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Images in Neurology
May 28, 2021

Ondine’s Curse and Trismus in Association With Kelch-like Protein-11 Autoimmunity

Author Affiliations
  • 1Center for Sleep Medicine, Mayo Clinic College of Medicine and Science, Rochester, Minnesota
  • 2Department of Neurology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota
  • 3Olmsted Medical Center, Rochester, Minnesota
  • 4Division of Pulmonary Critical Care, Mayo Clinic College of Medicine and Science, Rochester, Minnesota
  • 5Department of Medicine, Mayo Clinic College of Medicine and Science, Rochester, Minnesota
  • 6Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota
JAMA Neurol. 2021;78(8):1016-1017. doi:10.1001/jamaneurol.2021.1557

A 45-year-old man initially presented with vertigo that resolved with low-dose oral prednisone. Three months later, he developed vertigo, binocular diplopia, dysarthria, dysphagia, and tinnitus. He reported difficulties opening his jaw, consistent with trismus (Video), and 18- to 23-kg weight loss over 6 months (body mass index [calculated as weight in kilograms divided by height in meters squared] of 23). Examination revealed dysarthria, spontaneous vertical nystagmus, bilateral ptosis, facial weakness, facial sensory deficits, trismus, and gait instability.

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