How not to miss autoinflammatory diseases masquerading as urticaria

Urticarial skin reactions are one of the most frequent problems seen by allergists and clinical immunologists in daily practice. The most common reason for recurrent wheals is spontaneous urticaria. There are, however, several less common diseases that present with urticarial rash, such as urticarial vasculitis and autoinflammatory disorders. The latter include cryopyrin‐associated periodic syndrome and Schnitzler's syndrome, both rare and disabling conditions mediated by increased interleukin‐1 secretion. Apart from the urticarial rash, patients are suffering from a variety of systemic symptoms including recurrent fever attacks, arthralgia or arthritis and fatigue. Autoinflammatory diseases are often associated with a diagnostic delay of many years and do not respond to antihistamines and other treatments of urticaria. Also, the chronic inflammation may lead to long‐term complications such as amyloidosis. It is therefore important not to miss these diseases when diagnosing and treating patients with chronic recurrent urticarial rash. Here, we present clinical clues and tips that can help to identify autoinflammatory disorders in patients presenting with chronic urticarial rash and discuss their clinical picture and management.     

Myelofibrosis presenting as spinal cord compression

This report describes a case of myelofibrosis presenting as spinal cord compression on account of extramedullary haemopoietic tissue encroaching upon the spinal cord from a large pelvic mass.     

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.     

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.     

An audit of current practice and management of metastatic spinal cord compression at a regional cancer centre

Metastatic spinal cord compression (MSCC) is an oncological emergency requiring prompt recognition and management to preserve neurological function and mobility. We performed an audit to assess current practice of MSCC against current best practice as outlined by NICE. Our retrospective audit identified 10 patients from January to December 2009 with confirmed MSCC. The most common primary tumours were prostate 3 (30%), breast 3 (30%) and lung 2 (20%). Pain was the main presenting symptom 9 (90%), followed by weakness 7 (70%) and sensory changes 1 (10%). 5 (50%) had MRI within 24 hours and only 6 (60%) underwent full MRI scan. 8 (80%) had corticosteroids before MRI scan. 6 (60%) received radiotherapy within 24 hours. Only 4 (40%) were referred to orthopaedics and none of these patients had been recommended surgery. Up 14 days following radiological confirmation of MSCC, the number of patients who were unable to walk increased by 20%. Only 5 (50%) were discharged during this period of study. Our audit reported a number of variances in management compared to NICE guideline. These can be improved by following a'fast track' referral pathway and regular education for junior doctors and primary care doctors.     

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.     

Delayed neuronal damage related to microglia proliferation after mild spinal cord compression injury

In order to investigate the mechanism of delayed progressive or secondary neuronal damage after the spinal cord injury, we developed a mild-compression injury model in the rat thoracic spinal cord. Our compression device consists of a soft silicone point of contact to the dura, in order to prevent violent injury that may cause axonal tears or hemorrhages in the spinal cord. Since rats often assume a 'standing' posture, i.e. raising head with lifting their fore-limbs, damage to the thoracic spinal cord was evaluated by measuring the frequency of 'standing', which effectively indicates hind limb function. Twenty-four hours after compression by a 20 g weight for 10 or 20 min, the standing frequency of the injured rat was almost the same as that of sham animals that underwent laminectomy without compression. However, the standing frequency decreased with time; the frequency of standing at 72 h was approximately 30-50% that of sham animals. In the compressed spinal cord tissue, microglial cells, detected by lectin staining, proliferated with time. An enormous amount of microglia was observed at 48 and 72 h after compression, although only a small amount of cells were positive to lectin staining at 24 h after the compression. These results suggest that our mild-compression spinal cord injury model showed late-onset or delayed neuronal damage that may be related to pathological microglia proliferation.     

Transendothelial vesicular transport of protein following compression injury to the spinal cord.

Routes of vascular leakage resulting in trauma-induced edema have not been clarified. To explore the problem we followed the fate of intravascular horseradish peroxidase (HRP) after compression injury to the thoracic cord (cats). At 90 seconds and 15 minutes, HRP was confined to the gray matter, occupying perivascular spaces, unexpanded extracellular channels, and cytoplasmic compartments of injured cells. Adjoining segments contained similar but lesser deposits. At 4 hours, tracer occupied the expanded extracellular spaces of the lesion's white matter; gray matter deposits were present up to 4 cm. distal. Vessels revealed no evidence of rupture. Open interendothelial junctions were not found. Counts of HRP-labeled vesicles in the endothelium of gray matter capillaries revealed a significant intensification of vesicular activity in the lesion and in adjacent areas up to 9 cm. caudal. Morphologically, labeled vesicles exhibited a wide diversity in shape and size. Typical pinocytotic (700A) and tubular forms measured 400 to 700 A in width; vacuolar forms measuring up to 0.7 mum. across were frequently observed. Continuity between the three types was often evident. Where basement membrane and perivascular clefts were not yet inundated with HRP, sites of vesicular emptying of HRP at the tissue front were identified. Serial sections revealed that vesicles may be contiguous from luminal to abluminal surfaces, thus providing facilitated transport pathways. The data suggest that vesicular transport plays a role in the genesis of trauma-induced edema.     

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。     

不同程度脊髓背侧压迫损伤模型大鼠脊髓损伤致伤器的设计

背景：随着对脊髓损伤研究的不断深入,国内外学者不断设计出各种脊髓损伤模型,主要包括脊髓挫伤、重物坠击、脊髓压迫、化学灼伤、放射性损伤及激素横断半横断损伤等,然而各种制备方法都存在一定的局限。 目的：设计脊髓损伤致伤器并建立不同程度脊髓损伤动物模型。 方法：实验自行设计包括致伤部件及传动装置的脊髓损伤致伤器,利用不同致伤质量m1=10 g,m2=20 g,m3=30 g及时间T1=3 s,T2=5 s,造成不同质量×时间暨不同程度SD大鼠脊髓背侧压迫损伤,分为m1T1,m2T1,m3T1,m1T2,m2T2,m3T2共6组,并设假手术组进行对照。 结果与结论：造模后各实验组BBB评分均低于假手术组(P < 0.01),m1T1组与其他各实验组相比差异有显著性意义(P < 0.01),建模后8周m1T2组BBB评分高于与m2T2组及m3T2组(P < 0.05)。建模后8周各组体感诱发电位和运动诱发电位潜伏期明显长于假手术组(P < 0.01)。除了m1T1组与m1T2组外,其他各组间建模后体感诱发电位潜伏期均明显延长(P < 0.05)。所有组建模后运动诱发电位潜伏期均明显延长(P < 0.05)。结果证实,实验所设计的脊髓损伤致伤器可制备出不同程度的脊髓背侧压迫损伤动物模型。 中国组织工程研究杂志出版内容重点：肾移植;肝移植;移植;心脏移植;组织移植;皮肤移植;皮瓣移植;血管移植;器官移植;组织工程全文链接：     

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.