Vasculitic Moyamoya syndrome in a patient with paroxysmal nocturnal hemoglobinuria: A case report and literature review

Abstract

Moyamoya syndrome (MMS) is a chronic and progressive occlusive cerebrovascular disease characterized by steno-occlusive changes at or around terminal carotid arteries or the initial segments of the anterior/middle cerebral arteries accompanied by the development of fragile collateral network, which is often secondary to an underlying acquired or inherited condition. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell genetic mutation disease manifesting as hemolytic anemia, marrow failure, smooth muscle dystonia, and thrombosis. 1 MMS secondary to PNH is rarely reported in the literature. 1-4 However, to the best to our knowledge, there has been no direct evidence about the vasculitic moyamoya changes of intracranial arteries in the situation of PNH. Here, we provide the direct evidence using intracranial This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Case report: a case of paroxysmal nocturnal hemoglobinuria with ischemic stroke as the first symptom

“…The general condition worsened as it progressed to hemorrhagic infarction. It is noteworthy that other rarer spectrum of cerebrovascular manifestations consist of TIA [5], ischemic stroke[6], Moyamoya syndrome [7], and posterior reversible encephalopathy syndrome[8] (Table 2). These neurological complications are non-speci c whether patients are complicated with PNH.…”

“…Strikingly, cerebral venous thrombosis (CVT) has been identi ed as the most common neurologic complication and caused one-third of mortality in PNH [4]. In addition, transient ischemic attacks (TIA) [5], ischemic stroke[6], Moyamoya syndrome [7], and posterior reversible encephalopathy syndrome [8] are critical components of cerebrovascular complications of PNH. The incidence of vein thrombosis is 6 times that of arterial [9].…”