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 MRI of the spinal cord classify transverse myelitis (TM) into acute partial or longitudinally extensive variants.
Confirmed by LP demonstrating increased WBC count and absence of infection, with or without spinal cord MRI revealing a cord lesion that enhances after gadolinium administration.
In the setting of a characteristic brain MRI, the acute partial variant suggests high future risk of developing multiple sclerosis.
In the setting of neuromyelitis optica-IgG autoantibody seropositivity, the longitudinally extensive variant suggests a neuromyelitis optica spectrum disorder.
Therapy for acute symptoms includes intravenous corticosteroids or plasmapheresis.
Preventive therapies include immunomodulatory drugs for acute partial TM at high risk for multiple sclerosis. Immunosuppressive therapies are used for longitudinally extensive TM at high risk for recurrent myelitis or neuromyelitis optica.
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. MRI 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 associated with multiple sclerosis, whereas longitudinally extensive lesions are associated with neuromyelitis optica spectrum disorders.
History and exam
- 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
- paroxysmal tonic spasms
- upper motor neuron signs: hyperreflexia, positive Babinski sign, limb spasticity
- sensory loss/sensory level
- dyspnea/respiratory distress
- MRI spinal cord
- MRI brain
- CSF cell count, cell differential, protein level, IgG index, oligoclonal bands
- CSF Gram stain, cultures (bacterial, tubercular, fungal), and India ink smear
- CSF PCR for HSV-1, HSV-2, VZV, Borrelia burgdorferi (Lyme disease), CMV, EBV, and West Nile virus
- CSF VDRL
- CSF cytology
- serum and CSF angiotensin-converting enzyme
- chest x-ray
- CT body (chest, abdomen, and pelvis)
- whole-body PET scan
- serology for HSV-1, HSV-2, VZV, CMV, EBV, West Nile virus
- HIV antibodies
- serum ANA, double-stranded DNA
- extractable nuclear antigen (including SSA and SSB)
- visual evoked potential
- therapeutic trial with corticosteroid
- spinal cord biopsy
- serum aquaporin-4 autoantibodies
Professor of Neurology
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 monograph.
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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