脑干与脊髓内肿物的组织病理和超微结构研究

目的：研究脑干与脊髓内肿物的类别、分布和组织病理与超微结构特点。方法：取７２例脑干与脊髓内肿物的活检材料，做半薄和超薄切片，光镜与电镜观察。结果：脑干肿瘤２３例，脊髓内肿瘤１５例，脑干血管畸形１９例，脊髓内血管畸形４例，其中３例伴有肿瘤。脑干肿瘤中星形细胞瘤最多，室管膜瘤很少。脊髓内肿瘤中室管膜瘤最多，星形细胞瘤次之，９例脊髓内肿瘤，包括６例室管膜瘤，两极有空洞形成，洞壁淤血水肿。血管畸形中海绵状血管畸形最多，静脉性和毛细血管扩张型较少，可见毛细血管扩张型向海绵状血管畸形的过渡和复合型。结论：星形细胞瘤在光镜下适于按ＷＨＯ的新分级法分为四级。在电镜下需根据核异型的程度和数量及胞质内细胞器的增减来考虑恶性度。室管膜瘤、神经鞘瘤、黑色素瘤和血管外被细胞瘤在电镜下常有特异的微细结构作为确切的诊断依据。脊髓内肿瘤两极的空洞形成，考虑与肿瘤压迫，循环障碍，水肿液蓄积有关。３例脊髓内海绵状血管畸形有肿瘤与空洞并存，其发生可能与肿瘤长期压迫、循环障碍、毛细血管持续扩张有关。     

DARS2基因新发突变导致伴脊髓和脑干受累及乳酸升高的脑白质病一例

目的 本文报道1例由DARS2基因突变所致伴脊髓和脑干受累及乳酸升高的脑白质病(LBSL),并对患者的临床资料、神经影像学特征以及基因检测结果进行分析.方法 收集首都医科大学宣武医院LBSL1例,对其进行病史询问及磁共振(MRI)检查、磁共振波谱(MRS)、基因检测等相关检查.结果 患者主要表现为走路姿势异常、行走不稳...     

脊髓积水空洞症的磁共振成像和临床

３７例脊髓积水空洞症患者的磁共振成像（ＭＲＩ）资料显示大多数病例的病变范围较大，且位于脊髓中央，与第四脑室无明显的交通。３７例中有３４例伴ＣｈｉａｒｉⅠ型畸形。推测脊髓积水可能是本病的早期阶段，所以临床表现很轻且缓慢发展。大多数患者缺乏Ｃｈｉａｒｉ畸形的临床表现。因此，对于本病患者有必要施行颈枕区的ＭＲＩ检查。     

脊髓积水空洞症的磁共振成像和临床

３７例脊髓积水空洞症患者的磁共振成像（ＭＲＩ）资料显示大多数病例的病变范围较大，且位于脊髓中央，与第四脑室无明显的交通。３７例中有３４例伴ＣｈｉａｒｉⅠ型畸形。推测脊髓积水可能是本病的早期阶段，所以临床表现很轻且缓慢发展。大多数患者缺乏Ｃｈｉａｒｉ畸形的临床表现。因此，对于本病患者有必要施行颈枕区的ＭＲＩ检查。     

脊髓空洞症合并吉兰-巴雷综合征一例

目的探讨脊髓空洞症(SM)合并吉兰-巴雷综合征(GBS)的临床特点、影像学及实验室检查特征、诊断标准。方法分析1例SM合并GBS临床资料。结果患者肌电图(EMG)提示周围神经轴索损害,脑脊液(CSF)未出现"蛋白-细胞"分离现象,胸椎MRI示胸7~8椎体水平脊髓内异常信号,考虑SM。结论 GBS脑脊液检查可能不出现"蛋白-细胞"分离现象;SM临床特点结合MRI即可确诊;两者的病因、发病机制均不同,为一合并症。     

渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

目的 探讨渗透性脱髓鞘综合征的临床和神经影像特点.方法 对4例渗透性脱髓鞘综合征患者的临床演变过程、CSF、头颅CT和MRI、EEG动态变化特点、治疗及预后进行分析.结果 4例患者均存在低钠血症,纠正后出现精神意识改变、构音和吞咽困难、四肢瘫痪、肌张力障碍等症状,临床过程有双相性.EEG出现一过性的重度异常.头颅CT及CSF均未见异常.MRI特征性影像晚于临床表现10 d以后出现,4例患者首次MRI均为阴性,7～13 d后复查才显示病灶.MRI示4例患者均存在脑桥外髓鞘溶解症病灶,T1WI加权低信号,T2WI加权高信号,对称性地累及双侧尾状核、豆状核、丘脑、脑岛叶皮质、海马头部等部位,其中3例同时存在脑桥中央髓鞘溶解症改变,呈脑桥基底部位对称性T1低、T2高信号的蝶形病灶;Flair加权异常信号更清楚.3例有好转或痊愈,其中1例遗留明显肌张力障碍.结论 渗透性脱髓鞘综合征与慢性低钠血症有关,合并低血钾、低血氯时可能更易发生.治疗时应尽量避免过快纠正,临床病程具有双相性.MRI的特征性改变出现较迟,复查MRI是非常必要的.     

渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

目的 探讨渗透性脱髓鞘综合征的临床和神经影像特点.方法 对4例渗透性脱髓鞘综合征患者的临床演变过程、CSF、头颅CT和MRI、EEG动态变化特点、治疗及预后进行分析.结果 4例患者均存在低钠血症,纠正后出现精神意识改变、构音和吞咽困难、四肢瘫痪、肌张力障碍等症状,临床过程有双相性.EEG出现一过性的重度异常.头颅CT及CSF均未见异常.MRI特征性影像晚于临床表现10 d以后出现,4例患者首次MRI均为阴性,7～13 d后复查才显示病灶.MRI示4例患者均存在脑桥外髓鞘溶解症病灶,T1WI加权低信号,T2WI加权高信号,对称性地累及双侧尾状核、豆状核、丘脑、脑岛叶皮质、海马头部等部位,其中3例同时存在脑桥中央髓鞘溶解症改变,呈脑桥基底部位对称性T1低、T2高信号的蝶形病灶;Flair加权异常信号更清楚.3例有好转或痊愈,其中1例遗留明显肌张力障碍.结论 渗透性脱髓鞘综合征与慢性低钠血症有关,合并低血钾、低血氯时可能更易发生.治疗时应尽量避免过快纠正,临床病程具有双相性.MRI的特征性改变出现较迟,复查MRI是非常必要的.     

渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

目的 探讨渗透性脱髓鞘综合征的临床和神经影像特点.方法 对4例渗透性脱髓鞘综合征患者的临床演变过程、CSF、头颅CT和MRI、EEG动态变化特点、治疗及预后进行分析.结果 4例患者均存在低钠血症,纠正后出现精神意识改变、构音和吞咽困难、四肢瘫痪、肌张力障碍等症状,临床过程有双相性.EEG出现一过性的重度异常.头颅CT及CSF均未见异常.MRI特征性影像晚于临床表现10 d以后出现,4例患者首次MRI均为阴性,7～13 d后复查才显示病灶.MRI示4例患者均存在脑桥外髓鞘溶解症病灶,T1WI加权低信号,T2WI加权高信号,对称性地累及双侧尾状核、豆状核、丘脑、脑岛叶皮质、海马头部等部位,其中3例同时存在脑桥中央髓鞘溶解症改变,呈脑桥基底部位对称性T1低、T2高信号的蝶形病灶;Flair加权异常信号更清楚.3例有好转或痊愈,其中1例遗留明显肌张力障碍.结论 渗透性脱髓鞘综合征与慢性低钠血症有关,合并低血钾、低血氯时可能更易发生.治疗时应尽量避免过快纠正,临床病程具有双相性.MRI的特征性改变出现较迟,复查MRI是非常必要的.     

表现为多种脑干损害症状的原发性脑桥出血1例报告

原发性脑桥出血引起多种脑干损害症状较少见,现报告1 例如下.     

以脊髓浸润为首发症状的慢性淋巴细胞白血病一例

1病例报告患者男,62岁。因“双下肢无力2个月余”于2011‐12‐25入院。入院前3个月无明显诱因下出现腰腹部束带感,持续3～4d后自行缓解;入院前2个月出现左下肢乏力并逐渐加重;入院前1个月左下肢已不能抬起,并出现右下肢乏力、排便困难。期间行腰椎M RI检查示轻度腰椎间盘突出,尝试针灸及牵引治疗均无好转;入院前1周行颈胸髓M RI示颈髓及胸髓内可见多发斑片状稍长 T1、稍长T2信号病灶,增强后无明显强化,予以地塞米松、甘露醇、B族维生素治疗,患者双下肢无力部分好转。为进一步诊治收入作者医院。入院体检：体温36.6℃,血压120／80 mmHg ,意识清楚,全身浅表淋巴结未触及肿大,肝脾肋下未触及,脑神经（－）,双上肢及右下肢肌力V级,左下肢肌力V －级,胸6水平以下针刺觉及振动觉减退（左侧尤为显著）,四肢腱反射（＋＋）,双侧病理征阴性。入院后血常规检查结果显示：白细胞29.6×109／L ,淋巴细胞绝对值21.9×109／L ,血红蛋白137 g／L ,血小板193×109／L。外周血流式细胞学检查结果：B细胞型CD19、CD20、CD79a均为（＋）;CD5、CD23、CD34、CD10均（－）。腰穿脑脊液检查结果：脑脊液无色透明,白细胞260×106／L ,淋巴细胞100％;葡萄糖、蛋白及氯化物均正常。脑脊液流式细胞学检查：B细胞型,CD19、CD20、HLA‐DR均（＋）;CD5、CD23、CD34、CD10均（－）。骨髓细胞学检查：淋巴细胞比例增高,占56.5％;骨髓增生明显活跃,中性粒细胞比例减低,红细胞系比例减低,巨核细胞增生活跃,血小板可见。骨髓活检检查：骨髓增生明显活跃,淋巴细胞比例增高明显。骨髓染色体检查：正常,核型46,XY。诊断：慢性淋巴细胞白血病（CLL）。2012‐01起予以FR方案化疗〔氟达拉滨40 mg（第1～3天）＋利妥昔单抗600 m g （第1天）〕,1疗程／4周,共6疗程;每次化疗同时给予鞘内注射地塞米松5 mg、甲氨蝶呤10 m g。化疗第1个疗程后患者神经系统症状体征完全消失,第3个疗程后复查脑脊液、血常规均正常。此后每半年定期随访,末次随访为2014‐03,血常规及脑脊液检查结果正常,脊髓M RI检查示髓内病灶完全消失,处于完全缓解期。     

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids with a Significant Elevation of β-2 Microglobulin Levels

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a relapsing-remitting disorder for which steroid administration is a key to control the progression. CLIPPERS can exhibit radiological features similar to malignant lymphoma, whose diagnosis is confounded by prior steroid administration. We report a case of CLIPPERS accompanied by abnormal elevation of β-2 microglobulin in the cerebrospinal fluid (CSF). A 62-year-old man started to experience numbness in all fingers of his left hand one year ago, which gradually extended to his body trunk and legs on both sides. Magnetic resonance imaging demonstrated numerous small enhancing spots scattered in his brain and spinal cord. CSF levels of β-2 microglobulin were elevated; although this often indicates central nervous system involvement in leukemia and lymphoma, the lesions were diagnosed as CLIPPERS based on the pathological findings from a biopsy specimen. We emphasize the importance of biopsy to differentiate between CLIPPERS and malignant lymphoma because the temporary radiological response to steroid might be the same in both diseases but the treatment strategies regarding the use of steroid are quite different.     

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report and review of literature

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of central nervous system with distinct clinical and radiological features. The etiopathogenesis of this rare entity remains to be understood. The histopathological findings closely resemble chronic inflammatory diseases like sarcoidosis and malignancies like lymphoma. With advancements in serology, immunopathology and radiology CLIPPERS is identified as a distinct entity that differs considerably in its clinical presentation, immunopathology, radiological findings and response to steroids. We describe a case that presented to us with progressive quadriparesis and lower cranial nerve deficits whose radiological and pathological findings were consistent with CLIPPERS. The patient had a good outcome with long term immunosuppression.     

The brainstem origin of monoaminergic projections to the spinal cord of the North American opossum: A study using fluorescent tracers and fluorescence histochemistry

The retrograde transport of fluorescent markers has been combined with the glyoxylic acid and Falck-Hillarp techniques to identify the origin of monoamine axons within the spinal cord of the North American opossum. Catecholamine axons arise from neurons located within the ventrolateral medulla, dorsal to the superior olivary complex, within the dorsolateral and rostrolateral pons and within the periventricular nuclei of the hypothalamus. Such neurons are most numerous within the dorsolateral pons where they are found dorsal and lateral to the motor trigeminal nucleus, within the nucleus locus coeruleus pars alpha and adjacent reticular formation as well as within the ventral part of the nucleus locus coeruleus. Neurons containing the fluorescent marker and catecholamines were interspersed with others containing only the injected marker with the possible exception of the nucleus locus coeruleus. Spinal axons of the indoleamine type arise from neurons within the nuclei pallidus, obscurus and magnus raphe, the nucleus reticularis gigantocellularis, the nucleus reticularis gigantocellularis pars ventralis, the nucleus reticularis pontis pars ventralis and the nucleus dorsalis raphe. The latter nucleus only innervates rostral cervical levels. Most of the above areas also contain many non-indoleamine neurons which were labelled by the injected marker. This was particularly true of the nucleus magnus raphe and the adjacent nucleus reticularis points pars ventralis after injections of fluorescent markers into the superficial dorsal horn.     

Multiple sclerosis and coexisting intradural extramedullary spinal cord tumour: a case report

We report the coexistence of multiple sclerosis (MS) and an intradural extramedullary spinal cord tumour in a 46-year-old woman with a 2-year history of MS. The patient presented with right hemitrunk and lower extremity paraesthesias, urinary incontinence, and intermittent lower right back and abdominal pain, which did not respond to pulse steroid therapy. A spinal magnetic resonance imaging (MRI) study revealed an intradural extramedullary spinal cord tumour in the lower thoracic spine, later diagnosed as schwannoma. We call attention to this rare association of MS and a spinal cord tumour, and emphasize the need for scrutiny of new and uncommon symptoms during the follow-up of MS patients. Received: 2 April 2002 / Accepted in revised form: 28 May 2002 Correspondence to V. Etus     

Primary spinal cord oligodendroglioma: case report and review of the literature

Objects The objectives were to present a case of pediatric spinal oligodendroglioma and review the existing literature written in English on the subject of human spinal oligodendrogliomas. A comparison of the clinical, radiologic, and pathologic characteristics, as they relate to those already described in similar cases, was also attempted.Methods Thorough evaluation of the patients clinical course was undertaken. Presenting symptoms and signs are reported. The perioperative radiologic features of the case are presented and the intraoperative details as well as the pathologoanatomic findings and follow-up history are provided. We subsequently performed a thorough search in the literature focusing on the number, characteristics, treatment modalities, and prognosis of patients with spinal cord oligodendrogliomas.Conclusions Spinal oligodendrogliomas are a distinctly rare type of nervous system tumor, especially in the pediatric population. An international registry addressing all of their clinical and pathobiological characteristics would be of great benefit to patients harboring these rare tumors.     

Assessing the rotation of the spinal cord in idiopathic scoliosis: a preliminary report of MRI feasibility

Purpose  Magnetic resonance imaging (MRI) quantification of the rotation of the spinal cord in patients with thoracic idiopathic scoliosis could also be used to detect different spinal cord rotational patterns. Methods  Ten patients with a thoracic or thoracolumbar scoliosis had axial T2-weighted MRI. The rotation of the spinal cord and vertebra were measured. The rotational data of the spinal cord and vertebra was compared to other collated data using non-parametric tests. Results  The vertebral tile was measured from 3° to 32° and the spinal cord tilt was measured from 3° to 39°. The spinal cord tilt was statistically correlated with the Cobb angle and the antero-posterior or and transverse diameter of the spinal cord. Conclusion  We showed that, even in case of moderate curve with very limited angular values and vertebral rotation, a significant spinal cord rotation occurred. However, our findings are very limited to discuss some hypothesis about scoliosis pathogeny or progression mechanism.     

Lumbosacral spinal subdural hematoma following burr hole craniotomy: case report and literature review

Spinal subdural hematoma is a rare complication of cranial surgery. This study reports a case of postcraniotomy lumbosacral spinal subdural hematoma in the absence of predisposing factors. A review of the literature is also presented.     

Fractionated radiosurgical management of intramedullary spinal cord metastasis: A case report and review of the literature

Objective and importance

Intramedullary spinal cord metastasis (ISCM) comprises 8.5% of central nervous system metastases and confers significant morbidity. Radioresistant histologies such renal cell carcinoma and melanoma are not generally amenable to long-term palliation with conventional radiotherapy while surgery has often been found to be technically challenging and frequently morbid. In this report, we present a patient with a C5 ISCM from renal cell carcinoma treated with fractionated stereotactic radiosurgery.

Clinical presentation

A 50-year-old gentleman with metastatic renal cell carcinoma presented with profound bilateral shoulder pain and upper extremity paresthesias. Magnetic resonance imaging revealed an intramedullary lesion at the level of fifth cervical vertebra (C5). Medical management and chiropractic manipulation proved to be ineffective. The patient was then treated with external beam radiation therapy, but continued to experience severe pain, paresthesias, and progressive, profound neurologic symptoms.

Intervention

The patient was referred to radiation oncology and neurosurgery for evaluation. Consideration was given to cordotomy and resection but the location and procedure was deemed to be high-risk and therefore was deferred. The decision was made to treat with fractionated stereotactic radiosurgery. A dose of 15 Gy was successfully delivered in 3 fractions to the 80% isodose line without complication or adverse effects. Twenty-six months following treatment, the patient was still alive, fully functional, and reported no pain and rare of paresthesias.

Conclusion

Fractionated stereotactic radiosurgery is a feasible, safe, and effective modality for the treatment of ICSM and should be carefully considered in the management of this difficult to treat condition.     

Primary germinoma of the spinal cord: a case report with 28-year follow-up and review of the literature

Germ cell neoplasms occur in extra-gonadal midline locations of the retroperitoneum, mediastinum, pineal gland, areas of the suprasellar cistern, and rarely in the spinal cord. We recently reviewed a case of an unresectable lumbar spinal cord tumor in a 16-year-old female previously diagnosed as metastatic poorly differentiated carcinoma. An extensive evaluation for a primary neoplasm at that time was unsuccessful and the patient was treated with local radiation therapy. Recently, additional histochemical and immunocytochemical studies were performed on the archival formalin-fixed paraffin-embedded material and the clinical history was reviewed. These ancillary studies (including positive immunohistochemical staining for placental alkaline phosphatase) support a diagnosis of intramedullary germinoma of the conus medullaris. This patient has enjoyed 28 years of disease free survival which reflects the radiosensitive nature of this neoplasm. These data lend support to the existence of a primary germinoma in the spinal cord and illustrate the utility of using histochemical stains and immunohistochemistry to assist in diagnosing this treatable neoplasm.