Effectiveness of immunotherapies in relapsing myelin oligodendrocyte glycoprotein antibody-associated disease.

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can cause optic neuritis, transverse myelitis, or acute disseminated encephalomyelitis (ADEM). Immunotherapy is often used for relapsing disease, but there is variability in treatment decisions. OBJECTIVE: The objective was to determine the annualized relapse rates (ARRs) and incidence rate ratios (IRRs) compared to pre-treatment and relapse-freedom probabilities among patients receiving steroids, B-cell depletion (BCD), intravenous immunoglobulin (IVIG), and mycophenolate mofetil (MMF). METHODS: Retrospective cohort study of patients with relapsing MOGAD treated at Mass General Brigham. ARRs and IRRs compared to pre-treatment, and relapse-freedom probability and odds ratio for relapse-freedom compared to prednisone were calculated. RESULTS: A total of 88 patients met the inclusion criteria. The ARR on IVIG was 0.13 (95% confidence interval (CI) = 0.06-0.27) and the relapse-freedom probability after at least 6 months of therapy was 72%. The ARR on BCD was 0.51 (95% CI = 0.34-0.77), and the relapse-freedom probability was 33%. The ARR on MMF was 0.32 (95% CI = 0.19-0.53) and the relapse-freedom probability was 49%. In pediatric-onset disease, MMF had the lowest ARRs (0.15, 95% CI = 0.07-0.33). CONCLUSION: IVIG had the lowest ARRs and IRRs compared to pre-treatment and the highest relapse-freedom odds ratio compared to prednisone, while BCD had the lowest. In pediatric-onset MOGAD, MMF had the lowest ARRs.

Neuromuscular Alterations Associated with COVID-19. A Scientific Literature Review

Introduction: The COVID-19 pandemic has brought short, medium, and long-term consequences on the quality of life of those affected. Among the complications are those related to the involvement of the nervous system and the structures involved in body movement, with sequelae that may be transitory and/or definitive, and require rehabilitation. Objective: Identify the neuromuscular alterations that affect body movement, associated with COVID-19. Material and methods: A search was made for observational works published in the SCOPUS, PubMed, EBSCO, and Nature databases between January 2020 and June 2022 under the PRISMA methodology, to answer the PICO question: what are the neuromuscular alterations that can potentially affect movement, associated with COVID-19? The established filters were type of study, language, age, availability, publication dates. The MeSH terms were SARS-Cov-2, COVID-19, Long Covid, Motor Activity Neuromuscular Diseases, Neurological Disorders, Guillain-Barré Syndrome, Myelitis Transverse, Stroke, Patient, Peripheral Nervous System Diseases. The methodological quality was evaluated according to STROBE and the level of evidence was established according to CEBM. Results: In the first search, 645 articles were identified. 637 were discarded by filters, titles, duplicate abstracts, methodological quality, and level of evidence. There were 8 articles selected for the present review in which neuromuscular alterations of central and peripheral origin were identified, such as myalgias, fatigue, polyneuroradiculopathies, CNS inflammation, among others, with clinical manifestations that affect movement. Conclusion: COVID-19 is a multisystemic disease that can affect the nervous system with symptoms of neuromuscular alterations that compromise body movement.

Introducción: La pandemia por la COVID-19 ha traído consecuencias a corto, mediano y largo plazo sobre la calidad de vida de los afectados. Entre las complicaciones se encuentran aquellas relacionadas con la afectación del sistema nervioso y las estructuras involucradas en el movimiento corporal, con secuelas que pueden ser transitorias y/o definitivas, y requieren rehabilitación Objetivo: Identificar las alteraciones neuromusculares que afectan el movimiento corporal, asociadas a la COVID-19. Material y métodos: Se hizo una búsqueda de trabajos observacionales publicados en las bases de datos SCOPUS, PubMed, EBSCO y Nature entre enero de 2020 y junio de 2022 bajo metodología PRISMA, para dar respuesta a la pregunta PICO: ¿cuáles son las alteraciones neuromusculares que potencialmente pueden afectar el movimiento, asociadas a la COVID-19? Los filtros establecidos fueron tipo de estudio, idioma, edad, disponibilidad y fechas de publicación. Los términos MesH fueron SARS-Cov-2, COVID-19, Long Covid, Motor Activity Neuromuscular Diseases, Neurological Disorders, Guillain-Barré Syndrome, Myelitis Transverse, Stroke, Patient, Peripheral Nervous System Diseases. La calidad metodológica se evaluó según STROBE y el nivel de evidencia se estableció según CEBM. Resultados: En la primera búsqueda se identificaron 645 artículos. Posteriormente se descartaron 637 por filtros, títulos, resúmenes duplicados, calidad metodológica y nivel de evidencia. Así, quedaron seleccionados 8 para la presente revisión, en los cuales se identificaron alteraciones neuromusculares de origen central y periférico, como mialgias, fatiga, polineuroradiculopatías, inflamación del SNC, entre otras, con manifestaciones clínicas que afectan el movimiento. Conclusión: La COVID-19 es una enfermedad multisistémica que puede afectar el sistema nervioso con síntomas de alteraciones neuromusculares que comprometen el movimiento corporal.

Mielite transversa por Zika vírus em paciente com Lúpus Eritematoso Sistêmico (LES) - relato de caso em Manaus - AM / Transverse myelitis by Zika virus in patient with Systemic Lupus Erythematosus (SLE) - case report in Manaus - AM

Objetivo: Relatar caso clinico de uma paciente com Mielite Transversa diagnosticada com Lúpus Eritematoso Sistêmico e Zika Vírus. Relato de Caso: Paciente diagnosticada em Lúpus Eritematoso Sistêmico (LES) com nefrite lúpica classe IV há 15 anos em remissão, iniciou quadro de mialgia difusa prejudicando deambulação. Após melhora espontânea do quadro, paciente permaneceu com queixa de astenia e cefaleia intensa unilateral esquerda recorrente, evoluindo com síncope, paraparesia em membro inferior em caráter progressivo ascendente seguido de crise convulsiva tipo tônico clônico generalizado de inicio disruptivo. Um primeiro exame do liquor cefalorraquidiano (LCR) foi solicitado, sem evidência de alteração na analise bioquímica simples. Outras alterações laboratoriais foram identificadas, além do método de reação em cadeia de polimerase (PCR) para Zika vírus na urina e liquor cefalorraquqidiano (após recoleta) detectáveis. Foram detectadas alterações à ressonância magnética compatíveis com mielite transversa. A investigação etiológica durou dois meses e meio, com duas internações no período. Apesar das manifestações neurológicas do Zika Vírus serem ordinariamente inespecíficas, raras e brandas, não se deve desconsiderar a hipótese diagnostica de mielite transversa em área endêmica para arboviroses com manifestações neurológicas mesmo após liquor cefalorraquidiano e outros exames inespecíficos com o achado clínico, considerando paciente remissiva do quadro de lupus eritematoso sistêmico há 15 anos

Objective: case report of a patient with transverse myelitis diagnosed with systemic lupus erythematosus and zika Virus. Case report: Patient diagnosed with Systemic Lupus Erythematosus (SLE) with class IV lupus nephritis for 15 years in remission, begins to have diffuse myalgia, impairing walking. After spontaneous improvement of the condition, the patient remained complaining of asthenia and recurrent left unilateral severe headache, evolving with syncope, progressive ascending paraparesis in the lower limb, followed by a generalized tonic clonic seizure type of disruptive onset. A first examination of the cerebrospinal fluid (CSF) was requested, without evidence of alteration in the simple biochemical analysis. Other laboratory alterations were identified, in addition to the polymerase chain reaction (PCR) for detectable Zika virus in urine and cerebrospinal liquor (after collection). Changes were detected on magnetic resonance imaging (MRI) compatible with transverse myelitis. The etiological investigation lasted two and a half months, with two hospitalizations in the period. Although the neurological manifestations of Zika Virus are ordinarily nonspecific, rare and mild, the diagnostic hypothesis of transverse myelitis in an endemic area for arboviruses with neurological manifestations should not be disregarded even after cerebrospinal fluid and unspecific with the other clinical, remissive of the clinical picture lupus erythemasous systemic 15 years ago.

Longitudinal extensive transverse myelitis following ChAdOx1 nCOV-19 vaccine: a case report.

BACKGROUND: Transverse myelitis (TM) is a relatively uncommon condition, and vaccine-associated myelitis is even rarer. Concern regarding neurological complications following vaccination escalated following the report of TM during the safety and efficacy trials of the COVID-19 vaccine. CASE PRESENTATION: We report the first case of Longitudinal Extensive Transverse Myelitis (LETM) in Malaysia following administration of the chimpanzee adenovirus-vectored (ChAdOx1 nCoV-19) vaccine. A 25-year-old female presented with bilateral lower limb weakness and inability to walk with a sensory level up to T8 with absent visual symptoms. Urgent gadolinium-enhanced magnetic resonance imaging (MRI) of the spine showed long segment TM over the thoracic region. Cerebrospinal fluid autoantibodies for anti-aquaporin-4 and anti-myelin-oligodendrocyte were negative. A diagnosis of LETM following vaccination was made, and the patient was started on a high dose of intravenous methylprednisolone. The patient eventually made a recovery following treatment. CONCLUSION: LETM is a rare but serious adverse reaction following vaccination. Previously reported cases showed an onset of symptoms between 10 to 14 days post-vaccination, suggesting a delayed immunogenic reaction. However, the incidence of myelitis in COVID-19 is much more common, far greater than the risk associated with vaccination.

An Anti-aquaporin 4 Positive Longitudinally Extensive Transverse Myelitis with Antecedent Necrotizing Pneumonia Associated with S. aureus

A 53-year-old male patient presented with quadriparesis during pulmonological hospitalization for the treatment of S. aureus associated necrotizing pneumonia. He was diagnosed with the aquaporin-4 (AQP4) positive longitudinally extensive transverse myelitis from pons to T3 level. Despite the administration of intravenous methylprednisolone and plasma exchange with appropriate antibiotics, the patient's neurological condition deteriorated and he died. Our case implies that an S. aureus associated necrotizing pneumonia can trigger an AQP4 positive neuromyelitis optica spectrum disorder and contribute to the devastating course.

Clinical features of late-onset neuromyelitis optica spectrum disorders / 天津医药

Objective To explore the clinical features of late-onset neuromyelitis optica spectrum disorders (LON?MOSD). Methods A retrospective analysis was performed to evaluate 61 patients with LONMOSD admitted to our hospital from January 2010 to May 2015. Results (1) The median age at onset was 57 (53, 63) years, male/female was 1∶3.7. Thirty-two patients (52.5%) had transverse myelitis (TM) and 16 patients (26.2%) had optic neuritis (ON) at the disease onset. Fifty-one patients (83.6%) experienced recurrent attacks. Forty patients (65.6%) showed abnormal brain magnetic resonance imag?ing (MRI). Spinal cord MRI showed more frequently present in thoracic regions (39.3%). (2) There were no significant differ?ences in clinical features between AQP-4 seropositive and seronegative groups. (3) By Spearman analysis, it was obvious that EDSS scores at acute phase and remission were positively correlated to AQP-4 antibody levels (rs=0.389, P0.05;rs=0.096, P>0.05). Conclusion LONMOSD patients are more prone to present with TM at onset and have more lesions in thoracic spinal cord and brain. The AQP-4 antibody titres can indicate the severity of disease in acute phase.

Clinical insights for early detection of acute transverse myelitis in the emergency department.

OBJECTIVE: Acute transverse myelitis (ATM) is characterized by motor weakness, sensory changes, and autonomic dysfunction. However, diagnosis of ATM is based on early-stage clinical features only (and clarification of the cause of disease), which are difficult for emergency department (ED) physicians owing to low incidence rates. We performed retrospective analysis of ATM in order to provide clinical insights for early detection. METHODS: Medical records of patients, who were finally diagnosed with ATM from January 2005 to February 2013, were investigated. Data, including demographics, clinical findings, and radiographic findings, were reviewed. RESULTS: Forty-six patients were included in the present study, with a mean age of 43.4 years. Sensory changes were identified in 45 patients (97.8%), motor weakness in 33 patients (71.7%), and autonomic dysfunction in 35 patients (76.1%). Thirty patients (65.2%) showed high signal intensity in T2-weighted magnetic resonance imaging (MRI), with lesions most frequently found in the thoracic level of the spinal cord (56.7%). There were discrepancies between sensory changes and levels of MRI lesions. Thirty-five patients (76.1%) were diagnosed with idiopathic ATM. Initial diagnostic impressions in the ED were herniated intervertebral disc (38.7%), stroke (19.4%), Guillain-Barré syndrome (12.9%), cauda equina syndrome (9.7%), ATM (9.7%), and others (9.7%). CONCLUSION: When a patient presents with motor weakness, sensory changes, or autonomic dysfunction, ATM should be initially considered as a differential diagnosis, unless the ED physician's impression after initial evaluation is clear.

Acute Transverse Myelitis in a Patient with Rheumatoid Arthritis Treated with Intravenous Immunoglobulin / 대한내과학회지

Acute transverse myelitis (ATM) is a heterogeneous syndrome characterized by acute spinal cord dysfunction resulting in paresis and sensory and autonomic impairment below the level of the lesion. The etiology of ATM includes parainfectious, paraneoplastic, drug-induced, systemic autoimmune disorders, and acquired demyelinating diseases. ATM is very rare in patients with rheumatoid arthritis (RA). A 79-year-old woman with RA, a recurring peptic ulcer, and congestive heart failure presented with acute weakness of both upper and lower extremities. She was diagnosed with ATM based on the results of laboratory and radiological tests. ATM is usually treated with high-dose glucocorticoid. However, we treated her with intravenous immunoglobulin (IVIG) considering the adverse events of high-dose glucocorticoid, and she improved. We report a case of ATM in a patient with RA successfully treated with IVIG.

Mielitis transversa asociada a virus herpes tipo-1 / Associated transverse myelitis herpes virus type -1

La mielitis transversa (MT) es un proceso inflamatorio que involucra áreas restringidas de la médula espinal (ME) ygenera síntomas motores, sensitivos y autonómicos. El término mielitis transversa aguda estaba reservado para los casos idiopáticos, pero en la actualidad se usa para abarcar el síndrome clínico general, así se desconozca su etiología.El virus herpes simple raramente se ha reportado como causa de mielitis. En pacientes inmunosuprimidos se ha encontrado que el virus herpes tipo 1 causa encefalitis y el herpes tipo 2 causa con mayor frecuencia mielitis.Aunque muchos pacientes presentan secuelas, un diagnóstico y tratamiento oportunos; combinando terapia antiviraly esteroides; disminuye el daño del sistema nerviosos central y mejoran la calidad de vida.

Transverse myelitis is an inflammatory process who involved restricted areas of the spinal cord causing motor,sensory and autonomic symptoms. The term acute transverse myelitis was reserved for idiopathic cases, but currently is used for the entire clinical syndrome include there of unknown cause.The herpes simplex virus has been rarely reported as a cause of myelitis.In Inmunosupressed patients the Herpes type 1 virus causes encephalitis and type 2 herpes most often cause myelitis.Although many patients have sequels, early diagnosis and treatment; combining antiviral therapy and steroids;decreases central nervous system damage and improved the quality of life.

Serum uric acid level and related clinical features in high risk syndrome of neuromyelitis optica / 中华神经科杂志

Objective To investigate serum uric acid (UA) levels and related clinical features in patients with high risk syndrome of neuromyelitis optica. Methods UA levels were measured in 51 patients with high risk syndrome of neuromyelitis optica including 34 with longitudinally extensive transverse myelitis (LETM) and 17 with optic neuritis (ON), 48 with neuromyelitis optica (NMO), 45 with other neurological diseases (OND) and 65 with healthy controls (HC). The disability severity was assessed by the expanded disability status scale (EDSS). Spinal lesions were viewed by MRI. Serum aquaporin-4(AQP4) antibody was tested in cell based immunofluorescence assay. Results Serum UA levels in LETM ( ( 189. 84 ±85. 65) μmol/L) and ON patients ( (222. 12 ±61.68) μmol/L) were significantly lower than that in OND ((315.90±71.36) μ mol/L) and HC ((291.05 ±76.64) μ mol/L) subjects (P＜0.01). No difference was found between LETM, ON and NMO groups. UA levels were significantly lower in females ( ( 158.24 ±55.92), (187.00±47.52), (198.21 ±62.62), (274.51 ±70.66)and (243.26±60.65) μmol/L)than in males ( ( 262. 09 ± 101.63 ), ( 262. 45 ± 62. 13 ), ( 298.90 ± 74. 14 ), ( 355.37 ± 50. 30 ) and (340. 34 ±58. 23) μmol/L) in all groups (t=3. 183, 2.578, 4.356, 4.365 and 6.579, all P＜0.05).UA levels in patients with high risk syndrome of NMO were not correlated with mono or relapse course,duration or status of serum AQP4 antibody. UA were negatively correlated with EDSS in patients with LETM (r= -0.714, P＜0.01). Conclusion Lower serum UA levels were found in patients with high risk syndrome of NMO and related to more severe symptoms in LETM group.

Neuromielitis óptica. Reporte de caso / Neuromyelitis Optica. Case Report

La neuromielitis óptica, también conocida como síndrome de Devic, es una enfermedad que combina la neuritis óptica y la mielitis transversa. Hace unos anos era considerada una forma de esclerosis múltiple; sin embargo, realmente es una enfermedad diferente, con características clínicas, imágenes, serología e inmunopatologia propias. El presente artículo presenta el caso de una mujer de 29 anos con un cuadro clínico que inicia en el quinto mes de postparto, con pérdida progresiva de la fuerza en miembros inferiores, asociada a perdida de la agudeza visual bilateral. El objetivo es hacer, mediante el estudio de caso, una revisión amplia de esta enfermedad, poco común, mediada por un proceso inmune y desmielinizante. Para ello, se resumen los parámetros epidemiológicos mas importantes y se presentan las posibilidades diagnosticas y terapéuticas disponibles actualmente.

Neuromyelitis Optical, also known as Devic’s Syndrome, is a disease which combines optic neuritis and transverse myelitis. Some years ago it was considered as a form of multiple sclerosis. Actually, it is consider as a different disease, on the basis of the clinical, imaging, serology and immunopatholoy profile. A case of 29 years old female patient is reported, based on her clinical findings which began in the fifth postpartum month, with progressive lower limb paresis, associated with bilateral vision loss. This paper attempts giving a synoptic overview of this uncommon immune mediated demyelinating condition; it summarises the most important epidemiological parameters and presents the diagnostic and therapeutic possibilities available today.

A case of acute transverse myelitis following chickenpox / 소아과

Acute transverse myelitis (ATM) in most patients is characterized by an abrupt onset of progressive weakness and sensory disturbance in the lower extremities with a preceding viral infection such as Epstein-Barr, herpes simplex, influenza, mumps and Varicella-zoster viruses (VZV). Although less frequent, some residual deficits including bladder dysfunction or weakness in the lower extremities may follow ATM, from which recovery usually begins within the first week of the onset of symptoms. In this report, we describe the case of a 9-year-old girl who experienced ATM following chickenpox and had bladder dysfunction as a sequela.

A Catastrophic-Onset Longitudinal Myelitis Accompanied by Bilateral Internuclear Ophthalmoplegia in a Patient with Systemic Lupus Erythematosus

Transverse myelitis (TM) extending from midbrain to the entire spinal cord accompanied by internuclear ophthalmoplegia is extremely rare but cause serious central nervous system complications in patients with systemic lupus erythematosus. We report a case of a 28-yr-old woman with TM extending from the midbrain to the conus medullaris longitudinally and internuclear ophthalmoplegia associated with systemic lupus erythematosus. Her neurological symptoms had an abrupt catastrophic onset and rapidly progressed to respiratory failure within 24 hr. Bilateral internuclear ophthalmoplegia was also followed by TM. Brain MR images showed definite brainstem lesions, which were deeply associated with internuclear ophthalmoplegia, and diffuse signal changes in the whole spinal cord, medulla, pons and midbrain. Clinical improvement of her ophthalmoplegia and of neurological dysfunction of the upper extremities was noted after prompt and aggressive treatment with intravenous pulsed methylprednisolone and cyclophosphamide. However, the neurological dysfunction of the lower limbs and bladder and colon paralysis were almost unchanged until six months passed.