Mizoribine for renal sarcoidosis: effective steroid tapering and prevention of recurrence

Sarcoidosis is a systemic disease of unknown etiology that is characterized by chronic non-caseating granulomatous inflammation with tissue destruction. It is an uncommon disease in children, and renal sarcoidosis in particular is very rare in adults and children. A 17-year-old boy with renal sarcoidosis was referred to our hospital with an initial diagnosis of pyelonephritis. Prior treatment with various antibiotics had not been effective, but tentative oral daily prednisolone (PSL) had partially ameliorated his symptoms, although the symptoms recurred during steroid tapering. We detected non-caseating granulomatous interstitial nephritis and numerous sclerotic glomeruli in a second biopsy specimen, compatible with the diagnosis of renal sarcoidosis. The patient was treated with pulsed methyl-prednisolone and oral daily doses of PSL and mizoribine (MZR). During the treatment with MZR, the PSL was successfully tapered, and the patient has since presented no signs of recurrence. Our treatment of this patient shows that treatment with MZR can allow steroid sparing and prevent recurrence in a patient with sarcoidosis.     

Sarcoidosis presenting as an isolated intramedullary tumor

We report a case of isolated intramedullary sarcoidosis. The patient developed progressive signs that indicated a spinal tumor, which were investigated with contrast-enhanced magnetic resonance imaging scans. Magnetic resonance imaging clearly revealed an intramedullary lesion, but the diagnosis of sarcoidosis was made on the pathological analysis of the surgical specimen. Magnetic resonance imaging with contrast enhancement is reported in a histologically proven case of intramedullary sarcoidosis. Only 12 other cases of isolated intramedullary sarcoidosis have been reported. We review and discuss these cases according to their clinical presentation, the segmental location of the granulomas in the spinal cord, preoperative and operative diagnoses, and signs for systemic sarcoidosis. In none of the cases was the diagnosis of intramedullary sarcoidosis made before surgery. We think that surgical therapy for intramedullary lesions is the best way to diagnose rare instances of benign lesions like sarcoidosis and to treat them in an appropriate manner.     

Cervical spinal cord injury caused by vertebral body fracture in a patient with concomitant OALL and OPLL

We encountered a case of cervical spinal cord injury associated with cervical vertebral dislocation fracture that occurred in a patient with concomitant ossification of the anterior and posterior longitudinal ligaments. The cervical vertebrae were injured by hyperextension in a car accident. On admission, shearing fracture was noted in the OALL region and vertebral body OPLL region over the posterior column at the fourth cervical level, but no dislocation or neurological findings were noted. Restlessness occurred and caused dislocation several hours after admission, leading to complete injury of the cervical spinal cord. The patient died of complications after 3 weeks. Vertebral body fracture complicated by both OALL and OPLL is very rare, and only four cases have been reported. Since vertebral injury causes delayed fracture/dislocation after a symptom-free interval in cases with ligament ossification, accurate clinical evaluation early after injury and early fixation are necessary.     

SPECT-CT图像融合技术对老年椎体压缩骨折患者选择椎体强化术靶椎体的应用价值

目的:探讨单光子发射计算机断层摄像联合同机CT扫描(single photon emission computed tomographyand computer tomography,SPECT-CT)图像融合技术对老年椎体压缩骨折患者选择椎体强化术靶椎体的应用价值。方法:2010年6月~2011年4月对4例因体内置入顺磁性材料而不能接受MRI的老年椎体压缩骨折患者应用Infinia Hawkeye 4图像融合系统行SPECT全身平面骨显像,然后对感兴趣区病灶行同机CT检查,用图像融合软件完成SPECT-CT图像融合,在SPECT-CT融合图像上出现核素浓集的椎体考虑为病椎,在同机CT重建图像上仔细分析病椎CT图像、排除肿瘤或感染性骨破坏可能,从而确立骨质疏松性椎体压缩骨折的临床诊断,并定位椎体强化术靶椎体。男1例,女3例;年龄59～79岁,平均67.4岁;病程8d～2个月,平均17d;因冠心病安装冠状动脉金属支架2例、心脏起搏器1例,体内置入不锈钢股骨头假体1例;诊断确立后实施椎体强化术,记录术前、术后2d的VAS和Oswestry功能障碍指数(ODI)以评估手术疗效,放射学检查随访以进一步证实SPECT-CT图像融合检查的准确性。结果:SPECT-CT融合图像中,3例患者单个椎体、1例2个椎体出现核素浓集,其中T12 2例、L1 1例、L2 1例、L3 1例,同机CT重建图像提示病变椎体中无肿瘤或感染性骨破坏征象。术前、术后2d VAS分别为8~9分、1.5~2.5分,术前、术后2d ODI分别为80%~90%、20%~30%,术后2d与术前相比改善明显。随访8~13个月,平均10个月,影像学随访病椎未出现迟发性骨破坏。结论:对于不能接受MRI检查的老年椎体压缩骨折患者,SPECT-CT图像融合技术可清楚定位核素浓集区的病变节段,通过分析核素浓集区骨结构特点可确定疾病性质;SPECT-CT图像融合技术在选择椎体强化术靶椎体上有一定实用价值。     

Differenzialdiagnosen einer granulomatösen Entzündung im anterioren Oberkiefer

This article presents an unusual case of a 36-year-old male patient who presented with an extensive osteolytic lesion in the anterior maxilla which mimicked a periapical lesion. Histological examination showed non-caseating granulomatous inflammation. Further radiographic and biochemical examinations revealed pulmonary sarcoidosis and solitary involvement of sarcoidosis in the anterior maxilla. On the basis of the case presented the differential diagnosis, therapy and the need for interdisciplinary co-operation are discussed.     

Klippel-Feil综合征的MRI诊断价值

目的探讨Klippel-Feil综合征的MRI表现及应用价值。方法对50例成人Klippel-Feil综合征患者进行MRI检查,观察椎体融合情况及伴发病变。结果颈椎椎体融合累及2个椎体43例,累及3个椎体4例,累及4个椎体2例,累及5个椎体1例。伴椎间盘膨出或突出46例,伴黄韧带肥厚34例,伴后纵韧带肥厚4例,伴椎体后缘骨赘形成9例,颈髓明显受压15例,伴颈髓损伤10例。结论 MRI检查易于诊断Klippel-Feil综合征及其伴发颈椎退变及颈髓损伤,值得应用。     

Tetraplegia secondary to cervical sarcoidosis. Case report.

This is the report of a case of vertebral sarcoidosis causing collapse of cervical vertebrae with resulting tetraplegia. The patient was successfully treated by decompression and subsequent anterior removal of the invaded three vertebral bodies, followed by an autologous bone graft.     

Multifocal extensive spinal tuberculosis (Pott's disease) involving cervical, thoracic and lumbar vertebrae

An unusual case is reported of a 53-year-old woman presenting with spinal tuberculosis involving cervical, thoracic and lumbar vertebrae. The patient originally presented with progressive quadriparesis, fever, night sweats and weight loss. Imaging studies demonstrated vertebral body destruction with and without paraspinal and/or intraspinal abscess in cervical, thoracic and lumbar regions. The laboratory studies confirmed the diagnosis of Pott's disease and the patient was placed on anti-tuberculosis chemotherapy. The anterior approach was used for removal of the infected necrotic material, bone grafting and instrumentation, in both cervical and thoracic vertebrae. The postoperative course was uneventful and the patient made an excellent neurological recovery. The present report is of the first published case of an extensive spinal tuberculosis involving cervical, thoracic and lumbar spine.     

Spinal trauma with a complete anterior vertebral body dislocation: a report of three cases

STUDY DESIGN: Retrospective review of three cases.Objectives:Severe trauma can be responsible for a complete spinal anterior dislocation with a 100% anterior slip of the vertebral body. Three cases of this uncommon lesion are reported. SETTING: France. METHODS: The data of three cases of complete spinal anterior dislocation with a 100% anterior slip of the vertebral body were retrospectively reviewed. RESULTS: In all the cases, the vertebral dislocation was responsible for a severe neurological deficit and all patients had severe associated lesions. The diagnosis was made on plain radiographs. In one case of a multilevel injury, an extensive instrumented spinal fusion was necessary. In spite of the severe injury, two neurological deficits improved thanks to pedicular fractures, which widen the canal. CONCLUSION: The therapeutic goal is to achieve emergent vertebral alignment, neurological decompression and solid spinal fusion. A posterior facilitates this. Reduction of vertebral dislocation can be difficult to achieve and it is therefore mandatory to perform complete arthrectomy of the injured levels before reduction. Especially in young patients, severe disc lesions secondary to the wide vertebral displacement make it necessary to perform circumferential fusion.     

Infiltrating spinal angiolipoma causing myelopathy: case report

We present a case of an infiltrating spinal angiolipoma demonstrating extension into the vertebral body and the spinal epidural space. The infiltration into the epidural space caused myelopathy. About 40 cases of spinal angiolipoma and angiomyolipoma have been reported; however, only a few cases have been the infiltrating type. The radiological findings were similar to those of vertebral hemangioma, but poor enhancement of the angiolipoma on contrast-enhanced computed tomographic scans differentiated between them. The infiltrating epidural tumor was removed, and the clinical symptoms improved remarkably. Total removal of the tumor and stabilization of the involved vertebral body using the anterolateral approach may be desirable when a diagnosis of angiolipoma or angiomyolipoma is confirmed preoperatively.     

Intradural, extramedullary spinal sarcoidosis: report of a rare case and review of the literature.

BACKGROUND: Spinal sarcoidosis represents a rare subgroup of neurosarcoidosis. Most spinal sarcoid lesions are intramedullary, and only eight cases of intradural, extramedullary sarcoid lesions have been reported hitherto. We describe the complete entity of intradural, extramedullary spinal sarcoidosis. METHODS: A 39-year-old woman presented with a 2-year history of slowly progressive numbness and paresthesia of the right medial upper arm followed by brachialgia and cephalgia. Magnetic resonance imaging revealed an intradural extramedullary T1 isointense and T2 hyperintense mass lesion near the right C3 nerve root exit C3, extending into the right neuroforamen. The cervical cord was not severely compressed. The mass lesion showed a high T2 signal. After intravenous administration of gadolinium-diethylenetriaminepentaacetic acid, there was marked enhancement of the process. RESULTS: The patient underwent a C2 to C3 laminoplasty and total resection of the intradural extramedullary tumor. The lesion encroached along the right neuroforamen, involving the C3 nerve root, and was grossly adherent to some of the rootlets. Histopathological examination of the specimen showed a noncaseating granulomatosis consistent with sarcoidosis. Postoperative testing did not reveal systemic involvement of sarcoidosis but bilateral hilar and mediastinal lymphadenopathy with normal lung parenchyma. The patient was therefore treated with corticosteroids. During the follow-up period of 16 months, the patient made a satisfactory recovery, though with persistent, distally pronounced sensory disturbance in the C3 or C4 root areas, and returned to work full-time. CONCLUSION: An extramedullary sarcoid lesion is rare. Unlike intramedullary sarcoid lesions, it can be totally removed. Even if systemic sarcoidosis is present, the patient can have an excellent recovery. From a review of the literature, we can hypothesize that intradural extramedullary spinal sarcoidosis may represent a very early stage of spinal sarcoidosis progressing to intramedullary intradural spinal sarcoidosis. The intradural extramedullary spinal sarcoidosis can be subdivided into a peripheral and a central subtype. Positive histological examination is required to establish the diagnosis since other diagnostic tests are unspecific.     

Intradural Extramedullary Sarcoidosis case report and review of literature

Spinal sarcoidosis represents a rare subgroup of neurosarcoidosis. Most spinal sarcoid lesions are intramedullary, and only a few cases of Intradural Extramedullary (IDEM) sarcoidosis have been reported till date.A thirty years-old female patient with intradural extramedullary sarcoid lesion in the cervico-dorsal spinal canal (C7-D1) without any systemic involvement of sarcoidosis is described. The patient presented with rapidly progressive compressive myelopathy with bladder involvement. She was treated with surgical removal of the lesion coupled with the administration of steroid and showed marked improvement.     

Correlation of anomalous vascular and osseous anatomy in congenital kyphosis type I. Case report

Congenital kyphosis Type I results from failure of formation of the vertebral body (VB). The clinical course is characterized by progressive deformity and neurological dysfunction unless the patient undergoes fusion. It has been postulated that failure of VB formation may be secondary to anomalies of the segmental arteries arising from the aorta. There is limited clinical evidence in the literature, however, to support this hypothesis. The authors report on the case of an adult patient with congenital kyphosis Type I in whom spinal angiography correlated vascular with vertebral anomalies. This 32-year-old man presented with back pain, lower-extremity paresthesias, and incomplete voiding, which had progressed over several years. Imaging revealed that the L-1 and L-2 vertebrae had not fully formed, causing kyphotic deformity such that the anterior portion of T-12 nearly articulated with that of L-3; this anomaly produced significant mobility in flexion and extension. Magnetic resonance imaging revealed distal spinal cord atrophy and signal change on T2-weighted images. Spinal angiography showed that the right and left segmental arteries at both L-1 and L-2 arose from a common trunk from the aorta. This was confirmed during subsequent T12-L3 anterior-posterior fusion. Postoperatively the patient experienced relief of pain and remained neurologically stable throughout a 3-year follow-up period. The presence of corresponding anomalies of the segmental arteries and vertebrae in this case of congenital kyphosis Type I indicates that a relationship may exist in the genesis of aberrant spinal vertebral and vascular structures.     

手术治疗胸腰椎结核79例临床分析

目的探讨胸腰椎结核一期病灶清除、植骨融合、内固定治疗的临床疗效。方法回顾分析自2002年9月至2008年7月我院手术治疗并获得随访的79例胸腰椎结核患者,病变范围为T5～S1,病灶累及1～2个椎体72例,大于等于3个椎体7例,其中跳跃性脊柱结核4例。手术方法：a）经前路病灶清除,椎体间植骨融合并内固定术;b）后路椎弓根固定术并前路病灶清除、椎体间植骨融合术。术后佩戴支具3～6个月,抗结核药物治疗12～18个月。定期门诊随访,进行实验室检查和影像学观察。结果该组患者随访2～9年,均获临床治愈。79例前路植骨病例,界面骨性融合时间平均4.7个月,后凸畸形平均矫正18.7°,随访期内丢失2°。合并截瘫29例,术后神经症状均得到明显改善。结论一期结核病灶清除、植骨融合内固定术是治疗脊柱结核的重要手段,有效的抗结核化疗是脊柱结核治愈的基础。     

颈椎骨折脱位合并单侧椎动脉损伤的手术治疗

目的探讨合并单侧椎动脉损伤的颈椎骨折脱位的手术治疗风险和临床效果。方法76例合并单侧椎动脉损伤的颈椎骨折脱位患者行前路减压、植骨融合内固定,其中颈椎脱位患者先试行闭合复位,不能复位者,先行后路切开复位,一期再行前路减压、植骨融合。结果76例患者均未出现健侧椎动脉损伤,术前神经功能正常者术后无一例出现神经损害症状,不全瘫患者术后均有不同程度恢复。结论合并单侧椎动脉损伤的颈椎骨折脱位进行合理的手术治疗具有良好的效果。     

Extranodal Rosai-Dorfman disease with multilevel lumbar spinal lesions

The authors describe the case of a 44-year-old man with multilevel lumbar spinal Rosai-Dorfman disease (RDD), a rare clinical entity. To the authors' knowledge, there have been only 2 cases of lumbar spinal involvement of RDD (epidural) reported in the literature, and the current case is the third but the only one showing lumbar spinal intradural involvement of RDD. This case of RDD mimicked a meningioma both clinically and radiologically. The patient underwent a procedure in which the tumor was excised, and postoperatively the patient made a clinically acceptable recovery. Vertebral canal involvement of RDD should be considered in the differential diagnosis of vertebral canal tumors. Resection is an acceptable treatment option.     

Infected primary non-Hodgkin lymphoma of spine

Primary bone lymphoma (PBL) comprises less than 5% of all malignant bone tumors and almost 7% of all extranodal lymphomas. Only 1.7% of all PBLs have been reported to involve the vertebrae. In our case, osteomyelitis was accidentally found during surgery, which might have resulted in the rapid collapse of vertebral body. This is the first report on primary lymphoma of the vertebrae with superimposed osteomyelitis in the English literature to the best of our knowledge. The patient reported here received anterior vertebrectomy and posterior interbody fusion with instrumentation for spinal instability. Tumor mass and the necrotic debris were removed. After the procedure, the patient received treatment with antibiotics and six cycles of chemotherapy. This case reminds us the possibility of hematologic seeding of bacteria in the tissue, especially with tumor necrosis. We suggest percutaneous needle aspiration for pathology and culture before making a decision whether or not to proceed with surgical decompression for fear of missing the occult bacterial infection.     

Vertebral hemangioma presenting with intermittent claudication

Summary The case of a patient with vertebral hemangioma and unusual clinical presentation is reported, with an attempt to explain these unusual clinical complaints. Vertebral hemangioma is a common and often asymptomatic tumor. Neurologic symptoms may appear due to pressure on the neural tissue caused by extraosseous extension. The patient reported here presented with intermittent claudication. Conventional radiography CT, and MRI revealed vertebral hemangioma at T5 and extraosseous extension compressing the spinal cord. Gravity-related vascular dilatation may induce further compression of the spinal cord and, thus, is thought to be the underlying event in the induction of the intermittent clinical symptoms. The lesion was treated with subtotal corpectomy after embolization and fusion with a strut iliac crest graft. At the 9-month follow-up, the patient was without complaints. We conclude that a diagnosis of vertebral hemangioma should be considered in cases of intermittent neurologic symptoms of the lower extremities.     

Dorsolumbar spine duplication.

We report a case of dorsolumbar spine duplication in a female patient. She had several vertebral anomalies, such as fused vertebrae, hemivertebrae and butterfly vertebrae, together with duplication of the spinal column in the lower thoracic and the whole lumbar tract. Clinically, she had no control of the sphincters, but her gait was only slightly affected. This is probably the third reported case of this rare anomaly.     

Metastatic tumors involving the cervical vertebrae: surgical palliation.

Pain, weakness, or paralysis from involvement of the spinal cord and nerve roots secondary to invasion of the vertebrae by a malignant tumor often can be avoided or alleviated by stabilization of the spine. Twelve patients with neoplastic infiltration of the cervical vertebrae were so treated. The operation of wiring, augmentation bone-grafting, and decompression of the spinal cord was successful after conservative methods failed. Indications for operation were: (1) unremitting pain in the neck, not relieved by bracing or radiation therapy; (2) a major degree of vertebral destruction with loss, or impending loss, of support for the head; (3) collapse of a vertebral body; or (4) neural deficit from local tumor invasion. A classification of our twelve patients into three groups helped to delineate the surgical procedure needed. The value of obtaining spinal stability and a solid fusion above and below the tumor was evident in eleven patients. For almost all of their survival time, they were comfortable. Surgical treatment may not appreciably extend the lenght of a patient's survival, but it generally improves the patient's quality of life.