Neurological manifestations of polyarteritis nodosa associated with the antiphospholipid syndrome

Abstract

The association between antiphospholipid syndrome (APS) and vasculitis is rare and continues to evoke great interest. We report a case with neurological manifestations of polyarteritis nodosa (PAN) and concurrent APS. Electromyography and neuromuscular biopsy of the limb showed an axonal polyneuropathy following obliteration and necrosis of medium sized arteries, initially suggesting PAN. This vasculitis was confirmed on visceral selective arteriography, with the presence of numerous aneurysms. Cerebral MRI revealed multiple cortical and subcortical signals in the fronto-parietal areas, corresponding to ischemic microvascular lesions of APS. This association was confirmed by the presence IgG anticardiolipid, the past medical history of skin necrotic lesions and central retinal obliteration. Pulse intravenous cyclophosphamide, oral prednisolone and curative anticoagulation led to stabilization for 8 months.