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

Abstract

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.

Keywords

• cerebral infarction
• "heart appearance" infarction
• magnetic resonance imaging
• digital subtraction angiography