Summary
Definition
History and exam
Key diagnostic factors
- 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
Other diagnostic factors
- back pain
- trunk/limb pain
- areflexia/hyporeflexia
- hiccups
- nausea/vomiting
Risk factors
- preceding infectious illness
- recent vaccination
- female sex
- history of recent physical trauma
- spinal injection
Diagnostic investigations
1st investigations to order
- 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
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
Treatment algorithm
Contributors
Authors
Cristina Valencia-Sanchez, MD, PhD
Department of Neurology
Mayo Clinic
Scottsdale
AZ
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
LA
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
UK
Disclosures
AC declares that he has no competing interests.
References
Key articles
Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002 Aug 27;59(4):499-505. Abstract
Sechi E, Shosha E, Williams JP, et al. Aquaporin-4 and MOG autoantibody discovery in idiopathic transverse myelitis epidemiology. Neurology. 2019 Jul 23;93(4):e414-20. Abstract
de Seze J, Lanctin C, Lebrun C, et al. Idiopathic acute transverse myelitis: application of the recent diagnostic criteria. Neurology. 2005 Dec 27;65(12):1950-3. Abstract
Weinshenker BG, Wingerchuk DM. Neuromyelitis spectrum disorders. Mayo Clin Proc. 2017 Apr;92(4):663-79.Full text Abstract
Weinshenker BG, O'Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999 Dec;46(6):878-86. Abstract
Cortese I, Chaudhry V, So YT, et al. Evidence-based guideline update: plasmapheresis in neurologic disorders. Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011 Jan 18;76(3):294-300.Full text Abstract
Rae-Grant A, Day GS, Marrie RA, et al. Practice guideline recommendations summary: disease-modifying therapies for adults with multiple sclerosis. Neurology. 2018 Apr 24;90(17):777-88.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available here.
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