海洛因海绵状白质脑病2例报告

海洛因中毒所致的海绵状白质脑病近年来陆续有报道。现将我们遇到的2例报告如下。     

海洛因海绵状白质脑病四例报告

海洛因海绵状白质脑病(heroin spongiform leucoencephalopathy，HSLE)是近年引起医学界重视的新病种，国内外文献报道不多。为提高对此病的诊断和治疗水平，该文报道作者医院近年收治的4例HSLE临床、影像学特点及治疗情况。     

海洛因海绵状白质脑病4例分析

病　例　介　绍例 1,男 ,37岁 ,无业 ,因戒毒后言语不清 ,行走不能一月余入院。患者有吸海洛因史 3年 ,烫吸法 ,每日 1～ 2 g。在家自行戒毒后 4日出现言语不清 ,饮水呛咳 ,双上肢运动笨拙 ,双下肢不能行走。反应迟钝 ,大小便失禁。查体 :神志清 ,言语缓慢 ,构音障碍。颅神经检查未见异常。四肢肌张力增高 ,肌力 级 ,腱反射 ( )～ ( )。双侧 Hoffman征 (+)。双侧指鼻试验、跟膝胫试验均 (+)。 Romberg征 (+)。记忆力、计算力下降。头颅 MRI示双侧顶叶、丘脑外侧核、桥脑、小脑半球、齿状核可见对称性长 T1 、长 T2 影 ,边缘清晰。CSF各…     

海洛因海绵状白质脑病一例报告及文献复习

患者 男性,45岁,因神志欠清3 d于2008年5月30日入院.家属代诉患者于5月28日下午出现神志欠清,不愿言语,当晚出现二便失禁,自服药物改善不明显.入院时症状包括:神志欠清,嗜睡,时而呓语,表情淡漠.家人代诉患者既往有吸毒病史10年,主要以烫吸为主.     

海洛因海绵状白质脑病

海洛因海绵状白质脑病是近年才引起医学界关注的少见疾病，其临床与病理改变颇具特点，但国外报告的文献很少，国内仅见数例报告。本文主要查阅近几年的文献，对该病的病因、病理机制、临床表现、影像学特点、诊断与鉴别诊断、治疗及预后各方面进行了总结。     

海洛因海绵状白质脑病一例报告及文献复习

患者 男性,45岁,因神志欠清3 d于2008年5月30日入院.家属代诉患者于5月28日下午出现神志欠清,不愿言语,当晚出现二便失禁,自服药物改善不明显.入院时症状包括:神志欠清,嗜睡,时而呓语,表情淡漠.家人代诉患者既往有吸毒病史10年,主要以烫吸为主.     

海洛因白质脑病

重点综述了海洛因白质脑病的成因 ,临床表现及病理与影像学特征 ,并突出其诊断标准     

海洛因海绵状白质脑病的病理学改变

目的 研究海洛因海绵状白质脑病(HSLE)的病理学改变.方法 应用常规染色和免疫组化染色(MBP、GFAP、NF、CD45、CD54、CD20)方法对3例HSLE死亡患者的脑组织进行病理检查.结果 HSLE的病理改变以脑白质海绵状变性为主,灰质很少受累.脑组织受累程度依次为小脑半球白质、内囊后肢、胼胝体压部、脑干、顶枕叶深部白质以及额颢叶深部白质,皮质以及皮质下弓状纤维不受累或仅轻微受累.HSLE白质损害以粗大的有髓神经纤维为主,髓鞘的损害重于轴突损害;脑组织无炎症细胞浸润,血管内亦无炎症介质沉积.结论 脑白质广泛海绵状白质变性是HSLE显著的病理学特点,不同部位脑白质损害程度与临床和影像学表现一致,轴突损害可能继发于髓鞘损害,HSLE脑组织中无血管炎性改变.     

海洛因海绵状白质脑病的临床及MRI(附3例报告)

目的 :研究海洛因中毒所致的海绵状白质脑病临床与磁共振成像特点。方法 :本文报告 3例并结合文献分析海洛因海绵状白质脑病临床和 MRI资料。结果 :本病的主要临床表现 ,起病前有明确的吸服海洛因病史 ,急性或亚急性起病 ,早期以构音障碍、步态不稳、小脑性共济失调等小脑损害症状和体征为突出表现。 MRI检查显示对称性双侧小脑半球、大脑半球后部、内囊后肢、胼胝体压部、脑干等皮质为主的多发性大片状长 T1、长 T2信号 ,加强后病灶无强化。脑病理特点是脑白质呈对称性海绵样脱髓鞘改变。结论 :本病的诊断主要依靠其临床特点、影像学及脑病理学检查 ,MRI对本病的诊断具有重要价值。     

海洛因海绵状白质脑病

<正> 海洛因是德国Bayer公司1898年生产出售的,化学成分为二乙酰吗啡,因其滥用超过临床使用范围,临床已不再使用。海洛因滥用与一些神经系统并发症有关,其急慢性中毒易导致严重的神经系统损害,发生诸如海绵状白质脑病、脑卒中、急性炎症脱髓鞘多发性神经病、癫痫、多发性神经病、横贯性脊髓病、急性横纹肌溶解症等疾病,其引起的白质脑病国内亦有报导。因脑组织活检或尸检发现脑白质呈现海绵状变性但无环死,故又称为海洛因海绵状白质脑病(Spongiform Leukoencephalopathy after Inhaling Heroun,SLIH)。     

Meningioangiomatosis: report of two cases and literature review

Meningioangiomatosis (MA) is a rare benign intracraneal lesion. The majority of cases are sporadic although the association of this lesion with familial neurofibromatosis (NF) type 2 is well known. NF-associated MA may be multifocal and is often asymptomatic and diagnosed at autopsy. Non-associated cases are usually symptomatic, occurs in children and young adults and frequently arise in leptomeninges and underlying cerebral cortex. In the present work, we describe two new non-associated cases of MA in two boys, seven and one year old with seizures that disappeared after surgical excision. Histopathologically, the lesion was predominantly cellular in one case and more fibrous in the other. From the literature review we concluded that sporadic cases present as single lesions which manifest by seizures or persistent headaches. Rarely MA has been described to coexist with meningiomas. Histopathologically, MA is characterized by a plaque-like proliferation of meningothelial and fibroblast- like cells surrounding small vessels and trapping islands of gliotic cortical tissue. The lesion does not show significant atypia, mitosis or necrosis. Although all cases of MA share unifying features, there are different degrees of histological presentation with cases predominantly cellular and others more fibrous and calcified. This could correspond to different stages in the evolution of the MA. Symptoms disappear with the complete excision of the lesion.     

Primary dural lymphoma: a report of two cases with review of the literature

Primary lymphoma arising in dura is exceedingly rare. We report the clinicopathologic findings of two patients with primary B-cell lymphoma of dura. Both were female, 38 and 45 years old. Prior to biopsy they were felt to have meningioma on preoperative magnetic resonance imagery. Histologically, tumors were classified as MALT-type lymphoma. Literature describe only 14 reports of similar entity. Primary lymphomas arising in dura appear to have a more favourable clinical course compared to PCNSL and may require a less aggressive treatment.     

尿毒症患者透析后舞蹈样并发症二例分析及文献复习

目的探讨尿毒症患者透析后舞蹈样并发症的临床表现和治疗方法。方法选择2例典型患者进行临床表现及影像学结果回顾性分析。结果尿毒症血透患者维生素B_1缺乏可由多种原因引起,常见于摄入过低和透析中丢失过多。维生素B_1不足,三羧酸循环紊乱,血乳酸增加,从而导致机体出现一系列相关症状。其临床表现多为威尼克氏脑病。结论对尿毒症血透发生基底神经节功能紊乱所引发的舞蹈样症的患者,诊断时应加强对本病的认识。血乳酸和脑基底节影像学检查可为本病提供诊断依据。维生素B_1静脉疗法对本病有明显的缓解作用。     

Meningiomas associated with hemorrhage: A report of two cases with a review of the literature

Two cases of extensive spontaneous hemorrhage related to intracranial angioblastic meningiomas are reported. One of these also had glandular and papillary formations with mucin production. A review of 115 meningiomas with significant hemorrhage including the two current instances disclosed that they occurred in the first to ninth decades of life but were most common in the fourth to eighth decades (85%), with a peak occurrence in the fifth decade (24%). The average age of 51 years was 9 years more than in those patients with non-bleeding meningiomas. The female- to-male ratio was 6:5 for the entire series. Bleeding meningiomas arose most often on the cerebral convexity followed by the parasagittal region, the lateral ventricle, and the sphenoid ridge. Bleeding most frequently involved multiple sites, and was followed in decreasing frequency by hemorrhage into the subarachnoid space, subdural space, intracerebral tissue including the peritumoral region, and intratumoral substance. Meningocytic menin-gioma was the most common type of bleeding meningioma, followed in decreasing frequency by the angioblastic, fibroblastic, transitional and malignant types. The mech-anism of bleeding was uncertain in many cases, and multiple factors are probably involved. Patients with bleeding meningiomas had worse prognoses than those with non-bleeding meningiomas.     

Radio-induced low-grade glioma: report of two cases and review of the literature

With the increasing number of cancer survivors, we can observe a population that will present a higher risk of developing secondary long-term toxicities related to adjuvant chemo and radiotherapy regimens. Among these, children surviving from acute lymphoblastic leukemia (ALL) that were treated with prophylactic cranial irradiation represent a group of patients at a high risk of developing secondary brain tumors. Radiation-induced intracranial tumors have been documented since 1950, and today, more than one-hundred cases have been described. We report our experience with two young patients who were hospitalized for low grade gliomas and had a positive anamnesis for ALL and consequent radiotherapy.     

复发性Fisher-Bickerstaff综合征2例临床分析

目的探讨复发性Fisher综合征(FS)叠加Bickerstaff脑干脑炎(BBE)的临床特点和疾病命名。方法分析2例复发性Fisher-Bickerstaff综合征的患者的临床资料,并复习相关文献。结果例1表现为复发性睡眠增多、走路不稳、视物成双,肌电图检查提示下肢神经源性损害;例2表现为复发性视物成双、走路不稳,第2次发病出现四肢无力,查体双下肢远端肌力3级,双侧Babinski征阳性,肌电图提示四肢神经源性损害;2例患者除具有眼肌麻痹和共济失调的症状外,都有中枢和周围神经受损的表现;依据患者的病史、查体、临床检测和疾病经过,诊断为复发性Fisher-Bickerstaff综合征,第2例患者叠加吉兰-巴雷综合征。结论具有周围神经损害的BBE和具有中枢神经损害的FS从临床表型上不能截然分开,Fisher-Bickerstaff综合征的概念更能准确地表述这类患者的表型特点。     

Relapse of herpes encephalitis after acyclovir therapy: report of two new cases and review of the literature

Relapse of herpes simplex virus (HSV) encephalitis following acyclovir therapy has been reported infrequently in children beyond the neonatal period. The pathogenic mechanism of the recurrence is not fully understood. We report two new cases that support a mechanism of latent HSV infection with reactivation of the disease. Our patients were 2 years (#1) and 8 months (#2) old at initial infection. Both presented with fever, lethargy, focal seizures, and focal motor abnormalities. Serum HSV antibodies (Abs) were negative. The patients were treated with acyclovir for 14 and 21 days, respectively. They were readmitted at 1 month, and 4 days after discharge, respectively, with recurrent lethargy, seizures, and choreo-athetoid movements. Serum and CSF HSV Abs were significantly increased. CSF PCR was positive. In patient # 2 acyclovir-sensitive HSV was isolated from a brain biopsy. Both patients were re-treated with acyclovir, but progressed to a neurovegetative state. In our cases, latent HSV infection and reactivation is the most likely explanation for recurrent encephalitis. The immuno-pathogenic mechanisms of the infection recurrence are discussed. Based on the reported cases in the literature, patients younger than 2 years of age and with lower total dose of acyclovir treatments have a higher risk of recurrence.     

Wernicke's encephalopathy associated with hemodialysis: report of two cases and review of the literature.

Two rare cases of Wernicke's encephalopathy (WE) in non-alcoholic patients on hemodialysis (HD) are reported. They presented with the clinical triad of WE (ophthalmoplegia, ataxia and disturbance of consciousness) and intravenous administration of thiamine led to complete elimination of these manifestations. Reduced plasma thiamine levels prior to the administration confirmed the diagnosis of WE. Interestingly, a reduction in plasma thiamine levels by about half was seen in one of the patients on HD, suggesting that thiamine, a water-soluble vitamin, can be depleted with HD. In the literature, nine HD-dependent patients have been reported to develop WE, seven of whom were diagnosed postmortem. Their premortem diagnoses included uremic encephalopathy, dysequilibrium syndrome and dialysis dementia, which can often complicate HD and present symptoms similar to those of WE. We therefore emphasize that WE, even though a rare complication, should be suspected in all patients on HD who present with at least one of the clinical triad of WE.