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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 …
Contributors SoS and AD collected information and prepared the draft. AG, SaS and VP edited and reviewed the manuscript. All authors reviewed, edited and approved the final version.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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