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

Juan Carhuapoma, Luis D'Olhaberriague, Steven R. Levine

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)51-56
Number of pages6
JournalJournal of Stroke and Cerebrovascular Diseases
Volume8
Issue number2
DOIs
StatePublished - Mar 1999
Externally publishedYes

Fingerprint

Sneddon Syndrome
Moyamoya Disease
Antiphospholipid Antibodies
Anticardiolipin Antibodies
Seizures
Livedo Reticularis
Lupus Coagulation Inhibitor
Cerebral Angiography
Proteins
Diplopia
Intracranial Hemorrhages
Immunoglobulin Isotypes
Vertigo
Transesophageal Echocardiography
Carbamazepine
Paresis
Venous Thrombosis
Headache
Electroencephalography
Arm

Keywords

  • Anticardiolipin Antibodies
  • Antiphospholipid Antibodies
  • Livedo Reticularis
  • Lupus Anticoagulant
  • Moyamoya
  • Sneddon's Syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery
  • Rehabilitation
  • Cardiology and Cardiovascular Medicine

Cite this

Moyamoya syndrome associated with Sneddon's syndrome and antiphospholipid-protein antibodies. / Carhuapoma, Juan; D'Olhaberriague, Luis; Levine, Steven R.

In: Journal of Stroke and Cerebrovascular Diseases, Vol. 8, No. 2, 03.1999, p. 51-56.

Research output: Contribution to journalArticle

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AU - Carhuapoma, Juan

AU - D'Olhaberriague, Luis

AU - Levine, Steven R.

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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.

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