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Critical Illness Polyneuromyopathy in a Child with Severe Demyelinating Myelitis

Division of Pediatric Critical Care, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

Ariane Willems, MD

Division of Pediatric Critical Care, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

Michel Vanasse, MD

Division of Neurology, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

Guy D'Anjou, MD

Division of Neurology, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

Yves Robitaille, MD

Department of Pathology, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

Catherine Litalien, MD

Division of Pediatric Critical Care, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

France Gauvin, MD, MSc

Division of Pediatric Critical Care, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada, france_gauvin{at}ssss.gouv.qc.ca

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.

Key Words: critical illness • polyneuropathies • transverse myelitis • neuromuscular diseases • demyelinating disease

This version was published on June 1, 2009

Journal of Child Neurology, Vol. 24, No. 6, 758-762 (2009)
DOI: 10.1177/0883073808330166


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