52yo male with multiple lower cranial neuropathies Teaching NeuroImages Neurology Resident and Fellow Section by Nathaniel M. Schuster, Stellios Karnezis,

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52yo male with multiple lower cranial neuropathies Teaching NeuroImages Neurology Resident and Fellow Section by Nathaniel M. Schuster, Stellios Karnezis, and Lucas Restrepo Neurology Volume xx(x):x-x 2015 © 2015 American Academy of Neurology

52-year-old male presented for 4 months of progressive hypophonia and dysphagia associated with refractory headache and bilateral otitis media refractory to multiple oral and intravenous antibiotics. Examination found asymmetric palatal elevation, aphonia, right trapezius atrophy with scapular winging, and right tongue deviation. Vignette Schuster et al. Neurology 2015; xx(x):x-x 2015 © 2015 American Academy of Neurology

Clinical examination prior to immunosuppression Schuster et al. Neurology 2015; xx(x):x-x 2015 © 2015 American Academy of Neurology Clinical Examination

Schuster et al. Neurology 2015; xx(x):x-x 2015 © 2015 American Academy of Neurology Left: T1 Pre-Contrast, Center: T1 Post-Contrast, Right: T1 Sagittal Oblique Post-Contrast Imaging

Serum proteinase 3 antibody level was 180 units (normal <21 units) and c-ANCA was 1:160, consistent with granulomatosis with polyangiitis (GPA; Wegener’s granulomatosis). GPA was confirmed by lung nodule biopsy showing multinucleated giant cells and necrosis. Cranial neuropathies are a recognized complication of GPA (1, 2). Collet-Sicard syndrome (CSS) represents cranial nerve 9-12 palsies with sympathetic sparing. Cranial nerves 9-11 exit the jugular foramen while cranial nerve 12 traverses the nearby hypoglossal canal. 52yo male with multiple lower cranial neuropathies Schuster et al. Neurology 2015; xx(x):x-x 2015 © 2015 American Academy of Neurology