Pathological laughter as a presenting symptom of acoustic schwannoma: report of two cases.

Two cases of acoustic schwannoma in a 48-year-old woman and 18-year-old male are reported. Both patients presented with an extremely unusual symptom of pathological laughter as a principal presenting symptom. Complete resection of the tumour via a retrosigmoid route resulted in an immediate resolution of pathological laughter.     

脑干肿瘤的显微外科治疗

本文报告了19例经显微外科手术治疗的脑干肿瘤,其中肿瘤全切4例,大部分切除9例,部分切除6例.除1例术后第8d死于消化道出血,2例症状加重外,其余均获得良好疗效.初步分析了脑干肿瘤的性质,好发部位,临床症状和体征.讨论了脑干肿瘤的手术指征,手术入路,手术要点,术后并发症的防治和综合治疗.     

Localizing brain stem lesions with brain stem auditory evoked potentials

Based on a survey of brain stem auditory evoked potentials (BAEPs) in animals and humans with localized brain stem lesions compared to the recognizability of BAEP components in normal materials, it was concluded that BAEP examination might localize lesions to a high or low level and diffusely within the brain stem. In 155 patients with multiple sclerosis, vascular disorders or other lesions from a neurological department, the involvement of the brain stem was clinically estimated to be negative, situated at a high or low level or diffuse. Correlation between location of the lesions assessed by clinical versus BAEP examination showed correspondence between negative and diffusely abnormal findings, but not with regard to levels within the brain stem. CT-scanning similarly failed to localize lesions to various levels. This was probably due to poorly circumscribed or widespread lesions. The main value of BAEP in neurological patients was to establish clinically silent lesions, especially in patients with multiple sclerosis and only spinal signs.     

Evaluation and graduation of brain stem auditory evoked potentials in patients with neurological diseases

Based on normative data from 40 subjects, criteria for evaluating brain stem auditory evoked potentials have been elaborated. This system contained six grades from normal to diffusely abnormal, and was used in evaluating recordings from 142 patients, including 38 patients with definite and 48 with probable and possible multiple sclerosis, 18 patients with brain stem infarctions, six patients with other organic brain stem lesions, six patients with a postcommotional syndrome and 26 patients without any brain stem involvement. A statistically significant difference was found between normal persons and patients without evidence of brain stem lesions versus patients with such evidence, as well as between subgroups of slightly and severely affected patients. The system was found useful as a way of describing recordings, in company groups of patients and in a follow up of a few patients.     

脑干肿瘤的手术治疗

手术治疗6例脑干肿瘤病人,次全切除2例,大部切除1例,部分切除3例.术后临床表现明显改善1例,改善3例.无变化2例,无一例术后死亡.作者认为脑干肿瘤手术治疗成功的关键在于手术适应证的选择.弥漫性脑于肿瘤不适合手术治疗,局限性脑于肿瘤、囊性脑干肿瘤、颈髓延髓交界处脑于肿瘤及背侧外生性脑干肿瘤适合手术治疗.其中囊性脑干肿瘤、颈髓延髓交界处脑于肿瘤、背侧外生性脑干肿瘤常能做到肿瘤的全切除或次全切除.     

瞬目反射及脑干听觉诱发电位对脑干梗死疗效的评价

目的探讨瞬目反射和脑干听觉诱发电位对脑干梗死患者疗效的评价作用。方法40例经临床症状、体征定位于脑干髓内并经头部MRI检查明确诊断的脑干梗死患者(脑桥梗死31例、中脑梗死4例和延髓梗死5例),分别计算治疗前后瞬目反射各成分的平均潜伏期;观察脑干听觉诱发电位波形及各波潜伏期、峰间期的变化。结果经治疗后40例患者临床症状及体征均有不同程度改善,治疗前后瞬目反射各成分平均潜伏期之间差异无统计学意义(P>0.05);3例脑桥梗死患者R1波恢复正常,临床基本痊愈。治疗前后脑干听觉诱发电位各波潜伏期及峰间期比较,差异亦无统计学意义(P>0.05);治疗后中脑梗死及脑桥梗死患者各有2例恢复正常,临床症状明显好转。结论瞬目反射与脑干听觉诱发电位均能敏感地反映脑干功能的变化,与临床表现具有一致性,但在发病15d内大部分病例的电生理活动不能恢复正常。     

Dissociative brain stem lesions in cephalothoracopagus twins

This report concerns unusual central nervous system (CNS) abnormalities in female cephalothoracopagus twins delivered after 29 weeks of pregnancy, and who died soon after birth. The two bodies, fused to a single umbilical cord, had one head and face, and four arms and four legs. Thorax, lungs, heart, esophagus and stomach were shared by both. In the CNS, there was one cerebrum with two separate pontes, cerebella, medulla oblongatae and spinal cords, which were set at 60d? to each other. The diencephalon was shared by the twins. In each brain stem, the nuclei of motor trigeminal, abducent, facial and hypoglossal nerves were rudimentary on the conjoined sides, but the neurons in other nuclei such as the locus ceruleus, pontine nucleus, superior and inferior olivary nuclei, and the area postrema were preserved bilaterally. The pyramidal tract in the brain stem and spinal cord was affected only on the conjoined side. These brain anomalies provide valuable information for investigations dealing with CNS development.     

Similarity on neural stem cells and brain tumor stem cells in transgenic brain tumor mouse models

Although it is believed that glioma is derived from brain tumor stem cells, the source and molecular signal pathways of these cells are still unclear. In this study, we used stable doxycycline-inducible transgenic mouse brain tumor models (c-myc⁺/SV40Tag⁺/Tet-on⁺) to explore the malignant trans-formation potential of neural stem cells by observing the differences of neural stem cells and brain tumor stem cells in the tumor models. Results showed that chromosome instability occurred in brain tumor stem cells. The numbers of cytolysosomes and autophagosomes in brain tumor stem cells and induced neural stem cells were lower and the proliferative activity was obviously stronger than that in normal neural stem cells. Normal neural stem cells could differentiate into glial fibrillary acidic protein-positive and microtubule associated protein-2-positive cells, which were also negative for nestin. However, glial fibrillary acidic protein/nestin, microtubule associated protein-2/nestin, and glial fibrillary acidic protein/microtubule associated protein-2 double-positive cells were found in induced neural stem cells and brain tumor stem cells. Results indicate that induced neural stem cells are similar to brain tumor stem cells, and are possibly the source of brain tumor stem cells.     

引起脑干受压胆脂瘤的临床评价

目的探讨引起脑干受压的胆脂瘤的临床表现,影像学特点,手术治疗及预后情况。方法经磁共振成像（MRI）和计算机体层摄影（CT）及手术证实的胆脂瘤9例,位于脑干腹侧面8例,脑干左侧1例。临床表现早期无症状或不典型,晚期可具有脑干轻微受压表现。全部行手术治疗,其中肿瘤及包膜全切2例,包膜及肿瘤次全切3例,包膜保留内容全切4例。结果4例术后出现化学性脑膜炎,经治疗后痊愈,5例表现为一组或多组脑神经障碍。结论①引起脑干受压的胆脂瘤,其影像学改变与临床表现不同步,影像学反映的病灶大,临床上却不能表现相应的症状。②手术治疗以保留神经功能为主,不必强行剥离肿瘤包膜。     

Ondine''s curse in listeria monocytogenes brain stem encephalitis

A case of necrotizing brain stem encephalitis due to listeria monocytogenes is described in a 48-year-old man who had brain stem encephalitis of complicated course and with selective destruction of the vasomotoric and respiratory centers. He developed that very rare Ondine's curse syndrome, being only able to breathe when awake. The literature on Ondine's curse and brain stem encephalitis due to LM is reviewed. Brain stem encephalitis has a mortality near 100%. The only treatment for Ondine's curse is lifelong artificial ventilation.     

脑干神经节胶质瘤的诊断及治疗

目的总结脑干神经节胶质瘤的诊治经验。方法回顾性分析10例脑干神经节胶质瘤的临床资料。肿瘤均位于延髓,其中单纯位于延髓3例,累及上颈髓3例,累及脑桥3例,累及小脑1例。均经枕下后正中入路,行显微手术治疗。结果肿瘤近全切除6例,大部分切除2例,部分切除2例。术后辅助放疗2例。病理诊断为神经节胶质瘤。随访3个月～12年,失访2例;存活7例,未见肿瘤复发;术后4个月因急性呼吸道梗阻死亡1例。结论显微神经外科手术是脑干神经节胶质瘤的首选治疗方法,手术主要切除突出到脑干外及脑干内相对表浅的肿瘤。脑干神经节胶质瘤预后较好,后组脑神经麻痹是其主要术后并发症。     

Seizures as the presenting symptom of brain tumours in children.

Seizures were the presenting clinical symptom in 10 (12%) of 81 consecutive children with a primary brain tumour treated in a tertiary paediatric oncology unit over 5 years. Nine patients experienced partial seizures, and in seven a waking electroencephalogram showed focal or lateralising abnormalities. Astrocytoma was the most common tumour histology. The delay in tumour diagnosis from the onset of seizures ranged from 2 weeks to 2 years with a mean of 6 months. Complete resection of the tumour was the only treatment in three patients and four underwent resection followed by radiotherapy and/or chemotherapy. Two patients died. Three patients became seizure free receiving no antiepileptic medication and the remaining five showed a 50-80% reduction in seizures between 2 and almost 5 years following treatment.