TY - JOUR
T1 - Challenges in the Clinical Recognition of Acute Flaccid Myelitis and its Implications
AU - Hayes, Leslie H.
AU - Hopkins, Sarah E.
AU - Liu, Shanshan
AU - Pardo, Carlos A.
AU - Garcia-Dominguez, Maria A.
AU - Oleszek, Joyce
AU - Yea, Carmen
AU - Ciftci-Kavaklioglu, Beyza
AU - Yeh, E. Ann
AU - Dean, Janet
AU - Sadowsky, Cristina L.
AU - Desai, Jay
AU - Wiegand, Sarah
AU - Farias-Moeller, Raquel
AU - Nash, Kendall
AU - Thakur, Kiran T.
AU - Vargas, Wendy S.
AU - Hong-Routson, Sue J.
AU - Yeshokumar, Anusha
AU - Zhou, Melissa S.
AU - Makhani, Naila
AU - Wilson-Murphy, Molly
AU - Bove, Riley
AU - Zhang, Bo
AU - Benson, Leslie A.
N1 - Funding Information:
This work was supported by the National Institutes of Health (Grant R01 NS108358 , to C.P.), National Institute of Neurological Disorders and Stroke (Grants K23NS105935-01 , to K.T. and K23NS101099 , to N.M.), National Institute of Child Health and Human Development (Grant K23HD098312 , to W.V.), US Department of Defense (E.Y.), Chicago Department of Public Health (S.H-R.), Bart McLean Fund for Neuroimmunology Research (C.P.), National Multiple Sclerosis Society (E.Y.), National Multiple Sclerosis Society Harry Weaver Scholars Award (to R.B.), Multiple Sclerosis Society of Canada (E.Y.), Canadian Institute for Health Information (E.Y.), Canadian Institutes of Health Research (E.Y.), National Institutes of Health (E.Y.), Office of Information and Resource Management (E.Y.), Mario Battaglia Foundation (E.Y.), SickKids Foundation (E.Y.), Canada Bulletin of Medical History Innovation Fund (E.Y.), Consortium of Multiple Sclerosis Centers (E.Y.), Stem Cell Network (E.Y.), Rare Disease Foundation (E.Y.), Biogen (E.Y.), UCB (J.D.), Novartis (J.D.), Ovid (J.D.), Aquestive (J.D.), Neurelis (J.D.), unrestricted educational funds from Teva (to E.Y.), and unrestricted educational funds from the Guthy-Jackson Foundation (to E.Y.). C.P. is an unpaid advisor to the AFM Task Force of the US Centers for Disease Control and Prevention. E.Y. has served on a scientific advisory panel for Hoffman-La Roche and Biogen and has received speaker’s honoraria from Novartis, Consortium of Multiple Sclerosis Centers, Multiple Sclerosis at the Limits, and the Canadian Rheumatological Association. R.B. reports research support from the National Multiple Sclerosis Society , Hilton Foundation , California Initiative to Advance Precision Medicine , Sherak Foundation , Akili Interactive , and Roche Genentech . L.B. receives research funding from Biogen , Alexion , ROHHAD Fight , and The Shore Foundation ; is a paid consultant to the national Vaccine Injury Compensation Program and separately to the Massachusetts Department of Public Health for acute flaccid myelitis; and is a member of the Centers for Disease Control and Prevention’s AFM Task Force and has advised on the development of the National Institutes of Health’s AFM Natural History Study, receiving travel support for both. The other authors declare no conflicts of interest.
Funding Information:
This work was supported by the National Institutes of Health (Grant R01 NS108358, to C.P.), National Institute of Neurological Disorders and Stroke (Grants K23NS105935-01, to K.T. and K23NS101099, to N.M.), National Institute of Child Health and Human Development (Grant K23HD098312, to W.V.), US Department of Defense (E.Y.), Chicago Department of Public Health (S.H-R.), Bart McLean Fund for Neuroimmunology Research (C.P.), National Multiple Sclerosis Society (E.Y.), National Multiple Sclerosis Society Harry Weaver Scholars Award (to R.B.), Multiple Sclerosis Society of Canada (E.Y.), Canadian Institute for Health Information (E.Y.), Canadian Institutes of Health Research (E.Y.), National Institutes of Health (E.Y.), Office of Information and Resource Management (E.Y.), Mario Battaglia Foundation (E.Y.), SickKids Foundation (E.Y.), Canada Bulletin of Medical History Innovation Fund (E.Y.), Consortium of Multiple Sclerosis Centers (E.Y.), Stem Cell Network (E.Y.), Rare Disease Foundation (E.Y.), Biogen (E.Y.), UCB (J.D.), Novartis (J.D.), Ovid (J.D.), Aquestive (J.D.), Neurelis (J.D.), unrestricted educational funds from Teva (to E.Y.), and unrestricted educational funds from the Guthy-Jackson Foundation (to E.Y.). C.P. is an unpaid advisor to the AFM Task Force of the US Centers for Disease Control and Prevention. E.Y. has served on a scientific advisory panel for Hoffman-La Roche and Biogen and has received speaker's honoraria from Novartis, Consortium of Multiple Sclerosis Centers, Multiple Sclerosis at the Limits, and the Canadian Rheumatological Association. R.B. reports research support from the National Multiple Sclerosis Society, Hilton Foundation, California Initiative to Advance Precision Medicine, Sherak Foundation, Akili Interactive, and Roche Genentech. L.B. receives research funding from Biogen, Alexion, ROHHAD Fight, and The Shore Foundation; is a paid consultant to the national Vaccine Injury Compensation Program and separately to the Massachusetts Department of Public Health for acute flaccid myelitis; and is a member of the Centers for Disease Control and Prevention's AFM Task Force and has advised on the development of the National Institutes of Health's AFM Natural History Study, receiving travel support for both. The other authors declare no conflicts of interest. Funding and disclosure information is available at www.jpeds.com.
Publisher Copyright:
© 2022 The Authors
PY - 2023/2
Y1 - 2023/2
N2 - Objectives: To explore the challenges in diagnosing acute flaccid myelitis (AFM) and evaluate clinical features and treatment paradigms associated with under recognition. Study design: This was a retrospective multicenter study of pediatric patients (≤18 years) who were diagnosed with AFM from 2014 to 2018 using the Centers for Disease Control and Prevention's case definition. Results: In 72% of the cases (126 of 175), AFM was not considered in the initial differential diagnosis (n = 108; 61.7%) and/or the patient was not referred for acute care (n = 90; 51.4%) at the initial clinical encounter, and this did not improve over time. Although many features of the presentation were similar in those initially diagnosed with AFM and those who were not; preceding illness, constipation, and reflexes differed significantly between the 2 groups. Patients with a non-AFM initial diagnosis more often required ventilatory support (26.2% vs 12.2%; OR, 0.4; 95% CI, 0.2-1.0; P = .05). These patients received immunomodulatory treatment later (3 days vs 2 days after neurologic symptom onset; 95% CI, −2 to 0; P = .05), particularly intravenous immunoglobulin (5 days vs 2 days; 95% CI, −4 to −2; P < .001). Conclusions: Delayed recognition of AFM is concerning because of the risk for respiratory decompensation and need for intensive care monitoring. A non-AFM initial diagnosis was associated with delayed treatment that could have a clinical impact, particularly as new treatment options emerge.
AB - Objectives: To explore the challenges in diagnosing acute flaccid myelitis (AFM) and evaluate clinical features and treatment paradigms associated with under recognition. Study design: This was a retrospective multicenter study of pediatric patients (≤18 years) who were diagnosed with AFM from 2014 to 2018 using the Centers for Disease Control and Prevention's case definition. Results: In 72% of the cases (126 of 175), AFM was not considered in the initial differential diagnosis (n = 108; 61.7%) and/or the patient was not referred for acute care (n = 90; 51.4%) at the initial clinical encounter, and this did not improve over time. Although many features of the presentation were similar in those initially diagnosed with AFM and those who were not; preceding illness, constipation, and reflexes differed significantly between the 2 groups. Patients with a non-AFM initial diagnosis more often required ventilatory support (26.2% vs 12.2%; OR, 0.4; 95% CI, 0.2-1.0; P = .05). These patients received immunomodulatory treatment later (3 days vs 2 days after neurologic symptom onset; 95% CI, −2 to 0; P = .05), particularly intravenous immunoglobulin (5 days vs 2 days; 95% CI, −4 to −2; P < .001). Conclusions: Delayed recognition of AFM is concerning because of the risk for respiratory decompensation and need for intensive care monitoring. A non-AFM initial diagnosis was associated with delayed treatment that could have a clinical impact, particularly as new treatment options emerge.
KW - child
KW - enterovirus
KW - misdiagnosis
KW - paralysis
UR - http://www.scopus.com/inward/record.url?scp=85139853947&partnerID=8YFLogxK
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U2 - 10.1016/j.jpeds.2022.09.012
DO - 10.1016/j.jpeds.2022.09.012
M3 - Article
C2 - 36115622
AN - SCOPUS:85139853947
SN - 0022-3476
VL - 253
SP - 55-62.e4
JO - Journal of Pediatrics
JF - Journal of Pediatrics
ER -