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.

An atypical case of acute transverse myelitis following COVID-19 infection.

ABSTRACT: Acute transverse myelitis is a rare condition that can follow a viral infection. At least 43 cases of COVID-19 associated with acute transverse myelitis have been presented in the literature. This case review highlights one such case in a young man. Although rare, acute transverse myelitis following COVID-19 can cause significant disability for patients. Clinicians must be equipped and encouraged to report and study cases of neurologic complications following COVID-19 infection to develop further treatment and cure.

Clinical Profile and Outcome of Pediatric Demyelinating Disorders in Calabar, Nigeria: A Case Series.

BACKGROUND: Demyelinating disorders of the central nervous system (CNS) are rare disorders characterized by inflammation and the selective destruction of CNS myelin. The incidence of this disorder is increasing in developed countries. Nigerian studies on the pediatric population on the subject are very scarce. AIMS: The aim of the study was to document the epidemiology, clinical profile, and impact of late presentation on the treatment outcome of demyelinating diseases of the CNS in pediatric patients. METHODS: The retrospective review of patients aged 1-15 years admitted in a tertiary hospital from January 2018 to December 2022 with various symptoms suggestive of demyelinating CNS disorders. The diagnosis was clinically and radiologically confirmed. Information retrieved from the case notes included patients' demographics, clinical symptoms and signs, number of days with symptoms to presentation in the hospital, results of the magnetic resonance imaging (MRI), treatment, and treatment outcomes. Data were entered in Excel sheet and results were presented in tables and percentages. RESULTS: The incidence of demyelinating disorders over the period was 0.013% (10 out of 769 patients admitted over the period). Acute demyelinating encephalomyelitis (ADEM) was the most common disorder seen in the study population (60%, n = 6), followed by transverse myelitis and two (20%) had optic neuritis (ON). Most of the patients with ADEM were in the 1-5-year age group. The female-to-male ratio was 2.3:1. Paraplegia, visual impairment, and ataxia were the most common clinical presentations in the study population. One of the patients met the criteria for the diagnosis of multiple sclerosis during follow-up. Human immunodeficiency virus (HIV) was identified as the cause of demyelination in one case. Most of the patients improved with steroids. CONCLUSION: ADEM was the most common clinical phenotype seen in this study. Patients with ADEM and ON had a better prognosis than transverse myelitis. Late presentation was also identified as a poor prognostic factor. Follow-up of cases is very important to monitor disease progression to multiple sclerosis.

Transverse myelitis associated with systemic lupus erythematosus (SLE-TM): A review article.

Systemic lupus erythematosus-related transverse myelitis (SLE-TM) is a rare but serious complication of SLE, which may result in significant morbidity. Its incidence is estimated between 0.5% and 1% of all SLE patients but may be the presenting feature in 30%-60% of these patients. Unfortunately, due to lack of high-quality studies, data regarding this condition remains limited. Its pathogenesis remains largely unknown and clinical presentation is variable. There are still no set guidelines regarding diagnosis, management, or monitoring and the role of autoantibodies remains controversial. In this review, we aim to summarize the available data regarding the epidemiology, pathogenesis, clinical features, management, and prognosis of this rare disease.

Comparison of acute flaccid myelitis and transverse myelitis in children and evaluation of diagnostic criteria.

BACKGROUND AND PURPOSE: Acute flaccid myelitis (AFM) and transverse myelitis (TM) are serious conditions that may be difficult to differentiate, especially at onset of disease. In this study, we compared clinical features of pediatric AFM and TM and evaluated current diagnostic criteria, aiming to improve early and accurate diagnosis. METHODS: Two cohorts of children with enterovirus D68-associated AFM and clinically diagnosed TM were compared regarding presenting clinical features, additional investigations, and outcome. Current diagnostic criteria for AFM and TM were applied to evaluate their specificity. RESULTS: Children with AFM (n = 21) compared to those with TM (n = 36) were younger (median 3 vs. 10 years), more often had a prodromal illness (100% vs. 39%), predominant proximal weakness (69% vs. 17%), and hyporeflexia (100% vs. 44%), and less often had sensory deficits (0% vs. 81%), bowel and/or bladder dysfunction (12% vs. 69%), and hyperreflexia (0% vs. 44%). On magnetic resonance imaging, brainstem involvement was more common in AFM (74% vs. 21%), whereas supratentorial abnormalities were only seen in TM (0% vs. 40%). When omitting the criterion of a sensory level, 11 of 15 (73%) children with AFM fulfilled the diagnostic criteria for TM. Of children with TM, four of 33 (12%) fulfilled the diagnostic criteria for probable/definite AFM. CONCLUSIONS: Although there is considerable overlap between AFM and TM in children, we found important early differentiating clinical and diagnostic features. Meeting diagnostic criteria for AFM in children with TM and vice versa underlines the importance of thorough clinical examination and early and accurate diagnostic studies.

Primary intramedullary spinal cord lymphoma misdiagnosed as longitudinally extensive transverse myelitis: a case report and literature review.

BACKGROUND: Primary intramedullary spinal cord lymphoma (PISCL) is rare and easily misdiagnosed with the lack of typical clinical features and non-specific imaging manifestations. CASE PRESENTATION: A 49-year-old man was admitted to our hospital because of persistent limbs numbness, pinprick-like pain in the posterior neck and unsteady gaits. He has brisk tendon reflexes and positive Babinski's sign. Magnetic resonance imaging (MRI) of the cervical spine showed an abnormal signal with aberrant reinforcement at medulla oblongata and the level of C1-C7. He was clinically diagnosed as longitudinally extensive transverse myelitis (antibody-negative). Steroid pulse therapy was administered and resulted in reduced symptoms. One month later, his situation was exacerbated compared to the onset. We launched a new cascade of steroid pulse therapy. But it did not improve his symptoms. Finally, the biopsy pathology confirmed PISCL. Chemotherapy, radiotherapy and zanubrutinib were administered and until now about 3 years into treatment the patient is still survival. CONCLUSIONS: Based on our case and literature review, we recommend that spinal onset patients react ineffectively to standard immunoglobulins or hormonal treatments or experience a relapse after a short time relief should take PISCL into consideration.

Extensive longitudinal acute transverse myelitis complicated by pulseless ventricular tachycardia and recent shingles vaccination.

This case describes a 50-year-old male with a history of psoriatic arthritis who presented to the emergency department with a chief complaint of ascending bilateral lower extremity paresthesia one week following a shingles vaccine. MRI of the patient's spine was significant for longitudinally extensive T2 hyperintensity involving the lower cervical spine with extension into the upper thoracic spine suggestive of acute transverse myelitis (ATM). The patient's hospital course was complicated by a self-limiting episode of pulseless ventricular tachycardia accompanied by a brief loss of consciousness. Initial treatment included IV solumedrol, however due to lack of clinical improvement after a 5-day steroid treatment, plasmapheresis was initiated. The patient's condition improved with plasmapheresis and he was subsequently discharged to a rehab facility with a diagnosis of ATM of unclear etiology. Extensive serology, cardiac and CSF studies failed to determine the cause of this patient's myelitis or pulseless ventricular tachycardia. The following case report explores the potential factors that may have contributed to this patient's symptoms.

Severe disease exacerbation after mRNA COVID-19 vaccination unmasks suspected multiple sclerosis as neuromyelitis optica spectrum disorder: a case report.

BACKGROUND: Since the beginning of the COVID-19 pandemic and development of new vaccines, the issue of post-vaccination exacerbation or manifestation of demyelinating central nervous system (CNS) disorders has gained increasing attention. CASE PRESENTATION: We present a case of a 68-year-old woman previously diagnosed with multiple sclerosis (MS) since the 1980s who suffered a rapidly progressive severe sensorimotor paraparesis with loss of bladder and bowel control due to an acute longitudinal extensive transverse myelitis (LETM) after immunization with the mRNA Pfizer-BioNTech COVID-19 vaccine. Detection of Aquaporin-4-antibodies (AQP4) in both serum and CSF led to diagnosis of AQP4-antibody positive neuromyelitis optica spectrum disorder (NMOSD). Treatment with intravenous corticosteroids and plasmapheresis led to a slight improvement of the patient's symptoms. CONCLUSIONS: Pathogenic mechanisms of post-vaccination occurrence of NMOSD are still unknown. However, cases like this should make aware of rare neurological disorders manifesting after vaccination and potentially contribute to improvement of management of vaccinating patients with inflammatory CNS disorders in the future. So far two cases of AQP4-antibody positive NMOSD have been reported in association with viral vector COVID-19 vaccines. To our knowledge, we report the first case of AQP4-antibody positive NMOSD after immunization with an mRNA COVID-19-vaccine.

Intramedullary parasite eggs, latent for three decades, mimicking acute transverse myelitis.

BACKGROUND: Intramedullary parasitic infection is extremely uncommon, and clinical presentation of Brown-Sequard syndrome is even rarer. CASE PRESENTATION: The authors report a case involving a 57-year-old woman with Brown-Sequard syndrome, in whom magnetic resonance imaging and clinical and epidemiological features were similar to those of acute transverse myelitis. Myelotomy suggested inflammation caused by latent parasite eggs in the spinal cord. Antiparasitic and steroid therapies were administered postoperatively. To the author's knowledge, this is the first report to describe a surgical experience for Taenia solium eggs in the spinal cord. CONCLUSION: Intramedullary parasitic infection is a diagnostic challenge that requires careful discrimination from other diseases. If parasite infection is suspected in a progressively deteriorating patient, myelotomy should be considered for rapid and accurate treatment.

Acute transverse myelitis progressing to permanent quadriplegia following COVID-19 infection.

As of January 2022, there have been over 350 million confirmed cases of COVID-19 in the world. The most common symptoms in those infected are fever, cough, malaise, and myalgia, however pulmonary, hematologic, gastrointestinal, renal, and neurologic complications have also been reported. Acute transverse myelitis (ATM) is an uncommon neurological syndrome characterized by acute or subacute spinal cord dysfunction that can lead to paresthesias, sensory and autonomic impairment, and even paralysis. Etiologies are often unclear; however, potential causes include infection, neoplastic, drug or toxin induced, autoimmune, and acquired. Treatment for ATM primarily consists of steroids and plasmapheresis, which often reverses any neurologic symptoms. ATM has rarely been reported as a complication of COVID-19 infections. A 43-year-old female presented to the emergency department for evaluation of progressive numbness and tingling in her legs ten days after developing upper respiratory symptoms from a COVID-19 infection. Physical examination and magnetic resonance imaging confirmed a diagnosis of ATM. During her hospital course, she experienced rapid progression of her paresthesias and developed complete loss of motor function in her upper and lower extremities. Within 48 hours after emergency department arrival, she required intubation due to worsening diaphragmatic and chest wall paralysis. Her treatment included a long-term steroid regimen and plasmapheresis, and unfortunately, she did not have any neurologic recovery. We present a very rare case of ATM progressing to complete quadriplegia following COVID-19 infection.

Case Reports of Acute Transverse Myelitis Associated With mRNA Vaccine for COVID-19.

Acute transverse myelitis (ATM) has been reported as rare complication of vaccination. Herein, we report 2 cases of ATM after the administration of an mRNA vaccine for coronavirus disease 2019 (COVID-19). The first one is an 81-year-old man who received the BNT162b2 vaccine. He presented with bilateral hand weakness. Spine magnetic resonance imaging (MRI) showed high signal intensity from the C1 to C3 vertebrae. The second is a 23-year-old woman who received the BNT162b2 vaccine and experienced tingling in her legs. Spine MRI showed a high signal intensity lesion at the conus medullaris. These patients were treated with intravenous methylprednisolone and their symptoms improved slightly. Careful follow-up is needed to identify adverse events after the administration of mRNA vaccines for COVID-19.

Late-onset neuromyelitis optica spectrum disorder: A case series from Iran.

INTRODUCTION: Neuromyelitis optica spectrum disorder (NMOSD) is a disabling autoimmune disease of the central nervous system that can start at ages of 50 or more, when it is called late-onset NMOSD (LO-NMOSD). Data on this disorder are sparse. In this cross-sectional study, patient characteristics of the disease were studied. LO-NMOSD patients of a tertiary center in Tehran were studied from 2016 to 2020. CASE REPORTS: Eight patients were identified, half of whom were men. The diagnostic delay was from no time-lapse to three years (mean: 0.62, SD: 1.06), which was significantly shorter than in early-onset patients. Seven patients (87.5%) tested positive for AQP4-IgG which was significantly higher compared to early-onset patients (p-value=0.01). Four patients (50%) had both transverse myelitis and optic neuritis as presenting symptoms, while three (38%) had just myelitis and only one (12%) had optic neuritis. CONCLUSION: There is discrepancy regarding different aspects of LO-NMOSD. Further studies are needed to clarify the subject in order to enhance diagnosis and treatment.

A case report of transverse myelitis in a patient with human immunodeficiency virus.

Acute or subacute transverse myelitis is a rare disease caused by inflammation of the spinal cord. The extensive differential diagnosis of this disease require physician to timely decide the necessary investigations and plan for effective treatment. We report a case of anomalous multifactorial transverse myelitis in a patient with human immunodeficiency virus (HIV) who responded well with multimodal therapy. The patient was given immunosuppressive agent and plasma exchange for the demyelinating disease as well as antibiotics for neurosyphilis, antiviral for cytomegalovirus (CMV) neurologic disease, and highly active antiretroviral therapy (HAART). Three months after his first presentation, patient showed full neurology recovery and suppressed HIV viral load.

Atypical presentation of transverse myelitis in the emergency department.

Transverse myelitis (TM) is an inflammatory disorder of the spinal cord characterized by acute onset sensory loss and motor weakness below the level of the lesion. The etiology of the disease is varied, and diagnosis of TM in the Emergency Department (ED) can be difficult owing to the low incidence of the disease, and frequently insidious onset. We report a case of a 59-year-old female who presented to the ED from home with left upper quadrant abdominal pain and flank pain of sudden onset. While in the ED, she experienced left lower extremity weakness and sensory changes that slowly progressed bilaterally, and ultimately was diagnosed with transverse myelitis. Transverse myelitis rarely diagnosed in the ED setting, but it is an important clinical consideration when evaluating ED patients presenting with both pain and progressive neurologic symptoms.

No laughing matter - Myeloneuropathy due to heavy chronic nitrous oxide abuse.

Chronic nitrous oxide abuse is a known cause of myeloneuropathy. Nitrous oxide irreversibly inactivates vitamin B12 causing demyelination of the dorsal spinal columns, clinically indistinguishable from that which is caused by vitamin B12 deficiency. We report a 37-year-old female who presented with ataxia, loss of lower extremity proprioception, demyelination of her cervical dorsal spinal columns, and other laboratory and physical exam findings consistent with nitrous oxide abuse. The patient reported daily use in excess of 500 nitrous oxide cartridges, also known as "whippits". Nitrous oxide myeloneuropathy should be included in the differential diagnoses for emergency medicine physicians when evaluating a patient with bilateral neurologic deficits and ataxia.

[Differential diagnostics of autoimmune inflammatory spinal cord diseases]. / Differenzialdiagnostik autoimmun-entzündlicher Rückenmarkserkrankungen.

Myelitis is an acute or subacute inflammatory syndrome of the spinal cord. Myelopathy, often used as a synonym and presenting with similar symptoms in clinical practice, can be caused by numerous, not primarily inflammatory etiologies and might also show a progressive disease course. Within the last decade the spectrum of autoimmune myelitis was significantly broadened as was the spectrum of diagnostic methods. Apart from the characteristic example of multiple sclerosis with short-length myelitis and neuromyelitis optica spectrum disorders with longitudinally extensive transverse myelitis, multiple rare but important differential diagnoses should also be considered. Magnetic resonance imaging and laboratory analyses of serum antibodies and cerebrospinal fluid are the most important diagnostic methods and are fundamental for rapid treatment decisions, subsequently with better prognosis. This article reviews representative diseases within the spectrum of autoimmune spinal cord diseases and their differential diagnoses.

Transverse Myelitis in Systemic Lupus Erythematosus: Clinical Features and Prognostic Factors in a Large Cohort of Latin American Patients.

BACKGROUND: Acute transverse myelitis (ATM) is an infrequent but severe complication of systemic lupus erythematosus (SLE). The purpose of study was to describe clinical features and prognostic factors of patients with SLE-related ATM. METHODS: In this medical records review study, data were collected from 60 patients from 16 centers seen between 1996 and 2017 who met diagnostic criteria for SLE and myelitis as defined by the American College of Rheumatology/Systemic International Collaborating Clinics and the Working Group of the Transverse Myelitis Consortium, respectively. Objective neurological impairment was measured with American Spinal Injury Association Impairment Scale (AIS) and European Database for Multiple Sclerosis Grade Scale (EGS). RESULTS: Among patients included, 95% (n = 57) were female, and the average age was 31.6 ± 9.6 years. Myelitis developed after diagnosis of SLE in 60% (n = 36). Symmetrical paraparesis with hypoesthesia, flaccidity, sphincter dysfunction, AIS = A/B, and EGS ≥ 8 was the most common presentation. Intravenous methylprednisolone was used in 95% (n = 57), and 78.3% (n = 47) received intravenous cyclophosphamide. Sensory/motor recovery at 6 months was observed in 75% (42 of 56), but only in 16.1% (9 of 56) was complete. Hypoglycorrhachia and EGS ≥ 7 in the nadir were associated with an unfavorable neurological outcome at 6 months (p < 0.05). A relapse rate during follow-up was observed in 30.4% (17 of 56). Hypoglycorrhachia and hypocomplementemia seem to be protective factors for relapse. Intravenous cyclophosphamide was associated with time delay to relapse. CONCLUSIONS: Systemic lupus erythematosus-related ATM may occur at any time of SLE course, leading to significant disability despite treatment. Relapses are infrequent and intravenous cyclophosphamide seems to delay it. Hypoglycorrhachia, hypocomplementemia, and EGS at nadir are the most important prognostic factors.

[Acute disseminated encephalomyelitis and myelitis associated with new coronavirus infection COVID-19. Case report].

Acute disseminated encephalomyelitis (AEM) and acute transverse myelitis (OPM) are autoimmune demyelinating diseases of the central nervous system. Two clinical observations of AEM and OPM developed after suffering acute coronavirus infection (SARS-CoV-2) are presented. Differential diagnosis was carried out with multiple sclerosis, encephalitis of an infectious nature, compressive myelopathy, and opticomyelitis. Both observations show an almost complete recovery of lost functions. The pathogenetic mechanisms of the development of AEM and OPM in patients with coronavirus infection are discussed. The onset of central nervous system dysimmune lesion in the context of coronavirus infection makes it necessary to monitor the clinical situation with the involvement of a neurologist for timely diagnosis and determination of therapeutic tactics that can reduce the degree of disability of patients.

Acute paraparesis in HIV-infected patient after initiation of highly active antiretroviral therapy.

Neurological syndromes occur in around 40-70% of HIV-infected people. Direct central nervous system involvement by the virus usually manifests as HIV encephalitis, HIV leucoencephalopathy, vacuolar leucoencephalopathy or vacuolar myelopathy. Indirect involvement is usually associated with neurotropic opportunistic infections which include tuberculosis, toxoplasmosis, cryptococcosis and viral encephalitis such as herpes simplex, varicella-zoster, cytomegalovirus and Human polyomavirus 2. We report a case of transverse myelitis in a recently diagnosed HIV patient who was otherwise asymptomatic initially and developed paraparesis after 1 month of initiation of antiretroviral therapy. After ruling out opportunistic infections and other causes of compressive and non-compressive myelopathy, development of transverse myelitis was attributed to immune reconstitution inflammatory syndrome in view of baseline low CD4 count and their improvement after HAART initiation. Prompt treatment with corticosteroids successfully reversed the symptoms.

MOG-IgG myelitis coexisting with systemic lupus erythematosus in the post-partum setting.

BACKGROUND: Longitudinally extensive transverse myelitis (LETM) accompanying systemic lupus erythematosus (SLE) is often due to coexisting aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder but has not been associated with myelin oligodendrocyte glycoprotein-IgG (MOG-IgG). OBJECTIVE AND METHODS: Case report at an academic medical center. RESULTS: A 32-year-old woman developed severe transverse myelitis (paraplegia) shortly after SLE onset in the post-partum period. Magnetic resonance imaging (MRI) revealed an LETM, cerebrospinal fluid showed marked inflammation, and testing for infections was negative. Serum live-cell-based assay for MOG-IgG was positive but aquaporin-4-IgG was negative. CONCLUSION: In patients with SLE and LETM, MOG-IgG testing should be considered, in addition to AQP4-IgG.