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Table 1.  

Characteristic Value
Sex
   M 19 (46.3)
   F 22 (53.7)
Median age, y, at onset (range) 4.07 (0.25–14.08)
Prodromal illness
   Confirmed 6 (14.6)
   Probable 15 (36.6)
   No 13 (31.7)
   Unknown 7 (17.1)
Confirmed specified
   URTI 5 (83.3)
   Gastrointestinal 1 (16.7)
Site of paralysis
   Known 39 (95.1)
   Unknown 2 (4.9)
Site of paralysis, upper limbs
   Unilateral 11 (28.2)
   Bilateral 3 (7.7)
Site of paralysis, lower limbs
   Unilateral 3 (7.7)
   Bilateral 13 (33.3)
Site of paralysis, upper and lower limbs
   Unilateral 5 (12.8)
   Bilateral 4 (10.3)
Bulbar/cranial nerve palsy
   Yes 14 (34.1)
   No 26 (63.4)
   Unknown 1 (2.4)
Reduced sensation
   Yes 6 (14.6)
   No 34 (82.9)
   Unknown 1 (2.4)
Bladder/bowel dysfunction
   Yes 17 (41.5)
   No 15 (36.5)
   Unknown 9 (22.0)
Median CSF protein level, g/L (range) 0.43 (0.11–1.18)
Elevated CSF protein level >0.55 g/L
   Yes 8 (19.5)
   No 29 (70.7)
   Unknown 4 (9.8)
Median CSF leukocyte count/mm3 (range) 38 (0–267)
Pleocytosis >5 cells mm3  
   Yes 33 (80.5)
   No 3 (7.3)
   Unknown 5 (12.2)

Table 1. Characteristics of cases of acute flaccid myleitis for 41 children <15 years of age, Australia, 2000‒2018*

*Values are no. (%) unless otherwise indicated. CSF, cerebrospinal fluid; URTI, upper respiratory tract infection.

Table 2.  

Characteristic No. (%)
Magnetic resonance conducted
   Yes 37 (90.2)
   No 4 (9.8)
   Unknown 0
Brain abnormal
   Yes 7 (18.9)
   No 30 (81.1)
Brain region
   White 1 (14.3)
Gray 1 (14.3)
   Botd 5 (71.4)
Brain stem abnormal
   Yes 5 (13.5)
   No 32 (86.5)
Spinal cord abnormal
   Yes 33 (89.2)
   Yes, no regional details 4 (10.8)
Spinal cord region abnormal
   Cervical 12 (36.4)
   tdoracic 3 (9.1)
   Lumbar 2 (6.1)
   Cervical-thoracic 7 (21.2)
   thoracic-lumbar 2 (6.1)
   Cervical-thoracic-lumbar 7 (21.2)
Restricted to gray matter
   Yes 37 (100.0)
   No 0
Conus and roots abnormal
   Yes 11 (29.7)
   No 26 (0.3)

Table 2. Characteristics of cases of acute flaccid myelitis, by magnetic resonance imaging, for 41 children <15 years of age, Australia, 2000‒2018*

CME / ABIM MOC

Using the Acute Flaccid Paralysis Surveillance System to Identify Cases of Acute Flaccid Myelitis, Australia, 2000‒2018

  • Authors: Liz J. Walker, MPH; Bruce R. Thorley, PhD; Anne Morris, MBBS, MPH, FRACP; Elizabeth J. Elliott, MD; Philip N. Britton, PhD, FRACP
  • CME / ABIM MOC Released: 12/16/2021
  • Valid for credit through: 12/16/2022
Start Activity

  • Credits Available

    Physicians - maximum of 1.00 AMA PRA Category 1 Credit(s)™

    ABIM Diplomates - maximum of 1.00 ABIM MOC points

    You Are Eligible For

    • Letter of Completion
    • ABIM MOC points

Target Audience and Goal Statement

This activity is intended for infectious disease clinicians, pediatricians, neurologists, public health officials, and other clinicians caring for patients with acute flaccid paralysis or acute flaccid myelitis.

The goal of this activity is to describe the epidemiology and clinical and diagnostic characteristics of acute flaccid myelitis cases identified by Australia's acute flaccid paralysis surveillance system from 2000 to 2018, which included 915 acute flaccid paralysis cases in children younger than 15 years, of whom a subset was reclassified to acute flaccid myelitis using the US Council of State and Territorial Epidemiologists case definition.

Upon completion of this activity, participants will:

  • Assess the clinical and diagnostic characteristics of acute flaccid myelitis cases in children younger than 15 years identified by Australia's acute flaccid paralysis surveillance system from 2000 to 2018
  • Evaluate the epidemiology and viral characterization of acute flaccid myelitis cases in children younger than 15 years identified by Australia's acute flaccid paralysis surveillance system from 2000 to 2018
  • Determine the clinical and public health implications of the epidemiology, clinical and diagnostic characteristics of acute flaccid myelitis cases in children younger than 15 years identified by Australia's acute flaccid paralysis surveillance system from 2000 to 2018


Disclosures

As an organization accredited by the ACCME, Medscape, LLC requires everyone who is in a position to control the content of an education activity to disclose all relevant financial relationships with any commercial interest. The ACCME defines "relevant financial relationships" as financial relationships in any amount, occurring within the past 12 months, including financial relationships of a spouse or life partner, that could create a conflict of interest.

Medscape, LLC encourages Authors to identify investigational products or off-label uses of products regulated by the US Food and Drug Administration, at first mention and where appropriate in the content.


Faculty

  • Liz J. Walker, MPH

    National Centre for Epidemiology and Population Health, The Australian National University, Australian Capital Territory, Australia; Australian Government Department of Health, Australian Capital Territory, Australia

    Disclosures

    Disclosure: Liz J. Walker, MPH, has disclosed no relevant financial relationships.

  • Bruce R. Thorley, PhD

    National Enterovirus Reference Laboratory, Victorian Infectious Diseases Reference Laboratory, Royal Melbourne Hospital, at the Peter Doherty Institute for Infection and Immunity, Victoria, Australia

    Disclosures

    Disclosure: Bruce R. Thorley, PhD, has disclosed no relevant financial relationships.

  • Anne Morris, MBBS, MPH, FRACP

    Australian Paediatric Surveillance Unit, the Children's Hospital at Westmead, New South Wales, Australia

    Disclosures

    Disclosure: Anne Morris, MBBS, MPH, FRACP, has disclosed no relevant financial relationships.

  • Elizabeth J. Elliott, MD

    Australian Paediatric Surveillance Unit, the Children's Hospital at Westmead, New South Wales, Australia; Paediatric Active Enhanced Disease Surveillance Network, National Centre for Immunisation Research and Surveillance, the Children's Hospital at Westmead, New South Wales, Australia; Sydney Medical School, Specialty of Child and Adolescent Health, the University of Sydney, New South Wales, Australia

    Disclosures

    Disclosure: Elizabeth J. Elliott, MD, has disclosed no relevant financial relationships.

  • Nathan Saul, PhD

    Australian Government Department of Health, Australian Capital Territory, Australia

    Disclosures

    Disclosure: Nathan Saul, PhD, has disclosed no relevant financial relationships.

  • Philip N. Britton, PhD, FRACP

    Paediatric Active Enhanced Disease Surveillance network, National Centre for Immunisation Research and Surveillance, the Children's Hospital at Westmead, New South Wales, Australia; Sydney Medical School, Specialty of Child and Adolescent Health, the University of Sydney, New South Wales, Australia

    Disclosures

    Disclosure: Philip N. Britton, PhD, FRACP, has disclosed the following relevant financial relationships:
    Served as an advisor or consultant for: BioFire Diagnostics

CME Author

  • Laurie Barclay, MD

    Freelance writer and reviewer
    Medscape, LLC

    Disclosures

    Disclosure: Laurie Barclay, MD, has disclosed no relevant financial relationships.

Editor

  • Thomas J. Gryczan, MS

    Copyeditor
    Emerging Infectious Diseases

    Disclosures

    Disclosure: Thomas J. Gryczan, MS, has disclosed no relevant financial relationships.

CME Reviewer

  • Amanda Jett, PharmD, BCACP

    Associate Director, Accreditation and Compliance
    Medscape, LLC

    Disclosures

    Disclosure: Amanda Jett, PharmD, BCACP, has disclosed no relevant financial relationships.

None of the nonfaculty planners for this educational activity have relevant financial relationship(s) to disclose with ineligible companies whose primary business is producing, marketing, selling, reselling, or distributing healthcare products used by or on patients.


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CME / ABIM MOC

Using the Acute Flaccid Paralysis Surveillance System to Identify Cases of Acute Flaccid Myelitis, Australia, 2000‒2018

Authors: Liz J. Walker, MPH; Bruce R. Thorley, PhD; Anne Morris, MBBS, MPH, FRACP; Elizabeth J. Elliott, MD; Philip N. Britton, PhD, FRACPFaculty and Disclosures

CME / ABIM MOC Released: 12/16/2021

Valid for credit through: 12/16/2022

processing....

Abstract and Introduction

Since 2012, the United States has reported a distinct syndrome of acute flaccid paralysis (AFP) with anterior myelitis, predominantly in children. This polio-like syndrome was termed acute flaccid myelitis (AFM). Australia routinely conducts AFP surveillance to exclude poliomyelitis. We reviewed 915 AFP cases in Australia for children <15 years of age during 2000‒2018 and reclassified a subset to AFM by using the US Council of State and Territorial Epidemiologists case definition. We confirmed 37 AFM cases by using magnetic resonance imaging findings and 4 probable AFM cases on the basis of cerebrospinal fluid pleocytosis. Nonpolio enteroviruses were detected in 33% of AFM cases from which stool samples were tested. Average annual AFM incidence was 0.07 cases/100,000 person-years in children <15 years of age. AFM occurred sporadically in Australia before 2010 but regularly since then, indicating sustained, albeit rare, clinical manifestation in children. The AFP surveillance system in Australia is well-positioned to identify future AFM cases.

Introduction

During 2012 and 2014, reports of a distinct syndrome of acute flaccid paralysis (AFP) with inflammation of the spinal cord restricted predominately to the gray matter occurred in children in the United States[1,2]. Clinicians referred to these patients as having a polio-like syndrome because stool samples were negative for polioviruses. To avoid confusion with poliomyelitis, clinicians called the syndrome acute flaccid myelitis (AFM). During August 2014, the US Centers for Disease Control and Prevention (CDC) put out a national call for cases of AFM and, during 2015, began passive surveillance for the condition under a standardized case definition[3]. By August 2021, a total of 665 cases were reported[4]. Outbreaks occurred biennially in late summer to autumn during 2014 (120 cases), 2016 (153 cases), and 2018 (238 cases)[5]. A smaller number of AFM cases were reported from Europe, Asia, South America, Africa, and Oceania[6]. The anticipated biennial outbreak in 2020 did not materialize, probably affected by coronavirus disease restrictions, such as mask wearing and physical distancing.

Children who have AFM show acute flaccid limb weakness, typically with asymmetric onset, affecting the arms more than the legs and proximal muscles more than distal muscles, with or without cranial nerve involvement[7]. Patients frequently have an acute antecedent illness, most commonly respiratory. Analysis of cerebrospinal fluid (CSF) often shows an increased leukocyte count, supporting an infectious etiology. Although uncommon, AFM can be life-threatening. The health of affected patients can deteriorate quickly; >50% of children in the United States are admitted to an intensive care unit, and 1/4 of these children require mechanical ventilation[3].

The standard for confirming a central nervous system viral infection is amplification of viral nucleic acid from CSF or detection of specific antibodies in the CSF[8,9]. Detection of viral nucleic acid can be insensitive because the virus might have already been cleared from the CSF between the period of illness onset and the paralysis that prompts a lumbar puncture[10]. The CDC detected nonpolio enteroviruses (NPEV) enterovirus D68 (EV-D68), enterovirus A71 (EV-A71), and coxsackievirus A16 in the CSF in a small number of patients, although NPEV were more often detected in specimens from other sites[3,11].

AFP Surveillance

AFP surveillance began in Australia during 1995 by the Australian Paediatric Surveillance Unit (APSU) as part of Australia’s commitment to the global eradication of poliomyelitis[12]. Every month, the APSU sends report cards to ≈1,500 pediatricians and other child health specialists in urban, rural, and remote regions, inquiring whether or not they have seen a newly diagnosed patient who has AFP and other selected conditions under surveillance[13]. Since 2000, the monthly response rate has been >90%. In late 2007, the Paediatric Active Enhanced Disease Surveillance (PAEDS) network was established in tertiary hospitals to identify children hospitalized because of AFP and to complement the existing APSU surveillance. The aim of PAEDS was to help maintain the annual detection rate of AFP for Australia (the World Health Organization [WHO] target is at least 1 nonpolio AFP case/100,000 children <15 years of age/year), improve stool collection (the WHO target for a polio-free country is 2 stool samples within 14 days of symptom onset for ≥80% of nonpolio AFP cases), and assist in excluding poliovirus infection. PAEDS nursing staff identify AFP cases by actively screening hospital admissions at sentinel sites and matching hospital data with the AFP case definition. The APSU and PAEDS network have research ethics committee approval, and both operate under a waiver of consent.

Stool samples from AFP cases are sent to the National Enterovirus Reference Laboratory (NERL), which performs virus culture for the isolation of poliovirus and screens specimens for enterovirus RNA and reverse transcription PCR. The Polio Expert Panel (PEP) is convened 6 times a year to review and classify cases as poliomyelitis, polio-compatible, or the most likely clinical diagnosis for nonpolio AFP cases by using clinical and laboratory data and expert judgement.

In the past few decades, many high-income countries, including the United States and the United Kingdom, have failed to meet the annual WHO AFP target because of progressive decreases in reported cases. With the apparent emergence of AFM, the PEP recognized that the longstanding AFP surveillance system of Australia, in a high-income setting, afforded a unique opportunity to retrospectively analyze existing data and apply the new AFM case definition to identify cases or clusters of AFM that might have occurred in Australia. We describe the epidemiology, clinical, and diagnostic characteristics of these AFM cases.