Last reviewed: November 2017
Last updated: September  2017

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
  • 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 gender
  • hx of recent physical trauma
  • spinal injection

Diagnostic investigations

1st investigations to order

  • 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
Full details

Investigations to consider

  • 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
  • urinalysis
  • 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 auto-antibodies
Full details

Emerging tests

  • CSF interleukin-6 (IL-6)
  • CSF 14-3-3 protein
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Professor of Neurology

Mayo Clinic

Scottsdale

AZ

Disclosures

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.

Peer reviewers VIEW ALL

Assistant Professor of Neurology

LSU Health Sciences Center

Shreveport

LA

Disclosures

AM declares that he has no competing interests.

Assistant Professor of Neurosciences

Hotchkiss Brain Institute

University of Calgary

Alberta

Canada

Disclosures

CT declares that he has no competing interests.

Consultant Neurologist

Clinical Director of Neurosciences

Department of Neurology

Queen's Hospital

Romford

UK

Disclosures

AC declares that he has no competing interests.

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