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.
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. 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
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
Other diagnostic factors
- back pain
- trunk/limb pain
- preceding infectious illness
- recent vaccination
- female sex
- history of recent physical trauma
- spinal injection
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
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
- HIV antibodies
- visual-evoked potential
- optical coherence tomography
- therapeutic trial with corticosteroid
- spinal cord biopsy
acute neurologic deficits
at risk for multiple sclerosis (typical demyelinating lesions on MRI)
aquaporin-4 autoantibody seropositive
myelin oligodendrocyte glycoprotein-IgG autoantibody seropositive
- Compressive myelopathy
- Infectious myelitis (e.g., TB)
- Anterior spinal artery occlusion
- Practice guideline: disease modifying therapies in adults with multiple sclerosis
- ACR appropriateness criteria: myelopathy
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