PDF(Pubmed)
Abstract：
BACKGROUND: Hopkins syndrome (HS) is a rare disorder presenting with acute flaccid paralysis of the limbs following an asthma attack. Neurologists encounter a diagnostic challenge if patients without a history of bronchial asthma develop neurologic features mimicking HS following acute respiratory distress. We report a case of HS occurring after a first episode of bronchial asthma associated with enterovirus D68 infection.
METHODS: A 5-year-old girl developed acute respiratory distress. On the fourth hospital day, both her legs became paralyzed except for slight muscle contraction in the right lower limb. Tendon reflexes in the lower limbs were diminished and there was a positive Babinski sign on the right. Sensation was normal in all modalities, and there was no uro-rectal disturbance. Spinal magnetic resonance imaging identified T2-hyperintense lesions with spinal cord edema, mainly involving the bilateral T11 to L1 anterior horns, with left side dominance extending to the left posterior horn. The neurological and neuro-radiological findings of our case were suggestive of HS; however, she had no history of bronchial asthma. An acetylcholine inhalation challenge eventually proved the presence of reversible airway hyper-responsiveness, allowing us to diagnose HS. We identified enterovirus D68 in the patient\'s intratracheal aspirates using a sensitive polymerase chain reaction assay. Intravenous immunoglobulin administrations at 2 g/kg2 for 5 consecutive days were repeated every month up to four times. After these treatments, the muscle strength of her right lower limb slightly improved while her left lower leg remained completely paralyzed.
CONCLUSIONS: This case emphasizes the importance of provocation tests to reveal the presence of airway hyper-responsiveness when a child shows neurological signs mimicking HS following acute respiratory distress. Furthermore, the present case suggests a possible link between HS and acute flaccid paralysis following lower respiratory tract infection by enterovirus D68.
METHODS: A 5-year-old girl developed acute respiratory distress. On the fourth hospital day, both her legs became paralyzed except for slight muscle contraction in the right lower limb. Tendon reflexes in the lower limbs were diminished and there was a positive Babinski sign on the right. Sensation was normal in all modalities, and there was no uro-rectal disturbance. Spinal magnetic resonance imaging identified T2-hyperintense lesions with spinal cord edema, mainly involving the bilateral T11 to L1 anterior horns, with left side dominance extending to the left posterior horn. The neurological and neuro-radiological findings of our case were suggestive of HS; however, she had no history of bronchial asthma. An acetylcholine inhalation challenge eventually proved the presence of reversible airway hyper-responsiveness, allowing us to diagnose HS. We identified enterovirus D68 in the patient\'s intratracheal aspirates using a sensitive polymerase chain reaction assay. Intravenous immunoglobulin administrations at 2 g/kg2 for 5 consecutive days were repeated every month up to four times. After these treatments, the muscle strength of her right lower limb slightly improved while her left lower leg remained completely paralyzed.
CONCLUSIONS: This case emphasizes the importance of provocation tests to reveal the presence of airway hyper-responsiveness when a child shows neurological signs mimicking HS following acute respiratory distress. Furthermore, the present case suggests a possible link between HS and acute flaccid paralysis following lower respiratory tract infection by enterovirus D68.
摘要：
背景：Hopkins综合征（HS）是一种罕见的疾病，在哮喘发作后表现为四肢急性弛缓性麻痹。如果没有支气管哮喘病史的患者在急性呼吸窘迫后出现模仿HS的神经系统特征，神经学家将面临诊断挑战。我们报告了一例与肠道病毒D68感染相关的支气管哮喘首次发作后发生的HS。
方法：一名5岁女孩出现急性呼吸窘迫。在第四个住院日，除了右下肢肌肉轻微收缩外，她的双腿都瘫痪了。下肢的肌腱反射减弱，右侧有一个积极的巴宾斯基征。所有模式的感觉都是正常的，也没有尿路直肠紊乱.脊柱磁共振成像发现T2-高强度病变伴脊髓水肿，主要累及双侧T11至L1前角，左侧优势延伸到左后角。我们病例的神经和神经放射学检查结果提示HS；然而，无支气管哮喘病史。乙酰胆碱吸入攻击最终证明存在可逆的气道高反应性，允许我们诊断HS。我们使用敏感的聚合酶链反应测定法在患者的气管内抽吸物中鉴定了肠道病毒D68。连续5天以2g/kg2静脉内免疫球蛋白给药每月重复多达4次。经过这些治疗,她的右下肢肌肉力量略有改善，而她的左小腿仍然完全瘫痪。
结论：本案例强调了当儿童在急性呼吸窘迫后表现出模仿HS的神经系统体征时，激发试验揭示气道高反应性的重要性。此外，本病例提示HS与肠道病毒D68感染下呼吸道后急性弛缓性麻痹之间可能存在联系.
方法：一名5岁女孩出现急性呼吸窘迫。在第四个住院日，除了右下肢肌肉轻微收缩外，她的双腿都瘫痪了。下肢的肌腱反射减弱，右侧有一个积极的巴宾斯基征。所有模式的感觉都是正常的，也没有尿路直肠紊乱.脊柱磁共振成像发现T2-高强度病变伴脊髓水肿，主要累及双侧T11至L1前角，左侧优势延伸到左后角。我们病例的神经和神经放射学检查结果提示HS；然而，无支气管哮喘病史。乙酰胆碱吸入攻击最终证明存在可逆的气道高反应性，允许我们诊断HS。我们使用敏感的聚合酶链反应测定法在患者的气管内抽吸物中鉴定了肠道病毒D68。连续5天以2g/kg2静脉内免疫球蛋白给药每月重复多达4次。经过这些治疗,她的右下肢肌肉力量略有改善，而她的左小腿仍然完全瘫痪。
结论：本案例强调了当儿童在急性呼吸窘迫后表现出模仿HS的神经系统体征时，激发试验揭示气道高反应性的重要性。此外，本病例提示HS与肠道病毒D68感染下呼吸道后急性弛缓性麻痹之间可能存在联系.
