Moyamoya syndrome associated with Sneddon's syndrome and antiphospholipid-protein antibodies

• Published in: Journal of stroke and cerebrovascular diseases 1999-03, Vol.8 (2), p.51-56
• Main Authors: Carhuapoma, Juan R., D'Olhaberriague, Luis, Levine, Steven R.
• Format: Article
• Language: English
• Subjects: Anticardiolipin Antibodies; Antiphospholipid Antibodies; Livedo Reticularis; Lupus Anticoagulant; Moyamoya; Sneddon's Syndrome
• ISSN: 1052-3057; 1532-8511
• DOI: 10.1016/S1052-3057(99)80054-8

Background: There are anecdotal reports of the rare combination of Sneddon's syndrome, lupus anticoagulant, and Moyamoya. To our knowledge, we now report the first case of anticardiolipin antibodies, Sneddon's syndrome, and Moyamoya. Methods: Case-report and systematic literature review. Results: A 37-year-old woman had 31/2 years of recurrent left-sided sensory-motor symptoms. More recently, she had experienced vertigo, diplopia, and imbalance. Medical history included headaches, labile hypertension, left arm venous thrombosis requiring anticoagulation, and cigarette smoking. On examination she had livedo reticularis, limited left eye abduction, and left hemiparesis. Magnetic resonance imaging (MRI) showed right frontal, left parieto-occipital and pontine high intensity lesions on T 2-weighted images consistent with ischemia and abnormally increased flow-void in the basal ganglionic regions. Conventional cerebral angiography showed a Moyamoya pattern. Transesophageal echocardiography and electroencephalogram were normal. Serologic studies were remarkable for anticardiolipin antibodies immunoglobulin G isotype only. She responded favorably to carbamazepine as treatment of presumptive focal seizures, and long-term anticoagulation. Seven other cases reported in the literature were found and reviewed, with different combinations of Moyamoya, Sneddon's syndrome, and antiphospholipid-protein antibodies. The mean age was 37 (range 18–59, SD±16) years, male/female ratio 3/5; clinical features included cognitive changes (4 pts), ischemic stroke (6pts), seizures (1pt), and intracranial hemorrhage (2pts). Anticoagulation/steroids/anti-platelet agents were empirically associated with a favorable survival and functional outcome in 6 cases. Conclusion: This case expands the spectrum of associations with Moyamoya, and in conjunction with a review of the literature, suggests that evaluation for antiphospholipid-protein antibodies is recommended in cases of Moyamoya syndrome.