Clinical characteristics and predictive factors of recurrent idiopathic transverse myelitis.

Background: Idiopathic transverse myelitis (iTM) is defined as an inflammatory myelopathy of undetermined etiology, even after a comprehensive workup to identify other possible causes. Generally, the characteristics of recurrent iTM are not clearly defined. This study aimed to identify the clinical characteristics and predictive factors of recurrence in patients with iTM. Methods: We retrospectively recruited patients with transverse myelitis (TM) who visited Chungnam National University Hospital between January 2011 and December 2021. We included patients who were followed up for at least 2 years and excluded those diagnosed with multiple sclerosis or neuromyelitis optica spectrum disorder (NMOSD) during the initial episode or follow-up period. Patients with iTM were categorized into two groups: monophasic idiopathic TM (mTM) and recurrent idiopathic TM (rTM). We compared the clinical characteristics and spinal magnetic resonance imaging findings between the two groups. Results: In total, 167 patients were reviewed, of whom 112 were excluded. Finally, we included 55 patients with iTM. In 55 patients, 11 (20.0%) and 44 (80%) were classified into the rTM and mTM groups, respectively. Male predominance was observed in the iTM, rTM, and mTM groups. The percentage of patients with low vitamin D levels was significantly higher in the rTM group (100.0%) compared with the mTM group (70%) (p = 0.049). In addition, longitudinally extensive transverse myelitis (LETM) was observed more frequently in the rTM group, in 8 of 11 (72.7%) patients, compared with 15 of 44 (34.1%) patients in the mTM group, with the difference being statistically significant (p = 0.020). In multivariate regression analysis, female sex, younger age at onset, low serum vitamin D level (<30 ng/mL), and LETM were risk factors for recurrence. LETM was a significant predictor of relapse in iTM (p = 0.043, odds ratio = 13.408). Conclusion: In this study, the clinical features of mTM and rTM are nearly indistinguishable. In conclusion, >20% of the patients with iTM experience recurrence, and LETM is the most significant risk factor for recurrence. In cases of recurrence, there is a favorable response to immunotherapy, and the prognosis is generally good. Although LETM may be the initial symptom of NMOSD, it may be manifestation of iTM, and in cases of idiopathic LETM, it is important to be mindful of the elevated risk of recurrence. Based on these results, idiopathic rTM has good clinical prognosis and response to immunosuppressive treatment.

Longitudinally Extensive Transverse Myelitis in a Patient With Systemic Lupus Erythematosus: A Case Report and Literature Review.

Longitudinally extensive transverse myelitis (LETM) is a rare but severe neurological complication of systemic lupus erythematosus (SLE). The existing literature contains only limited information about this condition. We present a case of a 38-year-old female with SLE who presented with quadriparesis. Magnetic resonance imaging (MRI) of the brain and spinal cord showed T2-weighted high signal intensity involving the brainstem, bilateral middle and inferior cerebellar peduncles, and C1-C7 spinal cord segments. Early intervention with high-dose methylprednisolone and cyclophosphamide was initiated, resulting in partial clinical recovery. A comprehensive literature review highlights the importance of early diagnosis and treatment, discusses the potential etiologies, and explores the prognostic factors influencing patient outcomes. This case report underscores the need for a high level of clinical suspicion and prompt therapeutic intervention to improve prognosis in SLE patients presenting with LETM.

Integrated Rehabilitation of Longitudinal Extensive Transverse Myelitis: Study of a Complex Case.

Longitudinal extensive transverse myelitis (LETM) is a rare neurological disorder portrayed by inflammation of the spinal cord spanning three or more vertebral segments. It can lead to severe symptoms such as weakness, sensory abnormalities, and dysfunction in various parts of the body. LETM can be idiopathic or associated with autoimmune conditions like Multiple Sclerosis (MS) and Neuromyelitis Optica (NMO). Diagnosis of LETM requires MRI imaging of the spine, and treatment often involves corticosteroids, intravenous immunoglobulins (IVIG), and plasma exchange. Physical therapy plays a crucial role in managing LETM, focusing on improving functional abilities, mobility, and quality of life. This study outlines a 62-year-old male who was admitted with a complaint of bilateral weakness of both upper and lower limbs, predominantly on the left side, seizures, falls, and stiffness of the left limbs. He also complained of a cough with sputum, cluster headache, bowels and bladder dysfunction, and impaired vision. Neurologic examination showed hypotonia and reduced muscle strength in all four limbs with impairment of the optic nerve. The following investigations were conducted: MRI, Chest X-ray, and ultrasound. He was advised for physiotherapy, after which he showed improvement in functional independence and a general recovery following the treatment.

The real-world applicability of the 2023 international myelin oligodendrocyte glycoprotein antibody-associated disease criteria in a Latin American cohort.

BACKGROUND AND PURPOSE: The diagnostic criteria for myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) were published in 2023. We aimed to determine the performance of the new criteria in Latin American (LATAM) patients compared with the 2018 criteria and explore the significance of MOG-IgG titers in diagnosis. METHODS: We retrospectively reviewed the medical records of LATAM (Argentina, Chile, Brazil, Peru, Ecuador, and Colombia) adult patients with one clinical MOGAD event and MOG-IgG positivity confirmed by cell-based assay. Both 2018 and 2023 MOGAD criteria were applied, calculating diagnostic performance indicators. RESULTS: Among 171 patients (predominantly females, mean age at first attack = 34.1 years, mean disease duration = 4.5 years), 98.2% patients met the 2018 criteria, and of those who did not fulfill diagnostic criteria (n = 3), all tested positive for MOG-IgG (one low-positive and two without reported titer). Additionally, 144 (84.2%) patients met the 2023 criteria, of whom 57 (39.5%) had MOG-IgG+ titer information (19 clearly positive and 38 low-positive), whereas 87 (60.5%) patients had no MOG-IgG titer. All 144 patients met diagnostic supporting criteria. The remaining 27 patients did not meet the 2023 MOGAD criteria due to low MOG-IgG (n = 12) or lack of titer antibody access (n = 15), associated with the absence of supporting criteria. The 2023 MOGAD criteria showed a sensitivity of 86% (95% confidence interval = 0.80-0.91) and specificity of 100% compared to the 2018 criteria. CONCLUSIONS: These findings support the diagnostic utility of the 2023 MOGAD criteria in an LATAM cohort in real-world practice, despite limited access to MOG-IgG titration.

Radiological features in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease-diagnostic criteria and lesion dynamics.

The spectrum of acquired pediatric demyelinating syndromes has been expanding over the past few years, to include myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), as a distinct neuroimmune entity, in addition to pediatric-onset multiple sclerosis (POMS) and aquaporin 4-IgG-seropositive neuromyelitis optica spectrum disorder (AQP4+NMOSD). The 2023 MOGAD diagnostic criteria require supporting clinical or magnetic resonance imaging (MRI) features in patients with low positive myelin oligodendrocyte glycoprotein IgG titers or when the titers are not available, highlighting the diagnostic role of imaging in MOGAD. In this review, we summarize the key diagnostic features in MOGAD, in comparison to POMS and AQP4+NMOSD. We describe the lesion dynamics both during attack and over time. Finally, we propose a guideline on timing of imaging in clinical practice.

A rare case of systemic lupus erythematosus-associated neuromyelitis optica spectrum disorder with cystic lesions and dual seropositivity for anti-AQP4 and anti-MOG antibodies.

Key Clinical Message: In patients with SLE, concurrent NMOSD can manifest with optic neuritis and transverse myelitis. AQP-4 antibody positivity confirms the diagnosis. Prompt treatment is critical to manage the acute symptoms and prevent relapses, as highlighted by a young patient's case with optic neuritis and extensive spinal cord lesions. Abstract: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system that affects the optic nerve and spinal cord. It is associated with autoantibodies against aquaporin-4 (AQP-4) and/or myelin oligodendrocytes glycoproteins. It is diagnosed based on clinical, radiological, and serological criteria, and treated with immunosuppressants in the acute phase. Long-term immunosuppression is essential to prevent potential relapses. In this case report, we present the case of a 19-year-old female patient with systemic lupus erythematosus (SLE), who presented with blurriness and loss of vision in her left eye. Optical coherence tomography was normal, but a gadolinium-enhanced cervico-dorsal MRI showed multiple lesions extending from the brainstem to the C7-T1 junction suggestive of longitudinally extensive transverse myelitis (LETM), the largest of which was a cystic lesion at the cervico-spinal junction. A contrast injection also revealed left optic neuritis. Cerebrospinal fluid analysis showed elevated IgG and red blood cell count, but no oligoclonal bands. The patient tested positive for AQP-4 autoantibodies, confirming the diagnosis of NMOSD. Treatment with intravenous methylprednisolone led to partial improvement, but the patient experienced a relapse with severe neurological symptoms, including tetraplegia and bladder and bowel dysfunction. This case illustrates the importance of considering NMOSD in the differential diagnosis of patients with SLE who present with optic neuritis and/or myelitis, especially when MRI findings are suggestive of LETM. Early diagnosis and adherence to treatment are crucial to prevent further relapses and deleterious sequelae.

S. aureus bacteremia with acute transverse myelitis case report: Utility of molecular techniques.

We report a rare case of bacteremia with concomitant acute transverse myelitis (ATM) without evidence of a primary infectious focus or secondary localization due to Staphylococcus aureus in a 60-year-old man admitted for hyperpyrexia, quadriplegia, and respiratory failure. Bacterial ATM is a rare clinical entity with confusing clinical presentation and challenging diagnosis; isolated bacterial infections of the spinal cord without secondary localization or contiguous foci are exceptionally rare, as is S. aureus as the cause of infection. In this case, a rapid etiologic diagnosis was made possible by close collaboration between clinicians, infectious disease specialists and clinical microbiologists combined with extended molecular testing on CSF guided by incoming results.

Paraneoplastic myelitis associated with Waldenström macroglobulinemia responsive to treatment with ibrutinib - venetoclax: A case report.

Waldenström macroglobulinemia (WM) is a B-cell lymphoproliferative malignancy characterized by IgM paraproteinemia and presence of lymphoplasmacytic cells in the bone marrow. Isolated longitudinally extensive transverse myelitis (LETM) is a rare manifestation of WM. We report a rare case of paraneoplastic LETM in a 68-year-old male with treatment-naïve WM (MYD88 L265P mutation in bone marrow aspirate), who responded to ibrutinib and venetoclax therapy. Our patient presented with a two-month history of unsteadiness, tingling, and numbness in both hands and feet, that progressed to bilateral leg and arm weakness. Based on radiographic findings, a diagnosis of paraneoplastic LETM was made and he was treated acutely with IV methylprednisolone followed by a quick oral prednisone taper. However, he subsequently relapsed and symptomatically worsened while on rituximab therapy. Accounting for worsening anemia, our patient was enrolled in a Phase II trial evaluating the effects of ibrutinib-venetoclax therapy in treatment naïve WM. After three months of study therapy, he had a complete response of myelopathy symptoms and MRI lesions. Our observation of sustained disease response in this patient may support a role for concurrent BTK and BCL2 inhibition in paraneoplastic myelitis associated with B-cell lymphoproliferative disorders. However, this observation needs to be validated in larger cohort studies and potentially in clinical trials if further data are supportive.

A tropical myelitis.

Spinal cord inflammation is a rare presentation of schistosomiasis infection. The present report describes the case of a young patient presenting subacute medullary symptoms revealing extensive longitudinal myelitis related to schistosomiasis, also known as bilharzia. The diagnosis was based on detection of parasite eggs in stool. The patient was treated with Praziquantel, corticosteroids and plasma exchanges, leading to a favorable clinical course.

Acute Transverse Myelitis in a Patient With Type 2 Loeys-Dietz Syndrome: A Report of a Rare Case From India.

Loeys-Dietz syndrome (LDS) is a very rare connective tissue disorder with autosomal dominant inheritance, characterized by the involvement of the cardiovascular, musculoskeletal, and cutaneous systems, along with dysmorphic facial features. Currently, there are limited data regarding this disease. This case presents a clinical observation of a 17-year-old boy with acute onset of sensorimotor paraparesis and genetically confirmed LDS. The predominant symptoms of LDS include arterial aneurysms, arterial tortuosity, hypertelorism, and bifid uvula. However, this constellation of symptoms is not found in all patients with the disease. Genetic confirmation is essential for an accurate diagnosis. The prognosis for LDS differs from its mimics, such as Marfan syndrome, Beals syndrome, Ehlers-Danlos syndrome, and Shprintzen-Goldberg syndrome. Management of the disease warrants a multidisciplinary approach to address its various manifestations. Such an approach can help increase the life expectancy and improve the quality of life for these patients.

Neuromyelitis optica spectrum disorder: Exploring the diverse clinical manifestations and the need for further exploration.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder characterized by inflammatory assaults on the central nervous system (CNS), particularly on the optic nerves and spinal cord. In recent years, a wider range of clinical manifestations of this complex disease have been observed, emphasizing the importance of gaining a more profound understanding beyond optic neuritis (ON) and transverse myelitis (TM). CURRENT KNOWLEDGE: This study explores the many clinical symptoms of NMOSD, including common and uncommon presentations. Distinctive aspects of ON, TM, and diencephalic/brainstem syndromes are examined, highlighting their unique characteristics in contrast to conditions such as multiple sclerosis. We also discuss extra-CNS involvement, such as unusual signs, including muscle involvement, retinal injury, auditory impairment, and rhinological symptoms. AIMS AND OBJECTIVES: Our study intends to highlight the wide range and complexity of NMOSD presentations, emphasizing the significance of identifying unusual symptoms for precise diagnosis and prompt management. The specific processes that contribute to the varied clinical presentation of NMOSD are not well understood despite existing information. This emphasizes the necessity for more study to clarify the mechanisms that cause different symptoms and discover new treatment targets for this complex autoimmune disorder. CONCLUSION: It is essential to acknowledge the complex and varied clinical manifestations of NMOSD to enhance diagnosis, treatment, and patient results. By enhancing our comprehension of the fundamental processes and investigating innovative therapeutic approaches, we may aim to enhance the quality of life for persons impacted by this illness.

Trans-spinal magnetic stimulation combined with kinesiotherapy as a new method for enhancing functional recovery in patients with spinal cord injury due to neuromyelitis optica: a case report.

BACKGROUND: Experimental studies have shown that repetitive trans-spinal magnetic stimulation (TsMS) decreases demyelination and enables recovery after spinal cord injury (SCI). However, the usefulness of TsMS in humans with SCI remains unclear. Therefore, the main objective of this study is to evaluate the effects of TsMS combined with kinesiotherapy on SCI symptoms. We describe a protocol treatment with TsMS and kinesiotherapy in a patient with SCI due to neuromyelitis optica (NMO)-associated transverse myelitis. CASE PRESENTATION: A 23-year-old white male with NMO spectrum disorders started symptoms in 2014 and included lumbar pain evolving into a mild loss of strength and sensitivity in both lower limbs. Five months later, the symptoms improved spontaneously, and there were no sensorimotor deficits. Two years later, in 2016, the symptoms recurred with a total loss of strength and sensitivity in both lower limbs. Initially, physiotherapy was provided in 15 sessions with goals of motor-sensory recovery and improving balance and functional mobility. Subsequently, TsMS (10 Hz, 600 pulses, 20-seconds inter-trains interval, at 90% of resting motor threshold of the paravertebral muscle) was applied at the 10th thoracic vertebral spinous process before physiotherapy in 12 sessions. Outcomes were assessed at three time points: prior to physiotherapy alone (T-1), before the first session of TsMS combined with kinesiotherapy (T0), and after 12 sessions of TsMS combined with kinesiotherapy (T1). The patient showed a 25% improvement in walking independence, a 125% improvement in balance, and an 18.8% improvement in functional mobility. The Patient Global Impression of Change Scale assessed the patient's global impression of change as 'much improved'. CONCLUSION: TsMS combined with kinesiotherapy may safely and effectively improve balance, walking independence, and functional mobility of patients with SCI due to NMO-associated transverse myelitis.

Childhood Transverse Myelitis with Atypical Fever Presentation: A Case Report.

Transverse myelitis (TM) is a rare inflammatory spinal cord disorder, particularly uncommon in children. It is characterized by symptoms such as motor weakness, sensory disturbances, and autonomic dysfunction. This report describes a 10-year-old male presenting with bilateral lower limb weakness, urinary and fecal incontinence, and high-grade fever. Initial treatment at a local hospital with corticosteroids and antibiotics did not yield significant improvements, prompting advanced care at a tertiary facility. A magnetic resonance imaging (MRI) confirmed a longitudinally extensive TM lesion. Subsequent management with plasmapheresis led to satisfactory clinical improvement. This case highlights the importance of early consideration of TM in pediatric patients with acute neurological deficits and supports the use of aggressive therapeutic strategies for better outcomes.

Transverse Myelitis in a 72-Year-Old Male Presenting With Upper Extremity Weakness.

Acute transverse myelitis (TM) is a rare, acquired neuro-immune spinal cord disorder that can be idiopathic or related to a secondary disease. Clinical signs and symptoms include motor weakness, sensory alterations, and bowel or bladder dysfunction. Often TM occurs in the younger population or middle-aged adults. This patient's presentation is unique in the fact that he does not fall into either of these age categories. In this case, a 72-year-old male with a past medical history of hypertension and type 2 diabetes mellitus presented to the emergency department due to a five-day history of worsening weakness of the upper extremities bilaterally. In addition, the patient reported a new onset of abdominal wall numbness. The patient reported being at a theme park a few days prior, denying any injuries and only complaining of neck discomfort during the car ride home. Labs and imaging were quickly ordered for diagnostic purposes. The patient was diagnosed with TM using magnetic resonance imaging (MRI), lumbar puncture, and clinical signs. The etiology was later discovered to be due to a new diagnosis of Sjögren's autoimmune disease. The patient was treated with high-dose intravenous steroids for five days while being monitored for any neurologic changes. The plan was to continue steroids by mouth once discharged from the hospital. Due to poor adherence to discharge instructions, the patient was readmitted after presenting to the emergency department with worsening symptoms. Physicians need to recognize and diagnose TM quickly, as some etiologies are treatable and can prevent further damage to the spinal cord.

Longitudinally Extensive Transverse Myelitis in a Patient With Systemic Lupus Erythematosus, Recent Influenza Infection, and Nutritional Deficiencies in the Setting of Chronic Alcohol Use.

Longitudinally extensive transverse myelitis (LETM) is traditionally classified as an inflammatory disorder of the spinal cord spanning three or more vertebral segments. The differential diagnosis for TM is vast and can include infectious, nutritional, and can even be idiopathic in some reported cases. However, autoimmune etiologies such as systemic lupus erythematosus (SLE) can rarely present with neurological manifestations such as LETM. In this case report, we present a 33-year-old female with a prior history of SLE who developed an LETM in the setting of possible provoking factors such as nutritional deficiencies and a recent viral illness. In this case report, we highlight her clinical course, recovery, and working differential diagnosis after laboratory testing and neurological imaging. Finally, we discuss the different treatments that ultimately lead to her successful recovery after her prolonged clinical course.

Comparison between MRI-negative and positive results and the predictors for a poor prognosis in patients with idiopathic acute transverse myelitis.

BACKGROUND: Idiopathic acute transverse myelitis (IATM) is a focal inflammatory disorder of the spinal cord that results in motor, sensory, and autonomic dysfunction. However, the comparative analysis of MRI-negative and MRI-positive in IATM patients were rarely reported. OBJECTIVES: The purpose of this study was to compare MRI-negative with MRI-positive groups in IATM patients, analyze the predictors for a poor prognosis, thus explore the relationship between MRI-negative and prognosis. METHODS: We selected 132 patients with first-attack IATM at the First Affiliated Hospital of Nanchang University from May 2018 to May 2022. Patients were divided into MRI-positive and MRI-negative group according to whether there were responsible spinal MRI lesions, and good prognosis and poor prognosis based on whether the EDSS score ≥ 4 at follow-up. The predictive factors of poor prognosis in IATM patients was analyzed by logistic regression models. RESULTS: Of the 132 patients, 107 first-attack patients who fulfilled the criteria for IATM were included in the study. We showed that 43 (40%) patients had a negative spinal cord MRI, while 27 (25%) patients were identified as having a poor prognosis (EDSS score at follow-up ≥ 4). Compared with MRI-negative patients, the MRI-positive group was more likely to have back/neck pain, spinal cord shock and poor prognosis, and the EDSS score at follow-up was higher. We also identified three risk factors for a poor outcome: absence of second-line therapies, high EDSS score at nadir and a positive MRI result. CONCLUSIONS: Compared with MRI-negative group, MRI-positive patients were more likely to have back/neck pain, spinal cord shock and poor prognosis, with a higher EDSS score at follow-up. The absence of second-line therapies, high EDSS score at nadir, and a positive MRI were risk factors for poor outcomes in patients with first-attack IATM. MRI-negative patients may have better prognosis, an active second-line immunotherapy for IATM patients may improve clinical outcome.

Transverse Myelitis: A Case-Based Discussion of Infectious Etiologies.

Transverse myelitis (TM) is a frequently encountered inpatient neurological condition, usually with a broad differential of etiologies narrowed down by detailed history, temporal profile of symptom evolution, and pertinent diagnostic studies. We report a rare case of a 39-year-old man who presented with subacute onset of headaches and confusion, and three days later developed quadriplegia and areflexia. He was diagnosed with acute longitudinally extensive transverse myelitis (LETM) related to Epstein-Barr virus (EBV) superimposed on an initial presentation of streptococcal meningitis. As both etiologies are under-reported, we compare our case to the few similar cases in the literature to guide discussion of the clinical and radiologic findings of parainfectious TM related to EBV and streptococcal meningitis. Readers will have the challenge of attributing our patient's myelitis to one of these parainfectious sources and are encouraged to evaluate for rare infectious etiologies in acute settings.

Holocord myelopathy misdiagnosed as neuromyelitis optica spectrum disorder (NMOSD): A unique case of dural arteriovenous fistula at the craniocervical junction along first spinal nerve.

Background: Dural arteriovenous fistulas (DAVFs) at the craniocervical junction (CCJ) involving the first spinal nerve represent a particularly rare and challenging subtype of DAVFs, with holocord myelopathy secondary to cerebrospinal DAVFs being an exceedingly rare presentation. Case Description: We report the case of a 70-year-old woman who presented with progressive paraparesis over 2 weeks. Initial magnetic resonance imaging (MRI) of the spine showed extensive holocord myelopathy, leading to a misdiagnosis of inflammatory myelopathy and subsequent inappropriate steroid treatment at a local hospital, which exacerbated her neurological symptoms. On transfer to our institution and further evaluation with MRI and magnetic resonance angiography, a lower thoracic DAVF was initially suspected. However, comprehensive spinal angiography failed to localize the fistula, prompting cranial angiography, which ultimately identified a DAVF at the CCJ along the C1 nerve root, supplied by a small radiculomeningeal branch of the left vertebral artery. Successful management involved coagulation of the proximal draining vein, with follow-up imaging confirming complete fistula obliteration and resolution of the holocord edema. Conclusion: This case highlights the diagnostic and therapeutic challenges associated with DAVFs at the CCJ, particularly when presenting with holocord myelopathy. It underscores the importance of a high index of suspicion and the need for timely, accurate diagnosis and intervention to prevent permanent spinal cord damage in such rare and complex cases.

Neuropsychiatric Systemic Lupus Erythematosus: A Systematic Review.

Neuropsychiatric systemic lupus erythematosus (NPSLE) refers to the neurological and psychiatric manifestations of systemic lupus erythematosus (SLE), which remain poorly understood yet often have a profound effect on the lives of afflicted patients. The aim of this study is to synthesize the available information on the pathogenesis, diagnostics, management, and prognosis of this disease. Our hope is to increase awareness and call for further investigations that may optimize NPSLE patient outcomes and quality of life. We performed a literature review following the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines, resulting in 11 studies of inclusion. Within each study, we extracted data on epidemiologic factors, diagnostics, therapeutic modalities, and prognosis for each neuropsychiatric condition. The most widely discussed neuropsychiatric manifestations of SLE based on the American College of Rheumatology (ACR) classifications included status epilepticus (SE) and seizures, transverse myelitis (TM), and cognitive dysfunction. SE and TM had a prevalence of 1-2%, while cognitive dysfunction was nearly 38%. Diagnostics varied depending on symptom presentation but often included brain magnetic resonance imaging (MRI) and antibody testing. Treatment for NPSLE is still widely understudied, but concurrent treatment with immunosuppressants and anti-inflammatories for symptom control and more targeted immunotherapies based on the specific condition is often effective. Prognosis is highly symptom dependent, ranging from a 12.5% one-year mortality in SE and seizure patients to near resolution of symptoms in certain presentations including idiopathic intracranial hypertension and cerebellar ataxia. Further studies are needed to better understand the pathophysiology, diagnostics, and effective therapeutic measures for NPSLE. The severity of these manifestations and generally poor prognosis highlight the need for more research to accurately diagnose and treat this disease. While there is still little data available, this literature review serves to provide updated context on this condition.