The Cauda Equina Syndrome: A Rare Extra-Articular Manifestation of Ankylosing Spondylitis: A Case Report

A patient with long standing ankylosing spondylitis later developed a rare complication, the cauda equina syndrome, and associated lumbar thecal diverticulae. The pathogenesis of this condition and its investigation using radiological techniques are discussed.     

兔马尾神经慢性压迫后微血管改变的实验研究

目的研究马尾神经慢性压迫后微血管改变。方法采用中国本兔第七腰椎椎管内置入微型胶囊,定期注入一定量液体(形成马尾神经慢性压迫的动物模型),4周后将马尾神经灌注墨汁,用电镜观察其超微结构改变。结果压迫段以下马尾神经静脉扩张、瘀血、血管扭曲、纹理不清、染料外漏、微血管内皮细胞紧密连接开放,胶体碳颗粒通过血管内皮细胞紧密连接处。结论马尾神经受压平面以下静脉瘀血,导致血管内压增加,毛细血管内皮细胞损害,使其通透性增加,是继发以后病理改变的重要因素之一。     

Cauda equina compression by malignant lymphoma: long-term survival following multimodality therapy

Malignant lymphoma compression of the cauda equina is rare. In the literature only a few cases have been described with occasional long term survivors. We report another case of non-Hodgkin's lymphoma presenting with cauda equina compression. Significant palliation and long-term survival were achieved after therapy by decompressive, cytoreductive surgery; local irradiation; and chemotherapy.     

Aneurysm, arachnoiditis and intrathecal Au (gold)

This report is a 20-year follow-up of 14 patients treated with external beam craniospinal irradiation and intrathecal gold (10-45 mCi) for medulloblastoma. Six of the patients died within 2 years of treatment from persistent disease. No patients are alive without complications. Six of eight surviving patients developed arachnoiditis and cauda equina syndrome within 5 to 10 years of treatment. Seven of eight survivors developed aneurysms and/or cerebrovascular accidents 9 to 20 years after treatment. Four of the cerebrovascular events were fatal. Intrathecal gold pools in the basal cisterns and cauda equina delivering an extremely inhomogeneous dose throughout the neuroaxis. Its use is discouraged.     

A comparative study of ependymomas by site of origin

Sixty-one patients with histologically proven ependymoma were irradiated between 1954 and 1976. Supra-and infratentorial tumors occurred more often in children and spinal cord- cauda equina tumors more often in adults. Local control was achieved in four of 20 supratentorial, 13 of 26 infratentorial, three of seven intramedullary spinal cord, and seven of eight cauda equina tumors. Improved local control of infratentorial tumors was noted for patients who received higher biologically effective doses of radiation but no dose-response for supratentorial, spinal cord or cauda equina tumors could be found. Five-year actuarial survival was 56% for all patients, 35% for supratentorial, 59% for infratentorial, 57% for spinal cord and 83% for cauda equina tumors. Spinal metastases were pathologically documented in 5 of 46 (11%) patients with ependymomas above the foramen magnum. They were clinically evident in two patients and most common in patients with infratentorial tumors whose spines had not been irradiated. One patient who was irradiated externally for cauda equina tumor developed radiation myelopathy $\text{1}\text{2}$ years later; three of eight patients who received intrathecal gold 198 developed myelopathy and/or cauda equina syndrome $\text{3-}\text{1}\text{2}$ to 17 years later.     

Paragangliomas in the cauda equina region: clinicopathoradiologic findings in four cases

Summary Paragangliomas of the cauda equina are rare neuroendocrine tumors. Four cases of nonsecreting paraganglioma of the cauda equina, preoperatively misdiagnosed as neurinoma, are presented with an emphasis on the correlation between magnetic resonance imaging findings and pathological features. Although it is difficult to correctly diagnose paraganglioma preoperatively for intradural extramedullary tumors, especially in the cauda equina, paraganglioma should be included in differential diagnoses.     

Glioblastoma multiforme with bone metastase and cauda equina syndrome

Summary Remote metastases, leptomeningeal dissemination and spreading of the tumor by infiltration are rare complications of glioblastoma multiforme. The existence of different forms of spreading simultaneously is very rare. We present a case of a young patient with glioblastoma multiforme who was operated and subsequently received radiation therapy and cytotoxic treatment. Eight months following the operation, the patient developed cauda equina syndrome. Bone metastases of vertebrae and ribs, and direct infiltration of the recurrent tumor mass into the ethmoidal and frontal sinuses were found. The described patient demonstrates a very rare combination of all forms of spreading metastases in glioblastoma multiforme. The infiltration of the tumor mass into the sinuses and the direct permeation of the meningeal and dural venous system and of the leptomeninges, may explain the mechanism of the development of distant metastases and leptomeningeal dissemination in glioblastoma multiforme.     

The Narrow Cervical Spinal Canal

An element of developmental stenosis is present in a review of twelve patients presenting with cervical spondylotic myelopathy. Superimposed cervical spondylosis causes further canal narrowing to the extent that the minimal sagittal canal diameters of these patients measures from 5 mm to 10 mm. The maximal level of narrowing is at C3/C4 level where greatest stress is said to occur during neck movement. There is a possible association between cervical canal narrowing and lumbar canal stenosis (33% in this series). Advanced age of the patients and a moderately long history of CSM do not usually imply a poor prognosis. The control series show that cord compression signs are not present even with a sagittal diameter of 9 mm. This indicates that in CSM other factors are involved besides cervical canal stenosis. The most critical diameters are 6 mm and less. Under such circumstances surgery is indicated. Differentiation between CSM and a predominantly cauda equina compression syndrome does not generally pose a problem if attention is given to the importance of a very narrow cervical canal and the presence of an almost complete obstruction at cervical myelography.     

Giant Nondural-Based Cauda Equina Meningioma with Multiple Cysts

Summary A very rare case of a giant nondural-based cauda equina meningioma with multiple cysts was presented. Spinal meningioma most commonly occurs in the thoracic or cervical region and typically adheres to the dura. Only six cases of nondural-based meningioma have been reported in English literature. All occurred in the cauda equina region. These patients were predominantly female and younger than those with typical intraspinal meningioma. A 46-year-old woman had a 4-year history of lower back pain and right leg pain. Progressive weakness of both lower extremities occurred. Magnetic resonance imaging revealed a giant cauda equina tumor with multiple cysts from T₁₂ to L₄. Following laminectomies from T₁₁ to L₅ and intradural exposure, the tumor was found to be draped loosely by the roots of the cauda equina and attached to a root without any firm connection with dura mater. Complete removal of the tumor was achieved after microdissection of arachnoid and sacrifice of an involved rootlet of the cauda equina. The appearance of tumor was that of a typical neurilemmoma. However, histological and immunohistochemical analyses were consistent with meningioma. Nondural-based intraspinal meningiomas are very rare, particularly a giant tumor with multiple cysts as our presenting case. All of the cases previously reported, including our case, have been located in the cauda equina region. Most of the patients were female and were young, suggesting that the nondural-based cauda equina meningiomas are age- and sex-related. An accurate preoperative and operative diagnosis are difficult. Care must be taken in the management of cauda equina tumors resembling neurilemmoma which may in fact represent meningioma, particularly in the younger female.     

Combined paraganglioma and glioma of conus medullaris and cauda equina

A mixed paraganglioma and glioma occurred in the conus medullaris and cauda equina of a woman. The tumor was subdural, encapsulated, and vascular. It was first manifested with symptoms and signs of compression of the cauda equina when she was 19 years of age and was excised when she was 24. The tumor was attached to the conus medullaris, the dura mater, and a nerve root of the cauda equina. Striking light and dark cells with an organoid arrangement mingled with a few glial fibrillary acidic protein (GFAP)-positive neuroglia forming a combined paraganglioma and glioma. Twenty-three paragangliomas were reported in the literature including the present case. The sex and age of recorded 19 patients demonstrated ten males and nine females with an average age of 46.3 years. Twenty tumors were subdurally situated at the conus medullaris and cauda equina. Three neoplasms were epidural and thoracic in location.     

Neurosarcoidosis of the conus medullaris and cauda equina

An uncommon case of neurosarcoidosis of the conus medullaris and cauda equina is described in a 24‐year‐old man with a 4‐year history of urological symptoms and rash. Chest X‐ray and biopsy confirmed sarcoidosis and MRI diagnosed conus medullaris involvement.     

Leptomeningeal carcinomatosis from perineural invasion of a lip squamous cell carcinoma

Perineural invasion resulting in leptomeningeal carcinomatosis is a rare, but well‐recognized phenomenon in head and neck carcinomas. We report the rare case of a patient with a squamous cell carcinoma of the lip resulting in leptomeningeal carcinomatosis and review the relevant published work. A 51‐year‐old man presented with progressive facial paraesthesia after treatment for a recurrent squamous cell carcinoma of the lower lip. Cavernous sinus involvement was confirmed on MRI and he received stereotactic radiotherapy. He subsequently developed progressive lower limb neurological signs. An MRI showed multiple enhancing leptomeningeal nodules in the cervical and lumbar spine consistent with leptomeningeal carcinomatosis. Whole spine radiotherapy and dexamethasone resulted in short‐term stabilization of symptoms only and he rapidly succumbed to progressive neurological disease. To our knowledge, this is the first published report of a squamous cell carcinoma of the lip resulting in leptomeningeal disease of the cauda equina. It illustrates the potential aggressive natural history of squamous cell carcinomas with perineural invasion.     

Cauda Equina Paraganglioma: A Review

Although rare, cauda equina paraganglioma (CEP) is definitely established in the table of cauda equina tumours. There are no particular investigations to document its preoperative diagnosis, unless a vasomotor amine syndrome is present to guide the differential diagnostic spectrum. Magnetic resonance imaging is the examination of choice for the presurgical assessment, but there are still overlapping findings, mainly among CEP, ependymoma, schwannoma, and hemangioblastoma. It is also the only means for postoperative follow-up. Computed tomography may fail to reveal all intrathecal lesions. Total excision is the gold standard for lesions with such a benign or at least protracted clinical course, as observed with the CEP. In contrast to well-known oncologic principles in micro, ultrastructural, and immunohistochemical prediction analysis, these tumours behave in an independent fashion. Promising efforts are being conducted in immunohistochemistry and DNA search, but the mainstay of our knowledge regarding its natural history still stems from our sporadic observation of its clinical behaviour.     

Risk of cancer of unknown primary after hospitalization for autoimmune diseases

Cancer of unknown primary (CUP) is a heterogeneous syndrome diagnosed at metastatic sites. The etiology is unknown but immune dysfunction may be a contributing factor. Patients with autoimmune diseases were identified from the Swedish Hospital Discharge Register and linked to the Swedish Cancer Registry. Standardized incidence ratios (SIRs) were calculated for subsequent CUP and compared with subjects without autoimmune diseases. A total of 789,681 patients were hospitalized for any of 32 autoimmune diseases during years 1964–2012; 2,658 developed subsequent CUP, giving an overall SIR of 1.27. A total of 16 autoimmune diseases were associated with an increased risk for CUP; polymyositis/dermatomyositis showed the highest SIR of 3.51, followed by primary biliary cirrhosis (1.81) and Addison's disease (1.77). CUP risk is known to be reduced in long‐time users of pain‐relieving nonsteroidal anti‐inflammatory drugs (NSAIDs), such as aspirin. For patients with ankylosing spondylitis and with some other autoimmune diseases, with assumed chronic medication by NSAIDSs, CUP risks decreased in long‐term follow‐up. The overall risk of CUP was increased among patients diagnosed with autoimmune diseases, which call for clinical attention and suggest a possible role of immune dysfunction in CUP. The associations with many autoimmune diseases were weak which may imply that autoimmunity may not synergize with CUP‐related immune dysfunction. However, long‐term NSAID medication probably helped to curtail risks in some autoimmune diseases and CUP risks were generally higher in autoimmune diseases for which NSAIDs are not used and for these CUP appears to be a serious side effect.     

Paragangliomas of the cauda equina. Report of one case and review of the literature

The authors report one case of cauda equina paraganglioma and review the neurosurgical, radiological and pathological literatureon this rare tumour. Although it is difficult to distinguish preoperatively the paraganglioma from other tumours of the cauda equina, like neurinoma or ependymoma, this neuroendocrine tumour should be included in differential diagnosis.     

Neurologic complications of Merkel cell carcinoma

We describe a 61-year-old man with a multiple neurologic complication of Merkel cell carcinoma, a rare skin cancer. An enhancing brain mass, and cytologically proven leptomeningeal disease produced a succession of symptoms including seizures, bilateral radiculopathies, myoclonus, a cauda equina syndrome and altered mental status. Aggressive treatment prolonged his survival marginally.     

脊柱关节炎分类标准的演变

脊柱关节炎（ spodyloarthritis，SPA ）是一组互相关联的，通常侵犯脊柱、外周关节、关节周围结构的多系统炎性疾病，包括强直性脊柱炎（ ankylosing spondylitis， AS ）、银屑病关节炎（ psoriatic arthritis，PSA ）、反应性关节炎（ reactive arthritis，REA ）、赖特综合征（ Reiter＇s syndrome，RS ）、炎性肠病关节炎（ inflammatory bowel disease arthritis ）、幼年发病的脊柱关节病以及分类未定的脊柱关节病。     

Postradiation lumbosacral radiculopathy with spinal root cavernomas mimicking carcinomatous meningitis

Lumbosacral radiculopathy is a rare complication of radiotherapy and may be challenging to differentiate from diagnosis of a tumor recurrence. We reviewed the records of three patients with a past history of cancer and radiotherapy who were referred for suspicion of carcinomatous meningitis on lumbar MRI, but whose final diagnosis was radiation-induced lumbosacral radiculopathy. The three patients developed a progressive lumbosacral radiculopathy at 20, 13, and 47 years after lumbar radiotherapy delivered for renal cancer, Hodgkin's disease, and a seminoma, respectively. MRI showed a diffuse, nodular enhancement of the cauda equina nerve roots on T1 sequences, suggestive of leptomeningeal metastasis. A slowly progressive clinical course over several years and negative cerebrospinal fluid cytologic analysis ruled out the diagnosis of carcinomatous meningitis. Because of the radiologic findings, a biopsy was performed in two patients. In the first, a biopsy limited to the arachnoid excluded a malignant infiltration. In the second, a biopsy of the enhancing lesions demonstrated spinal root cavernomas. These observations, together with three recent case reports in the literature, delineate a syndrome of "radiationinduced lumbosacral radiculopathy with multiple spinal root cavernomas" that mimics carcinomatous meningitis on MRI. Its diagnosis is important in order to avoid inappropriate treatment and useless or dangerous spinal root biopsies.     

Posterior decompression and stabilization,and surgical vertebroplasty with the vertebral body stenting for metastatic vertebral and epidural cauda equina compression

We present the technique of combined posterior decompression and spinal instrumentation, and surgical (open) vertebroplasty using a novel system called vertebral body stenting (VBS) during a single session in a patient with metastatic vertebral and epidural cauda equina compression. J. Surg. Oncol. 2010; 101:253–258. © 2010 Wiley‐Liss, Inc.