Article thumbnail

Idiopathic Transverse Myelitis and Neuromyelitis Optica: Clinical Profiles, Pathophysiology and Therapeutic Choices

By Amer Awad and Olaf Stüve


Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins

Topics: Article
Publisher: Bentham Science Publishers
OAI identifier:
Provided by: PubMed Central

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.

Suggested articles


  1. (2001). [Devics neuromyelitis optica. Disease or variants of multiple sclerosis?].
  2. (2009). [Trial of rituximab in three patients with neuromyelitis optica]. Rinsho Shinkeigaku,
  3. (2000). 14-3-3 protein in the cerebrospinal fluid of patients with acute transverse myelitis. Lancet,
  4. (2010). A case of relapsing neuromyelitis optica treated with glatiramer acetate.
  5. (2000). A novel feature disclosed in opticospinal multiple sclerosis in Asians.
  6. (1999). A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease.
  7. (2002). A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica.
  8. (2004). A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet,
  9. (1998). A subgroup of multiple sclerosis patients with anticardiolipin antibodies and unusual clinical manifestations: do they represent a new nosological entity?
  10. (2001). Aboriginals with multiple sclerosis: HLA types and predominance of neuromyelitis optica. Neurology,
  11. (2007). Absence of aquaporin-4 expression in lesions of neuromyelitis optica but increased expression in multiple sclerosis lesions and normal-appearing white matter. Acta Neuropathol.,
  12. (2004). Activation of humoral immunity and eosinophils in neuromyelitis optica. Neurology,
  13. (1990). Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis.
  14. (1998). Acute myelitis with hyperIgEaemia and mite antigen specific IgE: atopic myelitis.
  15. (2001). Acute myelopathies: Clinical, laboratory and outcome profiles in 79 cases.
  16. (2007). Acute transverse myelitis in childhood: centerbased analysis of 47 cases. Neurology,
  17. (1998). Acute transverse myelitis in childhood: nine cases and review of the literature.
  18. (1973). Acute transverse myelopathy in adults. A follow-up study.
  19. (1986). Acute transverse myelopathy in childhood.
  20. Acute Transverse Myelopathy.
  21. (2005). An open label study of the effects of rituximab in neuromyelitis optica. Neurology,
  22. (2001). Anti-DNA antibodies are a major component of the intrathecal B cell response in multiple sclerosis.
  23. (2003). Aquaporin water channels: molecular mechanisms for human diseases.
  24. (2005). Association between systemic lupus erythematosus, rheumatoid arthritis, hyperprolactinemia and thyroid autoantibodies.
  25. (2009). Association of anti-Helicobacter pylori neutrophil-activating protein antibody response with anti-aquaporin-4 autoimmunity in Japanese patients with multiple sclerosis and neuromyelitis optica.
  26. (2008). Autoantibodies in inflammatory demyelinating diseases of the central nervous system. Swiss Med. Wkly.,
  27. (2002). Autoimmunity due to molecular mimicry as a cause of neurological disease.
  28. B cell therapy for rheumatoid arthritis: the rituximab (anti-CD20) experience.
  29. (1989). beta-specific stimulation of human T cells by staphylococcal toxins.
  30. (1998). Can electromyography predict the prognosis of transverse myelitis?
  31. (1973). Central nervous system disease associated with Mycoplasma pneumoniae infection: report of five cases and review of the literature. Pediatrics,
  32. (1996). Chronic recurrent transverse myelitis or multiple sclerosis.
  33. (2006). Clinical and MRI features of Japanese patients with multiple sclerosis positive for NMO-IgG.
  34. (2004). Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica.
  35. (1990). Clinical course and long-term prognosis of acute transverse myelopathy.
  36. (2009). Clinically isolated acute transverse myelitis: prognostic features and incidence.
  37. (1995). Cytomegalovirus-associated transverse myelitis in a non-immunocompromised patient.
  38. (1999). Cytomegalovirus-associated transverse myelitis in a nonimmunocompromised patient. Infection,
  39. (2003). Determinants of prognosis of acute transverse myelitis in children.
  40. (2000). Devic's neuromyelitis optica and HIV-1 infection.
  41. (2004). Devic's neuromyelitis optica treated with intravenous gamma globulin
  42. (1993). Devic's neuromyelitis optica: a clinicopathological study of 8 patients.
  43. (1998). Devic's neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurology,
  44. (2003). Devic's neuromyelitis optica: study of nine cases.
  45. (2009). Diagnosis of neuromyelitis spectrum disorders: comparative sensitivities and specificities of immunohistochemical and immunoprecipitation assays.
  46. (1996). Different superantigens interact with distinct sites in the Vbeta domain of a single T cell receptor.
  47. (2009). Distinctive retinal nerve fibre layer and vascular changes in neuromyelitis optica following optic neuritis.
  48. (2001). Effect of early interferon treatment on conversion to definite multiple sclerosis: a randomised study. Lancet,
  49. (2009). Effect of glatiramer acetate on conversion to clinically definite multiple sclerosis in patients with clinically isolated syndrome (PreCISe study): a randomised, double-blind, placebo-controlled trial. Lancet,
  50. (2008). Effects of erythropoietin on reducing brain damage and improving functional outcome after traumatic brain injury in mice.
  51. (2001). Efficacy of high dose steroid therapy in children with severe acute transverse myelitis.
  52. (2000). Familial neuromyelitis optica (Devic's syndrome) with late onset in Japan. Neurology,
  53. (2006). Fulminant Devic disease successfully treated by lymphocytapheresis.
  54. (2002). Functional activation of myelin-specific T cells by virus-induced molecular mimicry.
  55. (2006). Global brain atrophy after unilateral parietal lesion and its prevention by erythropoietin.
  56. (2005). Gluten sensitivity and neuromyelitis optica: two case reports.
  57. (2002). Growth factor treatment of demyelinating disease: at last, a leap into the light. Trends Immunol.,
  58. (1997). High dose methylprednisolone in severe acute transverse myelopathy.
  59. HLA-DRB association in neuromyelitis optica is different from that observed in multiple sclerosis.
  60. (2011). HLADPB1*0501-associated opticospinal multiple sclerosis: clinical,
  61. (2000). Hyperprolactinemia in opticospinal multiple sclerosis.
  62. (2003). Idiopathic recurrent transverse myelitis.
  63. (2007). Idiopathic transverse myelitis: corticosteroids, plasma exchange, or cyclophosphamide. Neurology,
  64. (2005). IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel.
  65. (2005). IL-6 induces regionally selective spinal cord injury in patients with the neuroinflammatory disorder transverse myelitis.
  66. (1997). Immune deviation following pulse cyclophosphamide/ methylprednisolone treatment of multiple sclerosis: increased interleukin-4 production and associated eosinophilia.
  67. (2008). Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica.
  68. (2002). Immunopathogenesis of acute transverse myelitis.
  69. (2006). Impact of IVIg on the interaction between activated T cells and microglia.
  70. (2000). Importance of paraclinical and CSF studies in the diagnosis of MS in patients presenting with partial cervical transverse myelopathy and negative
  71. (2009). In vitro comparison of the complementscavenging capacity of different intravenous immunoglobulin preparations. Vox Sang,
  72. (1996). Interferon beta-1b effects on cytokine mRNA in peripheral mononuclear cells in multiple sclerosis.
  73. (2007). Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination.
  74. (1997). Interleukin-6 (IL-6)--a molecule with both beneficial and destructive potentials.
  75. (2005). Intractable hiccup and nausea with periaqueductal lesions in neuromyelitis optica. Neurology,
  76. (2000). Intramuscular interferon beta-1a therapy initiated during a first demyelinating event in multiple sclerosis.
  77. (2006). Intravenous immunoglobulin ameliorates ITP via activating Fc gamma receptors on dendritic cells.
  78. (2003). Is Devic's neuromyelitis optica a separate disease? A comparative study with multiple sclerosis.
  79. (2001). Is methyl prednisolone useful in acute transverse myelitis? Spinal Cord.,
  80. (1992). Long-term follow-up of acute partial transverse myelopathy. Neurology,
  81. (2006). Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report.
  82. (1998). Magnetic resonance imaging findings in 22 cases of myelitis: comparison between patients with and without multiple sclerosis.
  83. (1987). Magnetic resonance imaging in isolated noncompressive spinal cord syndromes.
  84. (2004). Magnetiza- tion transfer and diffusion tensor MRI show gray matter damage in neuromyelitis optica. Neurology,
  85. Mechanism of action of mitoxantrone.
  86. (1995). Mechanism of action of mycophenolate mofetil.
  87. (2009). Mechanisms involved in synergistic anticancer effects of anti4-1BB and cyclophosphamide therapy.
  88. (2003). Mechanisms of action of intravenous immunoglobulin in autoimmune and inflammatory diseases.
  89. (2008). Mechanisms of disease: aquaporin4 antibodies in neuromyelitis optica.
  90. MR imaging of Devic's neuromyelitis optica.
  91. (2001). MS and neuromyelitis optica in Martinique (French West Indies). Neurology,
  92. (2003). Multiple sclerosis in the Japanese population. Lancet Neurol.,
  93. (1973). Necrotizing myelitis: a clinico-pathologic report of two cases associated with diplococcus pneumoniae and mycoplasma pneumoniae infections.
  94. (1984). Necrotizing myelopathy associated with malignancy. A clinicopathologic study of two cases and literature review. Cancer,
  95. Neuromyelitis optica (Devic disease) following varicella infection.
  96. (1994). Neuromyelitis optica (Devic's disease) following chicken pox.
  97. (2008). Neuromyelitis optica (Devic's disease) in a patient with syphilis.
  98. (1990). Neuromyelitis optica (Devic's syndrome) and pulmonary tuberculosis. Neurology,
  99. (2006). Neuromyelitis optica (Devic's syndrome) as first manifestation of systemic lupus erythematosus. Lupus,
  100. (2006). Neuromyelitis optica associated with myasthenia gravis: characteristic phenotype in Japanese population.
  101. (2006). Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression.
  102. (1975). Neuromyelitis optica following infectious mononucleosis.
  103. (2006). Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis.
  104. (2007). Neuromyelitis optica in a mother and daughter.
  105. (2006). Neuromyelitis optica in patients with myasthenia gravis who underwent thymectomy.
  106. (1968). Neuromyelitis optica versus subacute necrotic myelitis. II. Anatomical study of two cases.
  107. (1957). Neuromyelitis optica with increased intracranial pressure.
  108. (1984). Neuromyelitis optica with unusual vascular changes.
  109. (2003). Neuromyelitis optica: clinical predictors of a relapsing course and survival. Neurology,
  110. (2004). Neuromyelitis optica: current concepts.
  111. (1985). Neuromyelitis optica: two new cases and review of the literature.
  112. (2003). Neuromyelitis optica: what it is and what it might be. Lancet,
  113. (2000). Neurophysiological studies in acute transverse myelitis.
  114. (2008). NMO-IgG predicts the outcome of recurrent optic neuritis. Neurology,
  115. (2009). NMO-IgGnegative relapsing myelitis. Spinal Cord,
  116. (2004). Oligoclonal bands in Devic's neuromyelitis optica and multiple sclerosis: differences in repeated cerebrospinal fluid examinations.
  117. (2009). Optical coherence tomography helps differentiate neuromyelitis optica and MS optic neuropathies. Neurology,
  118. (2008). Optical coherence tomography in neuromyelitis optica.
  119. (2009). Optical coherence tomography is less sensitive than visual evoked potentials in optic neuritis. Neurology,
  120. (2004). Opticospinal multiple sclerosis: a pathogenetically distinct form?
  121. (2007). Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis.
  122. (1996). Pediatric Devic's neuromyelitis optica.
  123. (2002). Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology,
  124. (2001). Plasma exchange for severe attacks of inflammatory demyelinating diseases of the central nervous system.
  125. (2004). Plasma exchange for severe optic neuritis: treatment of 10 patients. Neurology,
  126. (1980). Possible beneficial effect of high-dose intravenous steroid therapy in acute demyelinating disease and transverse myelitis. Neurology,
  127. (1950). Post-infectious encephalomyelitis and multiple sclerosis; the significance of perivenous encephalomyelitis.
  128. (1998). Prognostic predictors of acute transverse myelitis.
  129. (2000). Progressive necrotic myelopathy: clinical course in 9 patients.
  130. (2002). Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology,
  131. (1998). Rapid recovery from transverse myelopathy in children treated with methylprednisolone.
  132. (1988). Rapid recovery of acute transverse myelitis treated with steroids.
  133. (2005). Reappraisal of Lhermitte's sign in multiple sclerosis.
  134. (1996). Recurrent myelitis.
  135. (1997). Recurrent optic neuromyelitis with endocrinopathies: a new syndrome. Neurology,
  136. (2004). Recurrent transverse myelitis associates with anti-Ro (SSA) autoantibodies. Neurology,
  137. (2000). Recurrent transverse myelitis with unusual long-standing
  138. (1991). Relapsing transverse myelitis. Neurology,
  139. (2009). Review: Cyclophosphamide in multiple sclerosis: scientific rationale, history and novel treatment paradigms.
  140. (2006). Revised diagnostic criteria for neuromyelitis optica. Neurology,
  141. (2005). Safety and efficacy of rituximab in patients with rheumatoid arthritis refractory to disease modifying antirheumatic drugs and anti-tumor necrosis factor-alpha
  142. (1997). Specialized membrane domains for water transport in glial cells: high-resolution immunogold cytochemistry of aquaporin-4 in rat
  143. (2008). Spectrum of pediatric neuromyelitis optica. Pediatrics,
  144. (2001). Spinal cord lesions of myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis) manifest eosinophilic inflammation.
  145. (2006). Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease).
  146. (1991). Subacute necrotizing myelopathy: MR imaging in four pathologically proved cases.
  147. (1993). Superantigens and their potential role in human disease.
  148. (1995). Superantigens of mouse mammary tumor virus. Annu Rev. Immunol.,
  149. (1994). T-cell activation by superantigens.
  150. (2005). The anti-inflammatory action of methotrexate is not mediated by lymphocyte apoptosis, but by the suppression of activation and adhesion molecules.
  151. (2007). The antiinflammatory activity of IgG: the intravenous IgG paradox.
  152. (1999). The clinical course of neuromyelitis optica (Devic's syndrome). Neurology,
  153. (1989). The early risk of multiple sclerosis following isolated acute syndromes of the brainstem and spinal
  154. (2003). The effects of intramuscular interferon beta-Ia in patients at high risk for development of multiple sclerosis: a post hoc analysis of data from CHAMPS.
  155. (1993). The George Hitchings and Gertrude Elion Lecture. The pharmacology of azathioprine.
  156. (1971). The pattern of neurological illness in tropical Africa. Experience at Ibadan,
  157. (1978). The prognosis of acute and subacute transverse myelopathy based on early signs and symptoms.
  158. (1992). The role of magnetic resonance imaging in acute transverse myelitis.
  159. (1993). The significance of brain magnetic resonance imaging abnormalities at presentation with clinically isolated syndromes suggestive of multiple sclerosis. A 5-year follow-up study.
  160. (2007). The spectrum of neuromyelitis optica. Lancet Neurol.,
  161. (1983). Thyroid autoimmunity in hyperprolactinaemic disorders. Acta Endocrinol. (Copenh),
  162. (2009). TNF-alpha and IL-10 downregula- tion and marked oxidative stress in Neuromyelitis Optica.
  163. (2000). Transverse myelitis associated with probable cat-scratch disease in a previously healthy pediatric patient.
  164. (2004). Transverse Myelitis: pathogenesis, diagnosis and treatment.
  165. (1998). Transverse myelitis. Comparison with spinal cord presentations of multiple sclerosis. Neurology,
  166. (1993). Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events.
  167. (1953). Transverse myelopathy in childhood.
  168. (2009). Treatment of neuromyelitis optica with mycophenolate mofetil: retrospective analysis of 24 patients.
  169. (2008). Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients.
  170. (1996). Western versus Asian types of multiple sclerosis: immunogenetically and clinically distinct disorders.