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acute neurologic illness with limb paralysis; acute flaccid myelitis (AFM)

Etiology: - enteroviruses play a role in the etiology of acute flaccid myelitis [10] - enterovirus D68 20% (< 1%), enterovirus A71 (< 1%) [5] - enteroviruses or rhinoviruses in 22% [2], 54% [5] - enterovirus & rhinovirus detected in 36% of respiratory samples tested at the CDC; 69% of these were enterovirus D68 [9] - no cases of poliovirus [4] - adenoviruses [3] - West Nile virus [3,9] - arechovirus, mycoplasma, Epstein-Barr virus, human herpesvirus 6 [9] Epidemiology: - children - 51 cases in children in 23 states (2014) - possible cluster of AFM in Washington state fall 2016 [3] - 2 confirmed cases, 7 suspected cases under evaluation [3] - 106 cases in 29 states Nov 2018 - 158 cases in 33 states have been confirmed Dec 2018 [7,8] - 186 cases in 39 states as of Dec 28, 2018 [8] - 193 confirmed pediatric cases from 41 states - most were in 2016 - most cases in summer & fall - most cases in boys - median age 6 years (range, 3 months - 21 years) [9] Clinical manifestations: - most patients have fever or respiratory symptoms 3-10 days prior to developing limb weakness [5] - 83% of patients report symptoms before onset of AFM, including respiratory (66%) & gastrointestinal (29%). - acute onset of focal limb weakness - 45% of patients hwith 3 or 4 limbs involved [9] - 36% present with quadriplegia [9] - 33% require mechanical ventilation [9] - 36% of patients without improvement in clinical status after 3 weeks [2] Special laboratory: - lumbar puncture with CSF analysis - no enterovirus or other pathogen [2] Diagnostic criteria: - age 21 years or younger - acute onset of focal limb weakness; onset on Aug. 1, 2014 or later - MRI showing a spinal cord lesion largely restricted to gray matter Radiology: - MRI of spinal cord - spinal cord lesion largely restricted to gray matter - cervical spine abnormalities in 80% [9] - brain MRI shows brainstem abnormalities in 38% [9] Complications: - no deaths reported [2] Management: - supportive - prognosis: spectrum of recovery is highly variable

General

neurologic disease

References

  1. Centers for Disease Control and Prevention (CDC) Investigation of Acute Neurologic Illness with Focal Limb Weakness of Unknown Etiology in Children, Fall 2014. http://www.cdc.gov/ncird/investigation/viral/sep2014.html
  2. Sofair A, Chavey WE Cause of Acute Limb Paralysis in Children Still Unknown Physician's First Watch, Jan 9, 2015 David G. Fairchild, MD, MPH, Editor-in-Chief Massachusetts Medical Society http://www.jwatch.org - Centers for Disease Control and Prevention (CDC) Notes from the Field: Acute Flaccid Myelitis Among Persons Aged <=21 Years - United States, August 1-November 13, 2014 MMWR Weekly. January 9, 2015 / 63(53);1243-1244 http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6353a3.htm
  3. Washington State Department of Health AFM Investigation. Nov 2, 2016 http://www.doh.wa.gov/Emergencies/AFMInvestigation
  4. Young K, Fairchild DG, Di Francesco L Acute Flaccid Myelitis Cases Confirmed in 22 States Physician's First Watch, Oct 17, 2018 David G. Fairchild, MD, MPH, Editor-in-Chief Massachusetts Medical Society http://www.jwatch.org https://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html
  5. McKay SL, Lee AD, Lopez AS, et al. Increase in Acute Flaccid Myelitis - United States, 2018. MMWR Morb Mortal Wkly Rep. ePub: 13 November 2018 https://www.cdc.gov/mmwr/volumes/67/wr/mm6745e1.htm
  6. Centers for Disease Control and Prevention (CDC) CDC Establishes AFM Task Force. CDC Press Releas. Nov 19, 2018 https://www.cdc.gov/media/releases/2018/p1119-cdc-establishes-afm-task-force.html
  7. Centers for Disease Control and Prevention (CDC) Weekly Update for Number of U.S. AFM Cases. Dec 3, 2018 https://www.cdc.gov/media/releases/2018/s1203-us-afm-case.html
  8. Lopez A, Lee A, Guo A et al. Vital Signs: Surveillance for Acute Flaccid Myelitis - United States, 2018. MMWR Morb Mortal Wkly Rep. ePub: 9 July 2019 PMID: 31295232 https://www.cdc.gov/mmwr/volumes/68/wr/mm6827e1.htm
  9. Ayers T, Lopez A, Lee A et al. Acute flaccid myelitis in the United States: 2015-2017. Pediatrics 2019 Oct 7; PMID: 31591135 https://pediatrics.aappublications.org/content/early/2019/10/03/peds.2019-1619
  10. Schubert RD, Hawes IA, Ramachandran PS et al Pan-viral serology implicates enteroviruses in acute flaccid myelitis. Nature Medicine (2019). Oct 21. PMID: 31636453 https://www.nature.com/articles/s41591-019-0613-1
  11. Centers for Disease Control and Prevention (CDC) Acute Flaccid Myelitis Home. AFM Investigation AFM in the United States. AFM Investigation https://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html