Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, a vasculitis affecting small and medium sized vessels usually affects the upper and lower respiratory tract, the kidneys, and the eyes. Neurologic manifestation in central nervous system (CNS) is less frequent than the peripheral and usually is in form of stroke. Few cases of lateral medullary ischemic stroke (Wallenberg syndrome) due to GPA have been reported. A 41 year-old female, presented with acute vertigo, nausea/vomiting, hiccups, dysphagia.

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