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A 25-year-old North American man came to Portugal on honeymoon. The week before, he had developed slowly progressive difficulty with both speaking and swallowing. At his wedding ceremony in the USA 2 days earlier, he had not been able to speak, saying “I do” using gestures, and could barely eat his own wedding cake. He deteriorated further after the wedding and by the following day he was unable to swallow.
Ten days before the onset of these symptoms, he had suffered a flu-like illness that resolved spontaneously over a couple of days. He was previously healthy, not taking any medication and not using any illicit drugs.
On examination, he was calm and alert, capable of following commands and could read and write which facilitated communication. He was anarthric, making only effortful guttural sounds and required nasogastric tube feeding because of severe dysphagia. He had bilateral ptosis and bilateral facial weakness. However, while his voluntary mouth opening was limited, it was normal when he yawned. His palatal movement was reduced but his gag reflex was intact, as was his jaw jerk. He could not cough voluntarily but had a reflex cough. He was unable to protrude or move his tongue from side to side. Ocular movements were normal and he had normal strength in his arms and legs with symmetrical tendon reflexes. …
▶ Videos 1 and 2 are published online only at http://jnnp.bmj.com/content/vol10/issue2
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