Ischemic Stroke in a Postpartum Patient: Understanding the Epidemiology, Pathogenesis, and Outcome of Moyamoya Disease

• Published in: Seminars in arthritis and rheumatism Vol. 35; no. 4; pp. 250 - 259
• Main Authors: Ishimori, Mariko L., Cohen, Stanley N., Hallegua, David S., Moser, Franklin G., Weisman, Michael H.
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA Elsevier Inc 01.02.2006; Elsevier
• Subjects: Adult; benign angiopathy; Biological and medical sciences; Brain Infarction - diagnosis; Depression, Postpartum - diagnosis; Diagnosis, Differential; Diseases of the osteoarticular system; Female; Humans; ischemic stroke; Medical sciences; Moyamoya disease; Moyamoya Disease - diagnosis; Moyamoya Disease - epidemiology; Moyamoya Disease - therapy; Moyamoya syndrome; Neurology; postpartum; Pregnancy; primary CNS vasculitis; Prognosis; Puerperal Disorders - diagnosis; Puerperal Disorders - epidemiology; Puerperal Disorders - therapy; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Vascular diseases and vascular malformations of the nervous system; EDAMS; MRI; CNS; DSA; IACNS; CSF; Moyamoya syndrome; postpartum; ischemic stroke; CRABP-I; ACA; primary CNS vasculitis; Moyamoya disease; MMD; ICA; BACNS; EMS; MCA; TIA; bFGF; CTA; EDAS; PACNS; TNF-α; benign angiopathy; C-section; EMAS; STA-MCA; MRA; GANS; Human; Cerebral infarction; Nervous system diseases; Stroke; Nishimoto Kudo disease; Prognosis; Pathogenesis; Rheumatology; Cardiovascular disease; Epidemiology; Cerebral disorder; Vascular disease; Vasculitis; Systemic disease; Central nervous system disease; Cerebrovascular disease; Takayashu arteritis
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2005.09.001

Moyamoya disease (MMD), or spontaneous occlusion of blood vessels in the circle of Willis, is a noninflammatory, nonatheromatous vasculopathy that often presents with ischemia or hemorrhage and has a tendency to affect young women. We discuss a case of a woman 7 days postpartum with new onset strokes, suspected initially to have benign angiopathy of the central nervous system (BACNS)/postpartum angiopathy versus primary CNS vasculitis. Cerebral angiography was suspicious for MMD and the patient underwent surgical revascularization. To review the epidemiology, presentation, diagnosis, differential diagnosis, treatment, prognosis, and postpartum maternal outcomes of MMD. The authors reviewed the English-language literature published between 1996 and 2004 using Medline databases. Additional articles were obtained from references described in literature obtained by the primary search. MMD is characterized by severe bilateral stenosis of the distal internal carotid arteries and their first branches, with prominent collateralization. Autoantibodies relating to endothelial cell dysfunction have been studied as possible pathogenetic mechanisms. The gold standard for diagnosing MMD is digital subtraction contrast angiography (DSA). MMD-related findings on imaging studies may, at times, be mistaken for those of vasculitis. A surgical approach may need to be considered. Despite treatment, the overall prognosis of MMD is worse in adults compared with children. Awareness of the specific features of MMD is crucial for clinical decision-making to avoid unnecessary exposure to aggressive immunosuppressive therapy. It is important for rheumatologists to consider MMD in the differential diagnosis of suspected CNS vasculitis.