TY - JOUR
T1 - Moyamoya syndrome associated with Sneddon's syndrome and antiphospholipid-protein antibodies
AU - Carhuapoma, Juan R.
AU - D'Olhaberriague, Luis
AU - Levine, Steven R.
N1 - Funding Information:
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 Ta-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 (6 pts), seizures (1 pt), and intracranial hemorrhage (2 pts). Anticoagulation/steroids / anti-platelet agents were empirically associated with a favorable sttrvival and functional outcome in 6 cases. Conclusion: This case expands the spectrum of associations with Moyamoya, and, in conjunctionw ith a review of the literature, suggests that evaluation for antiphospholipid-protein antibodies is recommended in cases of Moyamoya syndrome. Key Words: Moyamoya--Sneddon's Syndrome--~Anticardiolipin Antibodies--AntiphospholipidA ntibodies--LupusA nticoagulant--LivedoR eticularis Moyamoya is a condition that initially was defined as a disease, but it is currently better understood as a syndrome. It describes the association of bilateral intracranial From the Center for Stroke Research, Department of Neurology and Henry Ford Stroke Program, Henry Ford Hospital & Health Sciences Center, Detroit Campus of Case Western Reserve University, Detroit, MI. Received September 22,1998; accepted November 2, 1998. Supported in part by National Institutes of Health (NINDS) grants NS-R01-30896 and NS-P01-23393.
PY - 1999/3
Y1 - 1999/3
N2 - 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 T2-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.
AB - 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 T2-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.
KW - Anticardiolipin Antibodies
KW - Antiphospholipid Antibodies
KW - Livedo Reticularis
KW - Lupus Anticoagulant
KW - Moyamoya
KW - Sneddon's Syndrome
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U2 - 10.1016/S1052-3057(99)80054-8
DO - 10.1016/S1052-3057(99)80054-8
M3 - Review article
C2 - 17895140
AN - SCOPUS:77950208857
SN - 1052-3057
VL - 8
SP - 51
EP - 56
JO - Journal of Stroke and Cerebrovascular Diseases
JF - Journal of Stroke and Cerebrovascular Diseases
IS - 2
ER -