| Aim To explore the clinical features and surgical efficacy of 57 patients with moyamoya syndrome.Method Retrospective analysis the clinical data of 57 patients with MMS treatment from the Department of Neurosurgery in the 307 th Hospital of Chinese People’s Liberation Army from December 2002 to October 2013, Including: the onset of patients age, sex ratio, first symptoms, symptoms, basic diseases, suzuki in installment, imaging characteristics and surgical outcomes,and compared with the previous reports the clinical features and prognosis of the patients with moyamoya disease. Finally, induction of smoke syndrome overall clinical characteristics and surgical curative effect.Then grouped according to the age and with basic diseases situation, will < 18 children filtered and treated in the same period as the control group, 550 cases of children patients with moyamoya disease.Through the analysis of two groups of patients’ age, sex, first symptoms, symptoms, suzuki in installment, imaging characteristics and surgical curative effect, summarized summarized the clinical characteristics and surgical efficacy of moyamoya syndrome in children.57 cases of moyamoya syndrome patients, merge in 41 cases of thyroid function hyperfunction. For this part of the patient’s age, sex ratio, first symptoms, symptoms, basic diseases, suzuki in installment, imaging characteristics and surgical curative effect were analyzed, summarized the merged thyroid function hyperfunction of moyamo syndrome clinical characteristics and surgical outcomes. Result 57 cases of moyamoya syndrome patients including 13 cases of male, female 44 cases, ratio of 1:3.Onset age distribution, height is 25-35 years old. 57 cases of patients with the average age of 29.8 years, the male average age: 24.9 years old, female average age: 30.9 years.The basis Diseases of 57 patients include: 41 cases of thyroid function hyperfunction, 3 cases of hypothyroidism, 3 cases of neurofibroma, 3 cases of thyroid inflammation, 2 cases of systemic lupus erythematosus, 2 cases of ankylosing spondylitis, 1 case of psoriasis, accumulation of glycogen in 1 case, congenital heart disease in 1 case. In our group cases, by reviewing the history always, first symptoms of TIA: 21 cases, cerebral infarction: 21 cases, headache: 8 cases, cerebral hemorrhage:3 cases, dizziness: 3 cases, asymptomatic: 1 cases.12 children moyamoya syndrome patients, including 4 cases of male children, 8 cases of female children in 8 cases, the sex ratio of 1:2.The average age of 12 patients is 11.8 years, of which the average age of the male: 9 years old, female average age: 13.3 years.The basis diseases of 12 patients include: thyroid function hyperfunction of 7 cases, nerve fibroma in 2 cases, accumulation of glycogen in 1 case, congenital heart disease in 1 case, 1 cases of thyroiditis.In our group cases, by reviewing the history always, starting symptoms midbrain infarction: 5 cases, TIA: 3 cases, cerebral hemorrhage:2 cases, headache: 1 case, syncope:1 case. Before surgery, 5 cases of the disease progress. Over the same period in 550 cases of children patients with moyamoya disease treated male: 287, female: 263 cases, male to female ratio is about 1:1. The average age of 550 cases of children patients with moyamoya disease is 9.8 years, of which the male average age: 9.3 years old, female average age: 10.3 years. By reviewing the history always, starting in 550 cases of children patients with moyamoya disease symptoms are: TIA: 340 cases, headache: 95 cases, cerebral infarction: 80 cases,cerebral hemorrhage: 19 cases, dizziness: 10 cases,asymptomatic: 6 cases. In 550 cases of children patients with moyamoya disease progression in 48 patients. 12 cases of moyamoya syndrome of sex ratio, age, family history and contemporary moyamoya disease in children, there was no statistically significant difference(P > 0.05). In the starting symptoms, moyamoya syndrome group of children with cerebral infarction group(5/12) was significantly higher proportion of moyamoya disease [14.5%(80/550)], the proportion of patients with TIA(3/12) was lower than that in group moyamoya disease [61.8%(340/550)], differences were statistically significant(P < 0.05). In the progress of the clinical symptoms, moyamoya syndrome group compared with moyamoya disease progress occurred more [than 5/12 of 8.7%(48/550)], the difference was statistically significant(P < 0.05), 12 patients merged basic diseases include: thyroid function hyperfunction(7 cases), nerve fibroma(2 cases), accumulation of glycogen(1 cases), congenital heart disease(1 case), thyroiditis(1 case). Imaging features of 12 cases of children moyamoya syndrome patients, 12 cases of moyamoya syndrome patients, DSA examination in 11 cases, including head head MRA examination in 1 case. Results 12 cases were bilateral lesions, 1 case of combined cycle lesions. 11 cases with DSA examination, suzuki stage mainly concentrated in 3 ~ 4, first stage 4 sides, second stage 2 sides, thrird stage 6 sides, fourth stage 8 sides, fifth stage 2 sides. 41 cases on the basis of hyperthyroidism disease clinical characteristics of moyamoya syndrome, in which men and women sex ratio, 8 cases of male, female 33 cases, male to female ratio: 1:4. Average age 29.34 + 29.34(7-56), age distribution: 0-4:0 case, 5-9:2 cases, 10-14:4 cases, 15-19:3 cases, 20-24:4 cases, 25-29:7 cases, 30-34:8 cases, 35-39:5 cases, 40-44:3 cases, 45-49:3 cases, 50-54:1 case, 55-59:1 case, 60-64:0 case, 65-69:0 cases, > = 70:0 case. Presented single peak, at the age of 25 to 34 age most, accounts for about 36.6%. Men with an average age of 22.25, female with an average age of 31.06. First symptoms: TIA: 16 cases, cerebral infarction: 16 cases, headache: 6 cases cerebral hemorrhage:1 case, dizziness: 1 case, asymptomatic: 1 case. Progress symptoms: 19 cases of the disease progresses, the cerebral infarction: 17 cases,cerebral hemorrhage:2 cases. In the surgical treatment of 57 cases patients with moyamoya syndrome carry out EDAS+ medicine related treatment of 52 cases, 43 patients were not in attack or apparent ease symptoms. 12 cases of surgical treatment for children, 1 case in 12 patients at higher risk for anesthesia and Not perform surgery. The other 11 cases were effectively control the basic diseases, carried out EDAS surgery. Of which7 cases of bilateral, 4 cases of unilateral. 1 case appeared transient glossolalia and a right upper limb weakness in the 2 days postoperatively, the rest in perioperative patients with no surgery related complications occurred. Follow-up, 12 patients were followed up for 6 ~ 66 months, mean follow-up time was(23 + 16) months, none of the patients lost to follow-up. 11 patients who underwent surgical EDAS patients, clinical symptoms were improved to varying degrees, postoperative m RS score [median 0, P25-75(0-1)] significantly lower than the preoperative m RS score [median 2 points, P25-75(1-2), P < 0.05].Conclusion moyamoya syndrome with moyamoya disease have different clinical characteristics and disease progress there exist certain differences. To effectively control the basic diseases and the timely and effective EDAS therapy can prevent disease progression, and improving the prognosis of patients. At the same time for such patients, the forward curative effect remains to be seen, postoperative close follow-up is of great significance. |