An Aberrant Case of Neuromyelitis Optica Spectrum Disorder With a Review of Literature.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare, acquired demyelinating condition predominantly affecting middle-aged women and is characterized by spinal cord inflammation and optic neuritis. Anti-aquaporin 4 (AQP4) antibodies are typically seen in NMOSD. However, myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) shares clinical and imaging similarities. In NMOSD, longitudinally extensive spinal cord lesions (LESCLs), optic neuritis predominantly affecting the posterior aspect of optic nerves, and optic radiations are seen on magnetic resonance imaging (MRI). The brain parenchymal lesions particularly involve the dorsal medulla (area postrema). The report presents a case of a 26-year-old female with recurrent episodes of weakness, pain, and sensory symptoms in both upper and lower limbs who was initially treated for multiple sclerosis. Upon experiencing new symptoms of blurred vision and ataxia, an MRI of the spine and brain was performed, which showed short-segment cervical cord involvement and a lesion in the conus medullaris, raising the suspicion of NMOSD. Subsequent antibody testing confirmed the presence of anti-AQP4 antibodies. While the involvement of the conus medullaris is classically associated with MOGAD, unusual findings in the present case highlight the importance of comprehensive imaging evaluation and raising awareness among clinicians and radiologists regarding the imaging spectrum of NMOSD, thus facilitating timely diagnosis and tailored treatment strategies.

Neurophysiological findings in conus medullaris infarction-a case report and systematic review of the literature.

INTRODUCTION: Conus medullaris infarction (CMI) is a rare vascular phenomenon that has been scarcely reported in the literature. While previous studies have described the clinical and radiological features of CMI, little attention has been paid to its associated neurophysiological findings. METHODS: We present a case of idiopathic CMI and its neurophysiological findings, then present our findings from a systematic review of other reports of CMI with neurophysiological features found via PubMed search. RESULTS: Nine articles describing ten cases of CMI with associated neurophysiological data were found, in addition to our case. Out of all 11 cases, 7 cases (64%) had absent F-waves on the first nerve conduction study (NCS) performed as early as 4 h after onset, 5 of whom demonstrated reappearance of F-waves on subsequent follow-up NCS. Seven patients (64%) had diminished compound muscle action potentials (CMAPs), which was usually detectable on NCS performed between day 8 and day 18 of onset. None of them showed recovery of CMAPs in follow-up studies. Four patients (36%) had absent H-reflexes and two patients (18%) had sensory abnormalities. Electromyography (EMG) was reported in seven patients (64%), showing reduced recruitment as early as day 1 of onset, and denervation potentials as early as 4 weeks after onset. CONCLUSION: Absent F-waves and diminished CMAPs are the most common NCS abnormalities in CMI. Absent F-waves are detectable very early but tend to recover on subsequent NCS, while diminished CMAPs are detectable later but do not seem to resolve. Further research to determine the utility of neurophysiological studies in CMI diagnosis and prognostication is needed.

Case report: A cystic capillary hemangioma located at the conus medullaris mimicking hemangioblastoma.

Capillary hemangiomas, usually found in skin and mucosal tissues, are rarely encountered within the spinal cord, presenting a significant diagnostic challenge. We report a rare case of intradural extramedullary capillary hemangioma at the conus medullaris in a 66-year-old female patient. Our initial diagnosis leaned towards a cystic hemangioblastoma based on MRI findings due to the presence of cystic formation with an enhanced mural nodule. However, surgical exploration and subsequent pathological examination revealed the lesion as a capillary hemangioma. To the authors' knowledge, this case may represent the first documented instance of a spinal capillary hemangioma that mimics a cystic hemangioblastoma.

Cauda equina, conus medullaris and syndromes mimicking sciatic pain: WFNS spine committee recommendations.

Introduction: Cauda equina syndrome (CES), conus medullaris syndrome (CMS), and sciatica-like syndromes or "sciatica mimics" (SM) may present as diagnostic and/or therapeutic dilemmas for the practicing spine surgeon. There is considerable controversy regarding the appropriate definition and diagnosis of these entities, as well as indications for and timing of surgery. Our goal is to formulate the most current, evidence-based recommendations for the definition, diagnosis, and management of CES, CMS, and SM syndromes. Methods: We performed a systematic literature search in PubMed from 2012 to 2022 using the keywords "cauda equina syndrome", "conus medullaris syndrome", "sciatica", and "sciatica mimics". Standardized screening criteria yielded a total of 43 manuscripts, whose data was summarized and presented at two international consensus meetings of the World Federation of Neurosurgical Societies (WFNS) Spine Committee. Utilizing the Delphi method, we generated seven final consensus statements. Results and conclusion: s: We provide standardized definitions of cauda equina, cauda equina syndrome, conus medullaris, and conus medullaris syndrome. We advocate for the use of the Lavy et al classification system to categorize different types of CES, and recommend urgent MRI in all patients with suspected CES (CESS), considering the low sensitivity of clinical examination in excluding CES. Surgical decompression for CES and CMS is recommended within 48 h, preferably within less than 24 h. There is no data regarding the role of steroids in acute CES or CMS. The treating physician should be cognizant of a variety of other pathologies that may mimic sciatica, including piriformis syndrome, and how to manage these.

Disseminated Tuberculosis Revealed by Conus Medullaris Tuberculoma.

A 29-year-old man from Comoros presented with rapidly progressive paraplegia and sexual dysfunction. Magnetic resonance imaging (MRI) showed a contrast-enhanced conus medullaris lesion. Differential diagnoses included tumors, abscesses, and inflammatory diseases. Neurosurgery was delayed to complete examinations. Cerebral MRI showed three abscesses. Body computed tomography scan showed supracentimetric polyadenopathies, pulmonary nodules, prostatic lesion, and enhanced seminal vesicle, with hypermetabolism on positron emission tomography with 2-deoxy-2-[fluorine-18]fluoro-D-glucose scan. Histology of lymph node biopsy showed granulomatous infiltration without acid-fast bacilli, and positive polymerase chain reaction for Mycobacterium tuberculosis. Lymph node culture was positive after 2 months, urine culture after 3 weeks, but cerebrospinal fluid and sputum cultures were negative. A 1-year antituberculosis therapy was initiated, associated with corticosteroids because the patient developed tuberculosis-immune reconstitution syndrome, revealed by the recurrence of neurological symptoms. After 2 months the patient completely recovered and could run. MRI showed stability of the voluminous tuberculoma with decrease of medullary edema. Avoiding surgery in those cases may prevent iatrogenic neurological deterioration.

Survival and neurological outcomes following management of intramedullary spinal metastasis patients: a case series with comprehensive review of the literature.

Intramedullary spinal cord metastasis (ISCM), though rare, represents a potentially debilitating manifestation of systemic cancer. With emerging advances in cancer care, ISCMs are increasingly being encountered in clinical practice. Herein, we describe one of the larger retrospective single institutional case series on ISCMs, analyze survival and treatment outcomes, and review the literature. All surgically evaluated ISCMs at our institution between 2005 and 2023 were retrospectively reviewed. Demographics, tumor features, treatment, and clinical outcome characteristics were collected. Neurological function was quantified via the Frankel grade and the McCormick score (MCS). The pre- and post-operative Karnofsky performance scores (KPS) were used to assess functional status. Descriptive statistics, univariate analysis, log-rank test, and the Kaplan-Meier survival analysis were performed. A total of 9 patients were included (median age 67 years (range, 26-71); 6 were male). Thoracic and cervical spinal segments were most affected (4 patients each). Six patients (75%) underwent surgical management (1 biopsy and 5 resections), and 3 cases underwent chemoradiation only. Post-operatively, 2 patients had an improvement in their neurological exam with one patient becoming ambulatory after surgery; three patients maintained their neurological exam, and 1 had a decline. There was no statistically significant difference in the pre- and post-operative MCS and median KPS scores in surgically treated patients. Median OS after ISCM diagnosis was 7 months. Absence of brain metastasis, tumor histology (renal and melanoma), cervical/thoracic location, and post-op KPS ≥ 70 showed a trend toward improved overall survival. The incidence of ISCM is increasing, and earlier diagnosis and treatment are considered key for the preservation of neurological function. When patient characteristics are favorable, surgical resection of ISCM can be considered in patients with rapidly progressive neurological deficits. Surgical treatment was not associated with an improvement in overall survival in patients with ISCMs.

An Easy and Effective Method for Evaluating the Position of Conus Medullaris: Counting the Number of Vertebral Ossification Center Below the End of Conus Medullaris.

OBJECTIVE: This study aimed to ascertain the conus medullaris position by counting the number of ossification centers in the vertebral bodies below the conus medullaris endpoint (N) and assess its utility in screening for closed spinal dysraphism and tethered cord syndrome. METHODS: A total of 900 normal fetuses and 146 fetuses with closed spinal dysraphism or tethered cord syndrome were included in this study. The N values were tallied and compared along the spinal longitudinal plane. The receiver operating characteristic curve was utilized, and the cut-off value of N was analyzed. RESULTS: The counting of N was successfully performed in 856 normal and 146 abnormal fetuses. In the normal group, an increase in N with gestational age was observed. Specifically, in the subgroup of 17-20 wk fetuses, N was ≥6 in 117 out of 131 cases. This figure increased to 211 out of 213 in 21-24 wk and 512 out of 512 in 25-41 wk, respectively. Cases with N ≥7 accounted for 715 out of 856 fetuses in the 17-41 wk range. In the abnormal group, N was less than 7 in 152 out of 163 fetuses, showing statistical differences between the two groups. With a cut-off value of 6.5, specificity and sensitivity reached 93.3% and 83.5%. CONCLUSIONS: The counting of N was found to be a straightforward and efficient method for evaluating the position of the conus medullaris.

Recurrent Acute Disseminated Encephalomyelitis Presenting as Conus Medullaris Syndrome: A Case Report.

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that typically follows an infection or recent vaccination. Symptoms such as encephalopathy and focal neurological deficits appear weeks after the initial illness, leading to swift and progressive neurological decline. While ADEM in the brain has been well documented, reports of ADEM, specifically in the spinal cord, are relatively limited. A 58-year-old male presented with rapidly progressive bilateral lower extremity tingling, numbness, and mild gait disturbance approximately two days prior to visiting the emergency room. Spinal magnetic resonance imaging revealed a diffuse, longitudinal, high-signal lesion with mild enlargement of the conus and proximal cauda equina. The lesions were predominantly localized in the distal conus and cauda equina, and serial electrodiagnostic studies showed that the lesions progressed toward the proximal conus in tandem with symptom evolution and lacked clear lateralization. The patient was subsequently treated with high-dose steroids for seven days (intravenous methylprednisolone, 1 mg/kg). The patient's lower extremity weakness gradually improved and he was able to walk independently under supervision three weeks after symptom onset. In this case of spinal ADEM in a middle-aged adult, high-dose steroid treatment led to outstanding neurological recovery from both the initial occurrence and subsequent attacks.

Toward the End of the Funnel: The Ventriculus Terminalis-The State of Art of an Ancient Entity with a Recent History.

PURPOSE: The ventriculus terminalis (VT), also called the fifth ventricle, is a small cavity containing cerebrospinal fluid (CSF) that is in the conus medullaris in continuity with the central canal of the spinal cord. In adults, persistent VT is a very rare entity, and the diagnosis is incidental in most cases. Rarely, VT may become symptomatic for still-uncertain reasons but most often for its cystic dilatation. The management of these selected cases is still controversial and sometimes associated with unsatisfactory outcomes. METHODS: We performed a critical review of the existing literature on the management of symptomatic VT in adults. The etiology, pathophysiology, and treatment of VT are presented and discussed, focusing on the best timing for surgery. RESULTS: Conservative management, marsupialization, or the placement of a T drain have been reported. The existing classifications describe the most correct approach for each clinical presentation, but scarce importance has been given to the delay from symptoms' onset to surgical treatment. CONCLUSION: Although different cases have been described in the literature, this rare pathology remains unknown to most neurosurgeons.

Cystic Dilatation of the Ventriculus Terminalis: Examining the Relevance of the Revised Operative Classification Through a Systematic Review of the Literature, 2011-2021.

The literature features limited evidence on the natural history of the cystic dilatation of the ventriculus terminalis (CDVT) and its response to treatment. The goal of this study is to ascertain which impact the revised operative classification of CDVT had on the management of patients diagnosed over the past 10 years.Ten new clinical articles presenting a total of 30 cases of CDVT were identified and included for qualitative analysis. Two take-home messages can be identified: (1) Adequate consideration should be given to designing national pathways for referral to tertiary centers with relevant expertise in the management of lesions of the conus medullaris, and (2) we suggest that type Ia should be, at least initially, treated conservatively, whereas we reckon that the signs and symptoms described in types Ib, II, and III seem to benefit, although in some patients only partially, from surgical decompression in the form of cystic fenestration, cyst-subarachnoid shunting, or both.While the level of evidence gathered in this systematic review remains low because the literature on CDVT consists only of retrospective studies based on single-center series (level of evidence 4 according to the Oxford Centre for Evidence-Based Medicine (OCEBM)), the strength of recommendation for adopting the revised operative classification of CDVT is moderate.

High-Riding Conus Medullaris Syndrome: A Case Report and Literature Review-Its Comparison with Cauda Equina Syndrome.

INTRODUCTION: Conus medullaris syndrome (CMS) is a distinctive spinal cord injury (SCI), which presents with varying degrees of upper motor neuron signs (UMNS) and lower motor neuron signs (LMNS). Herein, we present a case with a burst fracture injury at the proximal Conus Medullaris (CM). CASE PRESENTATION: A 48-year-old Taiwanese male presenting with lower back pain and paraparesis was having difficulty standing independently after a traumatic fall. An Imaging survey showed an incomplete D burst fracture of the T12 vertebra. Posterior decompression surgery was subsequently performed. However, spasticity and back pain persisted for four months after surgical intervention. Follow-up imaging with single photon emission computed tomography (SPECT) and a whole body bone scan both showed an increased uptake in the T12 vertebra. CONCLUSION: The high-riding injury site for CMS is related to a more exclusive clinical representation of UMNS. Our case's persistent UMNS and scintigraphy findings during follow-up showcase the prolonged recovery period of a UMN injury. In conclusion, our study provides a different perspective on approaching follow-up for CM injuries, namely using scientigraphy techniques to confirm localization of persistent injury during the course of post-operative rehabilitation. Furthermore, we also offered a new technique for analyzing the location of lumbosacral injuries, and that is to measure the location of the injury relative to the tip of the CM. This, along with clinical neurological examination, assesses the extent to which the UMN is involved in patients with CMS, and is possibly a notable predictive tool for clinicians for the regeneration time frame and functional outcome of patients with lumbosacral injuries in the future.

Neurological Analysis Based on the Terminal End of the Spinal Cord and the Narrowest Level of Injured Spine in Thoracolumbar Spinal Injuries.

This study aimed to clarify neurological differences among the epiconus, conus medullaris, and cauda equina syndromes. Eighty-seven patients who underwent surgery for acute thoracolumbar spinal injuries were assessed. We defined the epiconus as the region from the terminal end of the spinal cord to the proximal 1.0 to 2.25 vertebral bodies, the conus medullaris as the region proximal to < 1.0 vertebral bodies, and the cauda equina as the distal part of the nerve roots originating from the spinal cord. On the basis of the distance from the terminal end of the spinal cord to the narrowest level of the spinal canal, the narrowest levels were ordered as follows: the epiconus followed by the conus medullaris and cauda equina. The narrowest levels were the epiconus in 22 patients, conus medullaris in 37 patients, and cauda equina in 25 patients. On admission, significantly more patients had a narrowed epiconus of Frankel grades A-C than a narrowed cauda equina. At the final follow-up, there were no significant differences in neurological recovery among those with epiconus, conus medullaris, or cauda equina syndrome. Anatomically classifying the narrowest lesion is useful for clarifying the differences and similarities among these three syndromes.

Ultrasound-based horizontal ranging in the localization of fetal conus medullaris.

BACKGROUND: Currently, there are a variety of methods for ultrasound to localize the conus medullaris. A concern is that measured values can be influenced by variations in spinal flexion and extension. OBJECTIVE: To overcome this limitation, the present study measures the horizontal distance (HD) between the end of the conus medullaris and the caudal edge of last vertebral body ossification in normal fetus at different gestational weeks, and analyzes the relationship between the measured value and fetal growth, as well as the utility of these measurements in assessing the position of the conus medullaris. METHODS: A total of 655 fetuses at gestational weeks 18-40, who underwent routine prenatal ultrasound, were selected in the study. We measured the distance between the end of the cone of the fetal spinal cord and the caudal end of the final vertebral ossification center (Distance1, D1), the distance between the end of the spinal cord cone and the intersection of the extension of D1 with the caudal skin (Distance2, D2), and HD. We analyzed the correlation between the measurements and gestational weeks, established normal reference values, the ratio of D1, D2 and HD to the commonly used growth parameters was calculated. The ratios of D1, D2, HD and the application value of each ratio phase were analyzed, and the reliability analysis of repeated measurement results among physicians was performed. RESULTS: D1, D2 and HD exhibited strong linear correlations with gestational weeks. Among the ratios of D1, D2 and HD to common growth parameters, D2/FL stabilized after 20 weeks of gestation and consistently exceeded 1. Repeatability tests between D1, D2 and HD showed good reliability (P> 0.05). CONCLUSION: D1, D2 and HD are significantly correlated with gestational age. Horizontal distance measurement can effectively determine the position of fetal conus medullaris, enabling rapid prenatal evaluation of low position of conus medullaris and excluding the possibility of tethered cord.

Tethered Cord Syndrome: Role of Imaging Findings in Surgical Decision-Making.

For infants presenting with urinary problems or lower extremity weakness, imaging is ordered to investigate spinal pathology. Tethered cord syndrome (TCS) often manifests without conclusive anatomic evidence. In our case, a premature infant presented with urosepsis and was found to have an asymmetric gluteal crease and a sacral dimple. Renal ultrasound showed mild hydronephrosis, and a cystourethrogram revealed bilateral high-grade vesicoureteral reflux. Ultrasound and magnetic resonance imaging demonstrated a borderline low-lying spinal cord at the mid-L3 vertebral level. Urodynamic testing to confirm neurogenic bladder could not be completed on the first attempt due to urinary tract infection and on the second attempt due to instrument intolerance. Despite the lack of conclusive imaging evidence of a tethered cord, enough supportive clinical data was present to proceed with surgical intervention with the goal of preventing the progression of neurological dysfunction. Because TCS is ultimately a clinical diagnosis, appropriate management should not be discouraged by inconclusive or borderline imaging findings.

Non-sedated fast spine magnetic resonance imaging in pediatric patients.

BACKGROUND: Traditional spine magnetic resonance imaging (MRI) protocols require sedation in young children and uncooperative patients. There is an increased interest in non-sedated pediatric MRI protocols to reduce risks associated with anesthetic agents and improve MRI access. OBJECTIVE: To evaluate the image quality of pediatric non-sedated fast spine MRI. MATERIALS AND METHODS: We retrospectively reviewed 69 pediatric non-sedated fast spine MRI exams performed in 57 patients. Two blinded readers provided image quality ratings for the evaluation of bones, cranio-cervical junction, cerebrospinal fluid (CSF) spaces, spinal cord, soft tissues, ligaments, and overall diagnostic quality on a 1-5 scale, and determined whether there was evidence of syringomyelia, abnormal conus medullaris position, or filum terminale abnormality. RESULTS: Mean patient age was 7.2 years (age range ≤ 1-17). Indications included syringomyelia (n=25), spinal dysraphism (n=4), combination of both syringomyelia and spinal dysraphism (n=8), and other miscellaneous indications (n=32). The inter-observer agreement ranged between moderate and very good for each variable (Cohen's weighted kappa] range=0.45-0.69). The highest image quality ratings were given to CSF spaces (mean image quality=3.5/5 ± 0.8) and cranio-cervical junction evaluations (3.5/5 ± 0.9). Overall diagnostic quality was worst in the <5 years group (P=0.006). Readers independently identified a cervical spinal cord syrinx in 6 cases, and 1 mm spinal cord central canal dilation in one case. Readers agreed on the position of the conus medullaris in 92% of cases (23/25 cases). CONCLUSION: Non-sedated pediatric spine MRI can be an effective diagnostic test to evaluate for spine pathology, especially syringomyelia, Chiari malformation, and conus medullaris anatomy.

Intramedullary Epidermoid Cyst of the Conus Medullaris: A Case Report and Literature Review.

Background: Central nervous system tumors are usually located in the brain, and spinal cord tumors account for approximately 20% of central nervous system tumors. Epidermoid cysts constitute <1% of all intraspinal tumors. It consists of squamous epithelial-lined cysts containing keratin, cholesterol, and cellular granules. Epidermoid cysts can be classified as congenital, acquired, extradural, extramedullary, or intramedullary according to etiology and location. The intradural intramedullary type is uncommon. Case Presentation: An 11-year-old female patient had back pain with radiation to both lower extremities but worsened on the left side for 5 years. On neurological examination, motor strength was 3/5 for hip flexion and knee extension bilaterally and 5/5 for other key muscle groups. Contrast-enhanced lumbosacral magnetic resonance imaging (MRI) revealed T1 hypointense and T2 hyperintense lesions in the L2-L4 intramedullary conus and cauda equina. Laminectomy and near total resection were done, and histopathological examination revealed an epidermoid cyst. On the third postoperative day, the patient was discharged with completely resolved back pain and an improvement in lower extremity motor power. At monthly follow-up visits for a further 6 months, her back pain and weakness completely resolved, and she had no neurologic deficits. A postoperative lumbosacral MRI was done and confirmed near total excision of the tumor. Conclusion: Intramedullary conus epidermoid cysts are rare but not unknown to neurosurgeons. MRI with diffusion-weighted images (DWI) is an imaging modality of choice. Asymptomatic patients can be conservatively treated. Once the patient has progressive symptoms and signs of compression, surgical excision is recommended. Meticulous electrocauterization may help decrease tumor regrowth in the remnant capsule, which is recommended. Avoiding leakage of cyst fluid into the subarachnoid space helps to avoid postoperative chemical meningitis. Radiotherapy is an option for the management of multiple recurrences.

Mature teratoma of conus medullaris: A case report and review of literature.

In conus medullaris, mature teratomas are rare. We report a case of a 40-year-old man who presented with urinary incontinence, low back pain, and muscle weakness. Magnetic resonance imaging revealed a mass in conus medullaris (T11-L1), further confirmed as a mature teratoma by pathological examination. We identified 63 cases of conus medullaris teratoma over the past two decades by systematically analyzing the case reports. Findings demonstrated that most cases were diagnosed in the fourth decade of life, with the majority of cases (57.6%) being male. Lower back pain, radiating pain in the extremities, hypoesthesia, and urinary dysfunction are the most common clinical presentations among patients with teratoma of conus medullaris. Mature teratoma is the dominant pathologic subtype of teratomas in this region, comprising more than 95% of cases. Our case highlights the importance of considering spinal teratoma as a differential diagnosis in patients presenting with urinary incontinence and lumbar pain.

Progressive conus medullaris lesions are suggestive of intravascular large B-cell lymphoma.

BACKGROUND AND PURPOSE: Spinal cord lesions are observed in 40% of all central nervous system lesions in intravascular large B-cell lymphoma (IVLBCL). However, because IVLBCL is a very rare disease, its clinical features are not well defined, which may delay appropriate diagnosis and treatment, whilst the acute to subacute course of brain lesions in patients with IVLBCL is well established. Therefore, this study aimed to clarify the clinical features of spinal cord lesions in patients with IVLBCL. METHODS: The medical records of patients with IVLBCL admitted to our hospital between 2010 and 2020 were searched. The inclusion criteria were preceding neurological symptoms without non-neurological symptoms and pathologically confirmed IVLBCL in various organs. Clinical features of spinal cord involvement in patients with IVLBCL were assessed and distinguished from those of brain involvement. RESULTS: Sixteen consecutive patients with IVLBCL were divided into two groups: six patients with spinal involvement (spinal cord type) and 10 patients with brain involvement (brain type). In the spinal cord type, four patients had chronic progression and two had subacute progression. Acute progression (0% vs. 80.0%) and sudden onset (0% vs. 50.0%) occurred significantly less frequently in the spinal cord than in the brain. All spinal cord lesions involved the conus medullaris. CONCLUSIONS: Spinal cord involvement in IVLBCL has a predominantly chronic progressive course that is exclusive to brain involvement. Conus medullaris lesions are suggestive of IVLBCL and are useful for early and accurate diagnosis and treatment.

Intramedullary Tuberculoma of the Conus Medularis in an Immunocompetent Young Adult with no Pulmonary Tuberculosis, the Challenges of Diagnosis and Management: A Case Report and Review of Literature.

Background and Objective: Intramedullary tuberculoma (IMT) of the conus medullaris is an extremely rare tumour that constitutes a diagnostic and management challenge in a resource-limited setting. We report a case of conus medullaris, IMT in a young immunocompetent, patient with no prior clinical features of pulmonary or extra-pulmonary tuberculosis. Case Summary: The patient presented with six months history of progressive and persistent mid back pain and slight weakness of both lower limbs of 3 months duration. Physical examination revealed a well-nourished man with power of 3/5 and hyperreflexia on both lower limbs. Chest radiograph and other investigations for tuberculosis were negative. Magnetic resonance imaging (MRI) of the lumbosacral spine showed fusiform expansion of the conus medullaris, with a well circumscribed, ring enhancing, intramedullary mass straddle between T12 and L1. Patient had gross total resection with no intraoperative monitoring assistance and no post-operative worsening of neurological function. Histology showed granulomatous lesion with central caseation in keeping with a tuberculoma. Patient was commenced on post-operative anti-tuberculous therapy with physiotherapy, with full motor recovery at six months post-surgical intervention and anti-tuberculous therapy. Conclusion: Intramedullary tuberculoma can be considered as one of the differential diagnoses of intradural, intramedullary tumour of the conus, even in immunocompetent individual with no clinical features of tuberculosis.

Diffuse midline glioma, H3K27-altered, of the conus medullaris presenting as acute urinary retention: illustrative case.

BACKGROUND: Diffuse midline glioma (DMG), H3K27-altered, is a rare, highly malignant central nervous system neoplasm that arises in midline structures. They are more commonly encountered in children and are rarely encountered in adults, usually in the thalamus or spinal cord. The presence of the H3K27 mutation in the H3F3A gene automatically classifies a tumor as World Health Organization grade IV. These tumors carry a grim prognosis, with an overall median survival of less than 1 year. OBSERVATIONS: The authors report the case of a 38-year-old male presenting with acute-onset urinary retention who was found to have an expansile, well-circumscribed mass involving the conus medullaris at the level of T12-L1. A T12-L1 laminectomy and tumor debulking were performed. Pathology revealed glial cells with astrocytic morphology among Rosenthal fibers, microvascular proliferation, and cellular atypia. The H3K27 mutation was confirmed. LESSONS: DMG, H3K27-altered, is a rarely encountered entity that can present in numerous midline structures. If localized to the conus medullaris, it may present as acute-onset urinary retention in a previously asymptomatic patient. Further investigation is needed to characterize its molecular and clinical features in adults to improve the management of those presenting with these tumors.