Department of Neurology, The First Affiliated Hospital of Zhejiang Chinese Medical University (Zhejiang Provincial Hospital of Traditional Chinese Medicine), 54 Youdian Road, Hangzhou, 310006, Zhejiang Province, China.
BMC Neurol. 2020 Oct 1;20(1):361. doi: 10.1186/s12883-020-01941-8.
Idiopathic hypereosinophilic syndrome (IHES) is associated with various organ system dysfunctions. Neurologic abnormalities have been previously noted in this syndrome. Cerebral infarction secondary to occlusion of large cerebral artery is rarely reported. Here we described a patient with IHES presented progressive multiple cerebral infarctions caused by bilateral middle cerebral artery occlusion.
A 55-year-old Chinese woman presented to our hospital with acute onset of right limbs weakness and slurred speech. Laboratory tests showed a significant eosinophilia of 5.29 × 10/L (normal, < 0.5), 49.9% of leukocytes. Brain magnetic resonance imaging (MRI) revealed multiple acute cerebral ischemic lesions. Magnetic resonance angiography (MRA) demonstrated stenosis in horizontal segment of right middle cerebral artery. A pretibial skin biopsy revealed eosinophilic infiltration around the capillaries in deep dermis and adipose tissue. The patient was given oral dual anti platelet agents and intravenous methylprednisolone. However, one week later, the patient presented significant neurological deterioration with right-sided hemiparesis and totally motor aphasia. Brain MRI and computed tomography perfusion (CTP) demonstrated new acute cerebral ischemia in left hemisphere. Digital subtraction angiography (DSA) revealed left middle cerebral artery completely occluded. The patient received a high-dose of intravenous methylprednisolone 500 mg per day and the eosinophil count quickly fell to normal within 2 days. She was transferred to a rehabilitation center and her neurological symptoms improved with modified Ranking Scale from 4 to 2.
IHES is one of the rare causes of acute ischemic stroke with large cerebral artery occlusion. An early high-dose of corticosteroids therapy should be considered in cases of IHES patients. Our case study is benefit to clinical diagnosis and treatment of cerebral infarction with IHES.
特发性嗜酸性粒细胞增多综合征（IHES）与各种器官系统功能障碍有关。以前在该综合征中已注意到神经系统异常。继发于大脑大动脉闭塞的脑梗死很少见报道。在这里，我们描述了一位患有 IHES 的患者，其表现为双侧大脑中动脉闭塞引起的进行性多发性脑梗死。
一位 55 岁的中国女性因右侧肢体无力和言语不清急性发作到我院就诊。实验室检查显示嗜酸性粒细胞显著增多，为 5.29×10/L（正常范围，<0.5），白细胞 49.9%。脑磁共振成像（MRI）显示多发急性脑缺血性病变。磁共振血管造影（MRA）显示右侧大脑中动脉水平段狭窄。小腿前皮肤活检显示真皮深层和脂肪组织中毛细血管周围嗜酸性粒细胞浸润。给予患者口服双联抗血小板药物和静脉甲基强的松龙。然而，一周后，患者出现右侧偏瘫和完全运动性失语等明显神经功能恶化。脑 MRI 和计算机断层灌注（CTP）显示左侧大脑半球新出现急性脑缺血。数字减影血管造影（DSA）显示左侧大脑中动脉完全闭塞。患者接受了每天 500mg 的大剂量静脉甲基强的松龙治疗，嗜酸性粒细胞计数在 2 天内迅速降至正常。她被转到康复中心，其神经症状从 4 分改善到 2 分。
IHES 是大血管闭塞性急性缺血性卒中的罕见原因之一。对于 IHES 患者，应考虑早期大剂量皮质类固醇治疗。我们的病例研究有助于 IHES 相关脑梗死的临床诊断和治疗。
