Intramedullary lesion resection as an effective treatment of spinal cord microscopic polyangiitis: a case report.

BACKGROUND: Microscopic polyangiitis (MPA), an autoimmune disease, is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The lungs and kidneys are the most common targets, whereas spinal cord involvement is rare. METHODS: We reported the clinical manifestations, diagnosis, and management of a patient with spinal cord MPA. RESULTS: The patient showed spinal compression symptoms and was diagnosed with MPA following magnetic resonance imaging (MRI) and histological examination. Spinal compression symptoms were completely relieved after intramedullary lesion resection and postoperative treatment with methylprednisolone. CONCLUSION: The diagnosis of MPA without typical manifestations can be challenging. MRI and histological examination are of great importance in spinal cord MPA diagnosis. Intramedullary lesion resection is an effective treatment for spinal cord MPA. Methylprednisolone is also recommended for postoperative treatment.

Spinal Cavernoma in an Infant: A Rare Pathological Entity with an Atypical Presentation.

INTRODUCTION: Cavernous malformation (CM) of the pediatric spine is uncommon, and its presentation especially in infancy is extremely rare. We report an unusual case of thoracolumbar intramedullary CM with hemorrhage in a 5-month-old male child. CASE PRESENTATION: The child presented with the predominant symptom of urinary retention, and the underlying neurological cause was initially overlooked. However, magnetic resonance imaging obtained after the onset of progressive limb weakness revealed a D11-L1 intramedullary lesion with features of intralesional bleed. Intraoperatively, the lesion showed evidence of hemorrhage and was completely excised. The final histopathology confirmed a cavernoma. CONCLUSION: Although rare, spinal CM can present with bleed in very young children. It is imperative to identify the subtle clinico-radiological findings and suspect such lesions, as an early treatment portends a good outcome.

Disseminated spinal myxopapillary ependymoma in an adult at initial presentation: a case report and review of the literature.

Disseminated spinal myxopapillary ependymoma (MPE) is extremely rare in adults. We report a 63-year-old man with chronic low-back pain found to have multiple MPEs in the thoracic, lumbar and sacral spine. Diagnostic and management strategies of disseminated MPE are discussed with a review of pertinent literature.

Surgical management of intramedullary cervical spinal sarcoidosis complicated by transient unilateral weakness: A case report.

Background: Sarcoidosis, a multisystem inflammatory non-caseating granulomatous disease, can present with neurologic lesions in up to 10% of patients. Case Description: A 57-year-old male presented with three months of worsening upper extremity radicular pain associated with dysmetria, hyperreflexia, bilateral Hoffman's, and positive Babinski signs. The contrast magnetic resonance imaging (MRI) showed a diffuse T2 signal hyperintensity and T1-enhancing 2.5 cm lesion extending sagittally between C4 and C6. The cerebrospinal fluid analysis showed a high protein level and lymphocytic pleocytosis. A cardiac positron emission tomography scan was consistent with the diagnosis of cardiac sarcoidosis. With the diagnosis of multisystemic/probable neurosarcoidosis, the patient was unsuccessfully treated with intravenous methylprednisolone, followed by infliximab. Due to severe cord compression/myelopathy, a C3-C6 laminectomy and C3-C7 posterior spinal fusion were performed. Postoperatively, the patient developed a transient right-sided hemiparesis. Over nine postoperative months, the patient had four relapses of transient repeated episodes of paresis, although follow-up cervical MRI scans revealed adequate cord decompression with a stable intramedullary hyperintense lesion. Conclusion: Patients with neurosarcoidosis respond unpredictably to surgical decompression and require prolonged medical care, which is often unsuccessful.

Abdominal Pain Mimicking a Neurological Disorder: A Case Report of Spinal Cavernous Malformation in a Pediatric Patient.

We present a case of a 13-year-old boy with abdominal pain initially misdiagnosed as gastrointestinal in origin. Despite initial outpatient management, his symptoms rapidly deteriorated, revealing a central-medullary cavernous malformation causing spinal cord compression. This case underscores the importance of a comprehensive pediatric examination and highlights new treatment approaches for spinal cavernous malformations.

Intradural extramedullary meningeal melanocytoma: a case report and literature review.

Primary meningeal melanocytomas are extremely rare, benign tumours arising from the leptomeninges. While they are considered to be benign lesions, there is potential for their growth and transformation into malignant melanomas. They are commonly found in the cervical spine, with a decreased incidence in the thoracic and lumbar regions. We present a case report of a 56-year-old man who presented to our unit with a 4-month history of lower limb weakness and a sensory level at T6. Magnetic resonance imaging shows an intradural extramedullary tumour. The patient underwent a thoracic debulking of the lesion with neurophysiological monitoring. Histopathology confirmed the diagnosis of melanocytoma of meningeal origin, with a low mitotic count. Our patient recovered well post-operatively with no complications. Surgical resection is an effective method to manage this tumour; however, adjuvant radiotherapy is advised due to the risk of recurrence and malignant transformation.

Long-Term Recurrent Intramedullary Arachnoid Cyst: Case Report and Literature Review.

Objectives This article reports the management of a case of a 32-year-old male who presented with progressive weakness in the lower limbs and spastic paraparesis secondary to an intramedullary arachnoid cyst (IMAC). For literature review, the authors used the phrase "intramedullary arachnoid cyst" in PubMed search engine. 23 articles describing cases with IMAC were included in this review, with a total of 26 patients. Materials and Methods We report a case with long term recurrant intramedullary arachnoid cyst and present a review on spinal intramedullary arachnoid cyst. Result IMAC is showing bimodal incidence and trending to occur below 10 years and after 30 years. However, rarely, it should be considered in the differential diagnosis of intramedullary cystic lesions. Authors suggest doing laminoplasty or fusion for the pediatric patients to prevent kyphoscoliosis deformity in the long run, but doing early surgery to gain better outcome. Resection of the cyst wall should be done as much as possible; if it could not be achieved, then marsupialization or cysto-subarachnoid shunt should be considered. Aspiration alone or fenestration is not enough to eradicate the cyst. Long-term and prospective studies are recommended to achieve the best treatment options. Conclusion Review supports early surgical treatment of symptomatic IMACs with resection of the cyst wall as much as possible.

Utility of Flexion and Extension MRI for Evaluating Isolated Cervical Spinal Cord Lesions: A Case Series.

The diagnosis of isolated spinal cord lesions is often challenging in clinical practice, and it is not uncommon for the etiology of such isolated lesions to remain unclear despite extensive workup and investigations. Magnetic resonance imaging (MRI) is extensively utilized for assessing spinal cord disease, despite certain radiological patterns suggesting certain pathologies, diagnostic uncertainty remains. Development of adjunct tests and techniques, radiographic or otherwise, is needed. Here, we present two cases in which flexion-extension cervical spine MRIs improved diagnostic ability by demonstrating dynamic cervical cord compression as an etiology for isolated intramedullary cervical spinal cord lesions.

Clinical and Imaging Characteristics of Non-Neoplastic Spinal Lesions: A Comparative Study with Intramedullary Tumors.

The features of non-neoplastic lesions are often similar to those of intramedullary tumors, and a differential diagnosis is challenging to obtain in some cases. A surgical biopsy, which is performed on highly invasive tumors, should be avoided in cases of non-neoplastic lesions. Therefore, an accurate diagnosis is important prior to treatment. We evaluated 43 patients suspected of having spinal cord tumors and, finally, were diagnosed with non-neoplastic intramedullary lesions via magnetic resonance imaging. The patients commonly presented with myelitis. The patients with non-neoplastic neurological diseases had a significantly shorter symptom duration than those with intramedullary astrocytomas. The proportion of patients with non-neoplastic neurological diseases who presented with lesions at the cervical spinal level and focal lesions on axial imaging but without a spinal cord enlargement was significantly higher than that of patients with intramedullary astrocytomas. The current study aimed to distinguish spinal cord tumors from non-neoplastic intramedullary lesions based on their distinct features.

Mycoplasma pneumoniae and Schistosoma mansoni co-infection in a young patient with extensive longitudinal acute transverse myelitis.

INTRODUCTION: Acute transverse myelitis is an uncommon inflammatory, intramedullary, disorder of the spinal cord. Spastic paraplegia, impaired sphincter functions, and sensory loss, with sensory level, are the clinical manifestations of this devastating disorder. The utilization of magnetic resonant imaging (MRI) contributes to the surge in the diagnosis of more ATM cases. Although the causes of ATM are numerous, both Mycoplasma pneumoniae and Schistosoma mansoni are uncommon causes and their co-existence in the same patient has not been reported before in Saudi Arabia. CASE: We report a 25-year-old ATM male patient presented with a history of sudden onset severe low back pain. Within four hours from the onset of the back pain, he became completely paraplegic with impaired functions of the bowel and urinary bladder sphincter. Furthermore, he lost all modalities of sensory functions in the lower limbs. His examination revealed spastic complete paraplegia with sensory level at T6. Clinical neurological examination revealed normal upper limbs and brain functions. The MRI of the cervico-dorsal spine showed extensive longitudinal hyperintense lesion extending from the upper cervical segments to the lower dorsal segments (extensive longitudinal transverse myelitis). A post-infectious immune-mediated predisposition was highly suspected due to the very high titers of anti-Mycoplasma pneumoniae IgM and IgG that were detected. The immunosuppressant therapy did not improve his paraplegia. A spinal cord biopsy revealed the presence of several Schistosoma mansoni ova surrounded by chronic inflammatory reactions and reactive gliosis. CONCLUSIONS: Both Mycoplasma pneumoniae and Schistosoma mansoni should be investigated in cases with extensive longitudinal ATM.

Distinguishing Intramedullary Spinal Cord Neoplasms from Non-Neoplastic Conditions by Analyzing the Classic Signs on MRI in the Era of AI.

Intramedullary lesions can be challenging to diagnose, given the wide range of possible pathologies. Each lesion has unique clinical and imaging features, which are best evaluated using magnetic resonance imaging. Radiological imaging is unique with rich, descriptive patterns and classic signs-which are often metaphorical. In this review, we present a collection of classic MRI signs, ranging from neoplastic to non-neoplastic lesions, within the spinal cord. The Differential Diagnosis (DD) of intramedullary lesions can be narrowed down by careful analysis of the classic signs and patterns of involvement in the spinal cord. Furthermore, the signs are illustrated memorably with emphasis on the pathophysiology, mimics, and pitfalls. Artificial Intelligence (AI) algorithms, particularly deep learning, have made remarkable progress in image recognition tasks. The classic signs and related illustrations can enhance a pattern recognition approach in diagnostic radiology. Deep learning can potentially be designed to distinguish neoplastic from non-neoplastic processes by pattern recognition of the classic MRI signs.

Early expansive single sided laminoplasty decompression treatment severe traumatic cervical spinal cord injury.

Background: Severe traumatic cervical spinal cord injury (tcSCI) is a disastrous event for patients and families. Maximizing spinal cord function recovery has become the primary therapeutic goal. This study investigated the effect of early extensive posterior decompression on spinal cord function improvement after severe tcSCI. Methods: A retrospective review of 83 consecutive patients who underwent extensive open-door laminoplasty decompression within 24 h after severe tcSCI (American Spinal Injury Association (ASIA) impairment scale (AIS) grade A to C) between 2009 and 2017 at our institution was performed. The patient clinical and demographic data were collected. Neurological functional recovery was evaluated according to the Japanese Orthopaedic Association (JOA) score system, ASIA motor score (AMS) and AIS grade. Results: Among the 83 patients initially included, the baseline AIS grade was A in 12, B in 28, and C in 43. Twenty-three patients (27.7%) had a high cervical injury. Cervical spinal stenosis (CSS) was identified in 37 patients (44.6%). The mean intramedullary lesion length was 59.6 ± 20.4 mm preoperatively and 34.2 ± 13.3 mm postoperatively (p < 0.0001). At the final follow-up visit, an improvement of at least one and two AIS grades was found in 75 (90.4%) and 41 (49.4%) patients, respectively. 24 (64.9%) patients with an improvement of least two AIS grades had CSS. The mean AMS and JOA score were significantly improved at discharge and the final follow-up visit compared with on admission (p < 0.0001). Conclusions: Our results suggest that early expansive laminoplasty decompression may improve neurological outcomes after severe tcSCI, especially in patients with CSS. Larger and prospective controlled studies are needed to validate these findings.

Spinal intramedullary tuberculoma in a 3-year-old girl.

BACKGROUND: Spinal intramedullary tuberculoma (IMT) is a rare manifestation of extrapulmonary tuberculosis (TB). Presentation of TB in the pediatric age group is a significant contributor to mortality. CASE DESCRIPTION: A young vaccinated girl presented to the neurosurgery department with difficulty walking and urinary incontinence. A magnetic resonance imaging performed outside the hospital showed a hyperintense intramedullary lesion extending from T6 to T9. The patient underwent T6-T9 laminoplasty with intramedullary lesion decompression under neuromonitoring. The dense adherence of the lesion to the cord and nerve roots permitted only debulking. Histopathological examination confirmed the diagnosis of tuberculoma. The patient was started on antitubercular treatment and was then subsequently discharged. After 8 months, the patient was reviewed and showed improvement in her symptoms and complete resolution of the lesion on imaging. The patient has now developed hydrocephalus on the latest computed tomography imaging, which may be due to tubercular meningitis or arachnoiditis. CONCLUSION: Complete resolution of spinal IMT is possible with a combined treatment approach.

The Central Dot Sign : A Specific Post-gadolinium Enhancement Feature of Intramedullary Spinal Cord Metastases.

PURPOSE: Peripheral enhancement characteristics on magnetic resonance imaging (MRI), namely the rim and flame signs, are specific for intramedullary spinal cord metastases (ISCM) compared to primary cord masses. The study compared the frequency of a novel finding-the central dot sign-in ISCMs versus primary intramedullary masses. METHODS: In this study 45 patients with 64 ISCMs and 64 control patients with 64 primary intramedullary cord masses were investigated and 2 radiologists blinded to lesion type independently evaluated MR images for the presence of a central dot sign: a punctate focus of enhancement in/near the center of an enhancing intramedullary mass. The frequency of this sign in the two patient groups was compared. RESULTS: A total of 63 enhancing ISCMs in 44 patients and 54 enhancing primary cord masses in 54 patients were included. The central dot sign was identified in 6% (4/63) of enhancing ISCMs in 9% (4/44) of patients and in none (0/54) of the enhancing primary cord masses (p = 0.038, per patient). The specificity for diagnosing ISCMs among spinal cord masses was 100%. The central dot sign was present in the axial plane only in two ISCMs and in the axial and sagittal planes in two ISCMs. The two ISCMs harboring the central dot sign also demonstrated both the previously described rim and flame signs, and two also demonstrated the rim sign alone. CONCLUSION: The central dot sign is not sensitive but highly specific for ISCMs compared to primary spinal cord masses. The rim and/or flame signs may or may not be concurrently present in ISCMs.

Surfer Myelopathy in Children: A Case Series Study.

OBJECTIVE: The cause of surfer myelopathy remains enigmatic and long-term follow-up outcomes are not well documented. In the present study, the mechanisms underlying surfer myelopathy in children are analyzed and the long-term follow-up outcomes are reported. METHODS: Clinical data from 3 institutions were retrospectively analyzed. Patients were assessed using the American Spinal Injury Association Impairment Scale (AIS) on admission and at follow-up. The mechanisms were studied by analyzing patients' medical history, magnetic resonance imaging, and magnetic resonance angiography. The prognosis of long-term follow-up was summarized. RESULTS: Thirty-one children were diagnosed with surfer myelopathy. Intramedullary high-intensity T2 signal from mid to lower thoracic level to conus was found during the acute stage. Follow-up magnetic resonance imaging in the subacute stage showed cranial progression of the T2 hyperintensity up to 1-10 vertebral segments, and no neurologic deterioration was found. Intramedullary lesion length between the complete and incomplete injury was significantly different (P < 0.01) in the subacute phase. Flow voids around nerve roots and in the epidural space were detected in 18 patients and 15 patients, respectively, on axial T2-weighted imaging. Enlarged tortuous veins were found in 3 of 6 patients who underwent spinal magnetic resonance angiography, which were discontinuous around nerve root. During long-term follow-up, no patients with AIS grade A recovered, and atrophic cord was observed in the later stage in 14 patients. Patients with incomplete injury had different recoveries. CONCLUSIONS: Surfer myelopathy in children is caused by spinal venous hypertension. The AIS grade on admission is a predictor of prognosis.

Intramedullary spinal cord involvement: a rare presentation of sarcoidosis.

Sarcoidosis is a systemic granulomatous disease of uncertain etiology, which predominantly affects the lungs, eyes, lymph nodes, and skin. Nervous system involvement (neurosarcoidosis) occurs in approximately 5-10% of patients, but spinal cord involvement is very rare, affecting <1% of patients. We present a rare case of intramedullary sarcoidosis.

Dorsal Spinal Intradural Intramedullary Epidermoid Cyst: A Rare Case Report and Review of Literature.

Epidermoid cysts are commonly seen intracranial lesions but their occurrence in the spine is rare. They account for <1% of all the benign tumors of the spine. These are benign epithelial-lined cysts filled with keratin. They are classified into two types: congenital or acquired. Congenital epidermoid cysts are more commonly associated with spinal dysraphic states such as syringomyelia, dermal sinus and spina bifida whereas the acquired cysts are associated with repeated lumbar punctures. Based on the location, they can be extradural, intradural, extramedullary, or intramedullary. Most of the epidermoids are intradural extramedullary. Intramedullary epidermoid cysts are very uncommon. We report a case of a 6-year-old female patient with dorsal epidermoid cyst with neurological deficits. Magnetic resonance imaging of the spine showed a well-defined lesion from D9 to D12 which was hypointense on T1W1 and heterogeneously hyperintense on T2W2. Surgery was performed to excise the lesion and to decompress the spinal cord. Histopathological examination of the excised lesion confirmed it as an epidermoid cyst.

Focal Intramedullary Spinal Cord Lesion in Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome: Toxoplasmosis Versus Lymphoma.

BACKGROUND: Neurologic complications are common in patients with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS). Although both the central nervous system (CNS) and the peripheral nervous system can be affected, 80% of patients with HIV/AIDS have CNS involvement during the course of their illness. The brain is the primary site of HIV/AIDS-associated CNS complications. Spinal cord involvement is rare, particularly focal intramedullary spinal cord lesions without any associated cerebral lesions. Among various opportunistic infections and malignancies, toxoplasmosis and CNS lymphoma are the most common causes of focal neurologic disease in patients with HIV/AIDS. Distinguishing between toxoplasmosis and CNS lymphoma is challenging, as the diseases have similar clinical presentations. CASE DESCRIPTION: In a woman with newly diagnosed HIV infection, myelopathy manifested as an isolated, single intramedullary spinal cord lesion. CONCLUSIONS: Common methods to distinguish the diagnoses of toxoplasmosis and CNS lymphoma are addressed. There should be a high index of suspicion for toxoplasmosis in patients with HIV/AIDS presenting with a focal intramedullary spinal cord lesion.

Identifying myelopathy caused by thoracic syringomyelia: a case report.

Myelopathy is a form of neurological disease caused by compression of the spinal cord. Upper and lower quarter screens are commonly used in identifying myelopathy, although most of the screen components demonstrate poor or unstudied diagnostic value. The purpose of this case report is to describe the diagnostic process in detecting syringomyelia, an intramedullary lesion that may cause myelopathy. The patient was a 47-year-old female with a thoracic syrinx that was discovered by spinal magnetic resonance imaging (MRI) following a complicated and delayed clinical diagnostic course. Following surgical intervention and a two-week inpatient rehabilitation stay, the patient was discharged using a rolling walker for ambulation and was performing most transfers with modified independence. A complicating pattern of signs and symptoms combined with a diagnostic process guided by poorly studied screen components demonstrates the diagnostic dilemma associated with identifying the cause of myelopathy within the thoracic spine. This also indicates the need for further investigation of individual and clustered components of the neurological screen to improve the ability to identify patients in need of complete imaging studies in a more timely fashion.