Transverse Myelitis Research Articles

Severe Acute Transverse Myelitis With a Rapid Progression in an Infant

Severe Acute Transverse Myelitis With a Rapid Progression in an Infant

Overview of emerging therapies for demyelinating diseases.

This paper provides an overview of autoimmune disorders of the central nervous system, specifically those caused by demyelination. We explore new research regarding potential therapeutic interventions, particularly those aimed at inducing remyelination. Remyelination is a detailed process, involving many cell types-oligodendrocyte precursor cells (OPCs), astrocytes, and microglia-and both the innate and adaptive immune systems. Our discussion of this process includes the differentiation potential of neural stem cells, the function of adult OPCs, and the impact of molecular mediators on myelin repair. Emerging therapies are also explored, with mechanisms of action including the induction of OPC differentiation, the transplantation of mesenchymal stem cells, and the use of molecular mediators. Further, we discuss current medical advancements in relation to many myelin-related disorders, including multiple sclerosis, optic neuritis, neuromyelitis optica spectrum disorder, myelin oligodendrocyte glycoprotein antibody-associated disease, transverse myelitis, and acute disseminated encephalomyelitis. Beyond these emerging systemic therapies, we also introduce the dimethyl fumarate/silk fibroin nerve conduit and its potential role in the treatment of peripheral nerve injuries. Despite these aforementioned scientific advancements, this paper maintains the need for ongoing research to deepen our understanding of demyelinating diseases and advance therapeutic strategies that enhance affected patients' quality of life.

Neuroborreliosis Presenting as Urinary Retention: Case Report.

Lyme disease is a tick-borne infectious disease caused by the spirochete Borrelia burgdorferi. Voiding dysfunction is a rare manifestation of neuroborreliosis with only a few cases reported. Here we describe a case of a 6-year-old male child with an acute urinary retention, paraparesis, and voiding difficulty in whom neuroborreliosis was diagnosed through serologic tests for antibodies, Western blot testing confirmation and intrathecal antibody synthesis. Magnetic resonance imaging (MRI) of the spine led to the diagnosis of acute transverse myelitis and a urodynamic study demonstrated detrusor areflexia. He received a 4-week course of intravenous ceftriaxone (2 g/d). The patient has recovered from the paraparesis but still suffers from a neurogenic bladder.

The presence of oligoclonal bands predicts conversion to multiple sclerosis in isolated myelitis

Acute transverse myelitis (ATM) is a disease characterized by inflammation of the spinal cord and may have various causes. In the context of this work, the distinction between isolated ATM and initial manifestation of autoimmune-mediated diseases of the central nervous system such as multiple sclerosis (MS) is crucial. Hence, the aim of this work was to identify predictive factors associated with the conversion to definite MS in a collective of individuals after their initial episode of isolated ATM (no initial identified cause). In this retrospective data analysis from the Vienna MS Database, all patients from Jan. 1, 1999, to Dec. 31, 2019, with a diagnosis of isolated ATM (according to the criteria of the Transverse Myelitis Consortium Working Group) who underwent lumbar puncture were extracted. Electronic medical records were reviewed on the availability of clinical data including therapy and follow-up, laboratory results including cerebrospinal fluid (CSF) analysis, evoked potentials (EP) as well as magnetic resonance imaging data. Among 42 patients with the diagnosis of isolated ATM, 12 (29%) were subsequently diagnosed with MS over a median follow-up period of 7.7 years. Univariately, MS converters were younger (32 years [25–39] vs. 42 years [31–50], p = 0.032), had a lower CSF/serum albumin ratio (29 [24–35] vs 37 [27–52], p = 0.037), lower CSF total protein (4.5 [2.8–4.8] vs. 5.5 [3.4–8.5], p = 0.023) and a higher proportion of CSF-specific oligoclonal bands (OCB; 83% vs. 30%, p = 0.002). In the multivariate regression analysis, the presence of CSF-specific OCB emerged as the sole predictive factor of subsequent MS diagnosis (OR: 14.42, 95% CI 1.39 to 149.48, p = 0.03). In a collective of 42 patients with isolated ATM and an MS conversion rate of nearly 30%, the only but highly predictive factor were CSF-specific OCB. This emphasizes the significance of conducting timely CSF analysis in such patients and underscores the need for tailored monitoring and follow-up strategies in this specific group.

Acute Transverse Myelitis in HHV-6 Infection

Acute Transverse Myelitis in HHV-6 Infection

Neurosyphilis Masquerading as Longitudinally Extensive Transverse Myelitis and Radiculopathy with an Initially Remitting Course

Neurosyphilis Masquerading as Longitudinally Extensive Transverse Myelitis and Radiculopathy with an Initially Remitting Course

Efficacy and Safety of Inebilizumab, an Anti-CD19 Monoclonal Antibody, for the Treatment of Neuromyelitis Optica Spectrum Disorder: Based on the N-MOmentum Trial

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathic disease of the central nervous system characterized by severe optic neuritis and transverse myelitis. The antibody against aquaporin 4 (AQP4), a water channel mainly expressed in astrocytes, is specific to NMOSD and may be detected in >70% of all cases. Inebilizumab is a humanized IgG1 monoclonal antibody against CD19. Anti-AQP4 antibodies are produced by CD19-positive plasmablasts, and inebilizumab administration significantly reduces the number of CD19-positive B cells and has therapeutic effects on NMOSD. The efficacy and safety of inebilizumab have been verified in the N-MOmentum trial, an international double-blind, placebo-controlled phase II/III study, in which Japanese patients also participated. Inebilizumab was approved for the treatment of NMOSD with AQP4-IgG in Japan in March 2021. In this review, we summarize the efficacy and safety of inebilizumab in the treatment of NMOSD and, focus on findings from the primary and additional analyses of the N-MOmentum trial. These results suggest that inebilizumab is effective and safe in preventing the recurrence of NMOSD in populations with different backgrounds and that long-term treatment with inebilizumab is beneficial.

I ANCA Kidding: ANCA-Associated, Pauci-Immune Crescentic Glomerulonephritis with Normal Kidney Function Presenting with Transverse Myelitis

I ANCA Kidding: ANCA-Associated, Pauci-Immune Crescentic Glomerulonephritis with Normal Kidney Function Presenting with Transverse Myelitis

Characterizing the clinical, radiological and serological features of children diagnosed with neuromyelitis optica spectrum disorder at pediatric neurology unit

Background. Neuromyelitis optica spectrum disorder is a rare autoimmune disease with a chronic inflammatory demyelinating characteristics that affects the central nervous. The aim of the study is to characterize the clinical, serological and radiological features of neuromyelitis optica disease in a sample of children from Iraq. Methods. A cross-sectional descriptive study has been carried out in the period from August 2019 to September 2020, in pediatric neurology ward in Children Welfare Teaching Hospital, Baghdad. In 12 retrospectively gathered cases, the medical registration of the ward was assessed. A total of 13 patients, aged 3-16 years were included. Results. Among the patients, girls represented 61.5% (8), and the females: males ration was (1.6:1). The patients' ages ranged from 3 to 16 years (mean 9.3 ± 4.02 years). All patients younger than 6 years were AQP-ve, in contrast to 15.3% in those older than 6 years. Negative AQP patients equally distributed between males and females. While females predominated in those with AQP positive status (5,71.4%). Weakness of the limbs and sphincteric disturbance were the first two common clinical features in both AQP positive and negative patients. The three most common brain sites insulted were deep white matter (10, 76.9%), periaqueductal area (5, 38.4%), and brainstem (4, 30.7%). All patients showed long spinal lesions, affecting predominantly the cervico-thoracic (61.53%). Conclusion. The demographic characteristics of the present cohort were comparative to that reported in the literature. Transverse myelitis phenotype was the most common and consistent one. Vomiting was more common in AQP +ve patients.

Cerebral Cortical Encephalitis and Other Meningocortical Manifestations of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children: Case Series and Review of the Literature.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is a neuroinflammatory disorder (MOGAD) with heterogeneous phenotype including paroxysms of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, brainstem demyelination, and encephalitis. Fluid-attenuated inversion recovery hyperintense cortical lesions in MOG-associated encephalitis with seizures, or FLAMES, is a manifestation of cerebral cortical encephalitis seen less frequently than other typical MOG antibody-associated disease presentations. Cases of FLAMES are rarer in children, and frequently initially misdiagnosed with infectious meningoencephalitis. Other meningocortical manifestations of MOG antibody-associated disease have been described and likely exist along a continuum. In this retrospective single-center case series, we describe the demographic, clinical, radiographic, laboratory, and electroencephalographic features of 5 children with clinicoradiographic features consistent with the spectrum of MOG-IgG-positive meningocortical syndromes.

Clinical characteristics and predictive factors of recurrent idiopathic transverse myelitis.

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. 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. 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). 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.

Neuroinflammatory Disorders of the Central Nervous System Associated with Monkeypox Virus Infection: A Systematic Review and Call to Action.

The Monkeypox virus, a zoonotic pathogen of the Orthopoxvirus genus, has shown a marked global spread, resulting in cases of neuroinflammatory disorders. This systematic review aims to summarize the central nervous system manifestations linked to monkeypox virus infection. We conducted a systematic review according to PRISMA guidelines. Databases such as PubMed, EMBASE, Cochrane Library, and Web of Science were searched up to September 2024. Inclusion criteria focused on monkeypox virus-positive patients with confirmed central nervous system inflammatory disorders, including encephalitis, meningitis, myelitis, and related neuroimmune diseases. From 770 screened articles, 38 studies were included. Of the 20 reported monkeypox virus-infected cases with central nervous system involvement, the most common manifestations were encephalitis (40%), encephalomyelitis (30%), and meningoencephalitis (10%). Additional rarer presentations included Guillain-Barré syndrome (5%) and transverse myelitis (5%). The mean age of affected individuals was 27.9±13.69 years, and 75% were male. Fever, fatigue, and myalgia were the predominant systemic symptoms, with neurological complications often appearing within 4.6±2.5 days post-infection. Diagnoses were confirmed by quantitative reverse transcription polymerase chain reaction, magnetic resonance imaging, and cerebrospinal fluid analysis. Treatment strategies varied, with antivirals (e.g., tecovirimat) and immunomodulatory therapies (e.g., methylprednisolone, immunoglobulins, and rituximab) utilized. Monkeypox virus infection is associated with severe neuroinflammatory disorders, with varying outcomes from complete recovery to mortality. Increased awareness, comprehensive diagnostic approaches, and targeted therapeutic interventions are critical in managing these cases. Further research is required to elucidate the mechanisms of neuroinflammation in the monkeypox virus and establish standardized treatment protocols.

Strength of Association between Coronavirus Disease 2019 and Neurological Disorders in Children: A Case-Control Study

Introduction: Evidence suggests potential neurological complications of coronavirus disease 2019 (COVID-19), particularly in adults. While case series have hinted at associations between COVID-19 and neurological disorders (NDs) in children, the extent of this link remains unclear. This study investigates temporal trends in NDs during the pandemic and assesses their potential association with COVID-19 infection in children. Methods: We analyzed national Thai hospitalization data (2017–2022) for children under 18 with specific NDs (acute transverse myelitis, central nervous system demyelination, neuromyelitis optica, optic neuritis, polyneuropathy, stroke). An interrupted time series analysis was employed to identify changes in the incidence trends of NDs following the declaration of the COVID-19 pandemic. A matched case-control analysis was conducted using data specific to the Thai COVID-19 outbreak period. This analysis aimed to estimate the association between recent/concurrent COVID-19 infection and NDs in children. A propensity score matching on age group, sex, and month of admission was performed before conducting logistic regression. Results: From 2017–2022, 1,721 children were admitted with NDs (2,474 admissions), with a male predominance (55%) and average age of 10.6 years. Significant slope change was observed in optical neuritis trends coinciding with the third COVID-19 wave. The case-control analysis included 468 cases and 2,340 controls. Children with NDs had a significantly higher prevalence of recent/concurrent COVID-19 (matched odds ratio: 1.95, 95% confidence interval: 1.21–3.16). Subgroup analysis revealed an association between stroke and recent/concurrent COVID-19 (matched odds ratio: 3.05, 95% confidence interval: 1.3–7.16). Thus, this study suggests an association between recent/concurrent COVID-19 and NDs, especially pediatric stroke.

Pruritus: An Overlooked Symptom of Spinal Tumors

Background Chronic itch is often associated with pain due to its complex nature. Neuropathic pruritus is defined as a debilitating form of chronic itching, about which not much is known to date. This condition may develop as a result of various neurological events and is associated with damage to the somatosensory nervous system, accounting for approximately 10% of chronic itching cases. Syringomyelia, transverse myelitis, radiculopathies, thoracic spine masses, brain tumors, strokes, and abscesses are among the conditions associated with neuropathic itching to date. Case presentation A 65-year-old female patient, with no known diseases and no regular medication usage, presented with a complaint of itching that had been ongoing for the past two years. Itching initially started in approximately five cm areas on the bilateral below-knee flexor surfaces. The patient, whose complaints persisted, was referred to the internal medicine outpatient clinic by dermatology. On physical examination, erythematous excoriated papules were observed in the areas affected by itching. The patient's blood sugar, liver and kidney function tests, complete blood count, erythrocyte sedimentation rate, thyroid function tests, urinalysis, stool parasitology were all normal or negative. The patient reported a sensation of coldness in the same area, was referred to neurosurgery to investigate the etiology of possible neuropathic itching. The patient's spinal imaging, revealed a spinal mass. She underwent surgery performed by a neurosurgeon, during which the spinal mass was completely removed. She reported that her itching had completely disappeared post-operatively. Conclusions The concept of itching as a variant of pain is not very new. Any damage occurring in the central nervous system or peripheral nervous system that affects the neurons responsible for transmitting and processing itch can lead to neuropathic itching. Focusing on spinal cord pathologies, any condition that damages the spinal cord may cause itching, depending on the level of damage. In cases of itching with dermatomal localization, where pain, hot or cold sensations, and paroxysmal itching are present, additional imaging methods or investigations for etiology should be performed.

MOG-antibody-associated transverse myelitis with the H-sign and unusual MRI enhancement: a case report and literature review.

Transverse myelitis is the second most common symptoms in myelin oligodendrocyte antibody-associated diseases (MOGAD), causing obvious clinical manifestation. T2-hyperintense lesions mainly restricted to the gray matter in the spinal cord on axial magnetic resonance imaging, produce the H-sign, which is thought to be the typical finding of MOGAD. Contrast enhancement can be observed in some cases of myelin oligodendrocyte antibody-associated transverse myelitis (MOG-TM). However, reports on the enhancement pattern associated with the H-sign are rarely seen. In this report, we describe a case of pediatric MOG-TM in which the H-sign was observed without enhancement, while the surrounding white matter exhibited enhancement. This pattern contradicts the previously observed gray matter involvement. Then we reviewed the literatures of myelin oligodendrocyte antibody-positive myelitis to focus on the neuroimaging features and discuss the implications of our finding.

Scrub Typhus Meningoencephalitis Presenting as Generalized Convulsive Status Epilepticus with Basal Ganglia and Extra-limbic Cortical Involvement, Complicated by Cortical Multifocal Myoclonus

Background Scrub typhus is an acute febrile infectious disease highly prevalent in the Asia Pacific region, often referred to as the “tsutsugamushi triangle.” This mite-borne rickettsial zoonosis is caused by Orientia tsutsugamushi, an intracellular Gram-negative organism that primarily targets endothelial cells. The resulting vasculitis leads to multisystem involvement. In terms of neurological manifestations, meningoencephalitis is the most common presentation of scrub typhus. Other frequent neurological manifestations include cranial nerve paresis, transverse myelitis, and polyneuropathy. Status epilepticus, while reported, is a rare presenting feature of this infection. Although scrub typhus has been documented to present as limbic encephalitis, there have been no previous descriptions in the literature of neuroradiological patterns affecting the basal ganglia or extra-limbic cortices in this condition. Case Report We report a case of a 23-year-old previously healthy woman who presented with scrub typhus meningoencephalitis. The condition manifested as encephalitis with involvement of the basal ganglia and extra-limbic cortices. She presented with generalized convulsive status epilepticus, which was complicated by cortical multifocal myoclonus. Discussion Scrub typhus can be a significant diagnostic challenge, potentially presenting with generalized convulsive status epilepticus and mimicking both clinical and radiological features of arboviral encephalitides, such as those caused by West Nile and Japanese encephalitis viruses. Furthermore, as demonstrated in this case, its radiological presentation can resemble that of autoimmune encephalitis. Given that scrub typhus is amenable to treatment with antibiotics, such as doxycycline and azithromycin, which do not increase seizure risk, it should be considered in the differential diagnosis for patients presenting with seizures or encephalitis, especially in endemic areas.

Clinical characteristics of double negative atypical inflammatory demyelinating disease: A prospective study.

This study aimed to investigate the clinical characteristics and predictors of relapse in double negative atypical inflammatory demyelinating disease (IDD) and to explore potential antigenic targets by tissue-based assays (TBA) using rat brain indirect immunofluorescence. We compared the clinical, laboratory, and MRI data of double negative atypical IDD with other IDD patients. Serum samples were collected for TBA. The predictors of relapse were examined over a minimum of 24 months follow-up. In our cohort of 98 patients with double negative atypical IDD, there was no significant female predominance (58.2%, 57/98). The lesions primarily affected the spinal cord and brain stem, with fewer cases of involvement in the area postrema (5.1%, 5/98) and longitudinally extensive transverse myelitis (43.9%, 43/98). A total of 62.5% (50/80) patients tested positive for anti-astrocyte antibodies based on rat brain TBA. Over a median duration of 39.5 months, 80 patients completed the entire follow-up, and 47.5% (38/80) patients exhibited monophasic course. A total of 36% (18/50) patients positively for anti-astrocyte antibodies had a monophasic course, which is significantly lower than patients negatively for anti-astrocyte antibodies (66.7%, 20/30) (p = 0.008). The presence of anti-astrocyte antibodies (hazard ratio (HR), 2.243; 95% CI, 1.087-4.627; p = 0.029) and ≥4 cerebrum lesions at first attack (HR, 2.494; 95% CI, 1.224-5.078; p = 0.012) were risk factors for disease relapse, while maintenance immunotherapy during remission (HR, 0.361; 95% CI, 0.150-0.869; p = 0.023) was protective factor. Double negative atypical IDD are unique demyelinating diseases with a high relapse rate. Maintenance immunotherapy is helpful to the prevention of relapse, particularly in patients with anti-astrocyte antibodies or ≥4 cerebrum lesions at first attack.

Cytotoxic Lesion of the Corpus Callosum Caused by Dengue Virus Infection

ABSTRACT Dengue virus infection can cause a wide spectrum of clinical manifestations, ranging from mild fever to lethal dengue shock syndrome. Neurological manifestations include encephalitis, acute disseminated encephalomyelitis, Transverse myelitis, Guillain-Barre syndrome, and neuro-ophthalmic complications. We report a case of a 44-year-old male who was admitted to our hospital with high-grade fever, myalgia, retro-orbital pain, visual disturbances, and drowsiness. Hematological and serological parameters confirmed the diagnosis of dengue fever. Brain imaging demonstrated altered signal intensity in the splenium of the corpus callosum and eventually a diagnosis of a cytotoxic lesion of the corpus callosum (CLOCC) was made. The patient was managed conservatively and discharged 7 days after admission and imaging findings were resolved completely 3 weeks later.

Clinical spectrum of demyelinating disease of central nervous system and frequency of anti AQP4 and anti MOG among them: one-year single-center retrospective study

Background: Inflammatory demyelinating diseases of the central nervous system (CNS) are autoimmune conditions leading to significant neurological disability in adults. Recent classifications include myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) and neuromyelitis optica spectrum disorder (NMOSD) which pose diagnostic challenges due to overlapping clinical and radiological features. This study aimed to assess the clinical spectrum among adults and children diagnosed with CNS demyelinating diseases and to find the proportion of MOG and/or aquaporin-4 (AQP4) autoantibodies amongst them. Methods: This single-center, retrospective study examined 20 patients diagnosed with CNS demyelinating disorders between March, 2023 and February, 2024. Data pertaining to demographics, disease types, CSF analysis, MRI findings, treatment modalities, and serological profiles for anti-AQP4 and anti-MOG antibodies were collected from hospital records and evaluated. Results: Among 20 patients [median age, 34 years (IQR, 18.75); males (n=10) and females (n=10)], acute transverse myelitis (TM) was the most common demyelinating disorder at onset (60%) followed by optic neuritis (ON) (20%). CSF analysis found elevated protein levels in 53% and pleocytosis in 33% of patients. MRI findings revealed longitudinal extensive involvement in 52% of patients, predominantly affecting the cervical and dorsal spine. Serological testing identified 15% positive for anti-AQP4 and 10% for anti-MOG antibodies. MOG+ patients were significantly younger than AQP4+ patients (mean age 16.5 vs. 36.66 years, p=0.016). Both MOG+ patients were male, with 50% presenting with acute TM and 50% with acute disseminated encephalomyelitis. Among AQP4+ patients, the male-to-female ratio was 1:2, with 66.66% presenting with acute TM and 33.33% with ON. Conclusion: CNS demyelinating disorders primarily affect younger individuals, with TM as the most common initial disorder and extensive spinal involvement in cervical and dorsal regions. Serological testing identified three patients with anti-AQP4 and two with anti-MOG antibodies, providing valuable insights into the clinical spectrum of these disorders through cell-based assays.

Neuromyelitis Optica Spectrum Disorder: A Case Report

Neuromyelitis optica spectrum disorder also known as Devic's disease is an autoimmune condition where the body produces antibodies against Aquaporin-4 in the astrocytes. This affects the brain and spinal cord leading to numerous manifestations like paralysis, transverse myelitis, and optic neuritis. MRI and aquaporin-4 antibody (AQP4-IgG) are crucial for its diagnosis. Treatment of the acute stage involves plasma exchange and intravenous steroids. Steroids and immune modulators can do long-term management. This case highlights a 40-year-old woman who manifested a wide range of neurological symptoms, including paralysis and eyesight loss. She had blood testing, radiology, and a clinical evaluation to diagnose her condition. Neuromyelitis optica spectrum disorder was diagnosed in light of the investigations. This instance emphasizes the need for careful examination and consideration of any paralysis. To prevent patients from experiencing psychological, financial, or physical challenges, treating physicians should be vigilant for any findings related to conditions like neuromyelitis optica spectrum disorder.