title: Clinical features and disease progression in moyamoya disease patients with Graves disease.
abstract: OBJECTIVE
  The present study aimed to clarify the incidence and clinical features of disease progression in adult moyamoya disease (MMD) patients with Graves disease (GD) for better management of these patients.
  
  
  METHODS
  During the past 18 years, 320 adult Chinese patients at West China Hospital were diagnosed with MMD, and 29 were also diagnosed with GD. A total of 170 patients (25 with GD; 145 without GD) were included in this study and were followed up. The mean follow-up was 106.4 ± 48.6 months (range 6-216 months). The progression of the occlusive lesions in the major intracranial arteries was measured using cerebral angiography and was evaluated according to Suzuki's angiographic staging. Information about cerebrovascular strokes was obtained from the records of patients' recent clinical visits. Both angiographic progression and strokes were analyzed to estimate the incidences of angiographic progression and strokes using Kaplan-Meier analysis. A multivariate logistic regression model was used to test the effects of sex, age at MMD onset, disease type, strokes, and GD on the onset of MMD progression during follow-up.
  
  
  RESULTS
  During follow-up, the incidence of disease progression in MMD patients with GD was significantly higher than in patients without GD (40.0% vs 20.7%, respectively; p = 0.036). The interval between initial diagnosis and disease progression was significantly shorter in MMD patients with GD than in patients without GD (p = 0.041). Disease progression occurred in both unilateral MMD and bilateral MMD, but the interval before disease progression in patients with unilateral disease was significantly longer than in patients with bilateral disease (p = 0.021). The incidence of strokes in MMD patients with GD was significantly higher than in patients without GD (48% vs 26.2%, respectively; p = 0.027). The Kaplan-Meier survival curve showed significant differences in the incidence of disease progression (p = 0.038, log-rank test) and strokes (p = 0.031, log-rank test) between MMD patients with GD and those without GD. Multivariate analysis suggested that GD may contribute to disease progression in MMD (OR 5.97, 95% CI 1.24-33.76, p = 0.043).
  
  
  CONCLUSIONS
  The incidence of disease progression in MMD patients with GD was significantly higher than that in MMD patients without GD, and GD may contribute to disease progression in MMD patients. The incidence of strokes was significantly higher in MMD patients with GD than in patients without GD. Management guidelines for MMD patients with GD should be developed.
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Jian-Bin
authors: JB
authors: Chen
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Ding
authors: D
authors: Lei
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Min
authors: M
authors: He
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Hong
authors: H
authors: Sun
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Yi
authors: Y
authors: Liu
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Heng
authors: H
authors: Zhang
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Chao
authors: C
authors: You
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Liang-Xue
authors: LX
authors: Zhou
authors: Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, People's Republic of China.
authors: Ling-Xue
authors: LX
authors: Zhou
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018450:Disease Progression; D005260:Female; D006111:Graves Disease / Q000150:complications*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009072:Moyamoya Disease / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology*; D055815:Young Adult
keywords: DSA = digital subtraction angiography; GD = Graves disease; Graves disease; MMD = moyamoya disease; MRA = MR angiography; T3 = triiodothyronine; T4 = thyroxine; TIA = transient ischemic attack; TSH = thyroid stimulating hormone; disease progression; fT3 = free triiodothyronine; fT4 = free thyroxine; moyamoya disease; stroke; vascular disorders