Subacute posttraumatic ascending myelopathy (SPAM): A potential complication of subarachnoid shunt for syringomyelia?

Context: Treatment of primary spinal syringomyelia is still controversial. Among others, shunting syrinx fluid to the subarachnoid, peritoneal or pleural space has been utilized with varying success. Shunt obstruction, migration, and infection represent the most common complications of these procedures.

Findings: The authors present the case of an 81-year-old woman who developed an unusual neurological deterioration resembling a subacute posttraumatic ascending myelopathy (SPAM) after the insertion of a syringosubarachnoid shunt for the treatment of slow-growing D10 syringomyelia.

Conclusion/Clinical Relevance: To date, no cases of SPAM secondary to the insertion of a syringosubarachnoid shunt for the treatment of syringomyelia have been reported. The potential pathogenesis related to this phenomenon is discussed.     

Arnold-Chiari畸形合并脊髓空洞症的外科治疗(附32例报告)

目的：探讨Chiari畸形合并脊髓空洞症的手术治疗方法。方法：分析我科收治的32例Chiari畸形合并脊髓空洞症患者进行手术治疗，以后颅窝减压、小脑扁桃体切除、脊髓空洞穿刺、松解四脑室正中孔蛛网膜粘连为主，达到解除后脑受压，解除粘连，恢复枕大孔区CSF循环通畅的目的。结果：随访24例患者中，临床症状完全消失18例，好转2例，术前有头痛、颈痈或肩臂病症状者均有显著减轻，3例自觉症状无变化，复查MRI显示21例空洞明显缩小。结论：后颅窝减压、松解四脑室正中孔蛛网膜粘连，恢复枕大孔区CSF循环通畅是手术治疗Chiari畸形合并脊髓空洞症的关键。     

血管内皮生长因子在脊髓空洞前状态中的表达及作用机制探讨

目的　通过观察脊髓空洞前状态中脊髓水肿程度和组织学变化 ,测定血管内皮生长因子的表达并探讨其作用机制。方法　常规组织学及电镜观察 ,应用干湿法测定脊髓空洞前状态中脊髓含水量 ,ELISA和免疫组织化学测定脑脊液和脊髓中VEGF表达含量。结果　组织学观察发现 ,Kaolin组动物在术后 2周内出现脊髓水肿且随时间延长逐渐加重 ,2 1d出现髓鞘和轴索损伤 ;脊髓含水量较对照组在术后第 1天轻度升高 (68.3 5± 0 .70 ) % ,第 3天水肿明显加重 (72 .70± 0 .88) % ,第 7、14天达到高峰 [(72 .92± 0 .86) %、(72 .18± 0 .5 5 ) % ] ,第 2 1天水肿开始缓解 ,但仍高于正常 (70 .0 3± 0 .77) %。Kaolin组动物脊髓、脑脊液中VEGF表达较正常对照明显增高 ,其强度变化趋势与脊髓水肿和组织学变化程度一致。结论　在实验性脊髓空洞症模型中 ,缺血水肿 ,组织含水量增高构成脊髓空洞前状态的主要表现 ;VEGF高表达在脊髓空洞前状态中的水肿形成中起重要作用。     

Experimental model of chronic tonsillar herniation associated with early stage syringomyelia

This report describes an experimental model of chronic tonsillar herniation and its effects on the spinal cord. In ten rats, a small piece of chemically induced mammary cancer was transplanted to the supraoccipital bone. In all cases, the transplanted cancers grew into the posterior fossa, destroying the supraoccipital bone and compressing the cerebellum extradurally. In six of the ten rats, tonsillar herniation was observed at 8–14 weeks after transplantation. Transdural infiltration of the tumor cells was not apparent in any animal. In those rats with tonsillar herniation (n=6), the spinal cord from the C5 to the T8 segments showed enlargement of the central canal without exception. Histological examination revealed the following changes: stretching and thinning of the ependymal cells; swelling of the astrocytic processes; and extracellular edema, predominantly in the dorsal gray matter, but also in the ventral inner portion of the dorsal column. In the control group (n=4) and those rats without tonsillar herniation (n=4), such histological changes of the spinal cord were not observed. Although the lesions can not be regarded as representing mature syringomyelia, they most likely constitute an earlier evolutionary stage.     

Surgical management of Chiari malformation type 1 associated to MCAP syndrome and study of cerebellar and adjacent tissues for PIK3CA mosaicism

BackgroundSubjects with Megalencephaly-Capillary Malformation-Polymicrogyria syndrome (MCAP) can present with a Chiari Malformation Type 1 and resulting alterations in cerebrospinal fluid (CSF) dynamics, which may require surgical treatment. The aim of this paper is to describe the features of children with MCAP who underwent surgical decompression for CM1, and to explore the PIK3CA variant allele frequency (VAF) identified in cerebellar parenchyma and other adjacent structures.MethodsThis study reviewed two cases of children with CM1 and MCAP who underwent surgical decompression treatment. These two cases were part of a national cohort of 12 MCAP patients who had CM1, due to their surgical eligibility. Tissue samples were obtained from the cerebellar tonsils and adjacent anatomical structures during the surgical procedures. Samples were then subsequently analyzed for PIK3CA postzygotic variants.ResultsIn both cases, alterations in CSF dynamics, specifically hydrocephalus and syringomyelia, were observed and required surgical treatment. PIK3CA targeted sequencing determined the VAF of the postzygotic variant in both cerebellar and adjacent bone/connective tissues.DiscussionThe recognition of a CM1 comorbidity in MCAP patients is of paramount importance when considering personalized treatment options, especially because these patients are at higher risk of developing complications during surgical decompression surgery. The variable PIK3CA VAF identified in the different analyzed tissues might help explain the heterogeneous nature and severity of anomalies observed in the volume of the posterior fossa structures in MCAP patients and associated CSF and venous disorders.     

Radiographic studies of the ventricles in syringomyelia

Summary Radiographic investigations of 171 patients with communicating syringomyelia have been reviewed. Hydrocephalus was found in onethird of the cases and has occasionally progressed after operation on the posterior fossa, sometimes with accompanying clinical deterioration. The outlets of the fourth ventricle were usually abnormal; tonsillar descent, arachnoiditis and both together were seen. Arachnoiditis correlated strongly with a history of difficult birth. The foramen of Magendie was sometimes patent and sometimes blocked. There was no consistent level of occlusion corresponding to a persistent roof of the fourth ventricle. The cisterna magna was usually small or obliterated but some examples of large cisterns or subarachnoid pouches were found. Radiological demonstration of a communication from the fourth ventricle to the syrinx occurred in only seven patients by positive contrast material and not by air. It is suggested that a sizable communication is rare at the time when patients seek treatment.     

Familial syringomyelia

Summary The clinical and neurological features of four siblings (2 , 2 ) affected by syringomyelia are described. A fifth sister was affected by an acoustic neurinoma. Since neither parent showed signs of syringomyelia, this is considered to be a datum substantiating the dysembryogenetic theory of the syringomyelia syndrome.We are grateful to Dr. M. Savoiardo for his many suggestions and for his interpretation of radiological features. We should like to thank Dr. L. De Lorenzi for allowing us to publish details about patients.     

后颅窝骨性扩大术治疗Chiari畸形合并脊髓空洞症

目的　介绍后颅窝骨性扩大术治疗Chiari畸形合并脊髓空洞症。方法　该手术分为两种亚术式 ,一种是枕颈固定扩大术 :取自体髂骨 5cm× 10cm ,打磨成“凹”字形。上端为齐头端 ,固定在后颅窝减压窗缘。缺口端为下端 ,固定在枢椎棘突上 ,将扩大的硬脑 (脊 )膜修补筋膜片固定在植骨片内面 ,使筋膜片贴附在植骨片内面 ,形成帐篷状 ;另一种是单纯骨性扩大术 :取自体髂骨 6cm× 8cm的梯形骨片 ,并利用较厚的髂骨缘打磨成弧形。植骨片固定在后颅窝减压窗上 ,弧形端构成枕骨大孔后缘。将扩大的硬脑 (脊 )膜修补筋膜片也固定在植骨片内面 ,使筋膜片贴附在植骨片内面 ,也形成帐篷状。结果　本组病例随访 2～ 7年 ,术后症状体征缓解总有效率为91.6% ,肌力比以前提高 2～ 3级。结论　该术式比其他治疗Chiari畸形合并脊髓空洞症的术式 ,治疗效果显著 ,疗效切实可靠     

小脑扁桃体下疝合并脊髓空洞症治疗分析

目的 探讨小脑扁桃体下疝合并脊髓空洞症的治疗.方法 回顾性分析1995年8月至2010年8月经治的29例小脑扁桃体下疝合并脊髓空洞症患者,均采用手术干预治疗,单纯行枕大孔减压术16例,行枕大孔减压加空洞-蛛网膜下腔分流术13例.术后随访6个月～3年观察治疗效果.结果 随访6个月～3年,单纯行后颅窝减压组中显效8例,有效6例,无效2例,恶化0例,总有效率为87.5％;后颅窝减压空洞-蛛网膜下腔分流组中显效6例,有效4例,无效2例,恶化1例,总有效率为76.9％,2组总有效率比较差异无统计学意义（P＞0.05）.结论 手术是小脑扁桃体下疝合并脊髓空洞症的首选治疗,因下疝和空洞致脊髓受损轻者减压术后恢复较好,而脊髓受损明显者则手术效果差.