Transverse Myelitis Overview



Focal inflammatory disorder of the spinal cord resulting in rapid onset of weakness, sensory alterations, and bowel or bladder dysfunction



Epidemiology:

 • 1400 new cases in the US each year

 • Bimodal peaks between the ages of 10 to 19 years and 30 to 39 years

 • 10-33% develop MS over a five to ten-year period

 • 75-90% of cases TM is monophasic



Pathophysiology of Transverse Myelitis:

 • Excessive activation of an immune response against the spinal cord that results in CNS inflammation and tissue damage

Mechanisms:

 • Idiopathic: etiology unknown

 • Infectious: Direct microbial infection versus molecular mimicry or super-antigen mediated disease

 • Immune Mediated



Transverse Myelitis - Clinical Presentation:

Injury to the spinal cord (Myelopathy) without cord compression (MRI) and findings of varying degrees of:

1. Weakness:

	- Weakness that preferentially affects the flexors of the legs and the extensors of the arms (pyramidal distribution of weakness)

	- Flaccidity -> Spasticity

	- Reflexes: decreased or absent initially -> Hyperreflexia develops later

2. Sensory Alterations:

	- Sensory features: paresthesias ascending from feet with or without back pain at/near level of myelitis

3. Autonomic Dysfunction:

	- Autonomic: Bowel/Bladder dysfunction, temperature dysregulation, bouts of HTN



Diagnostic Criteria:

 • Sensory, motor, or autonomic dysfunction located at the spinal cord

 • T2 hyperintense signal change on spinal MRI

 • No evidence of compressive cord lesion

 • Bilateral signs and/or symptoms

 • Clearly defined sensory level

 • CSF inflammation: CSF pleocytosis, elevated IgG index

 • Progression hours to days



Transverse Myelitis Diagnosis:

 • Must rule out compressive myelopathies!

 • Gadolinium-enhanced MRI of the spinal cord

	- Increased T2 signal and expansion of the cord

	- No cord compression

	- More than 2/3 of the cross-sectional area is involved

	- Focal enlargement

	- T2WI hyperintensity

	- Enhancement

	- TM and tumor the cord is swollen, while in MS and ADEM the cord is not swollen or less swollen

	- Lesion > 3 levels: Evaluate for NMO, SLE or Sjogren's

 • CSF:

	- Abnormal in 50% of cases

	- Pleocytosis: Mild lymphocytic

	- Pleocytosis > 100 cells: consider infectious myelitis

	- Elevated IgG index

	- Oligoclonal IgG bands (85 to 95% Predictive of MS)

 • Check Vitamin B12 and copper levels

 • NMO-IgG (anti-AQP4) should be tested and if negative, MOG-IgG test should be ordered

 • Infectious causes need to be ruled out

 • Brain MRI: Check for brain and/or optic nerve lesions suggestive of multiple sclerosis, NMOSD, MOG antibody disorders, or ADEM



Transverse Myelitis Etiologies:

 • Demyelination:

	- Multiple sclerosis (MS)

	- Neuromyelitis optica (NMO)

	- Idiopathic transverse myelitis

	- ADEM

	- Post vaccination

	- Myelin oligodendrocyte glycoprotein (MOG) antibody disorders

 • Infections:

	- Herpes zoster, West Nile virus

	- Herpes simplex virus, HIV, Hep A/B/CMV, TB

	- Treponema Pallidum

	- Lyme Disease Mycoplasma

	- Leptospirosis, Brucellosis

	- Dengue, EBV, Influenza, Enteroviruses

 • Idiopathic:

	- Post Infectious

	- Post Vaccine

 • Inflammatory Disorders:

	- SLE

	- Neurosarcoidosis

	- MCTD

	- Bechet disease

	- Sjogren

	- Vasculitis- Heroin

 • Paraneoplastic:

	- AntiGAD65, NMDAR, AntiCRMP IgG, Anti-Hu

	- Antiamphiphysin antibodies

 • Nutritional Deficiency:

	- Vitamin B12, E, D, copper



Transverse Myelitis Treatment:

 • Steroids

 • Plasma exchange

 • Immunomodulatory agents: IVIG

 • Cyclophosphamide



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