Moyamoya syndrome causing stroke in young women with type 1 diabetes.
Moyamoya syndrome is an idiopathic brain vasculopathy characterized by stenosis of major intracranial arteries. It often presents in patients with type 1 diabetes or thyroid disease and may have an autoimmune etiology. Moyamoya-related stroke poses a diagnostic challenge as initial symptoms and deficits vary greatly from classic ischemic stroke to encephalopathy, psychiatric, or seizure disorder.



We report 4 patients with type 1 diabetes and other autoimmune diseases who developed moyamoya-related stroke at a young age. Despite having long-term diabetes, these patients exhibited no evidence of dyslipidemia or other typical risk factors for atherosclerosis which might contribute to premature stroke. Three of the four patients underwent revascularization surgery while one patient received conservative management. All patients had improved neurologic function after treatment, some with residual deficits.



We highlight the importance of recognizing moyamoya syndrome in patients with pre-existing autoimmune diseases such as type 1 diabetes, as prompt diagnosis and treatment can have major impact on patient outcome and quality of life.
Division of Endocrinology, Metabolism and Lipid Research, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA. Electronic address: jing.hughes@wustl.edu.
Jing W
JW
Hughes
Division of Metabolism, Endocrinology, and Diabetes, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI, USA.
Jennifer A
JA
Wyckoff
Division of Pediatric Endocrinology and Metabolism, Washington University School of Medicine, St. Louis, MO, USA.
Abby S
AS
Hollander
Division of Diagnostic Radiology, Neuroradiology Section, Washington University School of Medicine, St. Louis, MO, USA.
Colin P
CP
Derdeyn
Division of Endocrinology, Metabolism and Lipid Research, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA.
Janet B
JB
McGill
D000293:Adolescent; D000328:Adult; D002545:Brain Ischemia; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000150:complications*; D005260:Female; D006801:Humans; D009072:Moyamoya Disease / Q000150:complications*; D011788:Quality of Life; D020521:Stroke / Q000209:etiology*; D016896:Treatment Outcome; D055815:Young Adult
Autoimmunity; Moyamoya; Stroke; Type 1 diabetes; Vasculopathy