Transverse myelitis

Last reviewed: 28 Dec 2022

Last updated: 22 Sep 2020

Transverse myelitis (TM) is a heterogeneous focal inflammatory disorder of the spinal cord characterized by acute or subacute development of motor weakness, sensory impairment, and autonomic dysfunction.

TM 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 TM into acute partial or longitudinally extensive variants.

The presence of TM is 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.

Definition

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][2] 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][4][5][2]

History and exam

Key diagnostic factors

age 10-19 or 30-39 years
motor weakness
paresthesias or sensory loss
bladder symptoms: urinary frequency, urgency, incontinence, or retention
bowel symptoms: incontinence or constipation
L'hermitte sign
McArdle sign
paroxysmal tonic spasms
upper motor neuron signs: hyperreflexia, positive Babinski sign, limb spasticity
sensory loss/sensory level
dyspnea/respiratory distress

More key diagnostic factors

Other diagnostic factors

back pain
trunk/limb pain
areflexia/hyporeflexia
hiccups
nausea/vomiting

Other diagnostic factors

Risk factors

preceding infectious illness
recent vaccination
female sex
history of recent physical trauma
spinal injection

More risk factors

Diagnostic investigations

1st investigations to order

MRI spinal cord
MRI brain
serum aquaporin-4 autoantibodies and myelin oligodendrocyte glycoprotein autoantibodies
cerebrospinal fluid cell count, cell differential, protein level, IgG index, oligoclonal bands
cerebrospinal fluid Gram stain, cultures (bacterial, tubercular, fungal), and India ink smear
cerebrospinal fluid, polymerase chain reaction for herpes simplex virus (HSV)-1, HSV-2, varicella zoster virus (VZV), Borrelia burgdorferi (Lyme disease), cytomegalovirus (CMV), Epstein-Barr virus (EBV), and West Nile virus
cerebrospinal fluid Venereal Disease Research Laboratory test
serum anti-nuclear antibody, double-stranded DNA
extractable nuclear antigen (including SSA and SSB)
serum and cerebrospinal fluid paraneoplastic auto-antibodies
other neural autoantibodies

More 1st investigations to order

Investigations to consider

serum and cerebrospinal fluid angiotensin-converting enzyme
chest x-ray
CT body (chest, abdomen, and pelvis)
whole-body PET scan
cerebrospinal fluid cytology and flow cytometry
serology for herpes simplex virus (HSV)-1, HSV-2, varicella zoster virus, cytomegalovirus, Epstein-Barr virus, and West Nile virus
urinalysis
HIV antibodies
visual-evoked potential
optical coherence tomography
therapeutic trial with corticosteroid
spinal cord biopsy

More investigations to consider

Treatment algorithm

ACUTE

acute neurologic deficits

ONGOING

idiopathic TM

at risk for multiple sclerosis (typical demyelinating lesions on MRI)

aquaporin-4 autoantibody seropositive

myelin oligodendrocyte glycoprotein-IgG autoantibody seropositive

Contributors

Authors

Cristina Valencia-Sanchez, MD, PhD

Department of Neurology

Mayo Clinic

Scottsdale

Disclosures

CVS declares that she has no competing interests.

Acknowledgements

Dr Cristina Valencia-Sanchez would like to gratefully acknowledge Dr Dean Wingerchuk, the previous contributor to this topic. DMW has received compensation from MedImmune for service on a clinical trial adjudication committee, from Caladrius for consulting services, and research support paid to Mayo Clinic from Alexion and TerumoBCT. DMW is an author of a number of references cited in this topic.

Peer reviewers

Alireza Minagar, MD

Assistant Professor of Neurology

LSU Health Sciences Center

Shreveport

Disclosures

AM declares that he has no competing interests.

Cory Toth, BSc, MD, FRCP(C)

Assistant Professor of Neurosciences

Hotchkiss Brain Institute

University of Calgary

Alberta

Canada

Disclosures

CT declares that he has no competing interests.

Abhijit Chaudhuri, DM, MD, PhD, FACP, FRCP

Consultant Neurologist

Clinical Director of Neurosciences

Department of Neurology

Queen's Hospital

Romford

Disclosures

AC declares that he has no competing interests.

Differentials
- Compressive myelopathy
- Infectious myelitis (e.g., TB)
- Anterior spinal artery occlusion
More Differentials
Guidelines
- Practice guideline: disease modifying therapies in adults with multiple sclerosis
- ACR appropriateness criteria: myelopathy
More Guidelines
Patient leaflets
Multiple sclerosis
More Patient leaflets
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Transverse myelitis

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Investigations to consider

Treatment algorithm

acute neurologic deficits

idiopathic TM

at risk for multiple sclerosis (typical demyelinating lesions on MRI)

aquaporin-4 autoantibody seropositive

myelin oligodendrocyte glycoprotein-IgG autoantibody seropositive

Contributors

Authors

Cristina Valencia-Sanchez, MD, PhD

Disclosures

Acknowledgements

Peer reviewers

Alireza Minagar, MD

Disclosures

Cory Toth, BSc, MD, FRCP(C)

Disclosures

Abhijit Chaudhuri, DM, MD, PhD, FACP, FRCP

Disclosures

Differentials

Guidelines

Patient leaflets

Log in or subscribe to access all of BMJ Best Practice

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