Histopathology of the filum terminale in children with and without tethered cord syndrome with attention to the elastic tissue within the filum

Purpose

To compare histologically transected fila from pediatric patients with tethered cord syndrome (TCS), with and without a low conus, with controls, focusing on collagenous and elastic tissue.

Methods

Thirty fila from patients with TCS, including 5 where minimal cautery was used prior to filum section, were compared with fila from 27 pediatric cadavers without TCS (controls). Sections of fila were stained with H&E, Masson trichrome and Verhoeff von Gieson elastic stains, and 7 with Gordon and Sweet’s reticulin stain.

Results

Fila from controls showed loose fibrous connective tissue (FCT) with thin and evenly dispersed elastic fibers (EFs). Reticulin fibers (RFs) were seen in blood vessel walls and nerve twigs. Fat was identified microscopically in 2 fila.All fila from patients with TCS had dense FCT. The EFs were in normal numbers in 17, and focally or diffusely decreased in 13. All 25 patients where the fila were cauterized during resection had thick and coiled EFs. Coiling was not seen when minimal cautery was applied. RFs were seen in blood vessel walls and nerve twigs. Fat was identified in 19 patients. Findings were similar, whether the conus termination was normal or low.

Conclusion

The fila of all patients with TCS, whether or not the conus was low, showed abnormal FCT. EFs were decreased in 48 % of patients; however, there were thick and coiled EFs in all patients. Coiling of EFs, initially thought to be an abnormality in patients, is considered most likely to be a result of cautery (i.e., artifactual/iatrogenic coiling).

     

Spinal cord and cauda equina compression in 'DISH'.

Diffuse idiopathic skeletal hyperostosis (DISH) has long been regarded as a benign asymptomatic clinical entity with an innocuous clinical course. Precise information is lacking in the world literature. Authors report the results of a retrospective analysis of 74 cases of DISH. Eleven patients presented with progressive spinal cord or cauda equina compression. In nine cases ossified posterior longitudinal ligament (OPLL) and in two cases ossified ligamentum flavum (OLF) were primarily responsible. Surgically treated patients (eight) had far better outcome as compared to the patients managed conservatively, as they had refused surgery. 'DISH' is neither a benign condition, nor it always runs a innocuous clinical course. In fact, in about 15% of the cases, serious neurological manifestations occur, which may require a major neurosurgical intervention.     

脊髓圆锥马尾区畸胎瘤合并终丝牵张型脊髓拴系综合征的手术治疗

目的 探讨脊髓圆锥马尾区畸胎瘤合并终丝牵张型脊髓拴系综合征（TCS）的临床特点、手术方法及其疗效。方法 回顾性分析2007年2月～2022年3月手术治疗的50例脊髓圆锥马尾区畸胎瘤合并终丝牵张型TCS的临床资料。结果 畸胎瘤内容物及囊性部分内壁剥离切除45例,囊壁次全切除加电灼5例;50例终丝均分离、切断。术后病理均为成熟囊性畸胎瘤及内终丝结构。术后随访6个月～14.5年,中位数75个月,按Hoffman脊髓功能评分,脊髓功能状态好转27例,不变22例,恶化1例;1例畸胎瘤内膜次全切除术后复发再次手术,其余49例未见肿瘤复发,无再拴系。结论 椎管内畸胎瘤多分布于脊髓圆锥马尾区,以慢性神经压迫为表现;对合并终丝牵张型TCS,一期行畸胎瘤切除术及终丝切断脊髓栓系松解术,疗效满意。     

Acute vertebral collapse and cauda equina compression in tertiary syphilis.

A patient with observed acute collapse of a lumbar vertebral body developed cauda equina compression. He was known to have contracted syphilis some 20 years before and, while he may well have suffered from tabetic spinal neuroarthropathy, histology of the collapsed vertebra showed features which indicate that an intra-osseous gumma could also have been responsible for his vertebral collapse and subsequent neurological deficit.     

Post-hemorrhagic hydrocephalus presenting as cauda equina syndrome in a patient with spinal dysraphism

Hydrocephalus has varied presentations in patients with a history of spinal dysraphism. This is a unique case of post-subarachnoid hemorrhage hydrocephalus presenting as cauda equina syndrome. We report on a 32-year-old woman with remotely repaired spinal defect who experienced subarachnoid hemorrhage and underwent anterior communicating artery aneurysm clipping. Post-operatively, she developed urinary and fecal incontinence as the sole presenting symptom of communicating post-hemorrhagic hydrocephalus. New neurological deficits in this population can also be attributed to recurrent cord tethering or syrinx, both of which were demonstrated on her lumbar spine MRI, but her incontinence resolved with external ventricular drain placement and cerebrospinal fluid diversion. There are few case reports of patients with closed neural tube defects and hydrocephalus and none in the adult population to our knowledge. Neurological change in patients with any history of spinal dysraphism may reflect altered cerebrospinal fluid dynamics affecting either end of the neuraxis.     

Intraventricular bleeding and transient hydrocephalus in a patient with hemorrhagic filum terminale ependymoma undergoing anticoagulation therapy with phenprocoumon

The authors discuss a rare case of hemorrhage in a filum terminale ependymoma presenting with acute paraparesis and transient hydrocephalic dementia in association with long-term phenprocoumon anticoagulation.     

Urinary incontinence in a patient with Duchenne muscular dystrophy and cord in the normal position with fatty filum terminale

Introduction To our knowledge, muscular dystrophy and specifically, Duchenne muscular dystrophy, has not previously been associated with gross intrathecal abnormalities.Case report We report a pediatric patient with Duchenne muscular dystrophy that was diagnosed a long time ago and recent onset urinary incontinence. MRI revealed a conus medullaris in a normal position with a fatty-infiltrated filum terminale. Operative intervention was performed entailing sectioning of the filum terminale. This resulted in resolution of the childs urinary incontinence.Conclusion Although seemingly rare and perhaps associated by serendipity, new, unusual neurological deficits in a patient with muscular dystrophy, especially of an upper motor neuron nature, should warrant MRI to rule out pathology of the spinal cord such as tethering.     

Cystic mature teratoma of the filum terminale in an adult. Case report and review of the literature

Intradural spinal teratoma is a very rare entity, more prevalent in childhood, that may associate with dysraphic defects. The authors report a 46 years old man with a cauda equina syndrome and an L1-2 tumor. An L1-2 laminectomy was performed and a mass was resected at the base of the filum terminale, which was in contact with the conus medullaris. Histopathological diagnosis was of cystic mature teratoma. Spinal teratomas may be found anywhere along the spine, but are more frequent in the cauda equina. We discuss the origin of these tumors and review the literature concerning these lesions.     

Leptomeningeal gliomatosis with spinal cord or cauda equina compression: a complication of supratentorial gliomas in adults

Seven patients with supratentorial gliomas developed leptomeningeal gliomatosis (LMG) without symptomatic recurrence at the primary tumor site. In all, severe back and radicular pain, often simulating disc disease, preceded the development of spinal cord or cauda equina dysfunction. In 4 instances, intracranial hypertension due to hydrocephalus developed prior to spinal involvement. Cytological examination of the CSF revealed malignant cells in only 2/7 but a myelogram was diagnostic in all 7. All patients received spinal irradiation (RT) and 5 received chemotherapy. Two patients with low-grade gliomas improved transiently; 5 with malignant gliomas responded poorly, became paraplegic over 4 months and eventually died of LMG. When fatal LMG occurs in young adults suffering from supratentorial glioma, the primary tumor is often quiescent. Hydrocephalus is often the first manifestation of LMG and, when it is detected, a myelogram and CSF cytology study should be performed in the hope that diagnosis and treatment of spinal cord lesion at a very early stage will prove beneficial. Irradiation of the entire spinal canal is probably required as there is a high risk of rapid development of new lesions in non irradiated segments of the spinal canal.     

Primary CNS lymphoma: lymphomatous meningitis presenting as a cauda equina lesion in an AIDS patient

Neurologic complications of the acquired immune deficiency syndrome (AIDS) most often present as brain dysfunction and/or a polyneuropathy. We describe a unique neurological problem of a woman with AIDS who presented with a cauda equina mass due to primary CNS lymphoma. She subsequently developed a fulminant lymphomatous meningitis and died. Although previously rare, primary spinal cord lymphoma is expected to be encountered more often with the current AIDS epidemic and may be difficult to distinguish from infectious mass lesions.     

Nitric oxide in cerebrospinal fluid and local inducible nitric oxide synthase after cauda equina compression in rats

We investigated the time course of changes in nitric oxide metabolite (NO2- plus NO3-: NOx) levels in the cerebrospinal fluid and the expression of local inducible nitric oxide synthase following cauda equina compression in rats. Cerebrospinal fluid NOx levels were significantly increased from 12 h to 3 days after compression, and decreased thereafter. Histologically, inducible nitric oxide synthase immunoreactivity was observed in macrophages that infiltrated the dura mater on days 1 and 3 after compression, but not in foamy macrophages in the parenchyma of the cauda equina observed afterwards. The pattern of NOx levels coincided with the appearance of inducible nitric oxide synthase labeled macrophages, indicating a critical role of these cells as the main synthesizers of NOx in the acute stage of cauda equina compression.     

Primary epidural lumbar Ewing sarcoma presenting as cauda equina syndrome in an adult patient who underwent surgical decompression: A case report

Ewing sarcoma (ES) is a rare primary neoplasm in the lumbar adult spine and may mimic a benign tumor. In this case, after a patient's three-month history of lower back pain and rapidly progressing leg numbness and weakness, magnetic resonance imaging (MRI) showed a mass in the third lumbar vertebra. At a two-month follow-up, imaging showed a fracture, compression and lesion enlargement. Decompression and fixation confirmed ES, and the patient began combined radiotherapy and chemotherapy. Two months postoperatively, residual ES was suspected on MRI. The patient underwent a second surgery, and histopathology confirmed necrosis. A six-month follow-up after the first surgery showed no tumor recurrence. This case supports the inclusion of ES in the differential diagnosis of pathologic spinal fracture. Early decompression and spinal fixation are critical for preserving neurologic and spinal functions in ES complicated by a compression fracture. Combined adjuvant radiotherapy and chemotherapy remain the standard therapeutic strategy.     

Bilateral chronic subdural hematoma in a young adult mimicking subarachnoid hemorrhage]

A 33-year-old man was admitted to our hospital with a sudden severe headache five days after the onset CT scan showed a slight high-density area in the basal cistern, mimicking subarachnoid hemorrhage (SAH), and diffuse brain swelling. However, conventional cerebral angiography and CT angiography failed to demonstrate aneurysms and vascular malformations. MRI showed bilateral subdural hematoma, but no SAH. Irrigation of liquefied subdural hematoma, causing high intracranial pressure, was carried out. Postoperative course was uneventful and his headache resolved within a day. The author presented a case of bilateral chronic subdural hematoma who presented with a sudden severe headache mimicking a SAH. Hyper attenuation in the basal cistern and subarachnoid space in CT, don't always indicate SAH. MRI, including fluid-attenuated inversion recovery (FLAIR) sequences, is useful in differentiating the "pseudo" SAH from "true" SAH, and lead to the right diagnosis.     

Ossified ligamentum flavum causing spinal cord compression in a patient with acromegaly

Acromegaly is a relatively rare neuroendocrine disorder associated with diffuse hypertrophy of bony and soft tissues due to growth hormone hypersecretion from a pituitary adenoma. Acromegaly can also cause numerous pathological changes in the spine, including degenerative osteoarticular disease, axial arthropathy, spinal stenosis, vertebral fracture and diffuse idiopathic skeletal hyperostosis (Forestier’s disease). Ossified ligamentum flavum (OLF) is a rare disorder that often presents as thoracic spinal stenosis, but to our knowledge has never been described in patients with acromegaly. Previously, no link has been established between these two entities. We present, to our knowledge, the first reported case of OLF in a patient with acromegaly who presented with thoracic spinal cord compression. OLF is a potential spinal manifestation of acromegaly and should be considered in the differential diagnosis of spinal stenosis or spinal cord compression in the context of growth hormone hypersecretion.     

Sexual function in men with cauda equina lesions: a clinical and electromyographic study

OBJECTIVE: To investigate the effects of cauda equina lesions on sexual function in men. METHODS: Sexual function was investigated in 46 men with long standing cauda equina/conus medullaris lesions. All had clinical and radiological findings supporting the diagnosis. The validated Slovene translation of the international index of erectile function (IIEF) was used. The responses were scored and sexual dysfunction categorised as absent, mild, moderate, or severe. The number of patients receiving help for sexual dysfunction was noted. Neurological examination of the trunk and lower limbs, electromyographic (EMG) evaluation of the sacral reflex, and quantitative EMG of the external anal sphincter muscles were done. RESULTS: Severe sexual dysfunction was reported by 35% of patients, moderate dysfunction by 24%, and slight dysfunction by 26%; normal sexual function was reported by 15%. Orgasmic function was slightly more impaired than erectile function, and sexual desire slightly less. The patients' age, but no findings on clinical neurological or EMG examination, correlated with sexual function. Only five men had received medical attention for sexual dysfunction. CONCLUSIONS: There is significant sexual impairment in men with lesions of the cauda equina or conus medullaris. This is poorly correlated with neurological and EMG findings and has received insufficient medical attention.     

Massive reduction of tumour load and normalisation of hyperprolactinaemia after high dose cabergoline in metastasised prolactinoma causing thoracic syringomyelia

In 1970 a 20 year old woman presented with a pituitary chromophobe adenoma for which she underwent transfrontal pituitary surgery. In 1978 she had to be reoperated on because of local tumour recurrence, resulting in hypopituitarism. Bromocriptine (5 mg/day) was given for 15 years, but the plasma prolactin levels remained elevated. In 2000 the patient presented with signs and symptoms suggestive of a spinal cord lesion at the mid-thoracic level. A magnetic resonance imaging (MRI) scan showed an extensive leptomeningeal mass extending from the brainstem to L5, with a thoracic syringomyelia at the T7-T8 level. The plasma prolactin level was very high (5114 microg/l). A biopsy showed the presence of a metastasised prolactinoma.On administration of high dose cabergoline, 0.5 mg twice a day orally, the plasma prolactin levels decreased within one month and then normalised within 26 months. Tumour load reduced considerably but unfortunately, her signs and symptoms did not improve. This case illustrates that a high dose dopamine agonist might be an important therapeutic option in patients with a metastasised prolactinoma.