Moyamoya vasculopathy with anti-SCL-70 antibodies: A case report and review of the literature

Abstract

Moyamoya disease and syndrome constitute two distinct pathological entities with a primary finding of progressive distal internal carotid artery occlusion, which results in either an ischemic or hemorrhagic sequela. While the disease entity stands as a primary arterial vasculopathy, moyamoya syndrome represents a secondary vasculopathy associated with a systemic inflammatory process. We describe a patient with an ischemic stroke and angiographic findings of moyamoya in the setting of positive antinuclear antibodies and anti-Scl-70 antibodies with clinical features of scleroderma on exam. A review of current literature identified three similar cases where immunosuppression in addition to secondary stroke prevention led to reduced frequency of neurological sequelae. These cases plus our own demonstrate that patients with moyamoya vasculopathy in association with anti-Scl 70 antibodies exist on a spectrum with either predominant scleroderma features or neurological symptoms. There are a limited number of cases reported of moyamoya vasculopathy in association with anti-Scl 70 antibodies. This case report demonstrates that not only may the association be more common than we think, but that it exists on a dynamic spectrum with variable clinical presentation and course.

Keywords: Autoimmune; Ischemic stroke; Moyamoya; Scleroderma.