Primary antiphospholipid syndrome complicated with moyamoya syndrome: a case report and literature review

doi:10.16150/j.1671-2870.2022.04.013

Abstract

Abstract:

Objective: To analyze the clinical data of a case of primary antiphospholipid syndrome (PAPS) combined with moyamoya syndrome (MMS), and to explore the clinical characteristics of APS combined with MMS. Methods:The clinical data of a patient with PAPS and MMS were collected and analyzed by searching the relevant literatures. Results: The patient was a 23 year old female with 2 pathological pregnancies. The disease started with cerebral infarction. Cerebrovascular imaging showed severe stenosis or occlusion of bilateral internal carotid arteries with collateral vessels formation, which was consistent with MMS findings. Abdominal CT showed splenomegaly. The symptoms were improved after anticoagulation and immunosuppressive therapy. Through literature review at home and abroad, 7 patients with APS complicated with smoky vascular changes with detailed clinical data were found, all of them were female, with an average age of 26.6 years. Their clinical manifestations were heterogeneous, in which limb weakness was a common symptom, with arteriovenous thrombosis, pathological pregnancy and persistent positive antiphospholipid antibody as the main clinical manifestations. A total of 6 patients were followed up in detail, and the symptoms of the 5 patients were improved after anticoagulation and immunosuppressive therapy. One patient died of cerebral hemorrhage. Conclusions: APS is an autoimmune disease involving multiple systems. The case report of APS with MMS is very rare. The anticoagulant or antiplatelet therapy of APS is contradictory to the risk of bleeding in the collateral vessels of MMS, so dual antiplatelet therapy should be carefully selected.

Key words: Antiphospholipid syndrome, Moyamoya syndrome, Pathological pregnancy, Ischemic stroke, Splenomegaly

CLC Number:

R743.9

SONG Luoqing, DAI Tingjun. Primary antiphospholipid syndrome complicated with moyamoya syndrome: a case report and literature review[J]. Journal of Diagnostics Concepts & Practice, 2022, 21(04): 497-503.

Figures/Tables 3

文献	年龄（岁）	主要临床表现	实验室检查	影像学表现			结果
Booth F, et al^[6]	7.5	嗜睡;头痛;运动性失语;偏侧肢体无力	血沉↑;aCL（+）	MRA示左侧颈内动脉明显狭窄;左侧前后交通动脉侧支血管烟雾状改变;MRI示左侧基底节区及额顶叶高信号影	华法林×5个月（INR保持2.5~4.0）减至低剂量ASA	30	语言及运动功能改善;出现认知障碍;脑血流量较前改善
Yamashita Y, et al^[7]	12	构音障碍;面部及肢体不自主运动;努南综合征表型	PTL↓;PT↑;APTT↑; aCL（+）;LA（+）; β₂GP1（+）;ANA（+）; GH↓	MRA示双侧颈内动脉狭窄,右侧为著伴远端血管烟雾状改变;MRI示右侧基底节区低信号影	ASA（100 mg/d）+生长激素+匹莫齐特(1.5 mg/d）×24个月	24	不自主运动完全好转;脑血流量明显改善
Shuja Ud Din MA, et al^[8]	41	面部及偏侧肢体无力	aCL（+）;β₂GP1（+）	MRA示右侧颈内动脉狭窄;右侧大脑中、后动脉侧支血管烟雾状改变;MRI示右侧颞叶、基底节区及双侧半卵圆区高信号影	ASA×2周	12	症状完全改善
Wang等^[5]	43	偏侧肢体无力	aCL（+）;β₂GP1（+）	CTA示双侧大脑中动脉闭塞;CT示双侧半卵圆区及基底节区多处低密度灶;B超示子宫腺肌症	ASA(100 mg/d)+氯吡格雷(75 mg/d)×7 d;ASA(100 mg/d)+氯吡格雷(75 mg/d)+曲普瑞林(3.75 mg/4周)	2	死亡（脑室出血）
左瑜等^[9]	17	头晕头痛;视力下降	PT↓ ;aCL（+）; LA（+）;β₂GP1（+）;	DSA示双侧颈内动脉远端及大脑前、中动脉近端闭塞伴侧支血管烟雾状改变;CT示蛛网膜下腔出血	ASA（100 mg/d）;甲泼尼龙（40 mg/d）→激素序贯口服;氨肽素（3 000 mg/d）	3	头痛、头晕及视力减退明显改善,血小板恢复正常
岳月红等^[10]	22	运动性失语;面部及肢体无力	aCL（+）;TT3↑; TT4↑;FT4↑;TSH↓; TRAb（+）	MRA示双侧颈内动脉末端狭窄伴远端血管烟雾状改变;MRI示左侧额叶及左侧基底节区大片状高信号影	抗血小板;控制甲亢	-	-
王梓等^[11]	44	头痛	aCL（+）	DSA示左侧大脑中动脉起始部闭塞伴侧支血管烟雾状改变	不详	2	症状完全改善

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