大剂量苯巴比妥治疗难治性惊厥性癫痫持续状态的临床观察

目的：观察大剂量苯巴比妥治疗难治性癫痫持续状态（SE）的效果,企为临床工作提供参考。方法：对急诊人院的21例以全身惊厥性SE为突出症状的患者,在针对病因进行积极治疗的同时,进行常规抗癫痫药物（AED）静脉滴注,若癫痫发作症状难以控制则改用大剂量苯巴比妥进行治疗,观察分析其治疗效果。结果：常规AED治疗时,患者24h内癫痫缓解率为20％;没有完全控制者,改用大剂量苯巴比妥静脉滴注后24h内癫痫均得到完全控制。结论：大剂量苯巴比妥治疗难治性SE是安全有效的方法,常规治疗方法无效时可以采用该方法进行治疗。     

结节性硬化症的癫痫手术治疗对发作及智力影响的研究

目的：探讨结节性硬化症TSC患者手术治疗癫痫对其远期疗效及智力的影响。方法：回顾性分析经手术治疗的9例伴癫痫的TSC患者的临床资料,总结发作症状学、EEG及头颅MRI的特点,随访远期发作的控制情况及智力水平。结果：术后随访24～30个月（平均26．1个月）,EngleI级8例,EngleIV级1例。8例手术治疗有效的患者中,5例智力水平显著提高,IQ平均增高18．2,手术时平均年龄为5．7岁。3例无明显变化,IQ平均增高2．3,手术时平均年龄为20．3岁。结论：在一部分TSC患者,当发作症状、MRI上的结节的部位、EEG痼样放电的部位一致时,手术治疗能有效的控制发作。手术时年龄较小的患者随发作的控制智力改善明显。     

结节性硬化症合并癫痫的手术治疗

目的：探讨结节性硬化症（TSC）合并难治性癫痫的手术适应征和手术方式,并介绍手术效果与经验。方法：回顾性分析行手术治疗的19例TSC合并难治性癫痫患者的临床资料。术前评估包括同步录像脑电图（V—EEG）监测、PET或SPECT检查、MRI和CT检查以及智商测试。本组病例中8例为局灶性癫痫、11例为双侧或多灶性癫痫,其中10例患者进行了皮层电图（ECoG）检查,4例不能而6例可以确定局限性癫痫起源灶。9例患者的IQ低于70分,3例IQ正常,7例有轻度的IQ缺陷。手术行胼胝体切开1例,病灶切除术4例,脑叶切除术5例,病灶切除联合脑叶切除术6例,胼胝体切开联合病灶切除术2例,胼胝体切开、病灶切除联合脑叶切除术1例。结果：预后11例患者达到EngleI级,5例Ⅱ级,3例为Ⅲ～Ⅳ级。不能进行致痫灶确切定位的4例术后仍有癫痫发作,与术前定位明确、术后无发作的患者比较差异有显著意义。性别、癫痫起始年龄和IQ与预后无明显相关性。结论：TSC合并局灶性癫痫患者手术治疗效果良好,是进行外科手术的适应征。手术对于TSC合并多灶性癫痫患者的效果比局灶性癫痫患者的效果差,但仍可能通过颅内电极EEG定位进行手术治疗。病灶切除和脑叶切除是治疗TSC的基本手术方式。     

40例癫（间）持续状态患者的临床特点及脑电变化特征

目的：分析不同类型癫痫持续状态（SE）患者的临床特点及脑电图（EEG）变化特征并观察临床疗效。方法：对40例SE患者进行临床分类并记录发作间期及发作期的EEG，静脉注射安定进行治疗并观察其临床疗效。结果：惊厥性SE28例，其中强直阵挛性SE24例，肌阵挛性发作SE1例，单纯部分运动性SE3例；非惊厥性SE12例，其中复杂部分性SE10例不典型失神SE2例，发作间期EEG25例记录到癫痫样放电；发作期EEG，8例病人行Video-EEG监测，7例记录到发作期典型癫痫样放电。1例表现为募集节律。疗效；治愈率95%，死亡率5%。结论：强直阵挛性SE常见，发作间期多可记录到癫痫样波形，肌阵挛性SE、非惊厥性SE临床诊断困难。应行Video-EEG监测；早期足量应用安定是安全有效的治疗措施。     

颅内电极脑电图监测对枕叶致痫灶的定位价值

目的：探讨颅内电极埋藏后视频脑电图（V-EEG）监测对枕叶致痫灶的定位价值。方法：对19例经无创方法难以定位致痫灶的药物难治性枕叶癫痫（OLE）病人,开顷埋置硬脑膜下条状、栅格状电极和深部电极,行V-EEG监测,记录发作间期及发作期脑电图（EEG）变化,确定发作起源区,再手术切除致痫灶。结果：16例埋置硬脑膜下电极,3例联合应用硬脑膜下电极和深部电极。颅内电极V-EEG监测36～192h（平均68h）,均记录到发作间期痫样放电及发作期EEG情况。行枕叶致痼灶切除术后,平均随访24个月,13例发作消失（EngelⅠ级）,4例偶发（EngelⅡ级）,2例发作减少75％以上（EngelⅢ级）。结论：在致痫灶定位困难的顽固性OLE中,开颅埋藏硬脑膜下电极、深部电极,行V-EEG监测,根据癫痫发作初始期异常放电的节律和范围,可精确定位致痫灶。     

癫痫外科颅内电极埋置方法、风险及对策

目的：探讨癫痫外科长期描记的颅内电极埋置手术方法及并发症的处理,提高手术的安全性与成功率。方法：回顾性分析2007年1月至2012年12月接受癫痫手术的403例患者行颅内电极埋置的经验,总结其适应证、操作方法、并发症及应对措施。结果：403例患者接受了408次颅内电极埋置手术,其中5例（i．2％）患者因电极覆盖范围不足而接受了第二次电极埋置术,平均监测时间3．7d（2～21d）。手术风险包括：术后颅内血肿29例（7．2％）,脑水肿5例（1．2％）,脑脊液漏11例（2．7％）,术口感染13例（3．2％）,颅骨骨髓炎5例（1．2％）,电极折断1例（0．2％）。没有因颅内电极埋置术而引起严重神经功能缺失和死亡者。结论：颅内电极埋置长程皮质脑电监测术是癫痫外科一项相对安全的侵袭性检查方法。术前完善无创性检查措施,合理制定手术方案,注意操作细节及技巧,可避免并发症的发生。     

拉莫三嗪及丙戊酸钠对锂-匹罗卡品致痫大鼠的神经保护作用及其对癫痫的治疗作用

目的：观察拉莫三嗪（LTG）及丙戊酸钠（VPA）对锂-匹罗卡品癫痫持续状态（SE）大鼠海马锥体细胞、齿状回门区神经元的保护作用及对癫痫的治疗作用。方法：用大鼠制作锂-匹罗卡品SE动物模型,分三组：SE对照组,LTG治疗组和VPA治疗组。此三组在SE后2h给予安定阻断痫性发作,再分别给予适量生理盐水（NS）、LTG、VPA治疗15d,比较各组海马锥体细胞、门区神经元计数、继发癫痫的发生率及苔藓纤维出芽的评分。另设有正常对照组（NS组）,不制模只给予NS“治疗”。结果：SE对照组、LTG组及VPA组齿状回门区均出现神经元丢失,VPA组及SE对照组均存在海马CA1神经元丢失,但LTG组海马CA1锥体神经元无丢失;三个模型组继发癫痫的发生率及苔藓纤维出芽的评分比较差异无统计学意义。结论：锂-匹罗卡品SE模型中,SE2h后给药,LTG能够对大鼠海马锥体细胞起到神经保护作用,VPA无明显保护作用,但两种抗癫痫药物均不能够阻断癫痫的发生及齿状回苔藓出芽。     

左乙拉西坦治疗青少年肌阵挛癫痫初步探讨

目的：探讨左乙拉西坦（LEV）对青少年肌阵挛性癫痫（JME）的疗效。方法：30例JME患者中,男16例,女14例,平均年龄19．63岁,分为两组。LEV治疗组15例,单用4例,与丙戊酸或（和）氯硝西泮合用11例,治疗剂量500～1000mg／d。随访时间2个月至9年,平均19．20个月;其他药物治疗组15例,包括丙戊酸3例,丙戊酸．＋氯硝西泮3例,单用氯硝西泮5例,妥泰3例,先用LEV后改用氯硝西泮1例,随访3～10年,平均23．08个月。结果：LEV组：发作终止4例（25％）,短期发作停止（3～24个月发作停止）8例（53％）,发作减少≥50％1例（7％）,总有效率为87％;其他药物组：发作终止2例（13％）,短期发作停止7例（47％）,总有效率为60％。两组经统计学处理差异无显著差异（P=0．215）。结论：虽然LEV组与其他药物组疗效比较差异无统计学意义,但因LEV安全性、耐受性及疗效均好,故LEV是治疗JME有希望的首选药物。     

脑高灌注综合征后癫痫

脑高灌注综合征（cerebral hyperperfusion syndrome，CHS）是以头痛、癫痫、意识障碍、局灶性神经功能缺损及颅内出血等为主要临床表现的症状群，常发生于颈动脉血运重建术后（主要是颈动脉内膜切除术后几小时至3周内），     

几种以皮层病灶起源的失神样癫痫发作患者的临床与脑电特征

目的：探讨几种由皮层病灶起源的失神样癫痫的发作分类,并就其临床与脑电特征进行分析讨论,为临床诊断失神样癫痫发作提供有价值的材料.方法：回顾性分析了我所近1年多来进行术前V-EEG监测到皮层病灶起源的24例具有明显失神样癫痫发作患者的临床、EEG及影像学资料.结果：24例中17例患者经头皮与颅内电极[皮层和（或）深部电极]描记EEG并进行了手术.失神发作时以额叶皮层起源放电者5例,颞叶7例,顶-枕叶5例 其余7例中4例属Lennox-Gastaut综合征,另3例因皮层多发病灶未进行手术.不同脑皮层部位起源的放电均可引起失神样发作,但其临床与脑电有各自的特点.脑电图出现3 Hz棘慢综合波时并非全是典型的失神样发作.结论： 失神样癫痫发作可分为全面性与部分性两大类,诊断失神样癫痫发作时应将EEG与临床资料进行综合分析,以免误诊.     

海洛因成瘾者停用海洛因后的脑功能情况-静息状态下fMRI研究

目的：探讨海洛因成瘾者停止用药后静息状态下脑功能的变化情况。方法：对海洛因成瘾者在停止使用海洛因3天（10人），1个月（10人）以及2个月（14人）时分别在静息状态下进行功能磁共振检测。并与正常对照进行比较。结果：成瘾者停用海洛因3天时出现额叶内侧，扣带回。颞上回等异常，停用1个月，额叶脑功能异常加重，海马功能出现异常，停用2个月，脑功能逐步恢复。结论：脑区的变化可能反映出海洛因成瘾者停药后渴求和复发可能性先增加。再下降的变化过程。额叶和海马发生变化时有时间上的关联性。本研究结果一方面为成瘾的动机假说提供了新的支持，另一方面提出海洛因依赖者在停药1个月左右复发的风险最大。     

Oro-facio-digital syndromes I and II: radiological methods for diagnosis and the clinical variations

In view of the different modes of inheritance and the different prognoses of the two oro-facio-digital syndromes, type 1 (OFD-I) and type 2 (OFD-II), it is important to establish a correct diagnosis in these patients. In this report two new patients with the OFD-I syndrome are presented. One of them (Case 1) had multiple congenital malformations and never made any mental contact. She died at the age of four months and autopsy of the brain revealed abnormalities typical of the syndrome, which are discussed. The other patient (Case 2) has so far had normal mental development. Although these two patients were affected to a very different degree, they both presented the clinical and radiological characteristics of the OFD-I syndrome. These two patients and previously reported cases of the OFD-I and OFD-II syndromes were compared with a patient with the OFD-II syndrome (Case 3), a patient reported earlier who is undergoing follow-up. The radiological features of the skeleton in the two syndromes are presented. The irregular mineralization of the hands and feet characteristic of OFD-I, but not of OFD-II, seems to offer a good opportunity to distinguish between these two syndromes. It is suggested that this finding is pathognomonic for the OFD-I syndrome.     

Longitudinal Flow Cytometry Identified “Minimal Residual Disease” (MRD) Evolution Patterns for Predicting the Prognosis of Patients with Transplant-Eligible Multiple Myeloma

Many questions about minimal residual disease (MRD) still need to be answered for multiple myeloma (MM). Flow MRD was monitored in 104 consecutive patients with MM after induction and at the 3rd, 6th, 9th, 12th, 18th, and 24th months post-transplant. Four MRD evolution patterns were revealed: Pattern 1 patients had persistent MRD-negative status after post-induction with no progression; pattern 2 patients had MRD-positive status postinduction but became MRD negative within 24 months post-transplant; pattern 3 patients had MRD-negative status postinduction but became MRD positive within 24 months post-transplant; and pattern 4 patients had persistent MRD-positive status after postinduction. Patients with MRD evolution pattern 1 had a better time to progression than did patients with the other evolution patterns (not reached versus not reached, versus 15.4 ± 2.4 months, versus 16.9 ± 3.0 months; log-rank test, P?=?.003, P?=?.000, and P?=?.000, respectively). Patients with MRD pattern 1 had a significantly longer overall survival than did patients with pattern 3 (not reached versus 35.2 ± 18.6 months; log-rank test, P?=?.000) and pattern 4 (not reached versus 23.8 ± 15.0 months, log-rank test, P?=?.000) but had a similar overall survival as pattern 2 patients (not reached versus not reached; log-rank test, P?=?.229). For progressing patients with MRD evolution pattern 2 or 3, the median interval of a sustained MRD-negative status was only 17 months and the median time from MRD reappearance to disease progression was only 4.6 months. A more complete MRD evolution pattern was developed to predict the outcomes for patients with MM. The optimal time of MRD assessment should include postinduction and 3rd and 24th month post-transplant. Regular MRD assessments will help detect relapse early. A sustained negative MRD status should last for at least 24 months.     

Clinical Analysis of Patients who Survived for Less than 3 Months After Brain Metastatectomy

In the patients with brain metastasis (BM), it is impossible to determine who will benefit from surgery because of limited survival. In an attempt to identify optimal candidates for brain metastatectomy, we analyzed patients who survived for <3 months after craniotomy for a single BM lesion. Between January 1st, 1997 and July 31st, 2007, 83 patients with a single BM underwent craniotomy. Of these patients, 25 patients (30.1%) died within 3 months of craniotomy. The primary lesions were non-small call lung cancer in 15, colon cancer in 6, and breast cancer, renal cell carcinoma, ovarian cancer, or esophageal cancer in one apiece. Of the 25 patients, 19 (79%) were of tumor stage IV and had extra-cranial metastasis. Eleven (44%) of the 25 primary cancers had a well-controlled status. Twelve patients (48%) had a Karnofsky Performance Scale (KPS) score of <70, and 13 (52%) were of Recursive Partitioning Analysis (RPA) class 3. Primary cancer status, RPA class, and functional status were found to be critical factors for consideration when selecting surgical candidates. In addition, adjuvant therapy was found to have an important role on survival.     

Stem cell transplantation nephropathy: a report of six cases.

Stem cell transplantation (SCT) nephropathy is 1 cause of chronic kidney disease in patients after allogeneic SCT. It is a thrombotic microangiopathic syndrome characterized by raised creatinine, hypertension, and anemia. The difference with thrombotic thrombocytopenic purpura (TTP)-like syndromes is that it occurs later than 3 months after SCT, has marked renal dysfunction, and occurs in the absence of other complications or nephrotoxic medication. Total-body irradiation (TBI) in combination with previous chemotherapy is most likely the cause. We describe 6 cases of SCT nephropathy that occurred in a cohort of 363 patients who received myeloablative allogeneic SCT. All patients had TBI with shielding of the kidneys. We discuss the course of the syndrome, treatment, and outcome of the patients.     

The psychobiology of minor head injury

Twenty-six consecutive admissions to an accident and emergency unit with minor head injury were examined. This was defined as a head injury warranting brief in-patient overnight stay but with a post-traumatic amnesia of less than 12 hours. Each patient had a neurological examination, a post-traumatic symptom check list completed, EEG power spectra analysis and auditory brain stem-evoked potential recordings, and a four-choice reaction-time measurement. These assessments were repeated six weeks later. Six months after the head injury a symptom check list was completed and four-choice reaction time measured again. Post-traumatic symptoms are persistent in half of all patients at six weeks and six months follow-up. The EEG power spectra showed a significant change in theta power between the first recording and the second one at six weeks, with relative reduction being noted. Approximately half of all patients had significant delays in brain stem conduction time at day 0. There was a trend towards a decrease in brain stem conduction time at six weeks, though in almost half the brain stem conduction time still remained abnormal at six weeks. Head-injured patients had prolonged choice reaction times at day 0 with serial improvement between then and six months, though the values at six weeks were still significantly longer than healthy controls. It is suggested that these findings reflect both cortical and brain stem damage following minor head injury, the brain stem damage being more persistent. There appear to be three patterns of recovery, half recovering within six weeks, a minority persisting over six months with persisting brain stem dysfunction and less than a third showing an exacerbation of symptoms with no evidence of brain stem dysfunction, the exacerbation being possibly a consequence of psychological and social factors.     

Theta-Band Oscillations as an Indicator of Mild Traumatic Brain Injury

Mild traumatic brain injury (mTBI) patients continue to pose a diagnostic challenge due to their diverse symptoms without trauma-specific changes in structural imaging. We addressed here the possible early changes in spontaneous oscillatory brain activity after mTBI, and their feasibility as an indicator of injury in clinical evaluation. We recorded resting-state magnetoencephalography (MEG) data in both eyes-open and eyes-closed conditions from 26 patients (11 females and 15 males, aged 20–59) with mTBI 6 days–6 months after the injury, and compared their spontaneous oscillatory activity to corresponding data from 139 healthy controls. Twelve of the patients underwent a follow-up measurement at 6 months. Ten of all patients were without structural lesions in MRI. At single-subject level, aberrant 4–7 Hz (theta) band activity exceeding the +?2 SD limit of the healthy subjects was visible in 7 out of 26 patients; three out of the seven patients with abnormal theta activity were without any detectable lesions in MRI. Of the patients that participated in the follow-up measurements, five showed abnormal theta activity in the first recording, but only two in the second measurement. Our results suggest that aberrant theta-band oscillatory activity can provide an early objective sign of brain dysfunction after mTBI. In 3/7 patients, the slow-wave activity was transient and visible only in the first recording, urging prompt timing for the measurements in clinical settings.     

Prognostic factors for outcomes of patients with refractory or relapsed acute myelogenous leukemia or myelodysplastic syndromes undergoing allogeneic progenitor cell transplantation.

Allogeneic progenitor cell transplantation is the only curative therapy for patients with refractory acute myelogenous leukemia or myelodysplastic syndromes. To identify prognostic factors in these patients, we performed a retrospective analysis of transplantation outcomes. Patients were selected if they had undergone an allogeneic transplantation between January 1988 and January 2002 and were not in remission or first untreated relapse at the time of transplantation. A total of 135 patients were identified. The median age was 49.5 years (range, 19-75 years). At the time of transplantation, 39.3% of patients had not responded to induction therapy, 37% had not responded to first salvage therapy, and 23.7% were beyond first salvage. Forty-one patients (30%) received unrelated donor progenitor cells. Eighty patients (59%) received either a reduced-intensity or a nonmyeloablative regimen. A total of 104 (77%) of 135 patients died, with a median survival time of 4.9 months (95% confidence interval, 3.9-6.6 months). The median progression-free survival was 2.9 months (95% confidence interval, 2.5-4.2 months). A Cox regression analysis showed that Karnofsky performance status, peripheral blood blasts, and tacrolimus exposure during the first 11 days after transplantation were predictive of survival. These data support the use of allogeneic transplantation for patients with relapsed or refractory acute myelogenous leukemia/myelodysplastic syndromes and suggest that optimal immune suppression early after transplantation is essential for long-term survival even in patients with refractory myeloid leukemias.     

Characteristics of breast cancer patients with central nervous system metastases: a single-center experience

The aim of this study was to assess the characteristics of breast cancer patients with central nervous system (CNS) metastases and factors associated with survival after development of CNS metastasis. One-hundred-forty-four patients with brain metastases were retrospectively analyzed. Median age at the time of brain metastasis diagnosis was 48.9. Median time between initial diagnosis and development of brain metastasis was 36 months. Fourteen cases had leptomeningeal involvement. Twenty-two patients (15.3%) had single metastasis. Ten percent of the patients had surgery, 94% had radiotherapy and 63% had chemotherapy. Median survival after development of brain metastasis was 7.4 months. Survival of patients with single metastasis was significantly longer than those with multiple metastases (33.5 vs. 6.5 months, p = 0.0006). Survival of patients who received chemotherapy was significantly longer than those who received radiotherapy alone (9.9 vs. 2 months, p < 0.0001). In multivariate Cox regression analyses, presence of single metastasis and application of chemotherapy were the only significant factors associated with better survival (p = 0.047 and p < 0.0001, respectively). Age at initial diagnosis or at the time of brain metastasis, time from initial diagnosis to development of brain metastasis, menopausal status, tumor stage, grade, hormone receptor or HER2 status individually were not associated with survival. In this study, survival after the diagnosis of CNS metastases appeared to be affected by patient characteristics rather than biologic characteristics of the tumor. This is probably secondary to the lack of effective treatment options in these patients and overall poor prognosis.     

Role of Palliative Radiotherapy in Brain Metastases

Background:

Brain metastases are a common manifestation of systemic cancer and exceed primary brain tumors in number and are a significant cause of neurologic problems. They affect 20-40% of all cancer patients. Aggressive management of brain metastases is effective in both symptom palliation and prolonging the life. Radiotherapy has a major role to play in the management of brain metastases.

AIM:

The aim of the study was to know the outcome of palliative radiotherapy in symptomatic brain metastases in terms of improvement in their performance status.

Materials and Methods:

This is a retrospective study of 63 patients diagnosed to have brain metastases and treated with palliative whole brain radiotherapy to a dose of 30 Gy in 10 fractions over two weeks between June 1998 and June 2007. Diagnosis was done in most of the cases with computed tomography scan and in a few with magnetic resonance imaging. Improvement in presenting symptoms has been assessed in terms of improvement in their performance status by using the ECOG scale.

Results:

Fifty-four patients completed the planned treatment. Eight patients received concurrent Temozolamide; 88% of patients had symptom relief at one month follow-up; 39/54 patients had a follow-up of just one to three months. Hence survival could not be assessed in this study.

Conclusion:

External beam radiotherapy in the dose of 30 Gy over two weeks achieved good palliation in terms improvement in their performance status in 88% of patients. Addition of concurrent and adjuvant Timozolamide may improve the results.