Objective:

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Background:

Polyarteritis nodosa (PAN) is a systemic non-ANCA small and medium vessel vasculitis. It is histologically distinguished by presence of necrosis and neutrophilic, eosinophilic and lymphocytic infiltration of the vessel wall. Central nervous system (CNS) is affected in approximately 5% of cases. Strokes occurring in the context of PAN vasculitis are most often lacunar (73% cases) as opposed to cortical infarcts from involvement of large intracranial vessels. Mainstay of treatment includes corticosteroids and once biopsy-confirmed, addition of cyclophosphamide. 

Design/Methods:

We present a case of a 70-year-old male with history of suspected polymyalgia rheumatica, previous leg DVT and left MCA stroke who presented to hospital with transient left sided weakness, intermittent diplopia, fatigue and jaw claudication. During admission he developed acute right sided weakness, aphasia and dysarthria. Imaging showed acute infarction in a watershed distribution bilaterally and angiogram confirmed presence of bilateral intracranial ICA stenosis and diffuse calibre irregularities of the ACA, MCA and extracranial ECA branches. Imaging done one year prior at the time of his MCA stroke had revealed normal caliber intracranial vessels without evidence of atherosclerotic disease. Patient was started on IV Solumedrol for suspected CNS vasculitis and temporal biopsy was performed. Over the following days, despite medical management, patient developed recurrent episodes of unilateral weakness concerning for hemodynamically significant stenosis through the stenosed ICAs. He required bilateral angioplasty and ultimately, decision was made to deploy a stent in the supraclenoid right ICA due to recurrent clinical events. Pathology ultimately revealed areas of necrosis and mixed acute and chronic inflammation, consistent with PAN vasculitis and cyclophosphamide was initiated. Patient had no recurrent ischemic events following the stent and he was functionally independent at 4 months.

Results:

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Conclusions:

This case demonstrated favourable outcomes and opens the door for future research investigating invasive treatment in CNS stenosing vasculitis related stroke.