Reversible cerebral vasoconstriction syndrome presenting as subarachnoid hemorrhage, reversible posterior leukoencephalopathy, and cerebral infarction

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe headache with or without additional neurological symptoms and reversible cerebral vasoconstriction. Unruptured aneurysm has been reported in some cases with RCVS. We report a severe case of a 53-year-old woman with RCVS having an unruptured cerebral aneurysm and presenting as cortical subarachnoid hemorrhage, reversible posterior leukoencephalopathy syndrome, and cerebral infarction. She was successfully treated with corticosteroids and a calcium channel blocker and the aneurysm was clipped. Her various complications are due to the responsible vasoconstriction that started distally and progressed towards proximal arteries. This case demonstrates the spectrum of presentations of RCVS, a clinically complicated condition.     

可逆性后部白质脑病综合征的发病机制

可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)是一种临床影像学疾病实体,主要以迅速进展的血压增高、头痛、呕吐、意识障碍、痢性发作为特征,神经影像学显示为双侧大脑半球对称性白质可逆性水肿(尤其是在大脑后部),通过及时和正确的治疗,临床症状和神经影像学改变可完全恢复.有关RPLS发病机制的两大假说--脑血管痉挛学说和脑血管过度灌注学说一直存在争论,目前多数学者仍然认同后者是造成脑水肿的主要原因.文章从RPLS的病因学、病理学和影像学特征阐述了其发病机制以及近年来对上述两大假说的新观点.     

Atypical reversible posterior leukoencephalopathy syndrome in thrombotic thrombocytopenic purpura

A 32-year-old man with an atypical form of reversible leukoencephalopathy syndrome (RPLS) caused by thrombotic thrombocytopenic purpura (TTP) is reported. In this particular case, a timely diagnosis of TTP was established primarily on the clinical findings, which led to the early initiation of plasmapheresis and resulted in excellent clinical recovery. The pathophysiological aspects of the relationship between TTP and RPLS are discussed in light of the clinical and radiological features (including diffusion- and perfusion-weighted magnetic resonance imaging studies) of this case. The mechanism for TTP-associated, or TTP-induced, leukoencephalopathy is suggested to be independent of hypertension and vasoconstriction. TTP-associated endothelial injury can play a major role as the inciting mechanism for the development of RPLS.     

Central nervous system lymphoma presenting as dysphagia

Two patients are presented having lymphomatous involvement of the central nervous system in whom dysphagia was the predominant symptom. All indicated studies failed to reveal evidence of direct esophageal involvement, and the dysphagia improved during treatment of the neural component. Esophageal manometric studies revealed abnormalities of the striated muscle portion of the esophagus. These two case histories suggest that invasion of the central nervous system with lymphoma may produce abnormalities of the neuromuscular control of esophageal function resulting in dysphagia.Supported by Department of the Navy, Clinical Investigation Program #0-06-1398.     

可逆性大脑后部白质脑病综合征16例的临床分析

目的探讨可逆性大脑后部白质脑病综合征的病因和临床特点,寻找早期诊断和治疗方法。方法选取该院2010．02～2013-02收治的16例可逆性大脑后部白质脑病综合征患者,回顾性分析其病因、临床表现特点、治疗及预后。结果16例患者中男6例,女10例。病因：高血压脑病7例（其中合并肾功能不全4例）,子痫4例,多发性大动脉炎2例,血栓性血小板减少性紫癜1例,系统性红斑狼疮1例,右颈内动脉内膜剥脱术后1例。临床表现以头痛、谵妄、意识障碍、癫痫发作性抽搐、视幻觉为主。头颅MRI检查可见大脑半球后部白质为主的T1加权呈短信号及12加权呈长信号的病灶,常双侧对称。经及时适当的脱水、降颅压,改善循环和高压氧等治疗,全组16例中,症状与体征消失者14例（87．5％）,明显好转者2例（12．5％）。结论可逆性大脑后部白质脑病综合征是一种具有自身病因、临床表现特点的临床综合征,经及时有效治疗,大多预后较好。     

Chronic lymphocytic leukemia presenting with symptomatic central nervous system involvement

Leukemic infiltration of the central nervous system (CNS) resulting in neurological manifestations is a rare complication of chronic lymphocytic leukemia (CLL). Furthermore, symptomatic CNS involvement as the initial presentation of previously undiagnosed CLL is extremely rare. In the present report, the authors describe a case of an 89-year-old female previously diagnosed with Alzheimer's disease who suddenly developed rapidly worsening mental changes. Cytological and immunocytological examinations of the lymphoid cells present on the cerebrospinal fluid (CSF) revealed CNS involvement by a clonal B-cell lymphoproliferative disorder, most consistent with de novo B-CLL expressing kappa light chain restriction. Subsequently, flow cytometric analysis done on the peripheral blood lymphocytes confirmed the diagnosis of B-CLL in this patient. Thus, this study shows the potential usefulness of immunocytological evaluation in detecting monoclonal lymphoid populations on CSF samples in adult patients presenting with altered mental status and CSF pleocytosis of lymphocytes.     

Primary central nervous system post-transplant lymphoproliferative disorder presenting as cerebral hemorrhage after unrelated bone marrow transplantation

Abstract: We present a rare case of cerebral hemorrhage due to Epstein–Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD). A 58-year-old man with myelodysplastic syndrome received allogeneic hematopoietic stem cell transplantation from an unrelated donor after being conditioned with fludarabine, melphalan, and total body irradiation. Tacrolimus and methotrexate were given for graft-versus-host disease (GVHD) prophylaxis. On day 23, he developed acute GVHD, which was successfully treated with prednisolone (PSL). The tapering of PSL failed because of extensive chronic GVHD involving the liver and lungs, and mycophenolate mofetil was added on day 244. On day 340, the patient suddenly complained of severe headache. Computed tomography confirmed subcortical hemorrhage, and he died on day 348. The autopsy revealed atypical lymphocytes infiltrating the brain and meninges, which were positive for B-cell-associated antigens and EBV-encoded RNA, and thus EBV-associated PTLD was diagnosed.     

老年人可逆性后部白质脑病综合征五例及临床和影像学特点分析

目的 分析我国老年人可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)患者的临床表现和影像学特点.方法 回顾报告5例老年RPLS患者的临床表现和影像学特点. 结果 本组老年男性患者1例,女性4例,平均年龄(63.0±2.4)岁,5例患者均在起病时并存高血压.最常见的临床表现依次为视觉障碍5例,头痛4例,癫痫4例,意识障碍3例,病理反射2例,偏瘫2例.CT检查示4例患者表现异常,MRI检查示5例患者均出现枕叶病变,颞叶受累2例,顶叶受累1例.4例患者出院平均(48.8±29.2)d后CT检查为阴性结果,死亡1例.结论 RPLS是典型的多因共果疾病,血管原性假说被影像学研究结果证实.神经影像学对诊断RPLS很重要,弥散加权像(DWI)有助于鉴别诊断RPLS和缺血性脑血管病.