Myelofibrosis presenting as chronic cholecystitis.

A 61 year old man presented with abdominal pain typical of chronic cholecystitis of one month's duration. Pallor was noted on examination and investigation uncovered myelofibrosis and a small gallstone. Cholecystectomy relieved the pain and pathological examination of the gall bladder showed widespread myeloid metaplasia. This is the first reported case of myelofibrosis presenting as chronic cholecystitis.     

Epidural angiolipoma with spinal cord compression

A case of epidural angiolipoma is reported. This tumor rarely occurs in the spinal canal. The most common location is in the thoracic region, and its histogenesis is probably congenital.     

Congenital generalized fibromatosis causing spinal cord compression

An unusual case of congenital generalized fibromatosis in which involvement of the spinal dura mater was accompanied by flaccid paralysis in the lower limbs is presented.     

Polycythaemia rubra vera and myelofibrosis with spinal cord compression

Spinal cord compression from extramedullary haemopoiesis within the spinal epidural space is a rare complication of myelofibrosis and polycythaemia rubra vera (PRV). A 69-year-old male with PRV (later transforming to myelofibrosis) who developed this complication is described here. Due to the uncertainty over its optimal treatment, previous case reports were systematically reviewed to define its presentations, treatments and outcomes. Including the present case this complication has been reported in 21 patients with myelofibrosis and PRV: 17 were male and the mean symptom duration was 7.6 months. Neurological improvement occurred in 14 patients and 12 survived. Seventy-five per cent of patients receiving combined treatment (irradiation with laminectomy or chemotherapy) showed neurological improvement and 100% survived. In contrast, 67% of those receiving single treatments exhibited improved neurology and only 33% survived. It is concluded that spinal cord compression in myelofibrosis and PRV has a high mortality, with combined treatment providing a better prognosis.     

体感诱发电位评价慢性压迫性脊髓症神经功能的病理机制

目的 探讨慢性压迫性脊髓症不同体感诱发电位(somatosensory evoked potential,SEP)变化对应的病理学机制.方法 20只SD大鼠经后路手术、颈椎管内(C5～C6水平)植入吸性聚氨酯胶片,该植入体在硬膜外逐渐吸水膨胀,形成对脊髓的慢性持续压迫.术前和造模后6个月检测SEP,并对慢性压迫脊髓行Micro-CT、组织学(HE染色)和组织化学(FLB染色)检测.结果 20只造模大鼠脊髓均出现侧后方明显压迫性形态学改变,Micro-CT显示脊髓灰质和白质扭曲变形.依据SEP变化分为Ⅰ(n=6)、Ⅱa(n=5)、Ⅱb(n=4)、Ⅲ(n=5)、Ⅳ(n=0)5类.SEP异常者脊髓后索髓鞘FLB染色显著减少(SEP异常:106±27;SEP正常:121±8;P=0.036),Micro-CT显示脊髓后索对比剂密度明显增加(SEP异常:95±5;SEP正常:87±6;P=0.041),后角内神经元也明显较少[SEP异常:(21±8)/mm2;SEP正常:(29±6)/mm2;P＞0.05].病理学上,SEP-Ⅰ型表现为脊髓中央管扩大;Ⅱa型表现为灰质内出血、静脉扩张和中央管缩小;Ⅱb型表现为灰质、白质排列紊乱,血管增生;Ⅲ型表现为神经元明显减少、白质-灰质结构不清,基质海绵样变.结论 慢性压迫性脊髓症不同类型的SEP变化反映了脊髓后索和灰质神经元损伤的严重程度,SEP作为脊髓功能预后评估的判断指标具有相应的病理学特征.     

Vocal cord dysfunction presenting as asthma.

A 14 year old boy presented with deteriorating asthma and marked stridor. Neither asthma nor stridor responded to an increase in anti-asthma medication, including high dose oral steroids. Indirect laryngoscopy revealed adduction of the vocal cords throughout the respiratory cycle, a phenomenon previously identified as a psychosomatic conversion reaction. When gently confronted with these findings and offered psychological assistance the boy's symptoms abated totally. After two sessions of hypnotherapy he has had better control of both his physical (asthma) and psychological problems.     

Idiopathic mediastinal fibrosis presenting as mediastinal compression syndrome

Mediastinal compression syndrome is a commonly seen entity. Mediastinal compression, mostly due to a space-occupying lesion, is distinct and different from mediastinitis/mediastinal fibrosis, which could also lead to superior vena cava syndrome. Idiopathic mediastinal fibrosis should also be considered as differential diagnosis of mediastinal structures with various radiological, CT and MRI and histological features if feasible. Medical therapy is disappointing while surgical cure has limitations. This interesting patient presented as mediastinal compression syndrome, which on investigation was postulated as idiopathic mediastinal fibrosis, as a diagnosis on exclusion of other causes, which is rare, hence is being reported.     

Osteological features in pure-bred dogs predisposing to cervical spinal cord compression

Relative to body size, midsagittal and interpedicular diameters of the cranial and caudal aspects of cervical vertebral foramina (C3–C7) were found to be significantly ( P < 0·05) larger in small breeds than in large breeds and Dachshunds, and also larger in Dachshunds ( P < 0·05) than in large breeds. This condition increases the risk for spinal cord compression resulting from relative stenosis of the cervical vertebral foramina, especially in large dogs, and this is also exacerbated by the typical shape of the vertebral foramina (i.e. dorsoventrally flattened cranially and bilaterally narrowed caudally). Within large dogs those breeds highly predisposed to cervical spinal cord compression were Great Danes (the breed with the smallest midsagittal vertebral foramen diameters from cranial C6 to cranial T1) and Doberman Pinschers, because of the most strikingly cranially dorsoventrally narrowed cone-shaped vertebral foramina at C6 and C7. The existence of a small midsagittal diameter in the cranial cervical spine was a high risk factor predisposing to spinal cord compression in small breeds and Dachshunds. Remarkable consistency was noted between the spinal level of the maximum enlargement of the spinal cord which previously was reported to be at C6, and the site of maximum enlargement of the vertebral canal currently stated in Dachshunds and small breeds. In large breeds the maximum enlargement of the vertebral canal tended to be located more caudally at the caudal limit of C7. The average age at which large dogs were most susceptible to noxious factors causing abnormal growth of the pedicles was determined to be 16 wk.     

Progressive spinal cord compression and degeneration of nerve fibers in rats

目的:探讨在进行性压迫性脊髓损伤过程中白质纤维溃变的规律.方法:采用自行设计的压迫装置制作进行性压迫性脊髓损伤模型.运用H-E、Luxol fast blue (LFB)、透射电镜和免疫荧光等方法,分别于压迫后1、3、7d观察脊髓白质纤维变化.结果:脊髓受压1d后,白质髓鞘化神经纤维出现水肿,排列疏松、髓鞘缺失等退行性溃变;随着压迫时间延长,神经纤维溃变加重,纤维数目逐渐减少,与对照组和正常组比较差异有统计学意义.髓鞘碱性蛋白阳性神经纤维变性,数量减少.结论:进行性压迫性脊髓损伤可诱发神经纤维脱髓鞘病变,并随着压迫时间推移溃变逐渐加重.     

脊髓受压损伤机制的三维有限元分析

目的 建立胸腰段脊髓的三维有限元模型,通过生物力学实验研究爆裂骨折时脊髓损伤的机制。方法 利用有限元仿真技术,对爆裂骨折时脊髓受压情况进行仿真模拟,并通过与已经验证的模型及体内、体外实验结果相对比的方式对仿真结果进行检验。结果 在爆裂骨折初期,脊髓白质的应变高于灰质。随着骨碎片位移的增加,灰质的应变逐渐上升,最终在骨碎片位移达到峰值时,白质的应变高于灰质。结论 脊柱发生爆裂骨折时,脊髓损伤的严重程度取决于脊髓后部组织的受累情况。脊髓前角（运动功能区）和后角（感觉功能区）发生创伤的顺序也对脊髓损伤的程度有重要影响。临床上,可以通过评估脊髓运动功能区和感觉功能区发生损伤的严重程度,更加准确判断患者病情。对于脊髓损伤过程中应变分布的进一步研究,能够在应对脊髓损伤时采取更为有效的治疗与预防措施。     

以脊髓压迫为首发症状的髓系肉瘤5例临床分析

目的 探讨以脊髓压迫为首发症状脊柱髓系肉瘤患者的临床表现、影像学特征及治疗方法。方法 采用回顾性横断面研究方法。纳入2014年1月—2017年12月长征医院骨肿瘤科收治的经骨髓穿刺及病理证实的脊柱髓系肉瘤患者5例,其中男3例、女2例,年龄15~54岁;肿瘤位于胸椎3例、腰椎2例。4例采取开放性外科手术治疗,出院后根据骨髓穿刺及病理结果,予以化疗及血液系统肿瘤规范化治疗;另1例行内科保守治疗(消炎镇痛、营养支持、化疗等)。分析患者治疗前后各项观察项目。结果 5例患者均有腰背部疼痛,伴下肢无力3例、双下肢不全瘫1例;X线检查均未见异常,CT和MRI表现为椎体骨质破坏或占位性病变。骨髓穿刺及术后肿瘤病理结果显示为急性粒细胞白血病4例,慢性粒细胞白血病1例;5例患者均经治疗后症状有所缓解。5例患者均获随访:1例术后行去甲氧柔红霉素+阿糖胞苷方案化疗5个疗程,并行异基因造血干细胞移植治疗,目前已随访至术后28个月,患者一般状态良好、无肿瘤复发;另外4例经化疗后出现复发,均死于感染,生存期5~26.5个月。结论 以脊髓压迫为首发症状的髓系肉瘤病例临床罕见,其影像学表现缺乏特异性,易出现误诊,骨髓穿刺及病理检查可确诊;当出现脊髓压迫表现时,建议早期行肿瘤切除神经减压术,术后辅以异基因造血干细胞移植和全身化疗。     

兔脊髓压迫器的研制及其评价

目的建立一种新型、可靠的脊髓压迫动物模型,为探索脊髓受压后的分子机制及临床治疗的研究奠定基础。方法根据兔椎骨及脊柱的形态特点,设计一种新型的兔脊髓压迫器,并用以制作兔脊髓急性压迫模型。运用改良Tarlov法、TTC、HE及Nissl染色验证该模型的可靠性。结果脊髓受压后,兔后肢的肌力减退、行动迟缓;TTC结果显示压迫段及其相邻节段均有不同程度的缺血,压迫后段还存在不同程度的出血及组织坏死;脊髓受压后出现组织水肿,神经元肿胀、核固缩,尼氏体减少甚至消失等病理改变。结论①用自行设计的压迫器制作兔脊髓压迫模型具有方法简便、科学、重复性强等特点;②本实验制作的兔脊髓压迫模型是用于研究脊髓压迫性损伤病理生理机制及临床治疗的理想模型。     

The dynamics of axolemmal disruption in guinea pig spinal cord following compression

Membrane damage has been postulated as a critical factor in mediating axonal degeneration in brain and spinal cord trauma. Despite compelling evidence of membrane disruption as a result of physical insults in both in vivo and in vitro studies, the dynamics of such damage over the time post injury in in vivo studies has not been well documented. Using a well-characterized in vivo guinea pig spinal cord compression model and horseradish peroxidase exclusion assay, we have documented significant membrane disruption at 1 hr, 3 days, and 7 days following injury. Furthermore, the membrane damage was found to spread laterally 10 mm beyond the center of original compression site in both rostral and caudal directions. A second-degree polynomial fit of the measured data predicts a bilateral spread of approximately 20-21 mm of membrane disruption from the epicenter of injury over a period of about 20 days. Thus, this study shows that membrane damage exists days, and possibly weeks, after spinal cord trauma in live guinea pigs. This provides the evidence necessary to investigate the role of membrane damage in triggering axonal deterioration in the future. Furthermore, this study has also revealed a long therapeutical window for membrane repair and functional enhancement following traumatic injury in the central nervous system.