Key result
Intravenous immunoglobulin administration partially improved right lower limb muscle strength in a 5-year-old girl who developed Hopkins syndrome following her first asthma attack associated with enterovirus D68.
Case Report (n=1)
This case highlights the importance of provocation tests for airway hyper-responsiveness in children with acute flaccid paralysis mimicking Hopkins syndrome after respiratory distress, and suggests a link with enterovirus D68.
May expand HS differential to post-first asthma with EV-D68; leaves open causality and need for prospective confirmation.
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. 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/kg 2 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. 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.
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Hayashi et al. (2018) conducted a case report in Hopkins syndrome following bronchial asthma associated with enterovirus D68 (n=1). Intravenous immunoglobulin was evaluated on Muscle strength improvement. Intravenous immunoglobulin administration partially improved right lower limb muscle strength in a 5-year-old girl who developed Hopkins syndrome following her first asthma attack associated with enterovirus D68.
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