双侧脑桥延髓内侧“心”型孤立性脑梗死临床和影像特征分析

Clinical and imaging features of “heart appearance” isolated cerebral infarction in bilateral medial pons-medulla oblongata

目的 探讨累及双侧脑桥延髓内侧特征性“心”型孤立性脑梗死的临床特点及影像学特征。方法 回顾性分析2019年5月及2020年2月广东药科大学附属第一医院神经内科收治的2例累及双侧脑桥延髓内侧“心”型孤立性梗死患者的临床及影像资料。计算机检索近10年来Pubmed、Web of Science、中国知网、万方等数据库收录的公开发表的脑桥延髓“心”型孤立性脑梗死患者的国内外文献数据,复习文献并总结分析。结果 2例患者病程均为进展性,临床上均表现为球麻痹、感觉障碍、进展性四肢瘫痪、呼吸受累。颅脑磁共振成像(MRI)显示脑桥延髓内侧典型的“心”型孤立性梗死。数字减影血管造影(DSA)显示病例1为椎基底动脉粥样硬化,病例2为椎基底动脉粥样硬化、狭窄及闭塞。24篇文献共纳入29例患者,延髓“心”型梗死21例,脑桥“心”型梗死8例。本中心和文献报道共计31例患者。男性28例,女性3例,发病年龄32~87(60.3±12.1)岁。出现球麻痹症状29例(29/31,93.55%),感觉障碍23例(23/31,74.19%),四肢瘫痪31例(100%),进展性病程29例(29/31,93.55%),累及呼吸23例(23/31,74.19%),预后不良24例(24/29,82.76%),死亡6例(6/29,20.69%)。29例患者行MRA、CTA或DSA检查,椎基底动脉无明显异常或仅存在粥样硬化7例(7/29,24.14%),椎基底动脉狭窄或发育不良11例(11/29,37.93%),椎基底动脉存在闭塞11例(11/29,37.93%)。结论 双侧脑桥延髓内侧“心”型孤立性梗死病例临床罕见,其在脑MRI上形态学特殊。此类患者病因常为椎基底动脉粥样硬化、狭窄或闭塞;病程常呈进展性,四肢瘫痪,常累及呼吸,病情危重,容易误诊、漏诊;应尽快行脑MRI及DSA检查,争取早诊断、早治疗,以改善预后。

Objective To investigate the clinical and imaging features of the characteristic “heart appearance” isolated cerebral infarction involving bilateral pons-medulla oblongata. Methods The clinical and imaging data of 2 patients with bilateral pons-medulla oblongata “heart appearance” isolated infarction treated in our hospital from May 2019 to February 2020 were retrospectively analyzed. The published domestic and foreign literature of patients with this disease collected in PubMed, Web of Science, CNKI, WanFang and other databases in recent 10 years were also searched by computer, and the literature was subsequently reviewed and analyzed. Results The course of the disease in both patients was progressive, with clinical manifestations of bulbar palsy, sensory disturbance, progressive quadriplegia, and respiratory involvement. Brain magnetic resonance imaging(MRI) showed a typical “heart appearance” isolated infarction in the medial medulla or pons. Digital subtraction angiography(DSA) revealed vertebrobasilar atherosclerosis in case 1 and vertebrobasilar atherosclerosis, stenosis, and occlusion in case 2. A total of 29 patients were contained in 24 pieces of literature, including 21 cases of medulla oblongata infarction and 8 cases of pontine infarction, all with “heart appearance”. Thereby a total of 31 patients, including 28 males, and 3 females, were reported by our center and the literature, with onset age of 32~87(60.3±12.1) years. Among them, there were 29 cases of bulbar palsy symptoms(29/31, 93.55%), 23 of sensory impairment(23/31, 74.19%), 31 of quadriplegia(100%), 29 with progressive disease course(29/31, 93.55%), 23 with respiratory involvement(23/31, 74.19%), 24 with poor prognosis(24/29, 82.76%), and death in 6 cases(6/29, 20.69%). Of the 29 patients examined by MRA, CTA or DSA, 7 had no obvious abnormality or only atherosclerosis in the vertebrobasilar artery(7/29, 24.14%), 11 had vertebrobasilar stenosis or dysplasia(11/29, 37.93%), and the remaining 11 showed vertebrobasilar artery occlusion(11/29, 37.93%). Conclusion Isolated “heart appearance” infarction of the bilateral medial pons-medulla oblongata is clinically rare and has special morphological features on brain MRI. The etiology of such patients is mainly vertebrobasilar atherosclerosis, stenosis or occlusion. The course of the disease is often progressive, quadriplegia, and involves breathing, with critical condition, which is prone to misdiagnosis and missed diagnosis. Brain MRI and DSA examination are suggested to be performed as soon as possible to facilitate early diagnosis and treatment to improve the prognosis.