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Critical illness polyneuromyopathy in a child with severe demyelinating myelitis
Tanja Adamovic1, Ariane Willems, Michel Vanasse
1Division of Pediatric Critical Care, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada.
Insights
This case report details an infant with demyelinating myelitis who developed critical illness polyneuropathy after corticosteroid treatment. The condition led to severe weakness and respiratory failure, ultimately proving fatal.
Area of Science:
- Pediatric Neurology
- Neuromuscular Disorders
Background:
- Acute demyelinating myelitis can present with severe neurological deficits in infants.
- Critical illness polyneuropathy and myopathy are serious complications in critically ill patients.
Observation:
- An 8-month-old boy with acute demyelinating myelitis developed limb weakness and respiratory failure.
- Initial treatment with corticosteroids was administered due to suspected spinal cord trauma.
- Electromyography and nerve conduction studies suggested Guillain-Barré syndrome, but treatment was ineffective.
Findings:
- Muscle biopsy revealed severe critical illness myopathy, while nerve biopsy showed mild neuropathy.
- The patient experienced no improvement and succumbed after 5 months.
- This suggests critical illness polyneuromyopathy may occur secondary to corticosteroid use in acute demyelinating myelitis.
Implications:
- Corticosteroid therapy in acute demyelinating myelitis may precipitate critical illness polyneuromyopathy in infants.
- This case underscores the need for careful monitoring and consideration of alternative treatments.
- Further research is needed to elucidate the mechanisms and optimal management strategies.
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.
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