Clinical Subpopulations in a Sample of North American Children Diagnosed With Acute Flaccid Myelitis, 2012-2016

Elrick, Matthew J.; Gordon-Lipkin, Eliza; Crawford, Thomas O.; Haren, Keith Van; Messacar, Kevin; Thornton, Nicole; Dee, Elizabeth; Voskertchian, Annie; Nance, Jessica; Muñoz, Laura S.; Gorman, Mark; Benson, Lina; Thomas, David L.; Pardo, Carlos A.; Milstone, Aaron M.; Duggal, Priya

doi:10.1001/jamapediatrics.2018.4890

Cited by 50 publications

(65 citation statements)

References 22 publications

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“…Patients reach their neurological nadir within hours to days. 34 Hyperacute onset (< 1 hour) should prompt consideration of an alternative diagnosis such as spinal cord stroke. 34 Respiratory failure requiring intubation and mechanical ventilation is common in AFM.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…34 Hyperacute onset (< 1 hour) should prompt consideration of an alternative diagnosis such as spinal cord stroke. 34 Respiratory failure requiring intubation and mechanical ventilation is common in AFM. 35 Intubation may also be required in patients with difficulty managing secretions due to bulbar weakness.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…In the U.S., the CDC has outlined a case definition designed to identify suspected cases of AFM; however, this definition is known to be nonspecific and captures other causes of acute flaccid paralysis. 34 Several conditions can cause a similar clinical syndrome to AFM, and it is essential that clinicians recognize the differentiating features (►Table 3). Guillain-Barre syndrome is the most common cause of acute flaccid paralysis, and patients often experience a preceding infection.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…Abnormalities of the spinal cord gray matter are identifiable on the initial MRI in all patients with AFM, regardless of the length of time elapsed between the onset of neurological symptoms and the imaging study. 34,35,[38][39][40] This being said, it is important to note that abnormalities can be relatively subtle with early imaging, and worsen over the initial days after presentation. T2 hyperintensity of the spinal cord gray matter with associated edema is the characteristic abnormality seen in AFM (►Fig.…”

Section: Investigations Mri Findingsmentioning

confidence: 99%

“…Typical electrophysiological changes include diminished or absent compound motor action potentials, diminished or absent Fwaves, and reduced recruitment of voluntary motor potentials followed by development of fibrillations or positive sharp waves. 6,10,12,34,42 Sensory abnormalities should be absent or minimal. 34 These findings can be interpreted as demonstrating a motor neuronopathy or neuropathy.…”

Section: Electrophysiology Findingsmentioning

confidence: 99%

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Acute Flaccid Myelitis: A Clinical Review

Murphy

Pardo

2020

Semin Neurol

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Acute flaccid myelitis (AFM) is an emerging disorder primarily affecting children that is characterized by acute flaccid paralysis accompanied by abnormalities of the spinal cord gray matter on magnetic resonance imaging. In most cases, prodromal fever or respiratory symptoms occur, followed by acute-onset flaccid limb weakness. Respiratory, axial, bulbar, facial, and extraocular muscles may also be affected. The clinical manifestations have been described as “polio-like,” due to striking similarities to cases of poliomyelitis. The primary site of injury in AFM is the anterior horn cells of the spinal cord, resulting in a motor neuronopathy. Seasonal peaks of cases have occurred in the United States every 2 years since 2012. However, AFM remains a rare disease, which can make it challenging for physicians to recognize and differentiate from other causes of acute flaccid paralysis such as Guillain–Barre syndrome, spinal cord stroke, and transverse myelitis. Epidemiological evidence suggests that AFM is linked to a viral etiology, with nonpolio enteroviruses (in particular enterovirus D68) demonstrating a plausible association. The epidemiology, possible etiological factors, clinical features, differential diagnosis, treatment, and outcomes of AFM are discussed in this review.

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Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

Section: Investigations Mri Findingsmentioning

confidence: 99%

Section: Electrophysiology Findingsmentioning

confidence: 99%

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Acute Flaccid Myelitis: A Clinical Review

Murphy

Pardo

2020

Semin Neurol

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Comparison of Spinal Cord Magnetic Resonance Imaging Features Among Children With Acquired Demyelinating Syndromes

Fadda

O’Mahony

et al. 2021

JAMA Netw Open

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IMPORTANCEThe recognition of magnetic resonance imaging (MRI) features associated with distinct causes of myelitis in children is essential to guide investigations and support diagnostic categorization. OBJECTIVE To determine the clinical and MRI features and outcomes associated with spinal cord involvement in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), multiple sclerosis (MS), and seronegative monophasic myelitis. DESIGN, SETTING, AND PARTICIPANTS In this cohort study, participants were recruited between 2004 and 2017 through the multicenter Canadian Pediatric Demyelinating Disease Study, which enrolled youth younger than 18 years presenting within 90 days of an acquired demyelinating syndrome. Of the 430 participants recruited, those with lesions on available spine MRI and anti-MOG testing performed on archived samples obtained close to clinical presentation were selected. Participants with poor-quality images and final diagnoses of nondemyelinating disease, antiaquaporin 4 antibody positivity, and relapsing seronegative myelitis were excluded. Data analysis was performed from December 2019 to November 2020. MAIN OUTCOMES AND MEASURES Spinal cord involvement was evaluated on 324 MRI sequences, with reviewers blinded to clinical, serological, and brain MRI findings. Associated clinical features and disability scores at 5 years of follow-up were retrieved. Results were compared between groups. RESULTS A total of 107 participants (median [IQR] age at onset, 11.14 [5.59-13.39] years; 55 girls [51%]) were included in the analyses; 40 children had MOGAD, 21 had MS, and 46 had seronegative myelitis. Longitudinally extensive lesions were very common among children with MOGAD (30 of 40 children [75%]), less common among those with seronegative myelitis (20 of 46 children [43%]), and rare in children with MS (1 of 21 children [5%]). Axial gray matter T2-hyperintensity (ie, the H-sign) was observed in 22 of 35 children (63%) with MOGAD, in 14 of 42 children (33%) with seronegative myelitis, and in none of those with MS. The presence of leptomeningeal enhancement was highly suggestive for MOGAD (22 of 32 children [69%] with MOGAD vs 10 of 38 children [26%] with seronegative myelitis and 1 of 15 children [7%] with MS). Children with MOGAD were more likely to have complete lesion resolution on serial images (14 of 21 children [67%]) compared with those with MS (0 of 13 children). CONCLUSIONS AND RELEVANCEThese findings suggest that several features may help identify children at presentation who are more likely to have myelitis associated with MOGAD. Prominent involvement of gray matter and leptomeningeal enhancement are common in pediatric MOGAD, although the pathological underpinning of these observations requires further study.

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Description of Restrictively Defined Acute Flaccid Myelitis

Berciano

Benito

2019

JAMA Pediatr

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uncomplicated Kawasaki disease (KD) who had previously normal coronary artery dimensions by echocardiography at diagnosis and at 1 to 2 weeks of illness. Maccora et al express concern about the 1.7% of our analysis cohort who had z scores at 6 weeks of more than 2.0. Although dimensions in the patients were present at 4 to 6 weeks, the degree of coronary artery dilation was mild at best and did not persist. Of these 8 patients, most 1 had a follow-up z score of 2.0 to 2.4, which would not be considered enlarged according to the 2017 American Heart Association Kawasaki Disease Guidelines. 2 Of the remaining 3 patients, the coronary artery dimensions normalized in 2 children at late follow-up, and the third patient had minimal dilation (z score of 2.1); again, not considered clinically concerning based on the 2017 American Heart Association guidelines. Indeed, the 2017 KD guidelines designate early aspirin use (first 4-6 weeks of illness) in patients with mild coronary artery dilation (z score ≥2.0 and <2.5) as a class IIa recommendation with Level of Evidence C (ie, consensus of experts and standard of care, but no clinical trials) and allowed for their discharge from care after 1 year if their coronary arteries normalize. 2 To make a change in guidelines, the findings in our study would need to be validated in other populations and settings. With these data, the risk/benefit ratio of the 6-week echocardiogram and continued low-dose aspirin therapy in this subgroup of patients with KD would need to be reconsidered by a guideline committee of experts. Indeed, guidelines are periodically updated precisely because newly published data continue to define best medical practices. In addition to our published article, 1 data suggest that baseline echo findings are the most reliable predictors of significant coronary artery complications in KD 3 and that thrombotic complications on longterm follow-up are exceedingly rare in patients even with small aneurysms (M.B.F. Son, MD, et al; unpublished data; 2018). For now, we reiterate our very strong support for obtaining highquality echocardiographic imaging of the coronary arteries in the earliest weeks after KD diagnosis, when coronary artery dimensions may be progressively dilating and additional antiinflammatory therapies can potentially prevent progression to significant coronary complications.

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Clinical Subpopulations in a Sample of North American Children Diagnosed With Acute Flaccid Myelitis, 2012-2016

Cited by 50 publications

References 22 publications

Acute Flaccid Myelitis: A Clinical Review

Acute Flaccid Myelitis: A Clinical Review

Comparison of Spinal Cord Magnetic Resonance Imaging Features Among Children With Acquired Demyelinating Syndromes

Description of Restrictively Defined Acute Flaccid Myelitis

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