Monofocal acute large demyelinating lesion mimicking brain glioma

Neurological Sciences - We report the case of a 34-year-old woman with clinical, neuroradiological and intraoperative histological findings, suggesting a low-grade astrocytic tumour. The...     

Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients

This retrospective analysis was undertaken to determine whether a subset of diabetic patients with demyelinating polyneuropathy were similar to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Ten patients meeting the clinical criteria for idiopathic CIDP were compared to nine patients with diabetes and demyelinating polyneuropathy. The diabetic patients with demyelinating polyneuropathy displayed clinical, electrophysiologic, and histologic features that were similar to those in CIDP patients. All six patients with diabetes and demyelinating polyneuropathy who were treated with immunomodulatory therapy showed a favorable response. Our study highlights the importance of investigating diabetic patients with polyneuropathy in an attempt to identify patients with demyelinating polyneuropathy, because of the likelihood of benefit in these patients from immunomodulatory treatment.     

Large demyelinating brain lesion mimicking a herniating tumor

We describe the case of a large brain lesion whose computed tomography appearance and clinical evolution mimicked a herniating tumor. The patient progressed to coma within 6 days of hospitalization despite high-dose steroid treatment. Emergency excision of the lesion was carried out. Histological analysis showed massive demyelination, axon preservation and no tumor cells. No lesions recurrence was seen during a 55-month follow-up. Recognition of such lesions through magnetic resonance imaging or spectroscopy may spare unnecessary surgery or biopsy. However, our case shows that such lesions may still require resection in the face of a rapid clinical progression and poor response to medical treatment. Received: 30 December 2000 / Accepted in revised form: 23 July 2001     

Chronic inflammatory demyelinating polyradiculoneuropathy with tumefactive central demyelination

A 46-year-old man with a 1-year history of distal paresthesias and mild distal weakness subacutely developed paralysis of the left hand. Electrodiagnostic evaluation revealed a demyelinating peripheral neuropathy that met criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Magnetic resonance imaging of the brain revealed a mass that enhanced with contrast, but revealed focal myelin loss with intense macrophage activity and axonal preservation on biopsy. The mass and hand weakness improved following steroid therapy. The combination of CIDP and central demyelination with mass effect broadens the spectrum of demyelinating disease in association with CIDP.     

Chronic inflammatory demyelinating polyradiculoneuropathy with autonomic involvement

We report a case of chronic acquired neuropathy predominantly affecting sensory and autonomic nerves. Investigations showed a demyelinating polyradiculoneuropathy with axonal degeneration and depletion of postganglionic noradrenergic fibers in the rectal mucosa. Intravenous immunoglobulin and corticosteroid administration were effective in alleviating symptoms and improving electrophysiological abnormalities. This neuropathy may be a novel variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), in which autoimmunoreactivity is directed not only against myelin but also against axon- or ganglion-composing protein. Autonomic nerve involvement does not exclude a diagnosis of CIDP.     

Chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis

We report a patient with chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis (PBC). Except for minimal biochemical abnormalities, clinical symptoms of PBC were not observed, and we diagnosed our patient with asymptomatic PBC from the results of a liver biopsy. Although the patient noticed little muscle weakness, an electrophysiological study demonstrated slow conduction velocities and prolonged distal latencies, with definite conduction blocks in the median, ulnar, and tibial nerves. The disturbed sensory pattern was asymmetrical, and sensory nerve action potentials were not evoked. From these observations, we diagnosed this patient with chronic inflammatory demyelinating polyneuropathy. Neuropathy associated with PBC is very rare. We must differentiate demyelinating neuropathy with PBC in patients with asymmetrical sensory dominant neuropathy with high immunoglobulin M titers, and investigate for the presence of anti-mitochondrial antibodies to rule out a complication of asymptomatic PBC.     

改进超声技术在脑胶质瘤术中应用

目的改进术中超声技术,评价超声在脑胶质瘤术中判断残余肿瘤价值。方法选择高频(12 MHz)专用术中探头,采用残腔腔内与残腔表面扫查结合的检查方法,在37例拟行肿瘤根治术脑胶质瘤患者常规显微手术结束时行超声检查。将超声影像与病理组织学检查结果对比,评价超声判断胶质瘤残余肿瘤的能力和价值。结果通过改进扫查方法,可获得高质量术中影像,降低了病理基础不确定的超声征象的出现率,避免了其对诊断的干扰。本组脑胶质瘤术中超声诊断残余肿瘤敏感性、特异性及准确性分别为65．71%(23/35)、98．34%(178/181)和93．06%(201/216)。假阴性诊断中的91．67%(11/12)发生于高分级胶质瘤,以胶质母细胞瘤明显。结论通过改进检查技术,获得高质量术中实时影像,超声可作为脑胶质瘤手术术中判断残余肿瘤安全可靠的影像手段。     

CNS involvement in Japanese patients with chronic inflammatory demyelinating polyradiculoneuropathy

Thirteen consecutive Japanese patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were studied by MRI, evoked potentials, and EEG. We found 3 of these patients exhibited symptoms of CNS disorders. Of these 3, 2 with abnormal MRI and visual evoked potentials, and one with abnormal brainstem auditory evoked potentials were detected. Another case without clinical CNS signs showed abnormal EEG findings. The subclinical CNS abnormalities found in the Japanese patients were considered to be less frequent than in cases from Western countries reported previously.     

Immunosuppressive treatment in refractory chronic inflammatory demyelinating polyradiculoneuropathy. A nationwide retrospective analysis

Background and purpose: There are other options open to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who are non‐responders to conventional treatment, including immunosuppressive and immunomodulatory agents (IA). The aim of this study was to assess whether the use of IA is able to increase the number of responders. Methods: Clinical and electrophysiological data of patients with refractory CIDP, followed at 10 Italian centres, were collected, and the clinical outcome (Rankin Scale) and drug side effects (SE) for the different therapies were analysed. Results: A total of 110 patients were included. These patients underwent 158 different therapeutic procedures with IA. Seventy‐seven patients were treated with azathioprine, 18 rituximab, 13 cyclophosphamide, 12 mycophenolate mofetil, 12 cyclosporine, 12 methotrexate, 11 interferon‐alpha and three interferon beta‐1a. The percentage of patients who responded to azathioprine (27%) was comparable to the percentage of responders to other therapies, after the exclusion of interferon beta‐1a that was not effective in any of the three patients treated. The percentage of SE ranges from 8% (methotrexate) to 50% (cyclosporine). Conclusions: One‐fourth of patients, refractory to conventional treatment, showed an improvement in their disability with IA. Methotrexate had the lowest SE; cyclosporine was associated with severe SE and often led to drug discontinuation.     

大鼠脑干胶质瘤模型的建立

目的 建立大鼠脑干胶质瘤模型,为脑干胶质瘤的研究提供动物实验平台.方法 将1×10~6个大鼠C6胶质瘤细胞通过立体定向头架注射到大鼠脑桥,种植后2周对大鼠行磁共振扫描观察,然后行灌流固定取脑,HE染色.结果 磁共振检查均发现肿瘤生长,肿瘤为长T1和T2信号,经大鼠舌静脉注射Gd-DTPA信号明显增强.HE染色显示肿瘤为胶质母细胞瘤,呈浸润性生长,瘤内新生血管丰富,假栅栏样坏死明显.结论 大鼠C6胶质瘤细胞株可以建立脑干胶质瘤模型,成瘤率高,重复性好.     

脑干占位病变治疗及相关问题探讨

目的探讨脑干占位病变外科手术治疗和非手术治疗的适应证、方法、手术入路、切除方式和并发症防治等问题.方法分析总结1999～2005年我院住院治疗的55例脑干占位病变的临床资料.接受显微手术治疗40例,其中颞下经天幕入路5例,经中孔一小脑延髓裂入路15例,经蚓体切开入路12例,经小脑半球切开入路2例,经桥小脑角脑干侧方入路4例,经纵裂一第三脑室入路2例.术后接受放疗和（或）化疗13例.非手术治疗15例,接受放疗5例,化疗3例.结果手术治疗者术后6个月随访,Karnofsky生活质量评分为90～100分8例,70～80分17例,40～60分6例,20～30分3例,10～0分6例（手术后1个月内死亡）.非手术治疗者住院后6个月随访,Karnofsky生活质量评分为90～100分1例,70～80分4例,40～60分2例,20～30分3例,10～0分5例（死亡4例）.结论脑干占位病变以出血性病变（包括海绵状血管畸形）、星型胶质细胞瘤和室管膜瘤多见.出血患者多数可在早期进行手术治疗,出血较少和已进入出血后期可采用非手术治疗.脑干肿瘤中胶质细胞瘤最常见,除弥漫生长型不适合手术和放化疗外,其他生长类型的肿瘤都可以进行显微手术治疗,术后根据病理性质接受放化疗.手术入路的选择和脑干切开的部位取决于病变在脑干中的位置,可以利用的神经功能缺损,患者可以耐受的神经功能缺损;在具体操作时采取循瘤原则、最短原则和避重就轻原则.经中孔-小脑延髓裂入路可以满足大部分经脑干背侧切除占位病变的需要.     

Reversible postictal MRI change mimicking structural lesion

A reversible change on magnetic resonance imaging (MRI) following generalised seizure mimicking a tumour-like structural lesion is reported in a 15-year-old patient. MRI revealed a left fronto-parietal cortico-subcortical lesion on T2 weighted images. The control MRI after 5 weeks from the onset revealed no pathological finding. The reversible MRI changes may be the result of a local brain swelling, and a defect of cerebral autoregulation during seizure at the site of activity. The transient nature of such neuroradiological findings have to be taken into consideration in the differential diagnosis because of their similar appearance on imaging to intrinsic brain tumours.     

脑胶质瘤浸润性和抗血管增生治疗

脑胶质瘤临床治疗效果不佳，这和其高度浸润、侵袭特性相关。当临床上发现脑内实体性占位时，瘤细胞已向周围正常脑组织广泛浸润、扩散，开颅手术只能切除可见的肿瘤实体占位，不能根除全部的肿瘤细胞。脑胶质瘤细胞的侵袭、转移和脑内血管以及肿瘤新生血管关系极为密切，抗血管增生治疗有可能对抗脑胶质瘤侵袭、转移，使其变成真正的局部占位病变，一定程度上提高了全切肿瘤实体占位后实际的临床效果，改善脑胶质瘤患者的预后。     

Clinical manifestations of chronic inflammatory demyelinating polyneuropathy with anti-cardiolipin antibodies

OBJECTIVE: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune syndrome where certain autoantibodies define clinicopathologic subgroups. In the present study, serum anti-cardiolipin antibodies (aCL) were evaluated. MATERIALS AND METHODS: We investigated aCL in sera from 21 patients diagnosed with CIDP in our hospital between 1991 and 2001. The four CIDP patients with aCL (aCL+) were compared with 17 patients without aCL (aCL-). RESULTS: All aCL+ patients displayed sensory-motor polyneuropathy, with severity and distribution of weakness resembling those in aCL- patients. Anti-nuclear antibody titer of aCL+ patients were significantly higher than those in aCL- patients. None of aCL+ patients presented clinical manifestations of primary anti-phospholipid syndrome (APS), such as thromboses or recurrent abortion. Although the aCL+ patients were older and had more complications and more severe pathologic features than aCL- patients, they responded well to steroid pulse or intravenous immunoglobulin. CONCLUSION: The aCL in CIDP apparently differ from 'autoimmune' aCL in APS, instead being among the autoantibodies pathologically involved in CIDP subgroups.     

脑肿瘤干细胞与胶质瘤生物治疗研究进展

脑肿瘤是中枢神经系统常见疾病之一,对人类健康乃至生命的危害极大。脑肿瘤发病率约占全身肿瘤的5％,占儿童肿瘤的70％,且近年来仍呈上升趋势。其中以胶质细胞瘤发病率最高,约占脑肿瘤总数的40.49％,综合发病年龄高峰位于30～40岁,或10～20岁。     

CT-guided stereotactic biopsies of brain stem lesions: personal experience and literature review

Abstract. Appropriate therapy of brain stem lesions should be guided by an accurate diagnosis. Clinical evaluation combined with modern neuroimaging techniques may nowadays approach the diagnosis but not always with accuracy, thus leading to erroneous treatment. We report a series of 11 patients who underwent stereotactic biopsy for brain stem lesions. In 8 patients, the lesion was approached transfrontally. In the remaining 3 patients, the lesion was approached via the suboccipital transcerebellar route. There was no surgical mortality. Precise histological diagnosis was achieved in all patients. Three patients (27.2%) suffered complications attributed to the procedure and were successfully treated conservatively. Histological results were in accordance with preoperative diagnosis in 9 patients (81.8%). Despite the limited number of patients, our data suggest that stereotactic biopsy of brain stem lesions is a safe technique that can obtain adequate tissue for histological diagnosis, thus providing each patient with the best available treatment.     

Chronic inflammatory demyelinating polyneuropathy after Campylobacter jejuni infection mimicking vasculitic mononeuritis multiplex in a diabetic

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a clinically heterogeneous disorder. Recent reports suggest that CIDP could be more frequent in diabetics. We report here a case of CIDP presenting as mononeuritis multiplex with accompanying cranial nerve involvement in an insulin-dependent diabetic who presented a preceding Campylobacter jejuni infection and misleading skin lesions. Treatment with intravenous immunoglobulins was successful. This case suggests that CIDP should be considered as a potential diagnosis in all diabetics with localized, acute or sub-acute onset, and multiple, or overlapping mononeuropathies.     

立体定向开放显微手术治疗脑内致痫小病灶

目的脑内致癫痫小病灶术前、术中的精确定位和病灶切除,是手术治疗效果的关键。探讨立体定向开放微创手术,皮层电极监测下切除脑内致痫小病灶的手术方法。方法53例症状性癫痫病例,CT、MR I检查有脑内小病灶(直径在0.5~3.0 cm),24 h视频脑电图确认致痫灶为脑内单发病灶。ASA 601S型立体定向仪CT引导辅助全麻环钻开颅,导针穿刺放置导管引导,显微镜下手术分离、切除病灶,皮层脑电图确认将致痫灶切除。结果病灶全切率达96.2%,术后50例得到随访,随访时间5~12个月,平均6.3个月,癫痫消失45例,脑电图检查记录到癫痫波11例,临床癫痫发作5例。因肺癌死亡3例。结论CT立体定向引导,显微手术切除颅内致痫小病灶,术中皮层电极确认将致痫灶切除,是一种定位精确、微创、安全、有效的治疗方法。     

大脑半球胶质瘤切除术中锥体束移位的初步研究

目的 探索大脑半球胶质瘤切除术中锥体束的移位情况及解决方法.方法 选择术前影像学诊断为额、颞、岛叶胶质瘤的患者63例,手术采用仰卧位下额颞入路,术前及术中应用MRI弥散张量成像技术进行扫描,并进行锥体束的追踪描计,对比术前及术中锥体束的位置.结果 锥体束在前后方向上18例发生向前移位,移位距离从0.9 mm至11.4 mm,42例发生向后移位,移位距离从1.7 mm至10.4 mm,3例无移位;锥体束在左右方向上31例发生向外移位,移位距离从1.5 mm至11.2 mm,30例发生向内,移位距离从1.3 mm至8.1 mm,2例无移位.结论锥体束的移位的方向和距离具有不可预测性,最佳解决方案是通过术中影像及神经导航的实时更新并予以保护.     

干细胞移植治疗脑胶质瘤的研究进展

尽管脑胶质瘤的治疗取得不断的进步，但其致残率和死亡率仍很高，其难以根除的主要原因是肿瘤细胞对脑组织的高度浸润性。传统的外科手术辅助放疗、化疗方法对正常神经组织的破坏及副作用很大，如放射性坏死、认知障碍和脑白质病等。近几年随着干细胞技术的兴起，利用干细胞作为载体的脑胶质瘤基因治疗受到广泛关注，该治疗方法的优势在于既能除去肿瘤细胞又有组织修复功能。本就干细胞治疗神经胶质瘤的细胞来源、肿瘤趋向性、治疗机理及体内监测等方面的现状及进展进行简要回顾和展望。[第一段]