Abstract
Purpose of review
Purpose of review Acute flaccid myelitis is a polio-like illness defined by the acute onset of flaccid paralysis in the setting spinal MRI demonstrating a longitudinal lesion in the gray matter of the cord. This paper aims to review the current state of knowledge and key clinical points for the diagnosis and management of acute flaccid myelitis.
Recent findings
Recent findings There were clusters of AFM noted in California and Colorado in 2014, with additional cases across the USA that year, and another spike in cases in 2016. Patients have been managed with classic treatments for transverse myelitis, but in general without benefit, although some colleagues have noted anecdotal improvement in individual patients. Our current practice at the Children’s Hospital of Philadelphia is to initiate therapy with intravenous immunoglobulin (IVIG) upon recognition of acute flaccid myelitis in hopes of boosting humoral immunity, and to provide an emphasis on rehabilitation services, including physical and occupational therapy. There is some data that suggests a connection to the virus enterovirus D68 (EV D68), but there has been no definitive link. Publications regarding longer-term outcomes in these patients are early in development and, thus far, only provide data for 6 to 12 months from onset.
Summary
Summary AFM is a serious illness with long-term consequences, and we have much to learn. Key areas in need of further investigation involve etiology, host susceptibilities, treatment options, and long-term outcome. Individual clinicians can assist in these efforts by the prompt reporting of cases of AFM to the US Centers for Disease Control and Prevention.
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References and Recommended Reading
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•• Messacar K, Schreiner TL, Van Haren K, Yang M, Glaser CA, Tyler KL, et al. Acute flaccid myelitis: a clinical review of US cases 2012–2015. Ann Neurol. 2016;80:326–38. This article provides the most comprehensive discussion of cases that occurred in 2014. It also provides a discussion of considerations for therapy.
Ayscue P, Van Haren K, Sheriff H, Waubant E, Waldron P, Yagi S, et al. Acute flaccid paralysis with anterior myelitis - California, June 2012-June 2014. MMWR Morb Mortal Wkly Rep. 2014;63:903–6. Available from: https://www.cdc.gov/mmwr/preview/mmwrhtml/mm6340a6.htm
Sejvar JJ, Lopez AS, Cortese MM, Leshem E, Pastula DM, Miller L, et al. Acute flaccid myelitis in the United States, August-December 2014: results of nationwide surveillance. Clin Infect Dis. 2016;63:737–45.
Centers for Disease Control and Prevention (CDC). AFM in the United States [Internet]. [cited 2017 Jan 1]. Available from: https://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html
Van Haren K, Ayscue P, Waubant E, Clayton A, Sheriff H, Yagi S, et al. Acute flaccid myelitis of unknown etiology in California, 2012-2015. JAMA. 2015;314:2663–71.
Aliabadi N, Messacar K, Pastula DM, Robinson CC, Leshem E, Sejvar JJ, et al. Enterovirus D68 infection in children with acute flaccid myelitis, Colorado, USA, 2014. Emerg Infect Dis. 2016;22:1387–94.
Hixon AM, Yu G, Leser JS, Yagi S, Clarke P, Chiu CY, et al. A mouse model of paralytic myelitis caused by enterovirus D68. PLoS Pathog. 2017:13.
• Maloney JA, Mirsky DM, Messacar K, Dominguez SR, Schreiner T, Stence NV. MRI findings in children with acute flaccid paralysis and cranial nerve dysfunction occurring during the 2014 enterovirus D68 outbreak. Am J Neuroradiol. 2015;36:245–50. This is a key review of imaging findings in AFM.
• Martin JA, Messacar K, Yang ML, Maloney JA, Lindwall J, Carry T, et al. Outcomes of Colorado children with acute flaccid myelitis at 1 year. Neurology. 2017;89:129–37. Martin, et al. provide the longest term follow-up data for patients with AFM (1 year).
Ruggieri V, Paz MI, Peretti MG, Rugilo C, Bologna R, Freire C, et al. Enterovirus D68 infection in a cluster of children with acute flaccid myelitis, Buenos Aires, Argentina, 2016. Eur J Paediatr Neurol. 2017;21(6):884–890.
Nelson GR, Bonkowsky JL, Doll E, Green M, Hedlund GL, Moore KR, et al. Recognition and management of acute flaccid myelitis in children. Pediatr Neurol. 2016;55:17–21.
Esposito S, Chidini G, Cinnante C, Napolitano L, Giannini A, Terranova L, et al. Acute flaccid myelitis associated with enterovirus-D68 infection in an otherwise healthy child. Virol J. 2017;14:4.
Centers for Disease Control and Prevention (CDC). Acute flaccid myelitis: interim considerations for clinical management. [Internet]. 2014. Available from: https://www.cdc.gov/acute-flaccid-myelitis/downloads/acute-flaccid-myelitis.pdf
Sabanathan S, Tan L, Thwaites P, Wills B, Qui P, van Doorn H. Enterovirus 71 related severe hand, foot and mouth disease outbreaks in South-East Asia: current situation and ongoing challenges. J Epidemiol Community Health. 2014;68(6):500–502
Zhang Y, Moore DD, Nix WA, Oberste MS, Weldon WC. Neutralization of enterovirus D68 isolated from the 2014 US outbreak by commercial intravenous immune globulin products. J Clin Virol. 2015;69:172–5.
Tyler KL. Rationale for the evaluation of fluoxetine in the treatment of enterovirus D68-associated acute flaccid myelitis. JAMA Neurol. 2015;72(5):493–4
O’Mahony J, Marrie RA, Laporte A, Yeh EA, Bar-Or A, Phan C, et al. Recovery from central nervous system acute demyelination in children. Pediatrics. 2015;136:e115–23.
Satbhai NG, Doi K, Hattori Y, Sakamoto S. Restoration of prehensile function for motor paralysis in Hopkins syndrome: case report. J Hand Surg Am. 2014;39:312–6.
Saltzman E, Vu A, Shwenker A, Raney S, Sneag D, Kevin Yakuboff SW. Nerve transfers for enterovirus D68-induced acute flaccid myelitis. Hand. 2016;97s:11.
McCoy DB, Talbott JF, Wilson M, Mamlouk MD, Cohen-Adad J, Wilson M, et al. MRI atlas-based measurement of spinal cord injury predicts outcome in acute flaccid myelitis. Am J Neuroradiol. 2017;38:410–7.
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Sarah E. Hopkins reports consulting fees from Shire Pharmaceuticals and receives salary support from the CDC for activities related to AFM surveillance.
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This article is part of the Topical Collection on Neuromuscular Disorders
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Hopkins, S.E. Acute Flaccid Myelitis: Etiologic Challenges, Diagnostic and Management Considerations. Curr Treat Options Neurol 19, 48 (2017). https://doi.org/10.1007/s11940-017-0480-3
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DOI: https://doi.org/10.1007/s11940-017-0480-3