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A 22-year-old man presented with progressive weakness and sensory loss in the left arm and leg for 1 year, imbalance while walking for 6 months, oscillopsia, dysphagia and urinary urgency for 3 months. Examination revealed a short neck, low posterior hairline and scoliosis. Downbeat nystagmus (DBN) was appreciated in the primary position of gaze. It increased in amplitude when the patient looked down and out, the so-called Daroff's sign1 (see online supplementary video). He also had lid nystagmus, right partial Horner's syndrome, lower cranial nerve palsies, bipyramidal …
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