Spinal cord magnetic resonance imaging in autosomal dominant hereditary spastic paraplegia

Hereditary spastic paraplegia (HSP) is a genetically heterogeneous group of neurodegenerative disorders characterized by progressive lower extremity weakness and spasticity. HSP pathology involves axonal degeneration that is most pronounced in the terminal segments of the longest descending (pyramidal) and ascending (dorsal columns) tracts. In this study, we compared spinal cord magnetic resonance imaging (MRI) in 13 HSP patients with four different types of autosomal dominant hereditary spastic paraplegia (SPG3A, SPG4, SPG6, and SPG8) with age-matched control subjects. The cross-section area of HSP subjects at cervical level C2 was 59.42±12.57 mm² and at thoracic level T9 was 28.58±5.25 mm². Both of these values were less than in the healthy controls (p<0.001). The degree of cord atrophy was more prominent in patients with SPG6 and SPG8 who had signs of severe cord atrophy (47.60±6.58 mm² at C2, 21.40±2.4 mm² at T9) than in subjects with SPG3 and SPG4 (66.0±8.94 mm² at C2, p<0.02; 31.75±2.76 mm² at T9, p<0.001). These observations indicate that spinal cord atrophy is a common finding in the four genetic types of HSP. Spinal cord atrophy was more severe in SPG6 and SPG8 HSP subjects than in other types of HSP we studied. This may suggest a different disease mechanism with more prominent axonal degeneration in these two types of HSP when compared with HSP due to spastin and atlastin mutations.     

外伤性截瘫并四肢骨折的治疗

目的：探讨胸腰椎骨折截瘫并发四肢骨折的最佳治疗方案。方法：36例患者中除1例尺骨骨折、2例挠骨骨折、手法复位外，余均行手术治疗，并遵循先脊柱后四肢的原则。对脊髓神经损伤的病例，早期行椎管减压内固定术，对四肢骨折按照治疗原则处理。结果：36例中按frankel标准评定，术前A级9例，术后恢复到B级3例，C级1例，D级1例；术前B级13例，术后恢复到C级8例；术前C级9例，术后恢复到D级5例，E级4例;术前D级5例，均恢复到E级。四肢骨折全部骨性愈合。结论：本组病例所采用的治疗方案和手术术式，创伤小，操作简单，固定牢靠，效果满意。     

Preoperative radiological assessment of fractures of the thoracolumbar spine causing traumatic paraplegia

As a sequel to preoperative radiological assessment, 32 patients with traumatic paraplegia due to injury at the thoracic or lumbar level were treated by anterolateral decompression. The results were satisfactory in 60% of patients. This paper describes the radiological methods that were employed and specifies the radiological assessment of these fractures. The prognosis in relation to the type of fracture is also considered.     

脑外伤后的神经保护

为改善脑外伤患者的预后,人们在实验和临床方面研究了许多神经保护药物,试图通过使用这些药物来降低脑外伤患者的死亡率和致残率,但迄今为止,未能发现一种药物能真正改善脑外伤患者的预后。本文首先介绍了神经保护的概念,在此基础对目前临床已使用或研究较多的神经保护措施进行评估,为神经保护药物的临床使用提供参考。     

腔内修复术成功救治外伤性主动脉夹层伴截瘫一例

主动脉夹层(aortic dissection,AD)发病急,病情凶险,临床表现复杂多变,易漏诊、误诊而死亡.AD以截瘫为主要临床表现的病例少见报道[1],我院收治1例,报道如下.     

Spinal cord infarction after solitary rib fracture

This article presents two cases of spinal cord ischemia and infarction following traumatic injury to the ribs and resulting in severe neurologic deficits. The mechanism of injury and diagnostic methods are discussed.     

Spinal subdural enhancement after suboccipital craniectomy.

PURPOSETo characterize transient intraspinal subdural enhancement (potentially mimicking the subarachnoid spread of tumor) seen on MR images in some children after suboccipital craniectomy for posterior fossa tumor resection.METHODSRadiologic and medical records of 10 consecutive children who had MR imaging for spinal staging after resection of posterior fossa tumor during a 9-month period were reviewed retrospectively. In addition, one case with similar findings of intraspinal enhancement on spinal staging MR images obtained at another institution was included in the review.RESULTSIntraspinal enhancement thought to be subdural was seen in four of 10 patients undergoing spinal staging MR imaging 6 to 12 days after surgery. In these four patients, MR studies 50 to 18 days later, without intervening treatment, showed resolution of the abnormal enhancement. A fifth patient (from another institution) with similar intraspinal enhancement underwent CT myelography 4 days later, which showed no subarachnoid lesions. No metastases have developed in any of these five patients during the 2.5- to 3.5-year follow-up period. conclusions: From analysis of the MR appearance and on the basis of prior myelographic experience, we suggest an extraarachnoid, probably subdural, location of this enhancement. Awareness of this phenomenon will reduce the rate of false-positive diagnoses of metastatic disease. Preoperative spinal staging should be considered for patients undergoing suboccipital craniectomy.     

胸主动脉腔内修复术后截瘫危险因素分析

【摘要】 目的 探讨胸主动脉腔内修复术（TEVAR）后发生截瘫的危险因素。方法 回顾性分析2015年12月至2021年2月在苏州大学附属第一医院接受TEVAR治疗的307例Stanford B型主动脉夹层或胸主动脉瘤患者临床和影像资料。根据术后有无截瘫发生将患者分为截瘫组（8例）和无截瘫组（299例）。观察患者临床指标及围手术期主动脉CTA检查结果。对两组间差异有统计学意义的临床指标进行单因素和多因素logistic 回归分析。 结果 术后截瘫总发生率为2.61%。两组患者间伴冠心病、假腔血栓、围手术期低血压、支架远端距腹腔干开口长度、支架远端真腔狭窄率参数差异有统计学意义（均P＜0.05）。单因素 logistic回归分析显示,围手术期低血压、支架远端距腹腔干开口长度、真腔狭窄率是截瘫发生的危险因素（均P＜0.05）。多因素logistic回归分析显示,围手术期低血压、支架远端距腹腔干开口长度、真腔狭窄率是截瘫发生的独立危险因素（均P＜0.05）。结论 围手术期低血压、支架远端距腹腔干开口长度、真腔狭窄率是TEVAR术后并发截瘫的预测因子。对于主动脉破口较低患者仍需寻找更优化方案封堵破口,保护脊髓供血,避免截瘫发生。     

Surgical outcomes after traumatic open knee dislocation

The purpose of this study is to describe the types of injuries and surgical treatments associated with open knee dislocations and to present the functional outcomes of these patients. Between 2001 and 2005, the medical records of patients that sustained traumatic open knee dislocations at our Level 1 Trauma Center were retrospectively reviewed. Initial surgical intervention was performed in all patients including placement of spanning external fixator, repair of vascular injuries if necessary, and irrigation and debridement of the open wounds. Ligamentous reconstruction was delayed until after limb salvage. The Short Form-12 was the primary outcome measure. Seven patients (five male, two female) had a mean age of 31.9 years (range 22–44) at the time of injury (five right, two left). Motorcycle accident was the most common cause (57%). Follow-up was a mean 27.6 months. The PCL was damaged in all patients. Three patients underwent angiography for absent/diminished pulses on initial exam with two requiring operative intervention. Three patients had associated common peroneal nerve injury (one iatrogenic). Ten (10.7) operative procedures were performed per patient (range 5–18) with an average of 6.6 debridements (range 2–11). Infection rate was 43% with one patient undergoing amputation for infection. Good to excellent results were found in 33% of patients. Most patients (86%) report some residual symptomatic or functional deficit. Due to the injury complexity in open traumatic knee dislocations, the surgical treatment is extensive and challenging. While infection rates are high, aggressive, individualized treatment can lead to satisfactory outcome although full return to activity is difficult to achieve using current treatment methods.     

Spinal vascular malformations: MR angiography after treatment

PURPOSE: To evaluate the role of magnetic resonance (MR) angiography in the assessment of spinal vascular malformation therapy. MATERIALS AND METHODS: Thirty-four patients with spinal vascular malformations (30 dural arteriovenous fistulas, two perimedullary arteriovenous fistulas, and two intramedullary arteriovenous malformations) underwent MR angiography and MR imaging before and after endovascular or surgical treatment. RESULTS: MR angiography showed residual flow in perimedullary vessels in seven patients with dural fistula after embolization with liquid adhesive. In all seven, treatment failure was confirmed with arteriography. Long-lasting disappearance of flow in perimedullary vessels was demonstrated at MR angiography in 22 patients with dural fistula. MR imaging demonstrated normalization of spinal cord volume in 16 of 22 patients and signal intensity on T2-weighted images in three patients. Disappearance of cord enhancement was observed in five of 21 patients and of perimedullary enhanced vessels in six of 13 patients. In one additional patient with dural fistula treated with embolization, early posttreatment MR angiography showed disappearance of flow in perimedullary vessels, which reappeared at follow-up and was consistent with reopening of a small residual fistula. Posttreatment MR angiography demonstrated transient reduction of flow in the nidus in two patients with intramedullary malformations treated with embolization. Permanent disappearance of flow in the perimedullary vessel was seen after endovascular treatment in two patients with perimedullary fistula. CONCLUSION: MR angiography is more sensitive than MR imaging in depicting residual or recurrent flow in peri- or intramedullary vessels, which indicates patency of the vascular malformation.     

Spinal deformity after intra-operative radiotherapy for paediatric patients

The purpose of this study was to clarify the incidence and characteristics of late-onset complications of the spine in children who underwent intra-operative radiation therapy (IORT) for common paediatric malignant tumours. 12 children with more than 4 years of follow-up after IORT were included and, in 11 of these, thoracic and/or lumbar spines were irradiated. To compare doses of irradiation to the spine with the resulting deformities, dose simulations of IORT were carried out on two selected cases using a radiation treatment planning system with a pencil-beam algorithm. The mean follow-up period was 135 months (range, 53–234 months). Radiographic reviews found spinal deformity in six patients. Only one patient was symptomatic and the spinal deformity was severe (Grade 3), whereas spinal deformity was mild in the remaining five patients without clinical symptoms (Grade 1). In all of the six patients, anterior wedge-shaped deformity was dominant, and scoliosis was found in only two patients. In one particular case with nephrectomy, irradiation had penetrated much deeper than usual at the site of nephrectomy, and dose distribution was asymmetric, causing clinically significant spinal deformity with scoliosis. In conclusion, specific deformities of the spine observed after IORT can be explained on the basis of dose distribution of the electron beam to the spine.Identification of late morbidity of paediatric cancer therapy has become increasingly important, as recent therapeutic developments have improved the survival rate of affected children. Radiation-induced bone injury is one of the hazardous problems: a high dose of radiation therapy impairs bone growth and gives rise to physical disabilities in affected children. Late complications following skeletal irradiation for childhood tumours have been reported previously [1–6].Intra-operative radiation therapy (IORT) is a technical refinement of radiation therapy that allows sparing of the dose-limiting tissues adjoining the target region. Following gross tumour removal, surgical displacement of critical organs or shielding of adjacent structures is carried out to minimise the radiation damage to the normal structure, and at the same time to deliver an effective irradiation dose to the therapeutic target in a single session. A high dose of electron beam irradiation with the proper acceleration energy can be delivered to residual tumours at the surgical site and neighbouring areas at high risk for microscopic disease. Combined with intensive chemotherapy, IORT has been applied successfully to paediatric malignant tumours [7–15]. In general, IORT has fewer and less severe adverse effects than conventional radiation therapy (external beam radiation therapy (EBRT)). However, the prevalence of arterial and ureteral stenosis following IORT has attracted particular attention recently [16]. The reason that IORT and EBRT are associated with different patterns of complications may result from differences in dose fractionation and distribution between electron beams and high-energy X-ray beams. Moreover, electron beams have a shorter range in bony tissue than X-ray beams and therefore present very different dose distribution patterns to X-rays. This suggests that the biological effects of IORT may be particularly unpredictable in the paraspinal region of patients. The purpose of this study was to clarify the incidence and characteristics of late-onset complications of the spine in children who underwent IORT for common paediatric malignant tumours (mostly for neuroblastoma). We focused on serial radiological changes and reviewed the medical records of long-term survivors retrospectively.     

急性脊髓创伤后神经肽含量变化的实验研究及临床观察

目的：旨在探讨神经肽在脊髓继发性损伤中的作用。方法：应用放射免疫分析（ＲＩＡ）技术首次系统观察了急性脊髓创伤患者脑脊液中及脊髓损伤大鼠脊髓组织中三种不同内源性阿片肽含量的动态变化。结果：强啡肽（ＤｙｎＡ）随损伤程度的加重进行性升高；β－内啡肽（β－ＥＰ）无明显变化；亮脑啡肽（Ｌ－ＥＫ）伤后含量降低或无明显变化，且与受伤程度无明显相关。临床观察为脊髓损伤越重，脑脊液（ＣＳＦ）中ＤｙｎＡ和β－ＥＰ升高越明显，回复至正常的时间越长，预后也越差。Ｌ－ＥＫ含量在完全性和不完全性截瘫患者伤后均无明显变化。结论：内源性阿片肽参与了脊髓继发性损伤的病理过程，其中ＤｙｎＡ的作用最重要。     

Revisiting Spinal Cord Infarction after Bronchial Artery Embolization

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Stanford B型主动脉夹层动脉瘤腔内隔绝术后截瘫的预防对策

目的 探讨Stanford B型胸主动脉夹层动脉瘤(TAD)腔内隔绝术(EVE)后截瘫或轻瘫的综合防治措施。方法 回顾在1998年至2001年实施的116例TAD EVE。对于可能发生截瘫的高危患者，术后常规给予地塞米松。结果 包括接受脊髓动脉造影者在内，成功释放并固定移植物的115例中，均无截瘫或轻瘫发生。结论 EVE虽避免了长时间主动脉阻断，但仍可因封闭肋间动脉造成脊髓的严重缺血，包括选择性脊髓动脉造影、糖皮质激素在内的综合性预防措施能减小发生截瘫／轻瘫的危险，而选择适当长度的移植物是其关键。     

脊髓损伤组织血小板活化因子变化与脊髓水肿的关系

采用改良的Ａｌｌｅｎ法打击猫脊髓致伤模型，测定伤后２小时－１周伤区及邻近脊髓组织血小板活化因子（ＰＡＦ）含量及水含量。结果表明，伤后２－７５小时伤区及邻近脊髓组织ＰＡＦ含量及水含量较对照组明显增高，而伤后１周时与对照组无显著性差异。提示ＰＡＦ在脊髓损伤后脊髓水肿的发生、发展过程中起重要作用。     

cerebellar atrophy after moderate-to-severe pediatric traumatic brain injury

BACKGROUND AND PURPOSE: Although the cerebellum has not attracted the same degree of attention as cortical areas and the hippocampus in traumatic brain injury (TBI) literature, there is limited structural and functional imaging evidence that the cerebellum is also vulnerable to insult. The cerebellum is emerging as part of a frontocerebellar system that, when disrupted, results in significant cognitive and behavioral consequences. We hypothesized that cerebellar volume would be reduced in children following TBI and wished to examine the relation between the cerebellum and known sites of projection, including the prefrontal cortex, thalamus, and pons. MATERIALS AND METHODS: Quantitative MR imaging was used to measure cerebellar white and gray matter and lesion volumes 1-10 years following TBI in 16 children 9-16 years of age and 16 demographically matched typically developing children 9-16 years of age. Cerebellar volumes were also compared with volumetric data from other brain regions to which the cerebellum projects. RESULTS: A significant group difference was found in cerebellar white and gray matter volume, with children in the TBI group consistently exhibiting smaller volumes. Repeating the analysis after excluding children with focal cerebellar lesions revealed that significant group differences still remained for cerebellar white matter (WM). We also found a relation between the cerebellum and projection areas, including the dorsolateral prefrontal cortex, thalamus, and pons in 1 or both groups. CONCLUSION: Our finding of reduced cerebellar WM volume in children with TBI is consistent with evidence from experimental studies suggesting that the cerebellum and its related projection areas are highly vulnerable to fiber degeneration following traumatic insult.