Asunto(s)
Meningitis Bacterianas , Mielitis Transversa , Infecciones Estreptocócicas , Streptococcus pyogenes , Humanos , Infecciones Estreptocócicas/complicaciones , Infecciones Estreptocócicas/diagnóstico , Streptococcus pyogenes/aislamiento & purificación , Mielitis Transversa/diagnóstico , Mielitis Transversa/etiología , Meningitis Bacterianas/complicaciones , Meningitis Bacterianas/microbiología , Meningitis Bacterianas/diagnóstico , Masculino , FemeninoRESUMEN
BACKGROUND: Retrospective studies suggest that spinal movement disorders, especially tonic spasms, are prevalent in NMOSD. However, there have been no prospective studies evaluating spinal movement disorders in NMOSD, MOGAD, and idiopathic transverse myelitis (ITM). METHODS: Patients referred to a tertiary neuroimmunology clinic for spinal cord demyelination (excluding MS) were evaluated. All patients answered a movement disorders survey and underwent a movement disorder-focused exam. Movement disorders were compared among patients with NMOSD with and without AQP4-IgG, MOGAD, and ITM. Patients with and without involuntary movements were also compared to identify predictors of spinal movement disorders. RESULTS: Sixty-three patients were evaluated from 2017 to 2021 (71% females, median age 49 years, range 18-72 years, median disease duration 12 months, range 1-408). Of the total, 49% had ITM, 21% had NMOSD without AQP4-IgG, 19% had NMOSD with AQP4-IgG, and 11% had MOGAD. Movement disorders were present in 73% of the total patients and were most frequent in NMOSD with AQP4-IgG (92%) and least frequent in MOGAD (57%). The most frequent spinal movement disorders were tonic spasms (57%), focal dystonia (25%), spinal tremor (16%), spontaneous clonus (9.5%), secondary restless limb syndrome (9.5%), and spinal myoclonus (8%). Multivariate analysis showed that longitudinally extensive myelitis and AQP4-IgG are independent risk factors for the development of spinal movement disorders, while MOG-IgG and African American race were associated with a lower risk of developing these movement disorders. CONCLUSIONS: Spinal movement disorders are highly prevalent in non-MS demyelinating disorders of the spinal cord. Prevalence rates exceed those reported in MS and retrospective NMOSD studies.
Asunto(s)
Acuaporina 4 , Trastornos del Movimiento , Mielitis Transversa , Neuromielitis Óptica , Humanos , Persona de Mediana Edad , Femenino , Mielitis Transversa/epidemiología , Adulto , Masculino , Anciano , Adolescente , Adulto Joven , Neuromielitis Óptica/epidemiología , Estudios Prospectivos , Trastornos del Movimiento/epidemiología , Acuaporina 4/inmunologíaRESUMEN
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.
Asunto(s)
Imagen por Resonancia Magnética , Mielitis Transversa , Humanos , Mielitis Transversa/diagnóstico por imagen , Mielitis Transversa/diagnóstico , Masculino , Femenino , Imagen por Resonancia Magnética/métodos , Pronóstico , Adulto , Persona de Mediana Edad , Médula Espinal/diagnóstico por imagen , Médula Espinal/patología , Estudios RetrospectivosAsunto(s)
Glicoproteína Mielina-Oligodendrócito , Mielitis Transversa , Humanos , Mielitis Transversa/tratamiento farmacológico , Mielitis Transversa/inmunología , Mielitis Transversa/diagnóstico , Glicoproteína Mielina-Oligodendrócito/inmunología , Femenino , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Imagen por Resonancia Magnética , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificación , Masculino , Resultado del Tratamiento , AdultoRESUMEN
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.
Asunto(s)
Enfermedades Desmielinizantes , Humanos , Nigeria/epidemiología , Niño , Femenino , Masculino , Adolescente , Preescolar , Estudios Retrospectivos , Lactante , Enfermedades Desmielinizantes/epidemiología , Enfermedades Desmielinizantes/diagnóstico , Imagen por Resonancia Magnética , Incidencia , Resultado del Tratamiento , Mielitis Transversa/epidemiología , Mielitis Transversa/diagnóstico , Neuritis Óptica/epidemiología , Neuritis Óptica/diagnósticoRESUMEN
BACKGROUND: Transverse myelitis (TM) is a demyelinating inflammatory disease that presents with motor, sensory, and autonomic dysfunction, which may be acute or subacute. COVID-19-associated TM has been described in a scarce number of patients. CLINICAL CASE: A 15-year-old previously healthy male patient with respiratory disease before his neurological deterioration presented to the emergency room after developing a complete medullary syndrome located at the cervical-dorsal level, with ascending and symmetric paraparesis that rapidly progressed to paraplegia, with sensory dysfunction from the T3 level, sphincter dysfunction and sudden ventilatory deterioration that required mechanical ventilation. Magnetic resonance imaging was compatible with acute TM. Inflammatory and non-inflammatory etiologies were discarded. In addition, a positive severe acute respiratory syndrome coronavirus 2 test was obtained. Treatment included steroid pulses and plasmapheresis, with an insidious evolution. CONCLUSION: COVID-19 is an infrequent cause of TM and should be suspected when other etiologies have been ruled out.
INTRODUCCIÓN: La mielitis transversa (MT) es una enfermedad inflamatoria desmielinizante que se presenta con disfunción motora, sensitiva y autonómica, de forma aguda o subaguda. La MT asociada al COVID-19 se ha escrito en un escaso número de pacientes. CASO CLÍNICO: Se presenta el caso de un masculino de 15 años previamente sano, quien cursaba con un cuadro respiratorio y que desarrollo un deterioro neurológico súbito que involucro un síndrome medular completo localizado en el nivel cérvico dorsal, con paraparesia simétrica que progreso a la paraplejia, con disfunción sensitiva desde el nivel medular de T3, disfunción de esfínteres y deterioro ventilatorio que requirió manejo avanzado de la vía aérea. Su resonancia magnética fue compatible con mielitis transversa aguda. Se descartaron causas inflamatorias y no inflamatorias de la patología. Además, se obtuvo un resultado positivo de SARS-COV-2. Se inició tratamiento con pulsos de metilprednisolona y plasmaféresis, con una evolución insidiosa. CONCLUSIÓN: El COVID-19 es una causa infrecuente de MT y debe sospecharse cuando otras causas han sido descartadas.
Asunto(s)
COVID-19 , Imagen por Resonancia Magnética , Mielitis Transversa , Humanos , Mielitis Transversa/diagnóstico , Mielitis Transversa/virología , Mielitis Transversa/terapia , COVID-19/complicaciones , COVID-19/diagnóstico , Masculino , Adolescente , Plasmaféresis/métodos , Respiración Artificial , Paraplejía/etiología , Paraplejía/virología , Paraparesia/etiologíaRESUMEN
Aims/Background Although electromyography has been extensively used in the diagnosis of neurological diseases, there is no comprehensive understanding of the electromyography manifestations of spinal dural arteriovenous fistula. Given the widespread use of electromyography in the diagnosis of neurological conditions, it is worthwhile to holistically analyse the electromyography findings of spinal dural arteriovenous fistula to differentiate it from neurological diseases that share similar clinical manifestations. The aim of this study is to evaluate whether electromyography can distinguish spinal dural arteriovenous fistula from longitudinally extensive transverse myelitis. Methods We holistically reviewed files of all patients who were diagnosed with spinal dural arteriovenous fistula or longitudinally extensive transverse myelitis at The First Medical Centre of PLA General Hospital from 1 January 2010 to 31 December 2020. We compared the symptomology, epidemiology, and imaging results of patients with spinal dural arteriovenous fistula and longitudinally extensive transverse myelitis, placing emphasis on their electromyography manifestations. Student's t test was used to analyse normally distributed data, while Chi-square test was used to compare classification statistics. Results Lesions of spinal dural arteriovenous fistula shown on images tend to appear at lower lumbar and sacral segments, whereas lesions of the cervical and upper thoracic segments are more characteristic of longitudinally extensive transverse myelitis. Spinal dural arteriovenous fistula patients and longitudinally extensive transverse myelitis patients overlap in terms of clinical manifestations. After comparison, the two groups of patients had different demographics (age, sex), onset mode, predisposing factors before onset, and electromyographic features. The electromyographic features of patients with spinal dural arteriovenous fistula were associated with neurogenic damage (p < 0.001). Conclusions In patients with spinal dural arteriovenous fistula, electromyography can help clinicians to identify early disease, avoid patient treatment delay, and eliminate unnecessary treatment.
Asunto(s)
Malformaciones Vasculares del Sistema Nervioso Central , Electromiografía , Mielitis Transversa , Humanos , Electromiografía/métodos , Masculino , Femenino , Mielitis Transversa/diagnóstico , Malformaciones Vasculares del Sistema Nervioso Central/complicaciones , Malformaciones Vasculares del Sistema Nervioso Central/fisiopatología , Malformaciones Vasculares del Sistema Nervioso Central/diagnóstico , Estudios Retrospectivos , Persona de Mediana Edad , Adulto , Anciano , Diagnóstico Diferencial , Enfermedades de la Médula Espinal/diagnóstico , Enfermedades de la Médula Espinal/fisiopatología , Imagen por Resonancia Magnética/métodosRESUMEN
BACKGROUND: Pediatric acute transverse myelitis (ATM) accounts for 20-30% of children presenting with a first acquired demyelinating syndrome (ADS) and may be the first clinical presentation of a relapsing ADS such as multiple sclerosis (MS). B cells have been strongly implicated in the pathogenesis of adult MS. However, little is known about B cells in pediatric MS, and even less so in pediatric ATM. Our lab previously showed that plasmablasts (PB), the earliest B cell subtype producing antibody, are expanded in adult ATM, and that these PBs produce self-reactive antibodies that target neurons. The goal of this study was to examine PB frequency and phenotype, immunoglobulin selection, and B cell receptor reactivity in pediatric patients presenting with ATM to gain insight to B cell involvement in disease. METHODS: We compared the PB frequency and phenotype of 5 pediatric ATM patients and 10 pediatric healthy controls (HC) and compared them to previously reported adult ATM patients using cytometric data. We purified bulk IgG from the plasma samples and cloned 20 recombinant human antibodies (rhAbs) from individual PBs isolated from the blood. Plasma-derived IgG and rhAb autoreactivity was measured by mean fluorescence intensity (MFI) in neurons and astrocytes of murine brain or spinal cord and primary human astrocytes. We determined the potential impact of these rhAbs on astrocyte health by measuring stress and apoptotic response. RESULTS: We found that pediatric ATM patients had a reduced frequency of peripheral blood PB. Serum IgG autoreactivity to neurons in EAE spinal cord was similar in the pediatric ATM patients and HC. However, serum IgG autoreactivity to astrocytes in EAE spinal cord was reduced in pediatric ATM patients compared to pediatric HC. Astrocyte-binding strength of rhAbs cloned from PBs was dependent on somatic hypermutation accumulation in the pediatric ATM cohort, but not HC. A similar observation in predilection for astrocyte binding over neuron binding of individual antibodies cloned from PBs was made in EAE brain tissue. Finally, exposure of human primary astrocytes to these astrocyte-binding antibodies increased astrocytic stress but did not lead to apoptosis. CONCLUSIONS: Discordance in humoral immune responses to astrocytes may distinguish pediatric ATM from HC.
Asunto(s)
Astrocitos , Mielitis Transversa , Humanos , Mielitis Transversa/inmunología , Animales , Femenino , Astrocitos/metabolismo , Astrocitos/inmunología , Niño , Ratones , Masculino , Adolescente , Células Plasmáticas/inmunología , Células Plasmáticas/metabolismo , Autoanticuerpos/inmunología , Autoanticuerpos/sangre , Ratones Endogámicos C57BL , Células Cultivadas , Preescolar , Inmunoglobulina G/inmunología , Inmunoglobulina G/sangre , Médula Espinal/metabolismo , Médula Espinal/inmunología , Médula Espinal/patologíaRESUMEN
A 57-year-old man presented with subacute-onset paraparesis, bilateral dysesthesia in his lower extremities, and bladder/bowel disturbance six weeks after being infected with severe acute respiratory syndrome coronavirus 2 infection [coronavirus disease 2019 (COVID-19)]. A neurological examination suggested transverse myelitis at the level of the lower thoracic spinal cord. However, repeated spinal magnetic resonance imaging (MRI) showed no abnormalities in the spinal cord. Laboratory and cerebrospinal fluid (CSF) tests ruled out other etiologies of myelitis, eventually suggesting COVID-19-associated myelitis. Aggressive immunosuppressive therapy, started soon after hospitalization, dramatically improved his symptoms. Early aggressive immunosuppressive therapy should therefore be considered in cases of MRI/CSF-negative myelitis associated with COVID-19.
Asunto(s)
COVID-19 , Inmunosupresores , Imagen por Resonancia Magnética , Humanos , Masculino , Persona de Mediana Edad , COVID-19/complicaciones , COVID-19/diagnóstico por imagen , Inmunosupresores/uso terapéutico , SARS-CoV-2 , Mielitis/diagnóstico por imagen , Mielitis/tratamiento farmacológico , Mielitis/líquido cefalorraquídeo , Mielitis/etiología , Mielitis Transversa/diagnóstico por imagen , Mielitis Transversa/tratamiento farmacológico , Mielitis Transversa/etiología , Resultado del Tratamiento , Médula Espinal/diagnóstico por imagen , Médula Espinal/patologíaRESUMEN
BACKGROUND: Spinal cord is one of the prominent targets of autoimmune mechanisms in Neuromyelitis Optica Spectrum Disorder (NMOSD). Rarely, NMOSD causes damage to the entire length of the spinal cord, from cervical segments to conus medullaris, which has not been characterized in the existing literature. MATERIAL AND METHOD: We reviewed medical records, demographic information, and magnetic resonance imaging (MRI) sequences of 174 NMOSD patients from January 2011 to January 2023 who were admitted to Isfahan Multiple Sclerosis center to find patients with whole spinal transverse myelitis (TM). RESULTS: Whole spinal TM was present in five patients (2.9 %). Three patients were seropositive for Aquaporin-4 (AQP4) antibody; Myelin Oligodendrocyte Glycoprotein antibody (MOG IgG) tested negative for all of them. Lower limb weakness was the most frequent clinical complaint. Two patients presented with optic neuritis; One patient reported having episodes of nausea and vomiting. These patients, overall, yielded a higher expanded disability status scale (EDSS) score than the other NMOSD patients. CONCLUSION: Whole spinal TM is a rare finding in NMOSD, which is strongly associated with a higher severity and a worse outcome of the disease. The role of anti-AQP4 antibodies in the extent of myelitis in NMOSD has yet to be investigated.
Asunto(s)
Acuaporina 4 , Mielitis Transversa , Neuromielitis Óptica , Humanos , Neuromielitis Óptica/diagnóstico por imagen , Neuromielitis Óptica/complicaciones , Neuromielitis Óptica/inmunología , Mielitis Transversa/diagnóstico por imagen , Mielitis Transversa/inmunología , Femenino , Adulto , Masculino , Persona de Mediana Edad , Acuaporina 4/inmunología , Imagen por Resonancia Magnética , Adulto Joven , Estudios Retrospectivos , Médula Espinal/diagnóstico por imagen , Médula Espinal/patología , Autoanticuerpos/sangreRESUMEN
A woman in her 40s presented with thoracic banding dysaesthesia and lower motor neuron weakness. Spinal imaging revealed a short segment of transverse myelitis and neurophysiology was suggestive of concurrent acute inflammatory demyelinating polyneuropathy. The patient improved with consecutive intravenous immunoglobulin and methylprednisolone treatment. Acute inflammatory demyelinating polyneuropathy is a progressive immune-mediated peripheral neuropathy which responds to intravenous immunoglobulin or plasmapheresis, whereas transverse myelitis is a central inflammatory syndrome usually treated with corticosteroid. We highlight differentiating features of the clinical presentation and the utility of investigations such as neurophysiology and MRI along with a review of treatment and the role for corticosteroid therapy.
Asunto(s)
Síndrome de Guillain-Barré , Inmunoglobulinas Intravenosas , Imagen por Resonancia Magnética , Metilprednisolona , Mielitis Transversa , Humanos , Mielitis Transversa/diagnóstico , Mielitis Transversa/complicaciones , Mielitis Transversa/tratamiento farmacológico , Femenino , Inmunoglobulinas Intravenosas/uso terapéutico , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificación , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/complicaciones , Síndrome de Guillain-Barré/terapia , Síndrome de Guillain-Barré/tratamiento farmacológico , Adulto , Diagnóstico DiferencialRESUMEN
BACKGROUND: While safety of influenza vaccines is well-established, some studies have suggested potential associations between influenza vaccines and certain adverse events (AEs). This study examined the safety of the 2022-2023 influenza vaccines among U.S. adults ≥ 65 years. METHODS: A self-controlled case series compared incidence rates of anaphylaxis, encephalitis/encephalomyelitis, Guillain-Barré Syndrome (GBS), and transverse myelitis following 2022-2023 seasonal influenza vaccinations (i.e., any, high-dose or adjuvanted) in risk and control intervals among Medicare beneficiaries ≥ 65 years. We used conditional Poisson regression to estimate incidence rate ratios (IRRs) and 95 % confidence intervals (CIs) adjusted for event-dependent observation time and seasonality. Analyses also accounted for uncertainty from outcome misclassification where feasible. For AEs with any statistically significant associations, we stratified results by concomitant vaccination status. RESULTS: Among 12.7 million vaccine recipients, we observed 76 anaphylaxis, 276 encephalitis/encephalomyelitis, 134 GBS and 75 transverse myelitis cases. Only rates of anaphylaxis were elevated in risk compared to control intervals. With all adjustments, an elevated, but non-statistically significant, anaphylaxis rate was observed following any (IRR: 2.40, 95% CI: 0.96-6.03), high-dose (IRR: 2.31, 95% CI: 0.67-7.91), and adjuvanted (IRR: 3.28, 95% CI: 0.71-15.08) influenza vaccination; anaphylaxis IRRs were 2.54 (95% CI: 0.49-13.05) and 1.64 (95% CI: 0.38-7.05) for persons with and without concomitant vaccination, respectively. CONCLUSIONS: Rates of encephalitis/encephalomyelitis, GBS, or transverse myelitis were not elevated following 2022-2023 seasonal influenza vaccinations among U.S. adults ≥ 65 years. There was an increased rate of anaphylaxis following influenza vaccination that may have been influenced by concomitant vaccination.
Asunto(s)
Vacunas contra la Influenza , Gripe Humana , Vacunación , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Anafilaxia/epidemiología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/epidemiología , Síndrome de Guillain-Barré/epidemiología , Síndrome de Guillain-Barré/etiología , Síndrome de Guillain-Barré/inducido químicamente , Incidencia , Vacunas contra la Influenza/efectos adversos , Vacunas contra la Influenza/administración & dosificación , Gripe Humana/prevención & control , Gripe Humana/epidemiología , Medicare/estadística & datos numéricos , Mielitis Transversa/epidemiología , Mielitis Transversa/etiología , Estaciones del Año , Estados Unidos/epidemiología , Vacunación/efectos adversosRESUMEN
INTRODUCTION: Acute transverse myelitis (ATM) is a rare neurological complication of Coronavirus disease (COVID-19) vaccines. Various vaccines have been linked to ATM, such as non-replicating viral vectors, ribonucleic acid, and inactivated vaccines. An ATM case is presented here involving the BNT162b2 vaccine leading to asymmetrical incomplete paraplegia and neurogenic bladder. CASE PRESENTATION: A 66-year-old male developed urinary retention one day after his second dose of the BNT162b2 vaccine, followed by rapidly progressing lower limb weakness. Clinical examination showed asymmetrical paraparesis, reduced sensation below the T8 level, including perianal sensation, and loss of ankle and anal reflexes. Laboratory tests were largely unremarkable, while the spine MRI revealed thickened conus medullaris with a mild increase in T2/STIR signal intensity and subtle enhancement post gadolinium. Following treatment with methylprednisolone, plasmapheresis, and immunoglobulin, and a rehabilitation program, the patient achieved good motor and sensory recovery, but the bladder dysfunction persisted. Single-channel cystometry indicated neurogenic detrusor underactivity and reduced bladder sensation, as evidenced by low-pressure and compliant bladder. The urethral sphincter appeared intact or overactive. The post-void residual urine was significant, necessitating prolonged intermittent catheterisation. DISCUSSION: Bladder dysfunction due to the COVID-19 vaccine-associated ATM is not as commonly reported as motor or sensory deficits. To our knowledge, this is the first case to highlight a neurogenic bladder that necessitates prolonged intermittent catheterisation as a consequence of COVID-19 vaccine-associated ATM. This report highlights the rare complication of the neurogenic bladder resulting from the BNT162b2 vaccine. Early detection and treatment are crucial to prevent long-term complications.
Asunto(s)
Vacuna BNT162 , Vacunas contra la COVID-19 , Mielitis Transversa , Vejiga Urinaria Neurogénica , Humanos , Masculino , Mielitis Transversa/etiología , Anciano , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria Neurogénica/terapia , Vacuna BNT162/efectos adversos , Vacunas contra la COVID-19/efectos adversos , COVID-19/complicacionesRESUMEN
BACKGROUND AND PURPOSE: This study was undertaken to raise awareness of a role of B cells in immune checkpoint inhibitor (ICI)-associated neurological immune-related adverse events (nirAE). METHODS: A systematic literature review was made, with case observations of a melanoma and a non-small cell lung cancer (NSCLC) patient who developed ICI-associated nirAE with cerebrospinal fluid (CSF) findings indicating B cell involvement. RESULTS: Two patients receiving ipilimumab/nivolumab for melanoma and chemotherapy/pembrolizumab for NSCLC developed nirAE in the form of myocarditis/myositis/myasthenia gravis overlap syndrome (triple M) and cerebellitis plus longitudinal transverse myelitis (c-LETM), respectively. Intrathecal inflammation with chemokine C-X-C motif ligand (CXCL13) elevation was present in both patients; the triple M case had acetylcholine receptor antibodies, antititin reactivity, altered CD4/CD8 T cell ratio in blood, and depressed programmed death-1 (PD-1) expression on CSF T cells; the c-LETM case showed intrathecal antibody production and plasma cells. Both patients insufficiently responded to first-line treatment. The NSCLC case improved upon administration of B cell-depleting therapy with rituximab, whereas the melanoma patient died before escalation therapy was initiated. Literature research revealed one additional ICI-associated LETM case with intrathecal CXCL13 elevation, three cases with ICI-associated aquaporin-4 antibody neuromyelitis spectrum disorder, and evidence of B cell-mediated toxicity based on antibody-mediated immune pathologies in ICI-associated immune-related adverse events. CONCLUSIONS: The case observations highlight the plethora of uncertainties in diagnosis and treatment of ICI-associated nirAE, exemplify the heterogeneity of immune mechanisms involved, and suggest a role of B cells, which may be underdiagnosed. Intrathecal CXCL13 may serve as a biomarker of B cell involvement in nirAE, supported by intrathecal immunoglobulin synthesis, presence of plasma cells, and/or recruitment of cognate immune cells.
Asunto(s)
Linfocitos B , Quimiocina CXCL13 , Inhibidores de Puntos de Control Inmunológico , Anciano , Femenino , Humanos , Anticuerpos Monoclonales Humanizados/efectos adversos , Anticuerpos Monoclonales Humanizados/administración & dosificación , Linfocitos B/efectos de los fármacos , Linfocitos B/inmunología , Quimiocina CXCL13/líquido cefalorraquídeo , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Ipilimumab/efectos adversos , Ipilimumab/administración & dosificación , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/inmunología , Melanoma/tratamiento farmacológico , Mielitis Transversa/inducido químicamente , Mielitis Transversa/inmunología , Nivolumab/efectos adversos , Nivolumab/administración & dosificación , Linfocitos T/inmunología , Linfocitos T/efectos de los fármacosRESUMEN
BACKGROUND: Eosinophils in cerebrospinal fluid (CSF) are an uncommon finding most often associated with parasitic infections, but have also been described in some neuroinflammatory disorders. Eosinophilic infiltration is not thought to be a typical feature of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). We aim to describe the rate of CSF eosinophil positivity in a cohort of pediatric MOGAD patients. METHODS: Single-center retrospective chart review of pediatric MOGAD patients. Clinical and laboratory data was collected from the electronic medical record and analyzed. RESULTS: Of 46 pediatric patients with positive serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) identified, 38 patients fulfilling internationally proposed MOGAD diagnostic criteria were included for analysis. 6 patients with MOGAD were excluded as no CSF data was available, and 2 patients with positive MOG-IgG but diagnosis more consistent with MS were excluded. Median age was 7.3 years, and 19/38 (50 %) were female. Acute disseminated encephalomyelitis (ADEM) was the most common presenting phenotype (23/38, 61 %), and other phenotypes included optic neuritis (10/38, 26 %), transverse myelitis (3/38, 8 %), and neuromyelitis optica spectrum disorder (NMOSD) (2/38, 5 %). 12 of 36 (33 %) patients with all lumbar puncture (LP) data available had CSF eosinophils present, with eosinophil mean of 3 % and range from 1 % to 18 % of CSF while blood cells. CONCLUSION: CSF eosinophils were present in one third of pediatric MOGAD patients, which is a higher rate than previously reported in either MOGAD or aquaporin-4 antibody positive NMOSD cohorts. Understanding the CSF composition of pediatric MOGAD patients helps to facilitate more prompt diagnosis and treatment and may shed light onto underlying pathologic mechanisms of disease with the goal to inform future therapeutic targets.
Asunto(s)
Autoanticuerpos , Eosinófilos , Glicoproteína Mielina-Oligodendrócito , Humanos , Glicoproteína Mielina-Oligodendrócito/inmunología , Femenino , Masculino , Niño , Estudios Retrospectivos , Eosinófilos/inmunología , Preescolar , Adolescente , Autoanticuerpos/líquido cefalorraquídeo , Autoanticuerpos/sangre , Encefalomielitis Aguda Diseminada/inmunología , Encefalomielitis Aguda Diseminada/líquido cefalorraquídeo , Encefalomielitis Aguda Diseminada/sangre , Encefalomielitis Aguda Diseminada/diagnóstico , Neuromielitis Óptica/líquido cefalorraquídeo , Neuromielitis Óptica/inmunología , Neuromielitis Óptica/sangre , Lactante , Mielitis Transversa/inmunología , Mielitis Transversa/líquido cefalorraquídeo , Mielitis Transversa/sangre , Neuritis Óptica/inmunología , Neuritis Óptica/líquido cefalorraquídeo , Neuritis Óptica/sangre , Enfermedades Autoinmunes Desmielinizantes SNC/líquido cefalorraquídeo , Enfermedades Autoinmunes Desmielinizantes SNC/inmunología , Enfermedades Autoinmunes Desmielinizantes SNC/sangreRESUMEN
We diagnosed a patient with dengue fever who developed acute onset of sensorimotor quadriparesis with bladder involvement, and facial nerve involvement. Despite initial negative results in routine investigations and cerebrospinal fluid analysis, spinal MRI confirmed longitudinally extensive transverse myelitis. The aetiological workup was negative, prompting an investigation into the presence of dengue in the cerebrospinal fluid, which returned positive. This case underscores the importance of considering rare neurological complications in dengue, the value of advanced diagnostic techniques and the potential effectiveness of tailored interventions in challenging cases.
Asunto(s)
Dengue , Mielitis Transversa , Mielitis , Humanos , Mielitis Transversa/diagnóstico , Dengue/complicaciones , Imagen por Resonancia Magnética/métodos , Cuadriplejía/complicaciones , Nervio Facial , Mielitis/complicacionesRESUMEN
INTRODUCTION: Acute transverse myelitis (ATM) refers to a rare severe acquired spinal cord inflammation, with a challenging diagnostic work-up and treatment. CASE PRESENTATION: We report the case of a 42-year-old patient who presented with loss of temperature and pain sensation beneath the C5 dermatome in her left side and reported a history of a possible respiratory tract illness 10 days ago. Within 2 days, clinical worsening was noted, compatible with Brown-Sequard syndrome. Spinal magnetic resonance imaging revealed a T2 sequence abnormal signal from level C4 to T3 and cerebrospinal fluid (CSF) studies showed only a mild pleocytosis mononuclear type. Extensive CSF and blood tests revealed only high Mycoplasma pneumoniae IgM and IgG titers. Treatment with high-dose intravenous methylprednisolone and oral azithromycin were administrated and the patient recovered completely within two months. DISCUSSION: We would like to highlight the importance for physicians to consider M. pneumoniae in their differential diagnosis as a potential cause when encountering patients with symptoms of ATM and inflammatory Brown-Sequard syndrome.
Asunto(s)
Síndrome de Brown-Séquard , Mielitis Transversa , Adulto , Femenino , Humanos , Síndrome de Brown-Séquard/etiología , Descompresión Quirúrgica , Imagen por Resonancia Magnética , Mycoplasma pneumoniae , Mielitis Transversa/complicacionesRESUMEN
We describe a patient with acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) with unique features, including concurrent transverse myelitis. A 2-year-old previously healthy girl had clinical findings consistent with AESD, occurring in association with influenza A infection. The posterior brain regions were most severely affected, resulting in cortical blindness. She also developed bilateral limb weakness, and spine MRI revealed transverse myelitis in the cervical region. She was treated acutely with intravenous methylprednisolone. Serum anti-myelin oligodendrocyte glycoprotein and anti-aquaporin-4 antibodies were negative, as was an anti-extractable nuclear antigen panel. Although her clinical presentation was severe, she improved dramatically over the following months, and 6 months following initial presentation, her parents felt she had returned to baseline. This is the first report of AESD occurring in combination with transverse myelitis. The co-occurrence of the two conditions is unlikely to be coincidental, suggesting that there may be a shared or overlapping immunological pathway involved. The patient's recovery was impressive, which could partially relate to the acute treatment with corticosteroids.
Asunto(s)
Mielitis Transversa , Convulsiones , Humanos , Femenino , Mielitis Transversa/tratamiento farmacológico , Mielitis Transversa/complicaciones , Preescolar , Convulsiones/etiología , Convulsiones/tratamiento farmacológico , Encefalopatías/complicaciones , Encefalopatías/tratamiento farmacológico , Imagen por Resonancia Magnética , Gripe Humana/complicaciones , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Metilprednisolona/administración & dosificaciónRESUMEN
INTRODUCTION: Transverse Myelitis is a rare inflammatory disorder of the spinal cord, characterized by the inflammation of the myelin sheath covering nerve fibers. Although rare, Transverse Myelitis holds significant clinical importance due to its potential life-altering consequences. The case report provides insight into the clinical presentation of Transverse Myelitis and the importance of Magnetic Resonance Imaging in confirming Transverse Myelitis. CASE PRESENTATION: A 27-year-old Nigerian female presented to a hospital facility after 2 months onset of paraplegia, urinary, and fecal incontinence. She was diagnosed with Acute Transverse Myelitis with Magnetic Resonance Imaging, a lacking imaging modality in Nigeria. On presentation, it was important to rule out spinal cord compression, a close differential to her presentation. Despite her late arrival at the facility, early diagnosis and prompt initiation of treatment with high-dose intravenous steroids and physiotherapy improved her quality of life. DISCUSSION: This case report reveals the poor health-seeking behavior in developing countries and the need for imaging modalities like Magnetic Resonance Imaging for improved diagnoses of rare neurological conditions such as Transverse Myelitis. The lack of healthcare infrastructure has led to clinical misdiagnosis, patient mismanagement, and underrepresentation of data in the country, underscoring the critical role of diagnostic tools for improved patient care pre-treatment and post-treatment. Additionally, follow-up of these patients is important to prevent the long-term sequelae of Transverse Myelitis like Neuromyelitis Optica or Multiple Sclerosis.