Intracranial hypertension with delayed puberty: a rare presentation of juvenile onset systemic lupus erythematosus

An adolescent boy presented with headache, bilateral papilloedema, growth retardation and absent secondary sexual characteristics. The diagnosis of intracranial hypertension was confirmed by increased intracranial pressure and normal neuroimaging of the brain except for partial empty sella and prominent perioptic cerebrospinal fluid (CSF) spaces. Evaluation showed an erythrocyte sedimentation rate of 150 mm/hr, positive antinuclear antibody, anti-dsDNA and antiribosomal P protein. Renal biopsy revealed diffuse segmental proliferative lupus nephritis (LN) class IV-S (A), which confirmed the diagnosis of systemic lupus erythematosus (SLE). Treatment of LN with intravenous pulse methylprednisolone and cyclophosphamide normalised the patient's CSF pressure and symptoms. In cases of intracranial hypertension, SLE must be considered. Growth retardation and absence of secondary sexual characteristics could coexist and may be presenting features of SLE. These manifestations point to advanced grades of LN, which could be asymptomatic and may be missed without a renal biopsy.     

带蒂椎板复合体回植钛网固定在椎管内肿瘤治疗中的应用

目的探讨带蒂椎板复合体回植、钛网固定,在椎管内肿瘤切除术中椎管重建的应用价值。方法对13例椎管内肿瘤患者保留双侧关节突关节,根据椎管内肿瘤的大小,采用磨钻或微型咬骨钳沿双侧关节突内侧缘打开一个或多个椎板,切断一端棘上和棘间韧带,将椎板复合体翻转,椎管内肿瘤切除后再将其原位回植,钛网固定,重建椎管完整性和脊柱的稳定性。结果 13例椎管内肿瘤全部成功切除,切除椎板数2～4个,平均(2.5±0.5)个。1例因粘连致硬脊膜破损,1例哑铃型神经鞘瘤术后6个月复发。随访12～27个月,平均(13±0.5)个月,所有病例回植物稳定,无塌陷及椎管狭窄,术后肢体瘫痪Frankel分级均有不同程度提高。结论椎管内肿瘤术中将带蒂椎板复合体翻转,术后用钛网回植固定,方法简便、安全,有利于保持椎管的完整性及脊柱的稳定性。     

神经内镜下切除椎管内肿瘤的临床研究

目的 探讨神经内镜下切除椎管内肿瘤手术特点及治疗效果.方法 采用半椎板或全椎板入路,神经内镜下手术切除12例椎管内(胸段至骶段)肿瘤,包括脊膜瘤3例,神经鞘瘤7例,皮样囊肿2例(其中合并脊髓栓系1例),并随访观察手术疗效.结果 12例肿瘤术中均全切.其中1例伴有脊髓栓系者并进行栓系松解术.术中及术后未出现明显并发症.全部患者术后症状均较术前有不同程度好转.术后随访1月-1.5年,患者症状进一步改善,McCormick评级均达Ⅰ级或以上.复查MRI未见复发.伴发有脊髓栓系者,其神经源性膀胱术后改善不明显,残余尿量多,后行膀胱造瘘手术治疗.结论 与传统显微镜下切除椎管内肿瘤手术比较,神经内镜手术具有明显特点:疗效明确,创伤小,恢复快,对脊柱稳定性影响小等.     

Paraneoplastic papilloedema in neuroblastoma

We report a case of bilateral papilloedema in an adult male with neuroblastoma, in the absence of hypertension or detectable intracranial disease. This complication has not previously been described in the English-speaking literature. Possible mechanisms are discussed.     

电视胸腔镜辅助下切除后纵隔神经源性哑铃形肿瘤

目的:探讨微创技术在后纵隔神经源性哑铃形肿瘤切除手术中的应用.方法:2001年3月-2004年3月对本院胸心外科11例后纵隔神经源性肿瘤患者与神经外科联合手术,小切口切除肿瘤椎管内部分,在电视胸腔镜辅助(VATS)下切除肿瘤椎管外部分.结果:10例患者用微创技术完整切除肿瘤,切除率100%,无1例出现血胸、神经根损伤、椎管内血肿、肺损伤及脑脊漏等并发症;1例因肿瘤巨大占据胸腔,附加一前外侧小切口,亦完整切除肿瘤.随访6个月至2年无1例复发.结论:与传统手术径路比较,背部小切口 VATS在后纵隔哑铃形肿瘤切除术中具有创伤小、暴露好、并发症少、Ⅰ期完整切除肿瘤、局部复发率低等优点,临床值得推广应用.     

椎管内非特异性外周T细胞淋巴瘤一例

非特异性外周T细胞淋巴瘤是一种少见的恶性肿瘤.早期诊断困难,预后不佳.复习北京大学第三医院神经外科收治的1例非特异性外周T细胞淋巴瘤患者,结合文献探讨其临床特点及合理的治疗方案.本例患者因进行性双下肢无力,突发截瘫入院.脊柱增强核磁共振示:T6～T8脊髓周围异常强化信号,脊柱多发椎体及附件骨质异常信号.初步诊断淋巴瘤,多系统受累.行椎板减压、硬膜外肿瘤切除术,术中见肿瘤环绕硬脊膜生长,硬膜囊缩窄变细.病理为非特异性外周T细胞淋巴瘤.术后患者接受化疗,4个月后随访双下肢肌力有部分恢复.非特异性外周T细胞淋巴瘤的中枢神经系统病变多发生于疾病进展期,但以椎管内占位表现为首发症状的罕有报道.患者的诊断和治疗需个体化,可积极考虑术后放、化疗.对于急性截瘫者,应尽早做充分椎板减压,切除肿瘤,明确病理诊断,为今后的放、化疗提供条件.但局部化疗的价值仍需进一步研究.     

血浆脂质结合唾液酸变化对颅内肿瘤疗效判断的意义

目的 :探讨血浆脂质结合唾液酸 (lipid boundsialicacid ,LSA)变化对颅内肿瘤疗效判断的意义。方法 :采用朱翔等改良硫代巴比妥酸比色法测定了 83例颅内肿瘤患者的血浆LSA ,并对其中 47例进行了术后 6～ 12个月的随访。结果 :良性肿瘤组于术后 1～ 3个月血浆LSA逐渐恢复至正常 ,而恶性胶质瘤患者 ,术后一个月LSA稍下降 ,经手术全切 ,一年内未复发者 12例 ,术后 6～ 12个月血浆LSA基本下降至正常水平 ;反之 ,经手术全切一年内复发者 12例 ,术后 6～ 12个月又升高 ,与术后一个月血浆LSA含量相比差异显著 (P <0 .0 1)。结论 :血浆LSA含量检测可作为颅内肿瘤的辅助诊断措施 ,动态观察有助于恶性胶质瘤预后的判断     

中枢神经系统孤立性纤维瘤的MRI特征及鉴别诊断

目的探讨中枢神经系统孤立性纤维瘤(SFT)MRI表现特征,提高对该病的正确诊断及鉴别诊断水平。方法收集8例经手术病理证实的SFT,其中6例为颅内SFT、2例为椎管内SFT,分析其部位、形态大小、信号特点及邻近脑膜和骨质改变等。结果 6例颅内SFT位于幕上,2例椎管内SFT位于颈段和胸段的硬膜下。肿瘤在T1WI多呈等或稍低信号,T2WI多呈稍高信号,内可见短T2信号和流空血管影,所有病灶增强扫描后均呈明显强化。1例颅内STF可见"脑膜尾征",2例椎管内SFT未见"脊膜尾征",2例颅内SFT邻近骨质受压变薄。6例颅内SFT在DWI上分别呈稍低信号、等信号和稍高信号。结论 MRI对中枢神经系统SFT的诊断及鉴别诊断具有一定的价值。     

Primary intraspinal mesenchymal chondrosarcoma: a case report and literature review

Mesenchymal chondrosarcomas are rare malignant tumors of the bone and soft tissue. Extraskeletal mesenchymal chondrosarcomas, especially those that arise in the central nervous system, are even rarer. Most of those described were intracranially located, with only a very few cases having been reported in an intraspinal region. Therapeutic experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. We report on a case of a 21-year-old man with back pain and bilateral progressive weakness and numbness of the lower extremities. A T1-weighted magnetic resonance image revealed a hypointense tumor located at the T8 level. The tumor was completely excised through a posterior approach. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. Spinal irradiation and chemotherapy were also administered for prevention of local recurrence and metastasis. The patient has been symptom-free for 1 year after surgery. Herein, we review the 22 cases of primary intraspinal mesenchymal chondrosarcomas in the literature and discuss their clinical presentations, pathology, imaging studies, treatments, and outcomes.     

Tetracycline and benign intracranial hypertension: report of five cases

Benign intracranial hypertension occurred in four young women taking tetracycline for acne; two were also taking vitamin A. In a fifth case a 14-year-old boy developed papilloedema after taking a short course of tetracycline for bronchitis. All symptoms disappeared soon after stopping the drugs, though in two cases the papilloedema persisted for many months. Benign intracranial hypertension should be sought in any young woman complaining of headache during treatment with tetracycline. Moreover, young women given vitamin A and tetracycline in combination for acne may be at special risk and should be kept under surveillance.     

影响正压性脑积水分流手术效果因素分析

目的：研究影响正压性脑积水分流手术效果的因素。方法：从NPH患者的病因、症状、颅内压。CSF蛋白含量、年龄、病程及腰椎穿刺放液试验等因素分析手术效果。结果：蛛网膜下脸出血、脑外伤、脑瘤术后所致的NPH手术效果好；临床以痴呆和步态不稳或痴呆和尿失禁为主要症状的手术效果好；腰穿放液试验有效、病程＜6个月、年龄＜60岁、颅内压波动易达到1．5kPa以上的手术效果好。CSF蛋白含量高易造成分流管堵塞，远期效果欠佳。结论：影响NPH手术疗效的因素复杂，综合分析各因素对判断手术效果有益。     

侵犯椎体及其附件的高颈段哑铃型椎管内肿瘤的显微外科治疗效果

目的探讨侵犯椎体及其附件的高颈段哑铃型神经鞘膜瘤显微外科手术及颈椎后路植骨固定的方法与特点.方法分析7例高颈段哑铃形椎管内肿瘤病人的临床特点、影像学特征、显微手术及颈椎后路植骨固定的方法与治疗结果等.结果 7例患者术前均经CT或MRI检查作出临床诊断,肿瘤位于颈1～2椎体平面2例,颈2～4椎体平面5例,均为哑铃形生长,沿椎间孔从椎管内延伸至椎管外,相邻颈椎椎体及其附件不同程度破坏、颈髓不同程度受压移位、椎动脉被肿瘤包绕2例,推挤移位5例,均行显微手术切除肿瘤并颈椎后路植骨固定术,全切5例,部分切除2例,治疗效果良好.结论高颈段哑铃形椎管内肿瘤多伴不同程度颈椎及其附件破坏,脊柱稳定性受损,切除肿瘤后,绝大多数患者需行颈椎后路植骨固定,以防术后椎体不稳造成脊髓压迫.     

53例椎管内肿瘤的临床分析

目的对椎管内肿瘤的诊断和手术治疗进行探讨.方法分析53例不同部位的椎管内肿瘤患者的临床表现、诊断和手术治疗.结果术前所有患者依其不同的临床表现,结合相应的检查明确诊断.53例神经鞘瘤中全切除37例,其余均为次全切除或大部切除,手术全切除率为69.8%.术后有5例肌力较术前略有减退,3例变化不明显,其余患者症状均有不同程度的改善或痊愈;1例为转移瘤患者,因全身衰竭在手术中死亡,手术有效率为83.0%.结论椎管内肿瘤患者术前确诊主要依赖MRI,根据肿瘤的部位,早期采取不同的手术方式治疗能取得满意的效果.     

脑膜癌病2例报道及文献复习

目的:探讨脑膜癌病的临床及脑脊液细胞学特征。方法:总结2例脑膜癌病的临床及脑脊液细胞学特征,并复习文献。结果:1例为慢性粒细胞白血病急性变,1例为乳腺癌术后;临床主要表现头痛、呕吐,无脊神经损害及脑膜刺激征。脑脊液检查颅内压增高,细胞数、蛋白升高,细胞学检查发现癌细胞。结论:脑脊液细胞学检查是诊断脑膜癌病的重要依据。     

Pseudotumour cerebri in association with polyarthritis, urticaria and cryoglobulinemia.

Pseudotumour cerebri is a disorder characterized by elevated intracranial pressure in the absence of hydrocephalus and intracranial mass lesions. The cause of this disease remains obscure but has been related to diverse underlying conditions. We report a patient with pseudotumour cerebri, polyarthritis, urticaria, hypocomplementemia and cryoglobulinemia. Serial lumbar punctures were unsuccessful in lowering the cerebrospinal fluid (CSF) pressure. A trial of prednisone, 40 mg daily for 4 weeks, resulted in clinical improvement but was ineffective in lowering the CSF pressure. Circulating immune complexes and complement activation may have played a role in the development of increased intracranial pressure. Cryoglobulinemia should be added to the list of disorders associated with pseudotumour cerebri.     

Clinical and pathological characteristics of primary intraspinal hemangiopericytoma and choice of treatment

Background  Primary intraspinal hemangiopericytoma is a rare malignant mesenchymal tumor with high rates of recurrence and metastasis. Surgery is the main therapeutic procedure for this lesion. This clinical research was undertaken to analyze the pathological characteristics, clinical course, and the choice of treatment for this lesion.
Methods  Twenty-three patients with primary intraspinal hemangiopericytomas were treated from 1987 to 2004. The clinical and imaging features, pathological findings, therapeutic procedures, and prognosis were analyzed retrospectively.
Results  Primary intraspinal hemangiopericytoma is more likely to attack middle-aged persons. The tumor mainly manifests as muscle weakness and sensor abnormalities. Microscopic examination showed slit-like vascular spaces and oral- or spindle-shaped cells with slightly acidic cytoplasm and oral nuclei. Tumors were subtotally resected in 11 patients, subtotally resected with postoperative radiotherapy in 4, totally resected in 5, and totally resected with postoperative radiotherapy in 3. Two patients were given spinal stabilization after total resection. Recurrence and metastatic rates were 50% and 0 in intradural patients. They were 73% and 27% in extradural patients, respectively.
Conclusions  The tumor should be resected en bloc with the neighboring dural mater to reduce recurrence and metastasis. Patients with subtotal resection need adjuvant radiotherapy. Patients with evident spinal involvement may benefit from spinal stabilization. The prognosis of the lesion arising from the dural mater is better.

     

颅内感染性疾病患者脑脊液中粒细胞集落刺激因子的检测分析

目的 证明细菌及其产物包括内毒素等是刺激和调节粒细胞集落刺激因子（Ｇ－ＣＳＦ）产生的主要物质。方法 实验采用酶联免疫法（双抗体夹心法）对５０例不同病因的脑膜炎患者脑脊液中的Ｇ－ＣＳＦ水平进行了测定。结果 化脓性脑膜炎，病毒性脑膜炎，结核性脑膜炎患者脑脊液中的Ｇ－产较正常值明显升高（Ｐ〈０．００１）其中以化脑升高最为明显。结论 Ｇ－ＣＳＦ可作为颅内感染性疾病患者诊断的重要依据。     

椎管内肿瘤的诊断及治疗

目的探讨椎管内肿瘤的临床特点及治疗疗法。方法回顾性的分析1999年7月～2008年6月我科经手术和病理明确诊断的67例椎管内肿瘤患者的临床症状和体征、影像学检查、病理类型、部位、手术治疗方式和疗效。结果MRI检查能对椎管内肿瘤准确定位,有41例（61．2％）做到定性诊断,有2例假阳性。63例良性肿瘤采用后路切除,内固定7例,4例转移性肿瘤采用前路切除,植骨内固定术;随访41例,随访时间9个月～8年,好转31例,复发或加重8例,死亡2例。结论M砌是诊断椎管内肿瘤最重要的手段,它能准确定位,有较高比例（61．2％）的定性诊断,因有假阳性,应密切结合临床。良性椎管内肿瘤早期诊断和手术,预后较好,转移性椎管内肿瘤的手术治疗应非常慎重。     

Bruns syndrome caused by intraventricular tumor

The Bruns syndrome is an unusual phenomenon, characterized by attacks of sudden and severe headache, vomiting and vertigo, triggered by abrupt movement of the head. The presumptive cause of the Bruns syndrome is a mobile deformable intraventricular lesion leading to an episodic obstructive hydrocephalus resulted from an intermittent or positional CSF obstruction with elevation of intracranial pressure due to a ball-valve mechanism. Although the old neurological literature recognized tumors as well as neurocysticercosis as causes of the Bruns syndrome, during the last 60 years only intraventricular neurocysticercosis was reported to cause this symptom-complex. Here, we report a 38-year-old woman with relapsing attacks of headache, vertigo, nausea, vomiting, and ataxia provoked by head rotation corresponding to the classical Bruns syndrome. The cranial MRI revealed a tumor in the third ventricle and a further tumor in the fourth ventricle, which could cause a transient obstruction of the CSF pathways. This unusual observation of the Bruns syndrome in a non-parasitary disease of the CNS adds the syndrome to the differential diagnosis of paroxysmal vertigo.     

Unusual giant intraspinal teratoma in an infant

There are few cases of giant pediatric intraspinal teratoma. We report a case of a 4-month-old female baby with giant intraspinal teratoma. Magnetic resonance imaging (MRI) of the spine revealed a large intradural tumor from the C7 to S2 level, with solid, cystic, and fatty components. Partial surgical removal of the tumor showed pathology of a mature cystic teratoma. The imaging diagnosis of intraspinal teratoma included the location, solid and cystic component, and fatty content. The MR techniques adopted included gradient echo sequences as used to detect teeth or calcification. The difficulties in surgical resection of this case are also presented.