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Acute flaccid myelitis in a polio-like syndrome

A previously healthy 5-year-old boy was admitted for fever, vomiting, and dyspnea, evolving with masticatory automatism, areflexia, and asymmetric flaccid tetraparesis, with preserved sensitivity. Magnetic resonance imaging (MRI) of the spine showed areas with symmetrical T2 hypersignals bilaterally in the anterior horns of the spinal cord, without contrast enhancement, in a “snake eye” sign (Figure 1).

FIGURE 1:
Magnetic resonance imaging of the spine, T2 sagittal (A) and T2 axial (B). Bilaterally symmetric circular to ovoid foci of high T2-weighted signals in the anterior horn cells of the spinal cord compatible with the “snake eye” sign (arrows in B).

Lumbar puncture identified anti-Epstein Barr virus (EBV) IgG and IgM antibodies in the cerebrospinal fluid.

Acute flaccid myelitis, an inflammation of the spinal cord that usually occurs after a viral disease, is characterized by flaccid paralysis of one or more members and may be associated with back pain, decreased sensitivity, and dysfunction of cranial nerves11. Sarmast SN, Gowda VK, Ahmed M, Gv B, Benakappa A. Acute flaccid myelitis - Clustering of polio-like illness in the tertiary care centre in Southern India. J Trop Pediatr 2019;65(4):309-14.,22. Andersen EW, Kornberg AJ, Freeman JL, Leventer RJ, Ryan MM. Acute flaccid myelitis in childhood: a retrospective cohort study. Eur J Neurol. 2017;24(8):1077-83.. Poliovirus was the most common cause before widespread vaccination in the 1950s. Currently, other enteroviruses are primarily responsible, such as Coxsackie and enterovirus 71. MRI typically shows spinal cord involvement in the anterior horns of the gray substance (“snake eye” sign), affecting one or more spine segments.

EBV is the etiological agent of infectious mononucleosis, generally a benign and self-limiting disease, capable of causing complications in the central nervous system, such as encephalitis, cerebellitis, polyradiculomyelitis, cranial and peripheral neuropathies and myelitis, the latter being rare but the most common presentation in children33. Singh JR, Ibraheem K, Jain D, Yogendra K. Myelitis: A Rare Presentation of Epstein Barr Virus. J Assoc Physicians India. 2019;67(3):93-5.. Neuroimaging findings on MRI are characterized by hyperintense T2-weighted lesions, and involvement of the anterior medullary horns may be present, with a “snake eye” sign.

Treatment is with antivirals, such as acyclovir, and corticosteroids33. Singh JR, Ibraheem K, Jain D, Yogendra K. Myelitis: A Rare Presentation of Epstein Barr Virus. J Assoc Physicians India. 2019;67(3):93-5.. Most patients survive, but permanent neurological deficit and death can occur.

ACKNOWLEDGMENTS

We offer our deepest thanks to the institutions that provided technical support for the development and implementation of this study.

REFERENCES

  • 1
    Sarmast SN, Gowda VK, Ahmed M, Gv B, Benakappa A. Acute flaccid myelitis - Clustering of polio-like illness in the tertiary care centre in Southern India. J Trop Pediatr 2019;65(4):309-14.
  • 2
    Andersen EW, Kornberg AJ, Freeman JL, Leventer RJ, Ryan MM. Acute flaccid myelitis in childhood: a retrospective cohort study. Eur J Neurol. 2017;24(8):1077-83.
  • 3
    Singh JR, Ibraheem K, Jain D, Yogendra K. Myelitis: A Rare Presentation of Epstein Barr Virus. J Assoc Physicians India. 2019;67(3):93-5.

Publication Dates

  • Publication in this collection
    13 Nov 2020
  • Date of issue
    2021

History

  • Received
    06 May 2020
  • Accepted
    29 May 2020
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