New Cluster of Acute Flaccid Myelitis in Western
Pennsylvania
Natan Cramer, MD; Neil Munjal, MD; Danielle Ware, DO; Sriram Ramgopal, MD; Dennis Simon, MD;
Megan C. Freeman, MD, PhD; Marian G. Michaels, MD, MPH; Christopher Stem, MD; Kavita Thakkar, MD;
John V. Williams, MD; Ashok Panigrahy, MD; Desiree N. W. Neville, MD; Sylvia Owusu-Ansah, MD, MPH*
*Corresponding Author. E-mail: sylvia.owusuansah@chp.edu.
Acute flaccid myelitis is a debilitating illness characterized by acute onset of limb weakness, with one or more spinal segments
displaying magnetic resonance imaging–confirmed gray matter lesions. Since the first outbreak in 2014, tracking by the Centers for
Disease Control and Prevention has demonstrated biennial epidemics in the United States, with a current outbreak occurring in 2018.
The cases of 3 children with acute flaccid myelitis who were initially thought to have common nonneurologic diagnoses are presented.
Emergency physicians need to be vigilant to recognize the subtleties of acute flaccid myelitis because the illness progression is rapid
and therapy is nuanced. [Ann Emerg Med. 2019;-:1-6.]
0196-0644/$-see front matter
Copyright © 2019 by the American College of Emergency Physicians.
https://doi.org/10.1016/j.annemergmed.2019.01.024
INTRODUCTION
Viral-mediated pathogenesis for acute flaccid myelitis
has been suggested, given similarities to poliovirus,
associations with enterovirus D68, and seasonal
variation.
1,2
There have been a total of 440 cases recorded,
with 116 in 2018 alone.
2
Acute flaccid myelitis typically
begins with a constitutional prodrome and most patients
present in early childhood.
1,3
Weakness develops and is
often asymmetric, with one or more limbs involved.
Diminished reflexes are appreciated.
1
The site most affected
is the cervical spine.
1
Gray matter lesions are initially
diffuse but become more localized to the anterior horn
cells. Enhancement is observed in the minority of
patients.
1,4
Cerebrospinal fluid pleocytosis is typical.
1
A
high degree of suspicion is required to promptly identify
cases of acute flaccid myelitis in patients with a
constitutional prodrome, focal motor deficit, and decreased
reflexes to prevent additional morbidity and establish early
neurorehabilitative efforts.
CASE REPORTS
On September 28 to 30, 2018, 3 previously healthy
immunized children younger than 5 years and from the
Pittsburgh area presented with acute flaccid myelitis
(Figure 1 and Table 1).
Case 1
A 3-year-old boy presented to the emergency department
(ED) with 3 days of fever, fatigue, and pharyngitis, and 24
hours of vomiting, with generalized weakness. Vital signs
were notable for fever and tachycardia. The patient was
unable to sit up but was able to move all extremities. He was
mildly hypoglycemic but was noted to have improved
strength after intravenous fluids. The patient was admitted
with the assumption that the weakness was a result of sepsis,
dehydration, and hypoglycemia. Within hours of admission,
he was noted to have decreased spontaneous movements.
Inpatient neurology consultation demonstrated weakness
in all extremities (power grade 1/5 in the left upper
extremity and 3/5 in all other extremities). There was no
difference in proximal or distal muscle strength. Light touch
sensation was intact. Deep tendon reflexes were 1þ
throughout and symmetric. His examination worsened over
10 hours from admission to flaccid quadriplegia and
respiratory failure, prompting emergency intubation.
Magnetic resonance image (MRI) demonstrated increased
T2 signal in the central gray matter of the cervical spine
and thoracic spine (vertebrae levels 7 to 10), as well as the
dorsal pons and midbrain. Because of initial concern for
transverse myelitis, intravenous methylprednisolone (30
mg/kg) was administered. On further deliberation, acute
flaccid myelitis was diagnosed and human intravenous
immunoglobulin (2 g/kg) was administered 5 days after
prodromal onset. Cerebrospinal fluid obtained after imaging
and steroid administration was notable for a lymphocytic
pleocytosis. He was transferred to inpatient rehabilitation
on hospital day 9. At 2 weeks after transfer, he
demonstrated minimal improvement in head control but
otherwise continued to have severe functional motor
impairment.
Volume -, no. - : - 2019 Annals of Emergency Medicine 1
PEDIATRICS/CASE REPORT