Transverse myelitis

Heterogeneous focal inflammatory disorder of the spinal cord characterized by acute or subacute development of motor weakness, sensory impairment, and autonomic dysfunction.

Causes motor weakness, a sensory impairment below the lesions, and bowel and bladder dysfunction.

Clinical characteristics and magnetic resonance imaging (MRI) of the spinal cord classify transverse myelitis (TM) into acute partial or longitudinally extensive variants.

Confirmed by lumbar puncture demonstrating increased white blood cell count and absence of infection, with or without spinal cord MRI revealing a cord lesion that enhances after gadolinium administration.

For people with a characteristic brain MRI, the acute partial variant suggests a high future risk of developing multiple sclerosis (MS).

For people with aquaporin-4-IgG autoantibody seropositivity, the longitudinally extensive variant suggests a neuromyelitis optica spectrum disorder.

Therapy for acute symptoms includes intravenous corticosteroids or plasma exchange (plasmapheresis).

Preventive therapies include immunomodulatory drugs for acute partial TM in patients at high risk for MS.

Immunosuppressive therapies are used for longitudinally extensive TM in people at high risk for recurrent myelitis or neuromyelitis optica spectrum disorder.

Transverse myelitis (TM) is a pathogenetically heterogeneous focal inflammatory disorder of the spinal cord characterized by acute or subacute development of motor weakness, sensory impairment, and autonomic dysfunction.[1]Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002 Aug 27;59(4):499-505. http://www.ncbi.nlm.nih.gov/pubmed/12236201?tool=bestpractice.com [2]Beh SC, Greenberg BM, Frohman T, et al. Transverse myelitis. Neurol Clin. 2013 Feb;31(1):79-138. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7132741/ http://www.ncbi.nlm.nih.gov/pubmed/23186897?tool=bestpractice.com Magnetic resonance imaging of the spinal cord reveals a focal hyperintense lesion and cerebrospinal fluid usually shows pleocytosis.

The causes of TM are heterogeneous, but partial TM (asymmetric, short cord lesions) is typically associated with multiple sclerosis, whereas longitudinally extensive lesions suggest neuromyelitis optica spectrum disorder.[3]Miller D, Barkhof F, Montalban X, et al. Clinically isolated syndromes suggestive of multiple sclerosis, part I: natural history, pathogenesis, diagnosis, and prognosis. Lancet Neurol. 2005 May;4(5):281-8. http://www.ncbi.nlm.nih.gov/pubmed/15847841?tool=bestpractice.com [4]Wingerchuk DM, Hogancamp WF, O'Brien PC, et al. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology. 1999 Sep 22;53(5):1107-14. http://www.ncbi.nlm.nih.gov/pubmed/10496275?tool=bestpractice.com [5]Weinshenker BG, Wingerchuk DM, Vukusic S, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006 Mar;59(3):566-9. http://www.ncbi.nlm.nih.gov/pubmed/16453327?tool=bestpractice.com [2]Beh SC, Greenberg BM, Frohman T, et al. Transverse myelitis. Neurol Clin. 2013 Feb;31(1):79-138. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7132741/ http://www.ncbi.nlm.nih.gov/pubmed/23186897?tool=bestpractice.com