title: Critical illness polyneuromyopathy in a child with severe demyelinating myelitis.
abstract: We report a child presenting with severe demyelinating myelitis complicated with critical illness polyneuropathy. This previously healthy 8-month-old boy presented with acute superior limb weakness, absent tendon reflexes, and respiratory failure. Spinal magnetic resonance imaging showed an extensive cervical demyelinating lesion. Spinal cord trauma was suspected and high doses of dexamethasone were administered. Electromyography and nerve conduction studies showed absence of compound muscle action potentials and sural nerve sensory action potential, which was suggestive of a severe Guillain-Barré syndrome. However, intravenous immunoglobulins did not induce any improvement. Afterward, sural nerve biopsy showed a mild neuropathy, but muscle biopsy revealed abnormalities compatible with severe critical illness myopathy. After 5 months of evolution without improvement, the patient died following withdrawal of life support therapy. This case highlights the possible occurrence of critical illness polyneuromyopathy when treatment with corticosteroids are used in patients with acute demyelinating myelitis.
authors.affiliation: Division of Pediatric Critical Care, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada.
authors.forename: Tanja
authors.initials: T
authors.lastname: Adamovic
authors.forename: Ariane
authors.initials: A
authors.lastname: Willems
authors.forename: Michel
authors.initials: M
authors.lastname: Vanasse
authors.forename: Guy
authors.initials: G
authors.lastname: D'Anjou
authors.forename: Yves
authors.initials: Y
authors.lastname: Robitaille
authors.forename: Catherine
authors.initials: C
authors.lastname: Litalien
authors.forename: France
authors.initials: F
authors.lastname: Gauvin
mesh_terms: D003907:Dexamethasone / Q000009:adverse effects / Q000627:therapeutic use; D004576:Electromyography; D017809:Fatal Outcome; D005938:Glucocorticoids / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology / Q000648:ultrastructure; D009188:Myelitis, Transverse / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D009431:Neural Conduction; D011115:Polyneuropathies / Q000150:complications* / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D003907:Dexamethasone