Enterovirus D68 (EV-D68) is an important cause of acute respiratory illness, especially in children.[1]Oermann CM, Schuster JE, Conners GP, et al. Enterovirus D68: a focused review and clinical highlights from the 2014 United States outbreak. Ann Am Thorac Soc. 2015 May;12(5):775-81.
http://www.ncbi.nlm.nih.gov/pubmed/25714788?tool=bestpractice.com
[2]Midgley CM, Jackson MA, Selvarangan R, et al. Severe respiratory illness associated with enterovirus d68 - missouri and illinois, 2014. MMWR Morb Mortal Wkly Rep. 2014 Sep 12;63(36):798-9.
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6336a4.htm
http://www.ncbi.nlm.nih.gov/pubmed/25211545?tool=bestpractice.com
[3]Foster CB, Friedman N, Carl J, et al. Enterovirus D68: a clinically important respiratory enterovirus. Cleve Clin J Med. 2015 Jan;82(1):26-31.
http://www.ccjm.org/index.php?id=107953&tx_ttnews[tt_news]=319161&cHash=311070af3357f93577156ab83fd358ec
http://www.ncbi.nlm.nih.gov/pubmed/25552624?tool=bestpractice.com
In 2014, the US experienced an outbreak of EV-D68, with numerous children requiring hospital admission due to severe lower respiratory illness and asthma. This outbreak coincided with an increase in acute flaccid myelitis (AFM) cases in the US, and there has been increasing evidence for causality of EV-D68 for this serious neurologic complication.[9]Lu E, Sinha A, Freeman MC. Clinical progress note: enterovirus D68 and acute flaccid myelitis. J Hosp Med. 2023 May;18(5):435-8.[10]Knoester M, Helfferich J, Poelman R, et al. Twenty-nine cases of enterovirus-D68-associated acute flaccid myelitis in Europe 2016: a case series and epidemiologic overview. Pediatr Infect Dis J. 2019 Jan;38(1):16-21.
https://journals.lww.com/pidj/fulltext/2019/01000/twenty_nine_cases_of_enterovirus_d68_associated.4.aspx
http://www.ncbi.nlm.nih.gov/pubmed/30234793?tool=bestpractice.com
[11]Messacar K, Asturias EJ, Hixon AM, et al. Enterovirus D68 and acute flaccid myelitis-evaluating the evidence for causality. Lancet Infect Dis. 2018 Aug;18(8):e239-47.
https://pmc.ncbi.nlm.nih.gov/articles/PMC6778404
http://www.ncbi.nlm.nih.gov/pubmed/29482893?tool=bestpractice.com
[12]Hu Y, Musharrafieh R, Zheng M, et al. Enterovirus D68 antivirals: past, present, and future. ACS Infect Dis. 2020 Jul 10;6(7):1572-86.
https://pmc.ncbi.nlm.nih.gov/articles/PMC8055446
http://www.ncbi.nlm.nih.gov/pubmed/32352280?tool=bestpractice.com
[23]Sejvar JJ, Lopez AS, Cortese MM, et al. Acute flaccid myelitis in the United States, August-December 2014: results of nationwide surveillance. Clin Infect Dis. 2016 Sep 15;63(6):737-45.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5709818
http://www.ncbi.nlm.nih.gov/pubmed/27318332?tool=bestpractice.com
[24]Centers for Disease Control and Prevention. Acute flaccid myelitis (AFM): AFM cases and outbreaks. Jun 2024 [internet publication].
https://www.cdc.gov/acute-flaccid-myelitis/cases/index.html
Respiratory illness
Most individuals infected have mild symptoms including runny nose, cough, sneezing, shortness of breath, or muscle aches; some patients have fever. Often patients with more severe disease have a history of asthma or chronic lung disease. These patients typically present with wheezing in EV-D68 infection.[36]Montes M, Oñate E, Muguruza A, et al. Enterovirus D68 Causing Acute Respiratory Infection: Clinical Characteristics and Differences With Acute Respiratory Infections Associated With Enterovirus Non-D68. Pediatr Infect Dis J. 2019 Jul;38(7):687-91.
https://journals.lww.com/pidj/Abstract/2019/07000/Enterovirus_D68_Causing_Acute_Respiratory.7.aspx
http://www.ncbi.nlm.nih.gov/pubmed/30985516?tool=bestpractice.com
[37]Caylan E, Weinblatt E, Welter J, et al. Comparison of the Severity of Respiratory Disease in Children Testing Positive for Enterovirus D68 and Human Rhinovirus. J Pediatr. 2018 Jun;197:147-53.e1.
https://www.jpeds.com/article/S0022-3476(18)30215-4/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/29655864?tool=bestpractice.com
Most patients with EV-D68 will have a mild self-limited upper respiratory tract infection for which specific diagnostic testing is not indicated. The illness may be suspected based on epidemiologic considerations. Often EV-D68 occurs as a seasonal outbreak in the late summer or fall. During outbreaks, emergency departments and hospitals may see an increase in the number of children requiring treatment for asthma or respiratory symptoms. In the hospital setting, respiratory viral testing may be indicated for epidemiologic purposes or for specific diagnosis in critically ill patients. Specific testing for EV-D68 should be considered in children with severe unexplained respiratory illness, even in the absence of fever.[35]Centers for Disease Control and Prevention. Non-polio enterovirus: clinical overview of enterovirus D68. Apr 2024 [internet publication].
https://www.cdc.gov/non-polio-enterovirus/hcp/clinical-overview
Because most patients have respiratory symptoms, the virus is generally tested for in respiratory samples, such as nasopharyngeal or oropharyngeal specimens. The standard method for detection is real-time reverse transcription polymerase chain reaction (rRT-PCR), and EV-D68 might be examined for using specific assays or as part of a multiplex respiratory viral panel. Many laboratories use methods that do not distinguish between different enteroviruses or between enteroviruses and rhinoviruses. For these assays, further testing with specific EV-D68 rRT-PCR or molecular sequencing is necessary if specific identification of EV-D68 is required. In the US, specific testing can be performed on a case-by-case basis through state or local health departments, or the Centers for Disease Control and Prevention (CDC).[38]Centers for Disease Control and Prevention. Non-polio enterovirus: laboratory testing for non-polio enterovirus. Apr 2024 [internet publication].
https://www.cdc.gov/non-polio-enterovirus/php/laboratories/index.html
Acute flaccid myelitis
Mounting evidence suggests a causal relationship between EV-D68 infection and polio-like AFM.[5]Pastula DM, Aliabadi N, Haynes AK, et al. Acute neurologic illness of unknown etiology in children: Colorado, August - September 2014. MMWR Morb Mortal Wkly Rep. 2014 Oct 10;63(40):901-2.
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6340a5.htm
http://www.ncbi.nlm.nih.gov/pubmed/25299607?tool=bestpractice.com
[6]Greninger AL, Naccache SN, Messacar K, et al. A novel outbreak enterovirus D68 strain associated with acute flaccid myelitis cases in the USA (2012-14): a retrospective cohort study. Lancet Infect Dis. 2015 Jun;15(6):671-82.
http://www.ncbi.nlm.nih.gov/pubmed/25837569?tool=bestpractice.com
[7]Ayscue P, Haren KV, Sheriff H, et al. Acute flaccid paralysis with anterior myelitis - California, June 2012-June 2014. MMWR Morb Mortal Wkly Rep. 2014 Oct 10;63(40):903-6.
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6340a6.htm
http://www.ncbi.nlm.nih.gov/pubmed/25299608?tool=bestpractice.com
[8]Messacar K, Schreiner TL, Maloney JA, et al. A cluster of acute flaccid paralysis and cranial nerve dysfunction temporally associated with an outbreak of enterovirus D68 in children in Colorado, USA. Lancet. 2015 Apr 25;385(9978):1662-71.
http://www.ncbi.nlm.nih.gov/pubmed/25638662?tool=bestpractice.com
[9]Lu E, Sinha A, Freeman MC. Clinical progress note: enterovirus D68 and acute flaccid myelitis. J Hosp Med. 2023 May;18(5):435-8.[10]Knoester M, Helfferich J, Poelman R, et al. Twenty-nine cases of enterovirus-D68-associated acute flaccid myelitis in Europe 2016: a case series and epidemiologic overview. Pediatr Infect Dis J. 2019 Jan;38(1):16-21.
https://journals.lww.com/pidj/fulltext/2019/01000/twenty_nine_cases_of_enterovirus_d68_associated.4.aspx
http://www.ncbi.nlm.nih.gov/pubmed/30234793?tool=bestpractice.com
[11]Messacar K, Asturias EJ, Hixon AM, et al. Enterovirus D68 and acute flaccid myelitis-evaluating the evidence for causality. Lancet Infect Dis. 2018 Aug;18(8):e239-47.
https://pmc.ncbi.nlm.nih.gov/articles/PMC6778404
http://www.ncbi.nlm.nih.gov/pubmed/29482893?tool=bestpractice.com
[12]Hu Y, Musharrafieh R, Zheng M, et al. Enterovirus D68 antivirals: past, present, and future. ACS Infect Dis. 2020 Jul 10;6(7):1572-86.
https://pmc.ncbi.nlm.nih.gov/articles/PMC8055446
http://www.ncbi.nlm.nih.gov/pubmed/32352280?tool=bestpractice.com
In 2014, an increase in AFM cases coincided with an outbreak of EV-D68-related severe respiratory illness in the US.[23]Sejvar JJ, Lopez AS, Cortese MM, et al. Acute flaccid myelitis in the United States, August-December 2014: results of nationwide surveillance. Clin Infect Dis. 2016 Sep 15;63(6):737-45.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5709818
http://www.ncbi.nlm.nih.gov/pubmed/27318332?tool=bestpractice.com
[24]Centers for Disease Control and Prevention. Acute flaccid myelitis (AFM): AFM cases and outbreaks. Jun 2024 [internet publication].
https://www.cdc.gov/acute-flaccid-myelitis/cases/index.html
In 2022, several US health systems reported increases in severe respiratory illness in children who tested positive for rhinovirus or enterovirus, with a higher proportion of EV-D68 positivity than in previous years. The CDC published an advisory urging healthcare providers to consider EV-D68 as a possible cause of acute severe respiratory illness, with or without fever in children, and to advise of the potential for an increase in AFM cases.[25]Centers for Disease Control and Prevention. Severe respiratory illnesses associated with rhinoviruses and/or enteroviruses Including EV-D68 – Multistate, 2022. Sep 2022 [internet publication].
https://archive.cdc.gov/#/details?url=https://www.cdc.gov/han/2022/han00474.html
The cardinal symptom of AFM is acute, flaccid weakness of one or more limbs.[39]Centers for Disease Control and Prevention. Acute flaccid myelitis (AFM): case definitions for AFM. Jun 2024 [internet publication].
https://www.cdc.gov/acute-flaccid-myelitis/php/reporting-patient/case-definitions.html
The majority of patients have prodromal fever, respiratory illness, or both, beginning a median of 6 days before the onset of weakness.[40]Stehbens WE, Wierzbicki E. The relationship of hypercholesterolemia to atherosclerosis with particular emphasis on familial hypercholesterolemia, diabetes mellitus, obstructive jaundice, myxedema, and the nephrotic syndrome. Prog Cardiovasc Dis. 1988 Jan-Feb;30(4):289-306.
https://www.sciencedirect.com/science/article/abs/pii/0033062088900205?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/3275991?tool=bestpractice.com
Thirty-five percent of patients have fever at the time of evaluation for weakness. Patients may also experience gait difficulty, back, neck or limb pain, or acute cranial nerve dysfunction.[40]Stehbens WE, Wierzbicki E. The relationship of hypercholesterolemia to atherosclerosis with particular emphasis on familial hypercholesterolemia, diabetes mellitus, obstructive jaundice, myxedema, and the nephrotic syndrome. Prog Cardiovasc Dis. 1988 Jan-Feb;30(4):289-306.
https://www.sciencedirect.com/science/article/abs/pii/0033062088900205?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/3275991?tool=bestpractice.com
[41]Centers for Disease Control and Prevention (CDC). Vital signs: Acute Flaccid Myelitis (AFM). Aug 2020 [internet publication].
https://www.cdc.gov/vitalsigns/afm2020
Other symptoms include difficulty holding up the head, decreased appetite, difficulty swallowing, and bladder or bowel changes, especially constipation.
Young children and their carers may not describe impaired limb function as weakness. If a child has new difficulty in feeding or dressing themselves, is limping, falling frequently, has difficulty throwing a ball overhead or getting out of a bathtub, or is suddenly using one limb less, he or she may have limb weakness.
Patients should have a comprehensive neurologic examination, evaluation for respiratory insufficiency and evaluation for autonomic manifestations (e.g., temperature instability, blood pressure lability). Sensory examination is often normal in patients with AFM.
Initial investigations include magnetic resonance imaging of the spine and brain, and analysis of cerebrospinal fluid, nasopharyngeal or oropharyngeal specimens, stool or rectal swab, and serum.[42]Centers for Disease Control and Prevention. Acute flaccid myelitis (AFM): clinical testing and diagnosis for acute flaccid myelitis. May 2024 [internet publication].
https://www.cdc.gov/acute-flaccid-myelitis/hcp/diagnosis-testing/index.html
Nerve conduction studies may also be useful in diagnosing AFM.[43]Centers for Disease Control and Prevention. Acute flaccid myelitis (AFM): testing and diagnosis for AFM. Jun 2024 [internet publication].
https://www.cdc.gov/acute-flaccid-myelitis/testing/index.html
Paralysis may be prolonged and recovery is often incomplete.
When a child presents with paralysis, the differential diagnoses include Guillain-Barre syndrome, traumatic neuritis, acute transverse myelitis, and toxin-mediated diseases such as botulism and tick paralysis. AFM may be caused by infection with several viruses in addition to EV-D68, such as other nonpolio enteroviruses, poliovirus, West Nile virus, and Japanese encephalitis virus.[44]Murphy OC, Messacar K, Benson L, et al. Acute flaccid myelitis: cause, diagnosis, and management. Lancet. 2021 Jan 23;397(10271):334-46.
https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(20)32723-9/fulltex
http://www.ncbi.nlm.nih.gov/pubmed/33357469?tool=bestpractice.com
Given the clinical similarity between AFM and acute flaccid limb paralysis caused by poliovirus, patients suspected of having AFM should also be tested for poliovirus infection.[45]Ma KC, Winn A, Moline HL, et al. Increase in acute respiratory illnesses among children and adolescents associated with rhinoviruses and enteroviruses, including enterovirus D68 - United States, July-September 2022. MMWR Morb Mortal Wkly Rep. 2022 Oct 7;71(40):1265-70.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9541033
http://www.ncbi.nlm.nih.gov/pubmed/36201400?tool=bestpractice.com
The World Health Organization routinely lists countries where endemic or vaccine-derived poliomyelitis is currently circulating:
Global polio eradication initiative
Opens in new window See Poliovirus infection.