Ischemic stroke in a postpartum patient: understanding the epidemiology, pathogenesis, and outcome of Moyamoya disease.
BACKGROUND
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.


OBJECTIVE
To review the epidemiology, presentation, diagnosis, differential diagnosis, treatment, prognosis, and postpartum maternal outcomes of MMD.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Ishimori
Mariko L
ML
Rheumatology Fellow, Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Cohen
Stanley N
SN
Hallegua
David S
DS
Moser
Franklin G
FG
Weisman
Michael H
MH
D000328:Adult; D020520:Brain Infarction / Q000175:diagnosis*; D019052:Depression, Postpartum / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D009072:Moyamoya Disease / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D011247:Pregnancy; D011379:Prognosis; D011644:Puerperal Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy