1 - Infectious Diseases Department, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, EPE, Portugal;
2 - Neurology Department, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, EPE, Portugal;
3 - Radiology Department, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Portugal
- congresso internacional, comunicação oral, 13th Excellence in Pediatrics 2021
Resumo:
Background: Predominantly grey matter involvement in longitudinal extensive spinal cord lesion is an acute predictor of either progressive presentation of common autoimmune disorders or insufficiently acknowledged entities such as paraneoplastic syndromes and peri-infectious acute flaccid myelitis. Environmental toxins and genetic disorders may be involved in the manifestation of this disease.
Case Presentation: A previously healthy, 11-month-year-old girl was admitted with a rapidly progressive arreflexic tetraparesis and worsening respiratory distress. Two days earlier, she had symptoms of upper respiratory illness and high fever and on the previous day before admission, she refused to walk and showed upper right arm paresis. Cerebrospinal fluid examination showed a normal cell count with elevated protein (75mg/dL). Neurotropic virus by multiplex-PCR was negative and immunoelectrophoresis revealed moderated blood-brain barrier disruption without intrathecal IgG production. Magnetic resonance imaging (MRI) showed longitudinally extensive homogeneous medullar lesion from D8 level to the medulla oblongata with signal alteration mainly localized in the central grey matter. Adenovirus was identified in respiratory secretions by quantitative multiplex-PCR testing. Anti-aquaporin 4 (AQP-4) antibodies, anti-myelin oligodendrocyte glycoprotein (MOG) antibodies and anti-N-methyl D-aspartate (NMDA) receptor (anti-NMDAR) antibodies were all negative. She was treated with ceftriaxone, ciprofloxacin, acyclovir, cidofovir and fluoxetine. Two weeks later, MRI showed new lesions in frontal and parietal left cortex consistent with encephalitis. Due to this clinical severity, intravenous immunoglobulin, pulses of methylprednisolone and plasmapheresis were administered. She was on mechanical ventilation for 20 days and required parenteral nutrition due to malnutrition. She started on intense rehabilitation with physical and occupational therapy with some improvement and her condition evolved to asymmetric spastic tetraparesis with mainly upper right arm and lower limbs involvement. HIV and primary immunodeficiency diseases were excluded.
Learning Points Discussion: Grey matter spinal cord lesion is a rare phenomenon and presents a diagnostic challenge in paediatrics where infectious aetiologies should be considered. Adenovirus can directly invade the anterior horn cells of the spinal cord, it has been reported neurological complications including aseptic meningitis, encephalopathy, encephalitis and myelitis. Regarding this potentially devastating condition with variable outcome, aggressive immunomodulatory treatment should be maximized early in the disease course, where time is of the essence.
Palavras-chave: adenovírus, grey matter myelitis