Pontine infarction in a patient with HELLP syndrome: сase report with a review

INTRODUCTION. The prevalence of HELLP syndrome ranges from 0.5% to 0.9%. The mortality rate during its development reaches 24%. One of the reasons for the unfavorable outcome of this complication is acute cerebrovascular accident, but the true frequency of this complication in HELLP syndrome is currently not specified. An even rarer complication is ischemic brainstem stroke. Progressive endothelial dysfunction leading to generalized microthrombosis and coagulopathy may be the cause of neurological complications in HELLP syndrome and requires a rapid differential diagnosis with other thrombotic microangiopathies (TMA). In case of negative dynamics after delivery, early initiation of plasma exchange or plasma filtration helps stabilize the condition and allows one to safely exclude other variants of TMA.
CASE PRESENTATION. The purpose of the publication is to present a clinical case of the development of brainstem stroke in a patient with an atypical course of HELLP syndrome without clinical and laboratory signs of liver failure, to summarize existing data on the frequency of strokes during pregnancy, risk factors, etiology, as well as diagnostic and treatment strategies.
CONCLUSION. TMA with the development of HELLP syndrome can be the cause of ischemic stroke; it requires an urgent differential diagnosis with hemorrhagic stroke, thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), an interdisciplinary approach to diagnosis and treatment, as well as early inclusion in complex therapy therapeutic apheresis.

引言。HELLP综合征（HELLP syndrome）的患病率为0.5%~0.9%，发病期间病死率可达24%。该并发症预后不佳的原因之一为急性脑血管意外（acute cerebrovascular accident），但目前尚未明确HELLP综合征中该并发症的实际发生频率。更为罕见的并发症为缺血性脑干卒中（ischemic brainstem stroke）。进行性内皮功能障碍（endothelial dysfunction）可引发全身性微血栓形成（generalized microthrombosis）与凝血病（coagulopathy），该病理过程可能是HELLP综合征患者出现神经系统并发症的病因，且需与其他血栓性微血管病（thrombotic microangiopathies, TMA）快速开展鉴别诊断。若分娩后患者病情无改善，尽早启动血浆置换（plasma exchange）或血浆滤过（plasma filtration）治疗有助于稳定病情，同时可安全排除其他TMA亚型。

病例介绍。本文旨在报告1例以非典型病程起病的HELLP综合征患者发生脑干卒中的临床病例，该患者无肝衰竭的临床与实验室征象；同时总结现有关于妊娠期卒中的发生频率、危险因素、病因及诊断与治疗策略的相关数据。

结论。伴HELLP综合征的血栓性微血管病可引发缺血性卒中（ischemic stroke），需与出血性卒中（hemorrhagic stroke）、血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura, TTP）、非典型溶血性尿毒症综合征（atypical hemolytic uremic syndrome, aHUS）进行紧急鉴别诊断，诊疗过程需采用多学科协作模式，并尽早将治疗性血液单采（therapeutic apheresis）纳入综合治疗方案。