Disseminated enteropathy-type T-cell lymphoma: cauda equina syndrome complicating coeliac disease

A patient with coeliac disease developed a progressive cauda equina syndrome in the 2 months prior to his death, for which no cause could be identified. At post-mortem examination this proved to be due to disseminated enteropathy-type T-cell lymphoma. Although rare, disseminated enteropathy-type T-cell lymphoma should enter the differential diagnosis in any patient with coeliac disease developing new neurological signs.     

Solitary localised hypertrophic neuropathy of the cauda equina

Localised hypertrophic neuropathy (LHN) is an uncommon non-neoplastic lesion, which is rare in the central nervous system. We report a patient with LHN of the cauda equina. Pre-operatively these lesions cannot be differentiated from schwannoma or other benign tumours or expansions of the nerve roots. Treatment is generally surgical, largely to confirm the diagnosis, and examination of the surgical specimen reveals an expansion of the nerve by “onion bulb” whorls of Schwann cells (S-100 positive). Multiple lesions may be associated with the generalised diffuse hypertrophic neuropathies or chronic inflammatory demyelinating polyneuropathies. It should be differentiated from intraneural perineuroma, which has a similar appearance but comprises perineural cells (epithelial membrane antigen positive). Adjuvant therapy is not required.     

Minimally invasive discectomy for the treatment of disc herniation causing cauda equina syndrome

Cauda equina syndrome (CES) is a condition associated with significant morbidity that requires definitive surgical decompression of the nerve roots to prevent permanent disability. Traditionally, wide open decompression has been advocated to obtain optimal decompression with minimal complications. Some have been reluctant to employ minimally invasive strategies to treat urgent conditions. The authors present a small series of four patients who presented with CES and were treated with minimally invasive discectomy (MID). Operative length times (mean ± standard deviation, 102.8 ± 30.9 minutes) were comparable to reported operative length for open laminectomies. All patients had either stabilization or resolution of symptoms at the 6-week follow-up examination. No patients suffered a cerebrospinal fluid (CSF) leak post-operatively. This series of patients presenting with CED at a single institution treated with MID demonstrates that MID can be used to successfully treat CES. Even large fragments can be safely removed without increased risk of a CSF leak.     

Sarcoidosis of the cauda equina: a report of three cases

Summary Three cases of sarcoidosis of the cauda equina are presented. In two there was no previous history suggestive of sarcoidosis and the presentation was one of a painful flaccid paralysis; in these the diagnosis was made after myelography and laminectomy with subsequent histology. The third patient had a previous diagnosis of lupus pernio and magnetic resonance imaging (MRI) was used to aid diagnosis. Only two previous cases have been reported of sarcoidosis presenting in the cauda equina with no other systemic manifestations. This is the first report of the use of MRI in sarcoidosis of the lower meninges.     

Tumors of the cauda equina: the importance of an early diagnosis

The natural history of cauda equina tumors was studied in 13 patients. Pain was the most prominent clinical manifestation. Neurological dysfunction mainly consisted of sensory disturbances. The pre-operative course was in 62% three years or longer.

A relation was found between the histology of the tumor and the delay of diagnosis: ependymomas were discovered much later than schwannomas. Other histological types of tumors were meningioma, sarcoma and metastasis of a medulloblastoma.

The effect of treatment, in nine cases only surgical, was excellent in seven of the 13 patients.

The prognosis was determined by histology and length of pre-operative period.     

Tumors in the cauda equina: A SEER analysis of tumor types and predictors of outcome

Cauda equina tumors are histologically diverse. International Classification of Diseases for Oncology (ICD-O3) confers dedicated site code (C72. 1) for cauda equina. This code is excluded during analyses of other primary spinal cord tumors. In this retrospective study, the Surveillance, Epidemiology and End Results (SEER) data for primary cauda equina tumors (PCET, C72. 1) excluding the tumors of spinal meninges (C70. 1) from 1992 to 2015 were reviewed. Demographic characteristics, tumor types, and clinical outcomes were analyzed using univariable analysis. Overall survival was estimated using Kaplan-Meier methods and compared for age, histology and treatment type. 293 patients with PCET met inclusion criteria. The most common tumors comprised schwannoma (32%), myxopapillary ependymoma (21%), malignant ependymoma (22%). The median age at diagnosis was 50 years (range < 1 year to 98 years), 57% of patients were males. 77% of the patients underwent surgery. Median follow up time for these patients was 70 months. Of the 293 patients, 250 (85%) were living at the end of 2015. The cause of death was tumor or CNS related in 15 patients. 136 patients were followed for <5 years, of which 102 were censored and 34 died (11.6%) before 5 years. Using univariable analysis, age at diagnosis (Hazard Ratio, HR 1.05; confidence interval, CI 1.03–1.07; p < 0.001), malignant tumor type (HR 2.88, CI 1.15–7.19, p = 0.0239) and absence of surgical intervention (HR 2.54, CI1.26–5.11, p = 0.0092) were predictors of increased mortality. Although most patients did well, older age and lack of surgical intervention were associated with worse survival.     

Metastatic malignant peripheral nerve sheath tumor of the cauda equina

Malignant peripheral nerve sheath tumor (MPNST) of the cauda equina is rare, with only four reported patients in the literature. We present a 70-year-old woman with a history of left sciatic nerve MPNST that had been treated by left hemipelvectomy 4 years prior. She then presented with disabling right leg weakness that progressed over 7 months. Spine imaging revealed an intradural extramedullary lesion from L5 to S1 with compression of the cauda equina. She underwent an L3-S1 laminectomy with subtotal resection of the lesion, which was diagnosed as a metastatic MPNST. At the 3-month postoperative visit, her lower extremity strength had returned to baseline. The only notable postoperative complication was a superficial wound infection that resolved with antibiotic treatment and wet-to-dry dressing changes. Thus, although uncommon, the spinal canal and cauda equina should be considered as possible locations for metastatic MPNST in the appropriate clinical scenario. To our knowledge, this is the first report of a patient with a metastatic MPNST to the cauda equina.     

神经电生理监测技术在圆锥马尾病变手术中的应用

目的探讨神经电生理监测技术在圆锥马尾病变手术中应用价值。方法回顾性分析110例圆锥马尾病变患者临床资料,其显微外科手术均在神经电生理监测下进行,感觉诱发电位(somatosensory evoked potential,SEP)和运动诱发电位(motor evoked potential,MEP)监测脊髓功能,肌电图(electromyography,EMG)确定肿瘤切除范围。结果显微镜下病变全切除92例(83.6%),次全或大部分切除18例(16.4%)。术后随访1~58个月,神经系统查体及JOA评分发现脊髓神经功能改善102例(92.8%),无变化4例(3.6%),下降4例(3.6%)。对病变切除前与切除后SEP潜伏期和波幅以及MEP潜伏期进行自身比较,脊髓神经功能改善和下降患者电生理监测指标改变差异有统计学意义(P0.05),脊髓神经功能无变化患者相关监测指标改变差异无统计学意义(P0.05),神经电生理监测指标的变化与术后脊髓神经功能改善情况基本相吻合。结论术中神经电生理监测可以实时了解脊髓神经功能的完整性,结合显微神经外科技术可以明显提高圆锥马尾病变的全切率,减少术后并发症,提高手术疗效及安全性。     

Malignant peripheral nerve sheath tumor of the cauda equina

Only one case of malignant peripheral nerve sheath tumor (MPNST) affecting the cauda equina region has been reported earlier. A 32-year-old male with congenital multiple subcutaneous swellings presented with low back pain, progressive paraparesis and bladder-bowel dysfunction. Magnetic resonance imaging (MRI) demonstrated a heterogenously enhancing intradural lesion at L2–L4. At operation, on opening the dura, multiple nodular, firm matted masses attached to the lumbosacral nerve roots were encountered. Peripheral lesions were partially excised. Histopathological exam revealed varied cellularity with necrosis and pleomorphic nuclei suggestive of MPNST. MRI features, pathophysiological characteristics and the literature are reviewed. Received: 2 February 2001 / Accepted in revised form: 13 June 2001     

Hemangioblastoma of the cauda equina

A very rare hemangioblastoma of the filum terminale with sciatic pain and partial cauda syndrome in a 36-year-old man is presented. The difference between solitary hemangioblastoma, hemangioblastomatosis and von Hippel-Lindau disease is stressed. The literature is reviewed and the diagnosis of cauda equina neoplasms is discussed. Even nowadays often the final diagnosis is made late and only after operation and microscopical tissue examination.     

Neuritis of the cauda equina,a chronic idiopathic polyradiculoneuritis in the horse

Summary Four cases of neuritis of the cauda equina (NCE) were studied by light and electron microscopy. Examination of sacral intradural rootlets revealed inflammatory cell infiltrates and an array of myelinated fiber changes which included myelin stripping by invading mononuclear cells and macrophages, as well as splitting and vesiculation of myelin lamellae without obvious participation by leukocytes. More distally in the extradural roots, there was marked granulomatous inflammation, and demyelinative changes were overshadowed by widespread evidence of irreversible axon damage. In all cases, unusual crystalline inclusions appeared in the endoneurium and sheaths of the rootlets and fascicles. The changes in NCE were compared and contrasted with those observed in acute and chronic inflammatory demyelinative neuropathies.     

Regeneration of cauda equina fibres after transsection and end-to-end suture

Summary The regeneration of nerve fibres in pigs was studied three months after transsection and end-to-end suture of the dorsal and ventral spinal nerve roots L 5 and S 1. Although no detailed quantitative analysis was performed, it was clearly evident that regeneration in motor roots is significantly more efficacious than in sensory roots. Probably as a reaction to Wallerian degeneration, an increase of histiocytes, fibroblasts and collagen fibrils, as well as a thickening of the sheaths was observed in the transsected and sutured roots. Heterotopic glial bundles were found in the regenerated dorsal roots.

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Zusammenfassung Die Regeneration in dorsalen und ventralen Nervenwurzeln der Cauda equina nach Durchtrennung und Naht am Schwein wurde nach dreimonatiger überlebenszeit untersucht. Obwohl keine detaillierten quantitativen Analysen durchgeführt wurden, konnte eindeutig festgestellt werden, da? die Regeneration in motorischen Wurzeln ausgiebiger was als in sensiblen Wurzeln. In den regenerierten Wurzeln wurde eine Zunahme von Histiozyten, Fibroblasten und Collagen sowie eine Verdickung der Wurzelhüllen beobachtet. In regenerierten dorsalen Wurzeln fanden sich heterotope, vom Myelon ausgewachsene Gliabündel.

     

Giant cauda equina schwannoma with dystrophic calcifications : case report and review of the literature

Giant spinal schwannoma of the cauda equine involving many nerve roots is rare, and ossification is usually not observed in the schwannoma. A 21-year-old man presented with a 12-month history of urinary dysfunction and numbness below the buttocks. Plain radiography showed scalloping of the posterior surface of the vertebral bodies from L4 to the sacrum, and magnetic resonance imaging and computed tomography revealed a giant cauda equina tumor with dystrophic calcification. The tumor was completely removed, with intraoperative neurophysiologic monitoring. Histopathologic examination showed that the tumor was a schwannoma. The patient's postoperative course was uneventful, with urinary function and numbness gradually improving. Although a giant schwannoma accompanied by dystrophic calcification is extremely rare, such a tumor can be removed safely and completely by meticulous dissection and careful neuromonitoring of the cauda equina spinal nerves involved in the tumor.     

Sclerosing fibrous tumor of the cauda equina: a fibroblastic variant of peripheral nerve tumors?

Summary The case of a 43-year-old man with an unusual mesenchymal tumor of the cauda equina is presented. A well-circumscribed firm tumor was found in the lower spinal canal at L1 level. Although a nerve root was involved, the adjacent dura mater or filum terminale was unrelated to the tumor. Microscopically, the tumor was rich in collagen and made up of irregularly intertwining fascicles of fibroblastic spindle cells lacking in nuclear atypia or mitotic activity. Partial broad hyalinization of collagen was another histological feature of the tumor. Histological and immunohistochemical studies failed to reveal any findings that suggested known fibrous neoplasms, such as schwannoma, neurofibroma and meningioma, originating in the nervous system. Ultrastructural features of the tumor cells were consistent with those of fibroblasts. Hence, the present tumor is regarded as a unique pure fibroblastic tumor (fibroma) derived from the interstitium of a nerve root in the cauda equina.     

Lesions of the cauda equina: a clinical and pathology review from the Armed Forces Institute of Pathology

We reviewed the clinical records of 149 patients with pathologically proved cauda equina lesions in order to define the relative frequency and clinical presentations of the various diagnoses. The most common pathology was ependymomas (47 patients) followed in frequency by nerve sheath tumors (35 patients), metastases (27 patients), nonependymal glial neoplasms (six patients), meningiomas (six patients), lipomas (five patients), paragangliomas (five patients) and various other diagnoses (19 patients). Mean patient age at presentation for the various lesions included: metastases (51.5 years), nerve sheath tumors (49.7 years), nonependymal glial tumors (46.5 years), paragangliomas (41.2 years), ependymomas (38.3 years), meningiomas (34.7 years), and lipomas (18.4 years). ANOVA showed that the relationship between age and diagnosis for these groups to be statistically significant at a high level (P=0.002). Low back pain was the most common symptom and occurred in 44 patients. Other symptoms included unilateral lower extremity pain or tenderness (24 patients), bilateral lower extremity pain or tenderness (16 patients), and bilateral lower extremity weakness (16 patients). No relationship between pathologic diagnosis and specific symptoms was found.     

Selective hypertrophy of the cauda equina nerve roots

Two patients are described with a previously unreported hypertrophic radiculopathy of the cauda equina with preservation of peripheral nerve function. In one removal of an associated bronchial carcinoid tumour led to marked improvement in the neurological condition suggesting a paraneoplastic cause. The second patient later developed an external ophthalmoplegia but the underlying aetiology remains obscure. Received: 19 February 2001, Received in revised form: 25 July 2001, Accepted: 2 August 2001     

Electrical stimulation and multichannel EMG recording for identification of functional neural tissue during cauda equina surgery

Electrical stimulation of structures within the surgical field was used to identify functional neural elements during 25 cauda equina operations. EMG responses from anterior thigh, posterior thigh, and anal sphincter muscles were recorded simultaneously using a multichannel signal averager. During nine operations, stimulation of a presumed filum terminale or other tissue produced clear EMG responses, prompting modification of surgical procedures. In one patient, this resulted in preservation of a flattened spinal cord which resembled a band of scar tissue. Some EMG responses were restricted to a single muscle group; these neural structures would probably not have been identified if only a single-channel EMG recording was used. Visual examination alone was not adequate for identifying functional neural elements, or for determining whether atretic-appearing nerve roots were functional. Electrical stimulation with multichannel EMG recording facilitates the preservation of functional neural elements and the optimization of surgical results in cauda equina surgery.     

Cerebrospinal fluid dissecting into spinal epidural space after lumbar puncture causing cauda equina syndrome: review of literature and illustrative case

Objects We report a case of epidural cerebrospinal fluid (CSF) leak after lumbar puncture caused by CSF dissecting into the spinal epidural space. The incidence of this phenomenon may be higher than suspected, although most cases may remain asymptomatic. Materials and methods A 4-year-old girl with new-onset seizure underwent a diagnostic lumbar puncture, the results of which were normal; 3 h later, she began experiencing severe low-pressure headaches and lower back pain, bilateral lower extremity weakness, numbness, and pain, and urinary retention when upright. Spinal MRI demonstrated extensive epidural CSF collection posterior to the thecal sac extending from the cervicothoracic junction to the sacrum. After 48 h in the supine position and gradual mobilization, the patient had complete resolution of symptoms and no neurological sequelae. Conclusion Patients usually recover without any neurological deficits after conservative treatment. Prone or lateral decubitus positioning immediately after lumbar puncture may decrease the incidence of this phenomenon.