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transverse myelitis

Etiology: 1) viral infections a) smallpox b) measles c) chickenpox (varicella) d) enteroviruses e) influenza 2) mycoplasma 3) spinal cord injuries or ischemia 4) immune reactions a) optic neuromyelitis b) multiple sclerosis 5) idiopathic Epidemiology: 1) no gender preference 2) peak incidence(s) 10-19 & 30-39 years of age 3) 1400 new cases/year in USA Pathology: 1) inflammation of both gray matter & white matter of the spinal cord 2) involves entire thickness of the spinal cord 3) limited longitudinal involvement 4) partial demyelinating myelitis may occur due to multiple sclerosis [2] Clinical manifestations: 1) sudden onset (over several hours-several weeks) 1) weakness to paralysis (below spinal level) 2) deep tendon reflexes (hyperreflexia), areflexia [8] 3) positive Babinski reflex 4) sensory spinal level loss (below spinal level) - as distingished from peripheral nerve distribution 5) symptoms are often asymmetric 6) low back pain 7) muscle spasms 8) malaise 9) headache 10) loss of appetite 11) paresthesias in the legs 12) autonomic dysfunction a) urinary retention, urinary incontinence b) fecal incontinence 13) mental status may be normal Laboratory: - cerebrospinal fluid analysis - increased CSF leukocytes (CSF leukocytosis) - negative Herpes simplex DNA - no CSF oligoclonal banding or elevated CSF IgG index Radiology: - magnetic resonance imaging (MRI) - MRI of the spine - contrast enhancement of the spinal cord - MRI of the brain (visualize entire CNS) - no brain lesions Complications: - multiple sclerosis (less likely than with optic neuritis) Differential diagnosis: 1) multiple sclerosis (MS) [7] - MRI lesions in the brain & CSF oligoclonal banding suggest MS 2) spinal cord ischemia, spinal cord infarction [7] 3) spinal cord compression from spondylosis or tumor 4) tropical spastic paraparesis 5) neurosarcoidosis [7] 6) neuromyelitis optica spectrum disorder 7) myelin oligodendrocyte glycoprotein autoimmunity 8) Guillain-Barre syndrome a) minimal sensory loss b) hyporeflexia or areflexia 9) arteriovenous malformation of the spine 10) embolism to the anterior spinal artery 11) cauda equina syndrome 12) anti-NMDA receptor encephalitis - markedly abnormal mental status Management: see multiple sclerosis 1) glucocorticoids - after excluding infection [2] - intravenous methylprednisolone 1 g/day for 3-7 days [2] 2) plasmapheresis or cyclophosphamide for glucocorticoid resistance [2] 3) symptomatic & supportive therapy 4) prognosis a) recovery usually begins 2-12 weeks after onset & may continue for up to 2 years b) most patients are left with disability - motor, sensory, & sphincter (bowel) deficits c) some patients show no recovery d) recurrence in a small percentage of patients

Related

multiple sclerosis (MS); includes clinically isolated syndrome

General

myelitis demyelinating disease

References

  1. Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
  2. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Greenberg BM, Thomas KP, Krishnan C et al Idiopathic transverse myelitis: corticosteroids, plasma exchange, or cyclophosphamide. Neurology. 2007 May 8;68(19):1614-7. PMID: 17485649
  4. Frohman EM, Wingerchuk DM. Clinical practice. Transverse myelitis. N Engl J Med. 2010 Aug 5;363(6):564-72 PMID: 20818891
  5. Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002 Aug 27;59(4):499-505. PMID: 12236201
  6. Scott TF, Frohman EM, De Seze J et al Evidence-based guideline: clinical evaluation and treatment of transverse myelitis: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011 Dec 13;77(24):2128-34 PMID: 22156988
  7. Zalewski NL, Flanagan EP, Keegan BM. Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses. Neurology. 2018 Jan 9;90(2):e96-e102. Epub 2017 Dec 15. PMID: 29247071
  8. NEJM Knowledge+
  9. NINDS Transverse Myelitis Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Transverse-Myelitis-Information-Page
  10. Transverse Myelitis Fact Sheet https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Transverse-Myelitis-Fact-Sheet