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1.
BMC Neurol ; 24(1): 241, 2024 Jul 11.
Artigo em Inglês | MEDLINE | ID: mdl-38992582

RESUMO

BACKGROUND: Pure red cell aplasia (PRCA) in neuromyelitis optica spectrum disorder (NMOSD) has not been reported before. This study presents a patient with NMOSD who developed PRCA. CASE PRESENTATION: A 54-year-old female was admitted in January 2023 for dysuria and progressive numbness and weakness of lower limbs. She had difficulty standing and walking in a straight line. Both lower limbs were positive for the Babinski and Chaddock signs. MRI showed abnormal signals in the spinal cord. Aquaporin-4-IgG (AQP-4-IgG) was positive (1:320), and NMOSD was confirmed. Intravenous immunoglobulin and methylprednisolone were given, and the symptoms improved. She received maintenance treatment with methylprednisolone tablets, and the dosage was gradually reduced. She was readmitted for fatigue, palpitations, and shortness of breath in May 2023. Bone marrow aspiration and biopsy showed elevated erythroid precursors and erythroid hypoplasia, with normal megakaryocytes and myeloid precursors. Chest CT showed no mediastinal lymph node enlargement or thymoma. PRCA secondary to NMOSD was diagnosed. Recombinant human erythropoietin was given. Her condition improved after 1.5 months, as indicated by blood cell count and imaging. CONCLUSIONS: This case suggests that PRCA can be secondary to NMOSD. A comprehensive immune function and bone marrow evaluation might be necessary if abnormal blood cells are found while managing NMOSD.


Assuntos
Neuromielite Óptica , Aplasia Pura de Série Vermelha , Humanos , Feminino , Neuromielite Óptica/complicações , Neuromielite Óptica/tratamento farmacológico , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/diagnóstico por imagem , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/complicações , Aplasia Pura de Série Vermelha/diagnóstico , Aplasia Pura de Série Vermelha/tratamento farmacológico , Aquaporina 4/imunologia
2.
J Integr Neurosci ; 23(2): 35, 2024 Feb 18.
Artigo em Inglês | MEDLINE | ID: mdl-38419451

RESUMO

BACKGROUND: Several results support the hypothesis that a group of pathologies falling within the Neuromyelitis Optica Spectrum Disorders (NMOSD) diagnostic criteria may coexist with Connective Tissue Diseases (CTD) in patients with a high susceptibility to autoimmune conditions. However, the relationship between NMOSD and rheumatologic diseases deserves further investigations to clarify all clinical aspects of this coexistence. We designed a systematic review and a proportional meta-analysis to estimate the association between CTD and MNOSD, with the aim of helping to plan the best strategy to achieve the most significant public health benefit for these conditions. METHODS: We conducted a systematic review of the literature published until February 2023, searching in four databases: PubMed, Web of Science, EmBase, and OVID. Then, we conducted a random-effects proportional meta-analysis and assessed the risk of bias of the included studies using the Joanna Briggs Institute checklist. RESULTS: The literature search yielded an overall result of 3176 publications (272 from PubMed, 880 from Web of Science, 634 from EmBase and 1390 from OVID). Of these, 29 were included in this systematic review. Analyzing studies that recruited unselected patients with Systemic Lupus Erythematosus (SLE) and Sjogren Syndrome (SjS), the pooled percentages of NMOSD overlapping were 0.6% (95% Confidence Interval [95% CI]: 0.1%-1.4%,) and 6.5% (95% CI: 4.7-8.6), respectively. Studies enrolling rheumatologic patients with nervous system symptoms involvement reported higher percentage of NMOSD (i.e., among SjS patients, a pooled percentage of 26.5%, 95% CI: 5.5-54.6%, was found). Similarly, recruiting patients with NMOSD, we found pooled percentages of SjS or SLE respectively of 7.0% and 3.5%. CONCLUSIONS: Our research found that the coexistence of these two disorders was more frequent in female rheumatologic patients with a SjS diagnosis with neurological manifestations and in neurologic patients for whom a SjS diagnosis was suspected. Similarly, NMOSD are less frequently found in SLE and very rarely incident in Mixed Connective Tissue Disease (MCTD) patients. These considerations should be taken into account in clinical experience of rheumatologists and neurologists, since early diagnosis of both conditions may influence the timing of immunosuppressive therapy and the prevention of systemic disabilities.


Assuntos
Doenças do Tecido Conjuntivo , Neuromielite Óptica , Neuromielite Óptica/epidemiologia , Neuromielite Óptica/complicações , Humanos , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Síndrome de Sjogren/complicações , Comorbidade
3.
J Assoc Physicians India ; 72(9): e22-e24, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39291531

RESUMO

A 32-year-old lady presented with acute paroxysmal facial pain in the V1-V2 distribution, lower cranial nerve dysfunction, and mild long-tract neurological signs. Paroxysmal facial pain with features of trigeminal autonomic cephalgia (TAC) and trigeminal neuralgia (TN) (Tic) was the unique presentation in this case, explained by involvement of the somatotopically arranged spinal trigeminal nucleus. She had two attacks of area postrema syndrome (APS) preceding the current symptoms, evaluated as a gastroenterological disorder, causing a delay in imaging and AQP4-seropositive diagnosis of neuromyelitis optica spectrum disorder (NMOSD). She recovered from brainstem symptoms with immunosuppression but continued to have painful tonic spasms and neuropathic pain as immediate sequelae. The untreated first attack, AQP4 positivity, and relapse within the 1st year are all risk factors for further relapses and long-term morbidity, as was the case with our patient.


Assuntos
Neuromielite Óptica , Cefalalgias Autonômicas do Trigêmeo , Neuralgia do Trigêmeo , Humanos , Feminino , Adulto , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/complicações , Neuralgia do Trigêmeo/etiologia , Neuralgia do Trigêmeo/diagnóstico , Cefalalgias Autonômicas do Trigêmeo/diagnóstico , Cefalalgias Autonômicas do Trigêmeo/etiologia
4.
Prague Med Rep ; 125(3): 232-238, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39171550

RESUMO

Primary Sjögren's syndrome is an autoimmune disorder that is characterized by lymphocytic infiltration of salivary and lacrimal glands. The extra-glandular manifestations might be arthritis, myalgia, glomerulonephritis, skin rashes, and neurologic involvement. One of the uncommon neurologic manifestations is neuromyelitis optica spectrum disorder (NMOSD). In the present case, an older woman is reported that was diagnosed with NMOSD secondary to keratoconjunctivitis sicca, which is rare in geriatric practice.


Assuntos
Neuromielite Óptica , Síndrome de Sjogren , Humanos , Feminino , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/complicações , Neuromielite Óptica/etiologia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Idoso , Ceratoconjuntivite Seca/etiologia , Ceratoconjuntivite Seca/diagnóstico
5.
Curr Opin Neurol ; 36(1): 10-18, 2023 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-36630210

RESUMO

PURPOSE OF REVIEW: The primary aim of this review is to describe the clinical course, salient imaging features, and relevant serological profiles of common optic neuritis (ON) subtypes. Key diagnostic challenges and treatment options will also be discussed. RECENT FINDINGS: ON is a broad term that describes an inflammatory optic nerve injury arising from a variety of potential causes. ON can occur sporadically, however there is particular concern for co-associated central nervous system (CNS) inflammatory syndromes including multiple sclerosis (MS), neuromyelitis optic spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD). The ON subtypes that often herald MS, NMOSD, and MOGAD differ with respect to serological antibody profile and neuroimaging characteristics, yet there is significant overlap in their clinical presentations. A discerning history and thorough examination are critical to rendering the correct diagnosis. SUMMARY: Optic neuritis subtypes vary with respect to their long-term prognosis and accordingly, require different acute treatment strategies. Moreover, delays in identifying MOGAD, and certainly NMOSD, can be highly detrimental because affected individuals are vulnerable to permanent vision loss and neurologic disability from relapses.


Assuntos
Esclerose Múltipla , Neuromielite Óptica , Neurite Óptica , Humanos , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/terapia , Aquaporina 4 , Glicoproteína Mielina-Oligodendrócito , Autoanticorpos , Neurite Óptica/diagnóstico , Neurite Óptica/terapia , Esclerose Múltipla/diagnóstico , Inflamação
6.
Osteoporos Int ; 34(5): 925-933, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36854747

RESUMO

PURPOSE: Interest in fractures in patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) has considerably increased in the last decade. However, few studies have compared the incidence of fractures between patients with MS and NMOSD using a nationwide database. This study aimed to evaluate the differences in the risk of fracture between patients with NMOSD and MS compared to that in healthy controls using cohort data from a Korean nationwide database. METHODS: In this retrospective cohort study, data from the National Health Insurance Service (NHIS) database from January 2010 to December 2017 were analyzed. A total of 1,217/1,329 patients with MS/NMOSD free of fractures at the index date were included. Matched controls were selected based on age, sex, and the presence of hypertension, diabetes mellitus, and dyslipidemia. The mean follow-up durations after the index date were 4.40/4.08 years for patients with MS/NMOSD and 4.73/4.28 for their matched controls. RESULTS: The adjusted hazard ratios (aHRs) with 95% confidence intervals of any, hip, and vertebral fractures were 1.81 (1.43-2.28), 3.36 (1.81-6.24), and 2.01 (1.42-2.99) times higher for patients with MS than for controls, respectively, and they were 1.85 (1.47-2.34), 3.82 (2.05-7.11), and 2.84 (1.92-4.21) times higher for patients with NMOSD than for controls, respectively. No significant differences were observed in the incidence of fractures between the MS and NMOSD groups. Patients with MS/NMOSD had a 1.8-fold higher risk of fracture than matched controls, and the risk of hip fracture was especially high (3- to 4-fold higher). CONCLUSIONS: Clinicians need to regularly assess patients with MS/NMOSD for the risk of fractures and take preventative measures to reduce it.


Assuntos
Fraturas Ósseas , Esclerose Múltipla , Neuromielite Óptica , Humanos , Neuromielite Óptica/complicações , Neuromielite Óptica/epidemiologia , Estudos de Coortes , Esclerose Múltipla/complicações , Esclerose Múltipla/epidemiologia , Estudos Retrospectivos , Imageamento por Ressonância Magnética
7.
Mult Scler ; 29(1): 150-153, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36437566

RESUMO

BACKGROUND: Recently, satralizumab (interleukin-6 receptor blocker) was approved for seropositive neuromyelitis optica spectrum disorder (NMOSD) patients. In SAkuraSky trial, mild neutropenia was reported in 15% of patients under satralizumab. Most neutropenias were transient; grade 3-4 was not related to serious infections. So far, no severe neutropenia (<100 cell/mm3) has been reported worldwide. METHODS: We present an aquaporin-4-antibody-positive NMOSD patient who developed severe febrile neutropenia 2 weeks after adding satralizumab to her azathioprine treatment. CONCLUSION: Analytic control for satralizumab is recommended at 4 weeks. However, we recommend this control at week 2, in order to closely monitor neutrophil count and prevent further complications.


Assuntos
Neutropenia Febril , Neuromielite Óptica , Humanos , Feminino , Neuromielite Óptica/complicações , Neuromielite Óptica/tratamento farmacológico , Aquaporina 4 , Anticorpos Monoclonais Humanizados , Autoanticorpos , Neutropenia Febril/induzido quimicamente
8.
Mult Scler ; 29(7): 819-831, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36786424

RESUMO

BACKGROUND: There is limited and inconsistent information on the prevalence of cognitive impairment in neuromyelitis optica spectrum disorders (NMOSD). OBJECTIVE: To assess cognitive performance and changes over time in NMOSD. METHODS: This study included data from 217 aquaporin-4-IgG-seropositive (80%) and double-seronegative NMOSD patients. Cognitive functions measured by Symbol Digit Modalities Test (SDMT), Paced Auditory Serial-Addition Task (PASAT), and/or Multiple Sclerosis Inventory Cognition (MuSIC) were standardized against normative data (N = 157). Intraindividual cognitive performance at 1- and 2-year follow-up was analyzed. Cognitive test scores were correlated with demographic and clinical variables and assessed with a multiple linear regression model. RESULTS: NMOSD patients were impaired in SDMT (p = 0.007), MuSIC semantic fluency (p < 0.001), and MuSIC congruent speed (p < 0.001). No significant cognitive deterioration was found at follow-up. SDMT scores were related to motor and visual disability (pBon < 0.05). No differences were found between aquaporin-4-IgG-seropositive and double-seronegative NMOSD. CONCLUSIONS: A subset of NMOSD patients shows impairment in visual processing speed and in semantic fluency regardless of serostatus, without noticeable changes during a 2-year observation period. Neuropsychological measurements should be adapted to physical and visual disabilities.


Assuntos
Esclerose Múltipla , Neuromielite Óptica , Humanos , Neuromielite Óptica/complicações , Neuromielite Óptica/epidemiologia , Estudos Prospectivos , Aquaporina 4 , Cognição , Imunoglobulina G , Autoanticorpos
9.
Mult Scler ; 29(10): 1240-1249, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37491849

RESUMO

BACKGROUND: We aimed to assess the frequency of new asymptomatic lesions on brain and spinal imaging (magnetic resonance imaging (MRI)) and their association with subsequent relapses in a large cohort of neuromyelitis optica spectrum disorder (NMOSD) patients in Argentina. METHODS: We retrospectively reviewed 675 MRI (225 performed during an attack and 450 during the relapse-free period (performed at least 3 months from the last attack)) of NMOSD patients who had at least 2 years of clinical and MRI follow-up since disease onset. Kaplan-Meier (KM) curves were used for depicting time from remission MRI to subsequent relapse. RESULTS: We included 135 NMOSD patients (64.4% were aquaporin-4-immunoglobulin G (AQP4-IgG)-positive). We found that 26 (19.26%) and 66 (48.88%) of patients experienced at least one new asymptomatic MRI lesion during both the relapse-free period and attacks, respectively. The most frequent asymptomatic MRI lesions were optic nerves followed by short-segment myelitis during the relapse-free period and attacks. KM curves did not show differences in the time taken to develop a new relapse. CONCLUSION: Our findings showed that new asymptomatic lesions are relatively frequent. However, the presence of new asymptomatic MRI lesions during the relapse-free period and at relapses was not associated with a shorter time to developing subsequent relapses.


Assuntos
Neuromielite Óptica , Humanos , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/complicações , Estudos Retrospectivos , Seguimentos , Encéfalo/diagnóstico por imagem , Aquaporina 4 , Imageamento por Ressonância Magnética , Autoanticorpos
10.
Mult Scler ; 29(14): 1872-1875, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37712413

RESUMO

Neuromyelitis optica spectrum disorder (NMOSD) in people living with HIV (PLWH) is rare and its management can be difficult. Here we report a case of an HIV patient with bilateral vision loss, who was diagnosed with AQP4-IgG-positive NMOSD in 2020 during the COVID-19 pandemic. Rituximab treatment was initiated after attack therapy with corticosteroids and plasma exchange. NMOSD and HIV disease remained stable, but SARS-CoV-2 immune response after repeated vaccinations was insufficient. After switching immunotherapy due to the lack of vaccination response to satralizumab, peripheral B cells reoccurred and a humoral immune response was observed after reapplication of SARS-CoV-2 vaccination. This case illustrates the challenges associated with the treatment of NMOSD in PLWH.


Assuntos
Infecções por HIV , Neuromielite Óptica , Humanos , Neuromielite Óptica/complicações , Neuromielite Óptica/terapia , Neuromielite Óptica/epidemiologia , Aquaporina 4 , Vacinas contra COVID-19 , Infecções por HIV/complicações , Pandemias , Autoanticorpos
11.
Alzheimer Dis Assoc Disord ; 37(1): 85-87, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-35838179

RESUMO

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease of the central nervous system. Although recent reports have noted that cognitive impairment is common in NMOSD, little longitudinal information is available on the trajectories of cognitive function in the disease. Here, we report a case of a 55-year-old woman with an 11-year history of NMOSD who visited our memory clinic for progressive memory loss. She was diagnosed with early-onset Alzheimer disease based on amyloid and tau positron emission tomography imaging biomarkers. This is the first report of early-onset Alzheimer disease in a patient with NMOSD. Complications of Alzheimer disease should be considered when patients with NMOSD exhibit rapid cognitive decline. More longitudinal studies of NMOSD with cognitive impairment are needed.


Assuntos
Doença de Alzheimer , Doenças Autoimunes , Disfunção Cognitiva , Neuromielite Óptica , Feminino , Humanos , Pessoa de Meia-Idade , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico , Doença de Alzheimer/complicações , Disfunção Cognitiva/complicações , Cognição
12.
BMC Neurol ; 23(1): 189, 2023 May 12.
Artigo em Inglês | MEDLINE | ID: mdl-37173637

RESUMO

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) is a group of autoimmune-mediated disorders of the central nervous system primarily involving the optic nerve and spinal cord. There are limited reports of NMOSD associated with peripheral nerve damage. CASE PRESENTATION: We report a 57-year-old female patient who met the diagnostic criteria for aquaporin 4 (AQP4)-IgG positive NMOSD with undifferentiated connective tissue disease and multiple peripheral neuropathy. In addition, the patient was positive for multiple anti-ganglioside antibodies (anti-GD1a IgG antibodies and anti-GD3 IgM antibodies) and anti-sulfatide IgG antibodies in serum and cerebrospinal fluid. After treatment with methylprednisolone, gamma globulin, plasma exchange, and rituximab, the patient's status improved and was subsequently discharged from our hospital. CONCLUSIONS: The neurologist should be aware of the unusual association between NMOSD and immune-mediated peripheral neuropathy undifferentiated connective tissue disease and nerve damage mediated by multiple antibodies may have combined to cause peripheral nerve damage in this patient.


Assuntos
Doenças Autoimunes , Neuromielite Óptica , Traumatismos dos Nervos Periféricos , Doenças do Tecido Conjuntivo Indiferenciado , Feminino , Humanos , Pessoa de Meia-Idade , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/líquido cefalorraquidiano , Autoanticorpos , Aquaporina 4 , Imunoglobulina G
13.
Curr Allergy Asthma Rep ; 23(11): 647-654, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37889429

RESUMO

PURPOSE OF REVIEW: Neuromyelitis optica (NMO) is an auto-immune disease essentially depicted by optic neuritis and transverse myelitis. Per se, NMO was initially believed to be a sub-type of multiple sclerosis with typical demyelinating cerebral lesions and optic nerve inflammation. More recently, corroborating lignes of evidence have strengthened the concept of the spectrum of diseases associated with NMO and more specifically with the role of anti-aquaporin-4 antibodies in the pathogenesis of disease. RECENT FINDINGS: In this article, we review the recent pathogenic findings in NMO and more interestingly the newly discovered role of anti-aquaporin-4 antibodies as key players in triggering cerebral lesions. The concept of spectrum of diseases associated with NMO is also discussed. These recent findings have paved in the further understanding of the pathogenesis underlying NMO and new treatments are currently being developed targeting anti-aquaporin-4 antibodies.


Assuntos
Doenças Autoimunes , Esclerose Múltipla , Neuromielite Óptica , Humanos , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/terapia , Neuromielite Óptica/complicações , Aquaporina 4 , Esclerose Múltipla/etiologia , Esclerose Múltipla/terapia , Autoanticorpos
14.
Neurol Sci ; 44(5): 1731-1737, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36645533

RESUMO

OBJECTIVES: We aimed to determinate the frequency of this association and compare the features of neuromyelitis optica spectrum disorder (NMOSD) with and without associated autoimmune diseases (AD) in a Latin American (LATAM) population in clinical practice. METHODS: We retrospectively reviewed the medical records of patients with NMOSD according to the 2015 diagnostic criteria. Patients from Argentina (n=77), Brazil (n=46), and Venezuela (n=17) were enrolled and classified into two groups as follows: with AD or without AD. Clinical, paraclinical (including aquaporin-4 antibodies (AQP4-ab) status), magnetic resonance imaging (MRI), and prognosis data were analyzed and compared. Kaplan-Meier (KM) and the Nelson-Aalen estimator analyses were performed to estimate both time and the cumulative hazard risk of disability reaching an EDSS≥4; and time for the first recurrence. RESULTS: Out of 140 patients, 33 (23.5%) patients had associated an AD at presentation. The most frequent associated AD was Hashimoto disease (n=10) followed by lupus (n=7) and Sjogren's syndrome (n=6). However, rituximab use (42.4% vs. 21.5%, p=0.02), female gender (82.2% vs. 100%, p=0.006), corticospinal lesions on MRI (0% vs. 12.5%, p=0.01) at onset, and positivity for antinuclear antibodies (21.2% vs. 48.4%, p=0.03) were significantly associated with NMOSD patients with AD in comparison to NMOSD patients without AD. No differences were found in other clinical and paraclinical aspects between groups. KM and Nelson-Aalen estimator analyses did not show differences between groups. CONCLUSION: NMOSD patients associated with AD were observed in 23.5%. In addition, NMOSD patients with and without associated AD were similar in most evaluated features.


Assuntos
Neuromielite Óptica , Síndrome de Sjogren , Humanos , Feminino , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/epidemiologia , Aquaporina 4 , Estudos Retrospectivos , Autoanticorpos , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia
15.
Eur Neurol ; 86(4): 256-262, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37100033

RESUMO

INTRODUCTION: The purpose of this study was to analyze IL-33 maybe as a biomarker especially with respect to intrathecal immunoglobulin G (IgG) synthesis which was involved in the immune-mediated process in the demyelinating disease of the central nervous system. METHODS: We aimed to determine the risk association of the serum and CSF levels of IL-33 in aquaporin-4 (AQP4)+neuromyelitis optica spectrum disorder (NMOSD) patients and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) patients compared with the control group. Levels of inflammatory (IL-2, IL-4, IL-6, and IL-10) markers and QAlb, the IgG index, and 24-h IgG synthesis rate were assessed in 28 AQP4+NMOSD patients and 11 MOGAD patients. Disease severity was assessed using the Expanded Disability Status Scale (EDSS). RESULTS: The level of IL-33 in serum decreased first but then increased gradually in AQP4+NMOSD and MOGAD. The serum level of IL-2, IL-4, and IL-10 increased more significantly and decreased more rapidly after methylprednisolone treatment. The level of IL-33 in CSF increased progressively in AQP4+NMOSD and MOGAD, especially in MOGAD. The QAlb levels were increased significantly in the CSF of MOGAD patients and AQP4+NMOSD patients on the acute stage of the disease. The IgG index and 24-h IgG synthesis rate were also increased significantly in the CSF of two groups similarly. CONCLUSIONS: Thus, we concluded that IL-33 may induce dysfunction of the blood-brain barrier and lead to intrathecal synthesis of immunoglobulin in the AQP4+NMOSD and MOGAD, especially in MOGAD. It maybe as a biomarker, at least in part, was involved in the demyelinating diseases of the central nervous system.


Assuntos
Neuromielite Óptica , Humanos , Aquaporina 4 , Biomarcadores , Imunoglobulina G , Interleucina-10 , Interleucina-2 , Interleucina-33 , Interleucina-4 , Glicoproteína Mielina-Oligodendrócito , Neuromielite Óptica/complicações
16.
Rev Neurol (Paris) ; 179(6): 533-547, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36781321

RESUMO

Many cases of aseptic meningitis or meningoencephalitis, unresponsive to antimicrobial treatments, have been reported recently in patients with established/new-onset central nervous system (CNS) inflammatory demyelinating diseases (CNSIDDs). Given the higher probability of infectious etiologies, CNSIDDs are rarely considered among the differentials in meningitis or meningoencephalitis cases. We gathered and tabulated cases of non-infectious, steroid-responsive meningitis or meningoencephalitis associated with neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein-associated disease (MOGAD). This conceptual review highlights the need to bolster routine infectious workups with immunological workups in cases of meningoencephalitis or meningitis where potential autoimmune etiologies can be suspected. Although differentiating CNSIDDs with meningeal involvement from infectious meningitis may not substantially affect acute treatment strategies, long-term management and follow-up of the two are entirely different. We also discuss future research directions and hypotheses on how CNSIDDs may be associated with meningitis-like presentations, e.g. overlapping glial fibrillary acidic protein astrocytopathy or autoimmune encephalitis, alterations in regulatory T-helper cells function, and undetected viral agents.


Assuntos
Encefalite , Meningite Asséptica , Meningoencefalite , Neuromielite Óptica , Humanos , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/terapia , Neuromielite Óptica/complicações , Encefalite/complicações , Glicoproteína Mielina-Oligodendrócito , Meningite Asséptica/etiologia , Meningite Asséptica/complicações , Meningoencefalite/diagnóstico , Meningoencefalite/etiologia , Autoanticorpos
17.
Zhonghua Nei Ke Za Zhi ; 62(6): 705-710, 2023 Jun 01.
Artigo em Zh | MEDLINE | ID: mdl-37263955

RESUMO

Objective: To investigate the misdiagnosis of area postrema syndrome (APS) manifesting as intractable nausea, vomiting and hiccups in neuromyelitis optic spectrum disease (NMOSD) and reduce the risk of misdiagnosis. Methods: We retrospectively analyzed data from NMOSD patients attending the Department of Neurology at the First Medical Center of PLA General Hospital between January 2019 and July 2021. SPSS25.0 was then used to analyze the manifestations, misdiagnosis, and mistreatment of APS. Results: A total of 207 patients with NMOSD were included, including 21 males and 186 females. The mean age of onset was 39±15 years (range: 5-72 years). The proportion of patients who were positive for serum aquaporin 4 antibody was 82.6% (171/207). In total, 35.7% (74/207) of the NMOSD patients experienced APS during the disease course; of these patients, 70.3% (52/74) had APS as the first symptom and 29.7% (22/74) had APS as a secondary symptom. The misdiagnosis rates for these conditions were 90.4% (47/52) and 50.0% (11/22), respectively. As the first symptom, 19.2% (10/52) of patients during APS presented only with intractable nausea, vomiting and hiccups; 80.8% (42/52) of patients experienced other neurological symptoms. The Departments of Gastroenterology and General Medicine were the departments that most frequently made the first diagnosis of APS, accounting for 54.1% and 17.6% of patients, respectively. The most common misdiagnoses related to diseases of the digestive system and the median duration of misdiagnosis was 37 days. Conclusions: APS is a common symptom of NMOSD and is associated with a high rate of misdiagnosis. Other concomitant symptoms often occur with APS. Gaining an increased awareness of this disease/syndrome, obtaining a detailed patient history, and performing physical examinations are essential if we are to reduce and avoid misdiagnosis.


Assuntos
Soluço , Neuromielite Óptica , Masculino , Feminino , Humanos , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico , Área Postrema , Estudos Retrospectivos , Soluço/etiologia , Soluço/complicações , Vômito/diagnóstico , Vômito/etiologia , Náusea/diagnóstico , Náusea/etiologia , Inflamação , Síndrome , Autoanticorpos , Erros de Diagnóstico , Aquaporina 4
18.
Beijing Da Xue Xue Bao Yi Xue Ban ; 55(6): 1118-1124, 2023 Dec 18.
Artigo em Zh | MEDLINE | ID: mdl-38101798

RESUMO

Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.


Assuntos
Neuromielite Óptica , Complicações na Gravidez , Síndrome de Sjogren , Adulto , Feminino , Humanos , Gravidez , Cesárea/efeitos adversos , Estado Terminal , Hidroxicloroquina/uso terapêutico , Hipestesia/complicações , Imunoglobulinas Intravenosas/uso terapêutico , Inflamação/complicações , Neuromielite Óptica/complicações , Neuromielite Óptica/terapia , Neuromielite Óptica/diagnóstico , Paralisia/complicações , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia , Rituximab/uso terapêutico , Síndrome de Sjogren/complicações , Esteroides/uso terapêutico , Transtornos da Visão
19.
J Pak Med Assoc ; 73(3): 471-475, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36932744

RESUMO

OBJECTIVE: To determine the prevalence of restless leg syndrome in patients with spinal cord injury using a consensus criterion. METHODS: The cross-sectional study was conducted from November 29, 2018, to February 28, 2021 at the departments of Neurology and Orthopaedic Surgery, King Edward Medical University, Mayo Hospital, Lahore, Pakistan, and comprised patients of either gender aged 18-80 years having spinal cord injuries. All the patients were interviewed using a 10-item questionnaire, and were assessed using the five-point consensus criteria of the International Restless Leg Syndrome Study Group. Data was analysed using SPSS 20. RESULTS: Of the 253 patients, 128(50.6%) were males and 125(49.4%) were females. The overall mean age was 38.6±14.2 years. Restless leg syndrome was present in 116(45.8%) patients, and 64(55.2%) of them were males (p>0.05). The mean duration of the symptoms was 18.9±16.9 months. Causes of spinal cord injury included metastasis 28(11.1%) multiple sclerosis 32(12.6%), neuromyelitis optica spectrum disorders 68(26.9%), tuberculous spondylitis 85(33.6%), trauma 24(9.5%) and viral myelitis 16(6.3%). CONCLUSIONS: Restless leg syndrome was prevalent in less than half the patients having spinal cord injury. It was more prevalent in males compared to females, but the difference was not significant.


Assuntos
Neuromielite Óptica , Síndrome das Pernas Inquietas , Traumatismos da Medula Espinal , Masculino , Feminino , Humanos , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Síndrome das Pernas Inquietas/epidemiologia , Prevalência , Estudos Transversais , Traumatismos da Medula Espinal/complicações , Traumatismos da Medula Espinal/epidemiologia , Neuromielite Óptica/complicações , Medula Espinal
20.
Pract Neurol ; 23(4): 314-316, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36639247

RESUMO

We report a case of conus medullaris syndrome presenting with lower limb and bladder symptoms. MR imaging showed an abnormality in the lowest part of the spinal cord as a first presentation of myelin oligodendrocyte glycoprotein (MOG)-associated disease. While such cord swelling can mimic a tumour, these patients respond well to corticosteroids, with good outcomes. MOG-associated disease is an immune-mediated syndrome distinct from aquaporin 4 antibody positive neuromyelitis optica syndrome and is now considered an independent entity. Although there can be overlapping phenotypes, there are also differences, and MOG-associated disease generally has a much better prognosis compared with aquaporin 4 antibody-positive neuromyelitis optica syndrome.


Assuntos
Neuromielite Óptica , Compressão da Medula Espinal , Humanos , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/tratamento farmacológico , Glicoproteína Mielina-Oligodendrócito , Aquaporina 4 , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/etiologia , Autoanticorpos
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