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Maria Soto-Maior Costa1; Rita Silva2; Gabriela Pereira3; Maria João Brito1

1- Unidade de Infecciologia Pediátrica, Área da Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central
2- Unidade de Neurologia Pediátrica, Área da Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central
3- Unidade de Cuidados Intensivos Pediátricos, Área da Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central

11th Excellence in Pediatrics. 6 de dezembro de 2019, Copenhaga, Dinamarca. Comunicação oral

Introduction: Acute flaccid myelitis (AFM) is a rare and recently described condition characterized by rapid progressive asymmetric weakness of the limbs, together with grey matter spinal cord lesions on magnetic resonance imaging (MRI). Recent outbreaks are predominantly associated with enterovirus D68 but others agents may be implicated.
Case description: A previously healthy 18-months-old girl presented with five days of progressive lethargy and asymmetric weakness of the lower limbs with constipation and urinary retention. She had high fever, cough and vomiting. Previous history of hand-foot-and-mouth disease six weeks earlier. Neurologic exam revealed global hypotonia, as she could not hold her head completely or sit without support, with diminished strength of the lower limbs, specially on the right (score 3/5), hyporeflexia and equivocal plantar responses. Spinal MRI showed cervical and brainstem lesions suggestive of myelitis and rhombencephalitis. Cerebrospinal fluid revealed pleocytosis 20.8/μL, with normal protein levels. She was admitted to the Pediatric Intensive Care Unit and started ceftriaxone, acyclovir, immunoglobulin 1g/kg for 2 days and methylprednisolone 30mg/kg/day for 5 days. Despite being negative in the cerebrospinal fluid, PCR for enterovirus was positive in respiratory secretions and stool. The remaining laboratory work was negative, including anti-aquaporin 4 and anti-MOG antibodies. Cerebrospinal fluid was negative for oligoclonal bands. There was no evidence of intrathecal synthesis. The child remained with spontaneous ventilation throughout the entire hospitalisation, rapidly recovered from bowel and urinary dysfunction, and presented stable. She underwent physical rehabilitation and feeding training, with gradual improvement of the motor manifestations until discharge. Six weeks after discharge, the girl showed some difficulty to stand up without using the upper limbs, but otherwise with a normal neurologic examination. Cell culture of enterovirus in stool was performed for viral identification.
Conclusions: There is no consensus about the management of AFM; therefore, most approaches are based on case reports and expert opinion. Aside the empiric antibiotic and antiviral coverage, most adopted regimens include immunoglobulin, glucocorticoids and plasma exchange, but none of these therapies has proven to be effective. It is essential to continue improving knowledge on AFM’s pathophysiology, in order to allow early recognition of the symptoms by clinicians and develop adequate treatment strategies.

Keywords: acute flaccid myelitis, acute myelopathy, enterovirus