Last reviewed: 5 Nov 2021
Last updated: 22 Sep 2020



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



Cristina Valencia-Sanchez, MD, PhD

Department of Neurology

Mayo Clinic




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.

Peer reviewers

Alireza Minagar, MD

Assistant Professor of Neurology

LSU Health Sciences Center




AM declares that he has no competing interests.

Cory Toth, BSc, MD, FRCP(C)

Assistant Professor of Neurosciences

Hotchkiss Brain Institute

University of Calgary




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




AC declares that he has no competing interests.

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