described in up to 50% of cases; in such cases, a follow-up examination after 2-7 days is recommended
  • complete blood count, inflammatory markers (e.g., ESR, CRP)
  • serologic screening
    • serologic tests for borrelia, Mycoplasma pneumoniae, and neurotropic viruses in the serum and CSF
    • direct detection of borrelia, herpes viruses, and enteroviruses in serum and CSF using PCR
  • autoimmune panels (IgM, IgA, and IgG, C3 and C4 components of complement and circulating immune complexes, antinuclear antibodies, etc.)
  • antibodies against aquaporin 4 (AQP4-IgG) in the serum
    • highly specific biomarker for neuromyelitis optica (NMO), especially in patients with longitudinally extensive transverse myelitis
  • testing for vitamin deficiencies (e.g., vitamin B12) and toxicology screens
  • screening for paraneoplastic syndromes if a malignancy is suspected
  • somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) to assess the functional integrity of sensory and motor pathways

Differential diagnosis of myelitis

  • spinal cord infarction (ischemia in the anterior spinal artery territory, AVM, aortic dissection)  Spinal AVM on MRI
    • older patients (> 50 years) are more likely to suffer spinal cord infarction
    • usually sudden onset (may not apply to AVM)
    • in the early phase, cerebrospinal fluid findings are negative; post-contrast enhancement of the lesion is usually absent (appears more in the subacute phase)
    • the spinal cord is enlarged, the lesion is hyperintense on T2 and DWI (restricted diffusion) Acute spinal infarct on DWI
    • multiple segments are affected, with the maximum occurrence in the upper thoracic and thoracolumbar segments
    • on T2, hyperintensity may also be visible in the vertebral body (infarction) 
  • hematomyelia  
  • spinal tumor
    • MRI shows mass lesion with or without enhancement
    • slowly progressive clinical course
  • external compression (hematoma, meningioma, neurinoma, etc.)  Epidural hematoma
  • vitamin B12 deficiency
    • subacute combined degeneration of the spinal cord
    • macrocytic anemia, elevated methylmalonic acid, and homocysteine levels
    • MRI may show hyperintensity in the posterior columns
  • toxic myelopathy
    • exposure to toxic substances (e.g., radiation, chemotherapy agents, illicit drugs)
    • history of toxin exposure
    • MRI may show diffuse spinal cord changes
  • degenerative spinal conditions
    • spinal stenosis or herniated disc with cord compression
    • gradual progression or fluctuation of symptoms
    • MRI showing structural abnormalities


Immunomodulatory therapy

  • high-dose IV corticosteroids
    • e.g., 1 g/day for 3-5 days followed by oral tapering
    • it has not been demonstrated that the administration of corticosteroids worsens the clinical outcomes in patients with spinal cord ischemia or infection
  • if corticosteroids fail, escalation of therapy proceeds with
  • plasmapheresis:
    • considered for patients not responding to corticosteroids
    • typically 5-7 exchanges
  • Intravenous Immunoglobulin (IVIG):
    • dose: 0.4 g/kg/day for 5 days (total dose 2g/kg)
    • used in refractory cases or when plasmapheresis is not available
  • disease-specific Treatments:
    • MS/CIS-related myelitis: Disease-modifying therapies (e.g., interferons, glatiramer acetate, natalizumab)
    • NMOSD: Immunosuppressive agents (e.g., azathioprine, rituximab, mycophenolate mofetil)
    • autoimmune myelitis: depending on the underlying condition, agents such as cyclophosphamide, methotrexate, or monoclonal antibodies
      • CPA dose: usually 500 – 1000 mg/ m2 IV

Specific, causal therapy

For example:

  • neuroborreliosis – ceftriaxone (LENDACIN) 2 g/day intravenously for 14–21 days
  • herpetic infection – acyclovir (HERPESIN)

General therapy

  • rehabilitation
  • prevention of complications (decubitus, contractures, secondary infections)
  • management of spasticity
  • urinary tract care


  • in more than 60% of cases, significant residual spinal symptoms persist, or no improvement occurs
  • better prognosis can be expected:
    • in younger individuals with a subacute onset and early clinical improvement
    • in myelitis due to MS/CIS, NMOSD, or neuroborreliosis that responds well to therapy
  • worse prognosis is associated with acute onset of myelitis (hours/days) with severe initial neurological impairment, incontinence, lesion detected in the cervical cord, and idiopathic etiology

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Acute transverse myelitis