Adamovic Tanja, Willems Ariane, Vanasse Michel, D'Anjou Guy, Robitaille Yves, Litalien Catherine, Gauvin France
Division of Pediatric Critical Care, Department of Pediatrics, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada.
J Child Neurol. 2009 Jun;24(6):758-62. doi: 10.1177/0883073808330166. Epub 2009 Mar 4.
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.
我们报告了一名患有严重脱髓鞘性脊髓炎并伴有危重病性多发性神经病的儿童。这名之前健康的8个月大男孩出现急性上肢无力、腱反射消失和呼吸衰竭。脊髓磁共振成像显示广泛的颈髓脱髓鞘病变。怀疑有脊髓损伤,给予了高剂量地塞米松。肌电图和神经传导研究显示复合肌肉动作电位和腓肠神经感觉动作电位缺失,提示严重吉兰-巴雷综合征。然而,静脉注射免疫球蛋白并未带来任何改善。之后,腓肠神经活检显示轻度神经病变,但肌肉活检显示与严重危重病性肌病相符的异常。经过5个月病情无改善的演变,患者在撤掉生命支持治疗后死亡。该病例突出了在急性脱髓鞘性脊髓炎患者中使用皮质类固醇治疗时可能发生危重病性多神经肌病。