Neuropediatrics 2019; 50(S 01): S1-S10
DOI: 10.1055/s-0039-1685426
Oral Communications
Georg Thieme Verlag KG Stuttgart · New York

Acute Flaccid Myelitis: An Emerging Disease with Several Challenges. A Retrospective Study of an Italian Cohort.

S. Bova
1   Department of Child Neurology, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie)
,
S. Olivotto
1   Department of Child Neurology, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie)
,
D. Tonduti
1   Department of Child Neurology, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie)
,
E. Alfei
1   Department of Child Neurology, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie)
,
S. Masnada
1   Department of Child Neurology, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie)
,
D. Dilillo
2   Department of Pediatrics, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie
,
V. Colombo
2   Department of Pediatrics, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie
,
C. Parazzini
3   Department of Pediatric Radiology and Neuroradiology, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie
,
S. Ferrario
4   Department of Pediatric Intensive Care Unit, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie
,
G. Bernardi
5   Laboratory of Clinical and Genetical Pathology, IRCCS Istituto Neurologico C Besta, Milano, Italie
,
G. Zuccotti
2   Department of Pediatrics, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie
,
P. Veggiotti
1   Department of Child Neurology, ASST Fatebenefratelli Sacco V. Buzzi Children’s Hospital, University of Milan, Milano, Italie)
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Publikationsdatum:
20. März 2019 (online)

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Background and Purpose: Acute flaccid myelitis (AFM) is a polio-like illness defined by the acute onset of flaccid paralysis associated with longitudinal lesion in the gray matter of the cord on spinal MRI. A connection with EV-D68 infection have increased in recent years and the evidence for a causal link is growing but a definite etiology has yet to be established. There were clusters of AFM in U.S. and Europe in 2014, 2016, and 2018. While most reports suggest some improvement, long‐term outcomes are thought to be poor, although there are limited data on the natural history of this condition. The efficacy of intravenous immunoglobulin (IVIG) has not been systematically studied even though IVIG are the only treatment that has been found to have some efficacy in preventing progression to neuroinvasive disease.

Methods: This is a retrospective cohort study. We describe clinical features, investigation findings, and outcome (follow-up from 3 to 27months) of a series of five children with AFM (3 months–12 years) observed in our hospital (2016 and 2018).

Results: In three children, initial symptoms were four-limb weakness with hypotonia followed by flaccid paralysis. Two children presented with rigor and back pain without severe weakness. Febrile illness, with respiratory (3/5) or gastrointestinal (1/5) symptoms preceded (3–14 days), the onset of neurological symptoms. One child required intensive care support because of respiratory paralysis and myocarditis. Cerebrospinal fluid examination showed pleocytosis with raised cerebrospinal fluid protein in all, intrathecal synthesis of IgM was documented in 3/5. Spinal MRI documented typical findings in the acute phase and normalized during the follow-up; brainstem and cerebellar involvement was documented in 2/5 children. EV-D68 infection was not documented. Four children showed complete clinical recovery at the last neurological assessment. One has persistent severe flaccid paraparesis.

Conclusion: AFM is a rare but severe disease, and we have much to learn about etiology, treatment options, and clinical and diagnostic definition. Case identification is dependent on awareness among clinicians. Adequate viral diagnostics on respiratory samples and the capability of laboratories to type EVs are crucial in order to identify case related to EV-D68 infection.