摘要: 目的 报告2 例临床罕见的可逆性胼胝体压部病变综合征病例,初步总结其诊断与治疗要点。方法与结果 回顾分析2 例患者之病因、临床表现、影像学特征和临床转归,并复习相关文献。 例1 主要表现为头晕,例2 则以精神障碍、发热、肌强直、震颤、外周血白细胞计数增加、血清肌酸激酶水平升高为主。临床表现均无特异性且无胼胝体离断综合征之证据,发病初期主要表现为上呼吸道感染症状,呈急性病程,经糖皮质激素等药物治疗14 和21 d 后症状与体征明显好转。头部MRI 共同特点为胼胝体压部孤立性病灶,呈类圆形,增强扫描病灶无强化;原发病缓解后胼胝体压部病灶完全消失。结论 可逆性胼胝体压部病变综合征是一种具有多种病因、独特的临床影像综合征,除骤然停用抗癫药物和感染等因素外,恶性综合征亦可能是诱因之一。本组2 例患者的诊断与治疗结果支持最近文献提出的其预后取决于导致该综合征的原发病而非胼胝体压部病变的观点。
关键词:
胼胝体,
脑疾病,
安定药恶性综合征,
磁共振成像
Abstract: Objective To report 2 cases of reversible splenial lesion syndrome (RESLES) and to summarize some key points in diagnosis and treatment of this disease. Methods The pathogenesis, clinical presentations, neuroimaging features and outcomes of 2 cases with RESLES were retrospectively analyzed and related literature was reviewed. Results Two patients, including a 24-year-old female and an 18-year-old male, presented with sudden-onset neurologic symptoms, which became prominent after the initiation of an upper respiratory infection. Both patients were previously healthy and had no history of seizure, usage of antiepileptic drugs, or any type of vaccination during the last 2 years. Case 1 just presented with non-vertiginous dizziness. However, Case 2 presented symptoms such as dysphrenia, fever, rigidity, tremor, leukocytosis and creatine kinase (CK) elevation. Their clinical presentations were nonspecific, without evidence of callosal disconnection syndrome. They were treated with corticosteroids. Clinically, both patients were greatly improved after 14 and 21 days. Cranial MRI showed an isolated lesion centered in the splenium of the corpus callosum. The lesions were round-shaped without contrast enhancement, and disappeared after complete remission of the underlying disease. Conclusions To our knowledge and according to previous reports, RESLES is a distinct clinicoradiological syndrome of varied etiology. In addition to sudden withdrawal of antiepileptic drugs and infection, RESLES can be caused by neuroleptic malignant syndrome (NMS). The outcome of the above mentioned cases supports the opinion that prognosis depends on the underlying disorder, but not on the presence or absence of the splenial lesion.
Key words:
Corpus callosum,
Brain diseases,
Neuroleptic malignant syndrome,
Magnetic resonance imaging
方旭明, 余晖, 徐竹, 陈红群, 楚兰. 可逆性胼胝体压部病变综合征:二例报告并文献复习[J]. 中国现代神经疾病杂志, 2014, 14(7): 586-591.
FANG Xu-ming, YU Hui, XU Zhu, CHEN Hong-qun, CHU Lan. Reversible splenial lesion syndrome: two cases report and review of literature[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2014, 14(7): 586-591.