Transverse Myelitis Research Articles (Page 1)

ABSTRACT Myelin oligodendrocyte glycoprotein antibody-associated disease (MOG AD) is a rare autoimmune demyelinating condition typically presenting with optic neuritis, transverse myelitis, or encephalitis. Its occurrence in immunocompromised individuals, particularly those with human immunodeficiency virus (HIV), is rare and presents unique diagnostic and therapeutic challenges. We report the case of a 70-year-old HIV-positive man who developed alexia without agraphia following treatment for opportunistic infections, including Pneumocystis jirovecii pneumonia and cytomegalovirus. Brain MRI revealed a non-enhancing hyperintense lesion in the medial left occipital lobe extending into the splenium of the corpus callosum. MOG-IgG was positive at a titer of 1:30, while aquaporin-4 antibodies and paraneoplastic panels were negative. Neuropsychological assessment confirmed selective impairment in visual word recognition with preserved writing ability, consistent with alexia without agraphia. The patient was treated with intravenous immunoglobulin (IVIG) without corticosteroids due to immunosuppressive concerns and demonstrated approximately 40% improvement in visual word recognition accuracy. At six months follow-up, no relapse was observed, and reading ability remained stable. This case represents the first reported instance of MOGAD presenting with alexia without agraphia in an HIV-positive individual, underscoring the importance of considering autoimmune demyelination in immunosuppressed patients with focal neurological deficits.

We sought to characterize the sixth most common finding in our neuroimmunological laboratory practice (tissue assay-observed unclassified neural antibodies [UNAs]), combining protein microarray and phage immunoprecipitation sequencing (PhIP-Seq). Patient specimens (258; 133 serums; 125 CSF) meeting UNA criteria were profiled; October 2022-September 2023. Top-ranking candidate antigens were validated in silico, by dual-staining confocal microscopy, and ≥ 1 protein-specific assay. Clinical data were reviewed. Among 21 patients, 11 autoantibodies were characterized (serum, 19; CSF, all 9 available). Autoantigens were CACNA1I, 1; CAMK2B, 2; CLIP2, 1; FMN2, 2; MAP1A, 2; MAP2, 5; NECAB1, 1; SNAP91, 3; SRCIN1, 1; SYNJ1, 1; SYT3, 2. Analytical validation was by confocal TIIFA (all), western blot (10/10 available), and cell-based assay (5/5 performed). Clinical accompaniments were: encephalitis, 6; brainstem encephalitis, 2; encephalomyelitis, 2; cerebellar ataxia, 2; longitudinally extensive transverse myelitis (LETM), 2; sensory neuronopathy, 1; peripheral neuropathy, 4, and movement disorders, 2. Inflammatory MRI abnormalities were identified in 5/16 patients (31%) with CNS disorders: T2 signal change (2), LETM (2), leptomeningeal enhancement (1). Seven of 8 (88%) had inflammatory CSF (pleocytosis, 5 [median 25.5 cells, range 7-294]; elevated IgG index/synthesis rate, 4; CSF-exclusive oligoclonal bands, 4). Six had paraneoplastic causation (lung cancer, 2; other, 4); 3 were postinfectious (1 each of COVID-19, HSV-1, and post-Group A streptococcal infection). Of 9 immunotherapy-treated patients, 5 improved. UNAs are partly accounted for by a repertoire of diverse mostly intracellular synaptic antigens. Their characterization is expedited by protein arrays and PhIPSeq. Further individual studies are needed to assess them as disease biomarkers.

Introduction Acute transverse myelitis in children is a rapidly progressive disease of unknown etiology, with some patients experiencing lifelong paralysis. Myositis ossificans (MO) refers to heterotopic ossification within soft tissues. Neurogenic MO is rare in clinical practice and is often misdiagnosed, particularly in pediatric patients who are unable to clearly describe their symptoms. Diagnosis typically relies on imaging studies. Methods This report describes a case of an 8-year-old child who developed paraplegia following a diagnosis of acute transverse myelitis and subsequently presented with MO of the iliopsoas muscle, likely resulting from the spinal cord injury in combination with a distal femoral fracture, although the ossification itself developed in the iliopsoas region rather than at the fracture site. Results The patient exhibited progressive limitation of hip joint movement during rehabilitation. X-ray imaging revealed high-density ossification in the iliopsoas region. After six months, the ossification had matured extensively, and surgical resection was performed. The patient's hip mobility improved postoperatively. During a 3-year follow-up period, there was no recurrence of ossification. Discussion This case highlights the potential development of neurogenic MO in pediatric patients with spinal cord injury, even when the ossification does not occur at the fracture site. We discuss the challenges in diagnosing and treating such conditions in children and review current diagnostic and therapeutic advancements. This case contributes to the understanding of neurogenic MO in pediatric patients and provides insights for managing similar conditions.

Evolution of neuroimaging features in children with acute flaccid myelitis compared to other forms of childhood myelitis.

Abstract Introduction Longitudinally extensive transverse myelitis (LETM) in children raises concerns for serious underlying autoimmune conditions. Neuromyelitis optica spectrum disorder (NMOSD), often associated with anti-aquaporin-4 (AQP4) antibodies, can present with LETM and is increasingly recognised in paediatric populations. Juvenile systemic lupus erythematosus (jSLE) may manifest with central nervous system involvement, including myelitis. While NMOSD and SLE can occur independently, their coexistence—especially at initial presentation—is rare and diagnostically challenging. We report a paediatric case of LETM that led to the simultaneous diagnosis of NMOSD and jSLE, highlighting the importance of early recognition, comprehensive autoimmune evaluation and multidisciplinary collaboration. Case description An 8-year-old girl presented on Christmas evening with sudden bilateral leg numbness, difficulty standing, back pain, and urinary retention. Her history was otherwise unremarkable, aside from an innocuous fall 10 days earlier. Prior to admission, she developed thoracic and lumbar back pain and constipation over four days. Examination revealed lower limb weakness (power 4/5), brisk reflexes, abdominal tenderness, and a sensory level at T9/T10, but no rash, fever or optic neuritis on ophthalmic evaluation. She had thromboyctopaenia (platelet count nadir of 42) and lymphopenia. MRI scan of the spine showed long-segment spinal cord dilation suggestive of transverse myelitis. She was treated with 5 days of IV methylprednisolone, which led to partial and comparatively quick improvements in her mobility. She was discharged on a tapering course of prednisolone commencing at 40 mg with a plan to wean over the course of 5 weeks and twice daily intermittent catheterization. Three weeks later, she was readmitted with fever and signs suggestive of meningoencephalitis; her inflammatory markers were elevated, and she responded to antibiotics and antiviral therapy with all cultures (urine, blood, CSF) and CSF PCRs negative. Serum and CSF aquaporin-4 antibody positivity supported a diagnosis of NMOSD, while her ANA and dsDNA positivity indicated systemic lupus erythematosus (SLE). There was no evidence of other systemic involvement, and repeat MRI showed near complete resolution of inflammation. There were no significant neurological sequelae aside from ongoing twice daily CIC, gait was normal though running was still impaired. This child met criteria for SLE and NMOSD which can co-exist with lupus. Discussion This case underscores the importance of recognizing autoimmune overlaps in children presenting with transverse myelitis, particularly when associated with positive autoantibodies. Autoantibodies associated with SLE and Sjogrens have been associated with CNS AQP4 antibodies. In these patients NMOSD can precede or follow their rheumatological diagnosis. The coexistence of SLE and NMOSD influences management and prognosis, requiring a multidisciplinary approach to optimize outcomes and prevent future relapses or neurological sequelae. There was input from tertiary and quaternary neurology teams as well as rheumatology, neurology, urology and radiology teams. Discussions considered whether all features present at diagnosis could be explained by just one or the other diagnosis. It was felt that both were co-existing, and the NMOSD was distinct from neuropsychiatric SLE with the presence of LETM driven by AQP4 antibodies. She met SLICC and 2019 ACR criteria for SLE though mainly immunological and haematological domains (strongly positive ANA and dsDNA, thrombocytopaenia, leucopaenia and positive DCT). Given the associated risk of recurrent CNS inflammation with AQP4 antibodies present the aim was to prevent further flares and following discussions with a specialist neurologist in neuroinflammation alongside local rheumatology and neurology she was commenced on MMF as maintenance therapy. A longer weaning course of prednisolone was also prescribed. She still undergoes regular reviews and remains a positive and stoic character. Her physical function has recovered however her bladder residual volume is still significant to require CIC. She has recently had an admission for a urinary tract infection as a complication of this. Key learning points • Consider autoimmune rheumatological diseases in cases of NMOSD and vice versa - the overlap is documented and has consequences for prognosis and treatment. • Management of these complex cases is multi-disciplinary, treatment should be guided by all specialties involved and specialist advice sought as needed. • NMOSD associated with autoimmune disease is considered more severe with more frequent relapses and worse prognosis, our case highlights an example of early intervention where prompt treatment with IV steroids and early initiation of DMARD have so far provided rapid initial recovery and a period of relative stability. • Trasnverse myelitis can be one of the neurological presentations of jSLE, which can be clinically, radiologically and immunologically differentiated from coexisting NMOSD, which has implications on planning the treatment too. • Although reported in adults, coexistence of jSLE and NMOSD is exceedingly rare, limited to a handful of case reports.

Baló's concentric sclerosis: A retrospective case series.

Assessment of different types of headaches, their prevalence, and contributing factors on neuromyelitis optica spectrum disorder patients.

Longitudinally-extensive transverse myelitis in the setting of recent Mycoplasma infection: Case series.

Longitudinally extensive transverse myelitis (LETM) is an immune-mediated disorder where spinal cord lesions extend over three or more contiguous vertebral segments, classically described in neuromyelitis optica spectrum disorders. We report a case where a 36-year-old man had concurrent leptospirosis and scrub typhus infection, presenting with LETM. In previous literature, LETM has been found with isolated leptospira and orientia tsutsugamushi infection; however, this case may be a rare association of LETM with concurrent diseases.

A 25-year-old patient, born in Eritrea who had lived for 7 years in Nigeria before he arrived in France 1 year ago, was referred for subacute and progressive tetraparesis evolving over 2 months. Examination revealed tetraparesis, impairment of sensory function below the C6 level and tetrapyramidal syndrome. Spine MRI showed increased T2 intensity at the C6 to T11 spinal cord levels consistent with a longitudinally extensive transverse myelitis. After gadolinium administration, a diffuse, nodular, and heterogeneous pattern of contrast enhancement was seen. Cerebrospinal fluid disclosed pleocytosis with 14% eosinophils, associated with an elevated protein level. Exhaustive analysis ruled out bacterial, viral, mycobacterial, or fungal infection. The diagnosis of spinal schistosomiasis was confirmed by detection of S. mansoni DNA in blood and cerebrospinal fluid by polymerase chain reaction (PCR). Treatment with praziquantel was initiated. The clinical course was favourable and follow-up spine MRI at 6 months disclosed a dramatic reduction in the T2 signal abnormality without contrast enhancement. Our case is notable because of the cervicothoracic involvement and because a post-therapeutic MRI was available, confirming the patient's favourable evolution.

A 19-month-old boy presented with gait instability and lower limb weakness, initially suggestive of Guillain-Barré syndrome or transverse myelitis. Clinical features including fever, rash, conjunctivitis, sterile pyuria and elevated inflammation markers supported a diagnosis of Kawasaki disease with bilateral hip arthritis. Treatment with intravenous immunoglobulins, steroids and aspirin led to rapid clinical recovery.

Abstract: Transverse myelitis (TM) is a rare inflammatory disease that causes injury to the spinal cord resulting in clinical manifestations of weakness, sensory changes, and autonomic dysfunction.This disease can attack all age groups, but the highest age range is 10-19 years and 30-39 years.This disease is acute, and shows dominant clinical neurological abnormalities.The cause of this condition is still unknown, and the diagnosis of TM can be assessed from clinical examination, neurological examination, radiology and laboratory. Case presentation: An 11-year-old female child patient came to the West Nusa Tenggara Provincial Hospital with complaints of weakness of the limbs felt since 4 days before admission accompanied by inability to urinate, abdominal bloating and pain. The patient then underwent further examination and the patient was diagnosed with transverse myelitis, and received drug management and medical rehabilitation Conclusion: Based on this case, the patient experienced sudden weakness in all four limbs which was idiopathic and diagnosed as Transverse myelitis. Although this condition is rare, it requires immediate management and appropriate rehabilitation to maximize the patient's motor, sensory and autonomic functions, to prevent permanent disability.

The rapid rollout of mRNA-based COVID-19 vaccines, including Pfizer-BioNTech's BNT162b2 and Moderna's mRNA-1273, has been instrumental in curbing the pandemic, demonstrating high efficacy and safety in the general population. However, concerns regarding neurological adverse effects, particularly in individuals with epilepsy (PWE), warrant scrutiny. Clinical data from case reports, multicenter studies, and meta-analyses (encompassing over 3000 PWE) indicate that most tolerate vaccination well, with seizure worsening in approximately 5% of cases, often transient and lower than post-COVID-19 infection rates. Rare severe events, such as status epilepticus, highlight vulnerabilities, though background seizure incidence remains comparable or lower than natural rates. This review examines potential neuroimmune mechanisms linking mRNA vaccination to seizure exacerbation, emphasising immune activation, neuroinflammation, and epileptogenesis. mRNA vaccines utilise lipid nanoparticles (LNPs) to deliver spike protein-encoding mRNA, eliciting robust immune responses. Potential triggers for seizures include cytokine storms (e.g., IL-1β, TNF-α, IL-6), blood-brain barrier (BBB) disruption, molecular mimicry with neuronal antigens, and autoantibody production, which may heighten neuronal hyperexcitability in susceptible individuals. Neurological side effects, including Bell's palsy, transverse myelitis, and herpes zoster reactivation, are more prevalent in mRNA platforms, potentially tied to LNP-induced inflammation or cross-reactive immunity. While evidence supports vaccination benefits outweighing risks for PWE, gaps persist in understanding individual predispositions. Future research should prioritise longitudinal studies, EEG monitoring, and AI-driven approaches for personalised risk assessment, mRNA optimisation, and pharmacovigilance. Integrating multi-omics and computational modelling could enhance vaccine safety, ensuring equitable protection for vulnerable populations.

Transverse myelitis is a rare neurological manifestation of antiphospholipid antibody syndrome. Most previously reported cases present with an acute or subacute onset whereas chronic presentations are extremely rare and pose a significant diagnostic challenge. We report a case of middle-aged female with a prior history of unprovoked deep vein thrombosis of the right lower limb who presented with gradually progressive symptoms suggestive of non-compressive cervical myelopathy. Blood investigations revealed a triple-positive antiphospholipid antibodies profile with no clinical or immunological evidence of the commonly associated systemic lupus erythematosus SLE. She was treated with high-dose steroids and mycophenolate mofetil and demonstrated mild to moderate clinical improvement after six months of follow-up.

Background: Inflammatory non-MS CNS demyelinating diseases includes a heterogeneous group of diseases with varying clinical presentation, extent of neural axis involvement and phenotype severity. This study was done to describe the demographic profile and clinical phenotype of these groups of diseases in order to provide data on the local patterns of disease presentation. Methods: A cross-sectional observational study comprising 65 patients was performed at Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar between October 2022 to June 2024 and data was collected on detailed demographic and clinical profile. Appropriate statistical methods were then applied and data analysis was done. Results: Neuromyelitis Optica Spectrum Disorder (NMOSD, 40%), Myelin Oligodendrocyte Glycoprotein Antibody Disease (MOGAD, 21.5%), and Acute Disseminated Encephalomyelitis (ADEM, 13.8%) were most common, followed by idiopathic primary central nervous system (CNS) demyelination, optic neuritis (ON), and longitudinally extensive transverse myelitis (LETM). Mean age was 40.2 years; 41.5% were aged 21–40. Among 15 pediatric cases, MOGAD, ADEM, and idiopathic ON predominated. NMOSD and MOGAD showed recurrent disabling patterns affecting spinal cord and optic nerves, while ADEM and CNS demyelination involved supratentorial regions; idiopathic LETM and ON affected spine and optic nerves exclusively. Conclusion: The study reflects the clinical heterogeneity amongst various groups of non-MS CNS inflammatory demyelinating diseases and also provides data on local demographic patterns.

Neuromyelitis optica spectrum disorder (NMOSD): Recent advances and insights from Taiwan.

Transverse myelitis (TM) is a spinal cord inflammatory condition that causes immediate sensory loss and motor paralysis below the lesion. The cause of the disease is diverse, and diagnosis of TM can be challenging due to the disease's low frequency and often subtle onset. We present the case of a 45-year-old female who was referred to the neurology ward with complaints of low back discomfort, urine retention, and inability to pass stool. She also complained about numbness in her inner thighs and perianal region. There were no complaints about her upper limbs. While in the neurology ward, she had left lower extremity paralysis and sensory abnormalities that gradually worsened bilaterally, eventually leading to a diagnosis of transverse myelitis. Transverse myelitis is a rare diagnosis, but it is an important clinical consideration when examining patients who arrive with pain and increasing neurologic symptoms. Sir Salimullah Med Coll J 2024; 32: 39-43

ABSTRACT Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system, characterized by severe relapses that can lead to blindness or paralysis. However, NMOSD presents with a broad spectrum of symptoms, including optic neuritis, transverse myelitis, and area postrema syndrome, along with distinctive findings on magnetic resonance imaging. In Japan, where the prevalence of NMOSD is relatively high, nationwide surveys and regional studies have revealed its detailed epidemiological and clinical characteristics. The discovery of aquaporin‐4 autoantibodies has not only distinguished NMOSD from multiple sclerosis but also provided critical insights into its immunopathogenesis, resulting in the development of molecular‐targeted therapies capable of inhibiting key immune pathways, including the complement system, interleukin‐6 signaling, and B‐cell‐mediated immunity. Several biological agents, such as eculizumab, ravulizumab, satralizumab, inebilizumab, and rituximab, have demonstrated high efficacy in preventing relapse in clinical trials. Although long‐term safety data remain limited for most agents, Japanese real‐world data support their effectiveness and safety. This review summarizes the clinical features, imaging characteristics, and current treatment strategies for NMOSD, with a particular focus on the therapeutic mechanisms of biological agents and the insights they provide into disease pathophysiology.

A rare case of West Nile virus leading to Acute Transverse Myelitis

We describe a case of tumefactive demyelinating lesion (TDL) that occurred during B-cell depletion therapy by ofatumumab and discuss its pathogenesis through serial magnetic resonance imaging and biopsied histopathology. Ten months after initiating subcutaneous ofatumumab following two episodes of transverse myelitis, the patient developed a large tumefactive lesion with closed-ring contrast-enhancement and intralesional microhemorrhages and was diagnosed as having a TDL by brain biopsy. After repeated intravenous methylprednisolone and plasma exchanges, the TDL gradually shrank, although a perilesional hypointense ring remained on susceptibility-weighted images. TDLs can occur even during B-cell depletion therapy; however, the underlying mechanism is not fully elucidated.