Critical illness polyneuromyopathy in a child with severe demyelinating myelitis

Autor(es): Adamovic Tanja,Willems Ariane,Vanasse Michel,D'Anjou Guy,Robitaille Yves,Litalien Catherine,Gauvin France

Resumo: 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.

Palavras-Chave: Critical illness; Polyneuropathies; Transverse myelitis; Neuromuscular diseases; Demyelinating disease

Imprenta: Journal of Child Neurology, v. 24, n. 6, p. 758-762, 2009

Identificador do objeto digital: 10.1177/0883073808330166

Descritores: Guillain-Barre Syndrome - Cytopathology ; Guillain-Barre Syndrome - Pathogenesis ; Guillain-Barre Syndrome - Proteins ; Guillain-Barre Syndrome - Antibodies ; Guillain-Barre Syndrome - Public health

Data de publicação: 2009