Heterogeneous focal inflammatory disorder of the spinal cord characterised 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 auto-antibody 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 characterised by acute or subacute development of motor weakness, sensory impairment, and autonomic dysfunction. 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.
History and exam
- presence of risk factors
- age 10-19 or 30-39 years
- motor weakness
- paraesthesias or sensory loss
- bladder symptoms: urinary frequency, urgency, incontinence, or retention
- bowel symptoms: incontinence or constipation
- L'hermitte sign
- McArdle's sign
- paroxysmal tonic spasms
- upper motor neuron signs: hyper-reflexia, positive Babinski's sign, limb spasticity
- sensory loss/sensory level
- dyspnoea/respiratory distress
- MRI spinal cord
- MRI brain
- serum aquaporin-4 auto-antibodies and myelin oligodendrocyte glycoprotein auto-antibodies
- 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 auto-antibodies
- 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
- HIV antibodies
- visual-evoked potential
- optical coherence tomography
- therapeutic trial with corticosteroid
- spinal cord biopsy
Department of Neurology
CVS declares that she has no competing interests.
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.
Assistant Professor of Neurology
LSU Health Sciences Center
AM declares that he has no competing interests.
Assistant Professor of Neurosciences
Hotchkiss Brain Institute
University of Calgary
CT declares that he has no competing interests.
Clinical Director of Neurosciences
Department of Neurology
AC declares that he has no competing interests.
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