Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 3.216
Filtrar
2.
Neurol India ; 69(5): 1412-1413, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34747827

RESUMO

Transverse myelitis in multiple sclerosis is typically a short cord lesion with patchy distribution. Rarely, longitudinally extensive transverse myelitis can be seen in those with highly active disease or frequent relapses. The recognition of this uncommon phenotype in multiple sclerosis is important as the treatment is largely different from other demyelinating diseases. We describe a patient with highly active relapsing-remitting multiple sclerosis on interferon beta-1a who developed LETM after multiple relapses.


Assuntos
Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Mielite Transversa , Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Humanos , Imageamento por Ressonância Magnética , Esclerose Múltipla Recidivante-Remitente/complicações , Mielite Transversa/etiologia , Recidiva Local de Neoplasia
3.
BMJ Case Rep ; 14(11)2021 Nov 12.
Artigo em Inglês | MEDLINE | ID: mdl-34772679

RESUMO

The spectrum of central nervous system demyelinating disorders is vast and heterogeneous and, often, with overlapping clinical presentations. Misdiagnosis might occur in some cases with serious therapeutic repercussions. However, introduction of several new biomarkers such as aquaporin-4 IgG and myelin oligodendrocyte glycoprotein IgG has made distinction between diseases such as multiple sclerosis and myelin oligodendrocyte glycoprotein antibody-associated disorder easier. Here, we report a case of a 15-year-old male patient with subacute multifocal neurological presentation without encephalopathy, eventually diagnosed as myelin oligodendrocyte glycoprotein antibody-associated disorder.


Assuntos
Esclerose Múltipla , Neuromielite Óptica , Adolescente , Aquaporina 4 , Ataxia , Autoanticorpos , Disartria , Humanos , Masculino , Glicoproteína Mielina-Oligodendrócito
4.
Acta Neurol Taiwan ; 30(3): 108-112, 2021 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-34841506

RESUMO

PURPOSE: Neuromyelitis optica (NMO) spectrum disorder and multiple sclerosis (MS) have similar clinical presentations which may make a diagnostic difficulty, especially when the data of aquaporin-4 (AQP4) antibody is not available. We reported the diagnostic and therapeutic dilemma of a woman with a delayed diagnosis of NMO spectrum disorder for more than 20 years. CASE REPORT: The patient was a 51 years old woman who suffered from several episodes of relapsing and remission of limbs weakness, visual impairment and gait disturbance since 29 years old. She was diagnosed as a case of MS and received treatment accordingly. Treatment with the use of Rebif was started since 2008-2012, and was then shifted to Fingolimod due to several minor attacks were still noted during this period. Serum AQP4-IgG was checked before the use of Fingolimod by using Enzyme-linked immunosorbent assay (ELISA) and the result showed sero-negative for this Ab. However, occasional minor attacks were still noted. In May 2018, severe relapsing developed and brain magnetic resonance imaging (MRI) showed marked progression of the brain lesion. Initially, progressive multifocal leukoencephalopathy was suspected, but both cerebrospinal fluid and serologic study for John Cunningham virus (JCV) were negative. AQP4-IgG was rechecked by using cell-based assay (CBA), and the result showed positive finding. Thereafter, her therapy was changed to NMO spectrum disorder regimen. CONCLUSION: It is worthwhile to recheck the serum AQP4-IgG if the initial study showed negative result by using ELISA since CBA has higher sensitivity than previous study method.


Assuntos
Neuromielite Óptica , Adulto , Aquaporina 4 , Autoanticorpos , Diagnóstico Tardio , Feminino , Humanos , Pessoa de Meia-Idade , Neuroimagem , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/tratamento farmacológico
5.
Front Immunol ; 12: 727750, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34721390

RESUMO

Neuromyelitis optica spectrum disorder (NMOSD), a relapsing autoimmune disease of the central nervous system, mainly targets the optic nerve and spinal cord. To date, all attempts at the establishment of NMOSD animal models have been based on neuromyelitis optica immunoglobulin G antibody (NMO-IgG) and mimic the disease in part. To solve this problem, we developed a rodent model by opening the blood-brain barrier (BBB) with low frequency ultrasound, followed by injection of NMO-IgG from NMOSD patients and complement to mice suffering pre-existing neuroinflammation produced by experimental autoimmune encephalomyelitis (EAE). In this study, we showed that ultrasound with NMO-IgG and complement caused marked inflammation and demyelination of both spinal cords and optic nerves compared to blank control group, as well as glial fibrillary acidic protein (GFAP) and aquaporin-4 (AQP4) loss of spinal cords and optic nerves compared to EAE mice and EAE mice with only BBB opening. In addition, magnetic resonance imaging (MRI) revealed optic neuritis with spinal cord lesions. We further demonstrated eye segregation defects in the dorsal lateral geniculate nucleus (dLGN) of these NMOSD mice.


Assuntos
Proteínas do Sistema Complemento/imunologia , Encefalomielite Autoimune Experimental/imunologia , Imunoglobulina G/imunologia , Neuromielite Óptica/imunologia , Animais , Aquaporina 4/metabolismo , Barreira Hematoencefálica/metabolismo , Modelos Animais de Doenças , Encefalomielite Autoimune Experimental/diagnóstico por imagem , Encefalomielite Autoimune Experimental/metabolismo , Feminino , Proteína Glial Fibrilar Ácida/metabolismo , Humanos , Imageamento por Ressonância Magnética , Camundongos Endogâmicos C57BL , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/metabolismo , Nervo Óptico/diagnóstico por imagem , Nervo Óptico/imunologia , Nervo Óptico/metabolismo , Medula Espinal/diagnóstico por imagem , Medula Espinal/imunologia , Medula Espinal/metabolismo , Ondas Ultrassônicas
6.
Ideggyogy Sz ; 74(9-10): 309-321, 2021 Sep 30.
Artigo em Húngaro | MEDLINE | ID: mdl-34657404

RESUMO

Treatment and new evidences in neuromyelitis optica spectrum disorder Illés Zs, MD, PhD Ideggyogy Sz 2021;74(9-10):309-321. Neuromyelitis optica spectrum disorder (NMOSD) is associated with antibodies against AQP4 in about 80% of the cases. In about one-fourth of seronegative cases, antibodies against the MOG protein are present in the serum (MOG-antibody associated disease, MOGAD). This article discusses off-label azathioprine and mycophenolate mofetil in the treatment of NMOSD and reviews the evidence-based clinical aspects of B/plasma cell depletion, antagonization of IL-6 signaling and blocking the complement pathway. The review also summarizes basic aspects of NMOSD pregnancy focusing on treatment, and the different therapeutic approach in MOGAD. In the recent two years, phase 3 clinical trials provided class I evidence for the efficacy and safety of rituximab (anti-CD20), inebilizumab (anti-CD19), tocilizumab (anti-IL6R), satralizumab (anti-IL6R), and eculizumab (anti-C5) in combination with other immunosuppressants or in monotherapy. The treatment approach in MOGAD is complicated by the monophasic course in about half of the cases and by the potential disappearance of MOG antibody. The necessity of maintenance treatment in MOGAD should be decided after tapered oral steroid. Immunosuppression is recommended in NMOSD during pregnancy and lactation, and this should be considered for optimal selection of treatment in fertile female patients. The new monoclonal antibodies broadened treatment options NMOSD, and the treatment strategy of MOGAD has become more straightforward.


Assuntos
Neuromielite Óptica , Anticorpos Monoclonais Humanizados , Aquaporina 4 , Feminino , Humanos , Neuromielite Óptica/tratamento farmacológico
8.
PLoS One ; 16(10): e0258165, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34597351

RESUMO

Brain aquaporin 1 (AQP1) and AQP4 are involved in cerebrospinal fluid (CSF) homeostasis and might participate in the origin of hydrocephalus. Studies have shown alterations of perivascular AQP4 expression in idiopathic normal pressure hydrocephalus (iNPH) and Alzheimer's disease (AD). Due to the overlapping of clinical signs between iNPH and certain neurological conditions, mainly AD, specific biomarkers might improve the diagnostic accuracy for iNPH. The goal of the present study was to analyze and quantify the presence of AQP1 and AQP4 in the CSF of patients with iNPH and AD to determine whether these proteins can be used as biomarkers of iNPH. We examined AQP1 and AQP4 protein levels in the CSF of 179 participants (88 women) classified into 5 groups: possible iNPH (81 participants), hydrocephalus associated with other neurological disorders (13 participants), AD (41 participants), non-AD dementia (32 participants) and healthy controls (12 participants). We recorded each participant's demographic and clinical variables and indicated, when available in the clinical history, the record of cardiovascular and respiratory complications. An ELISA showed virtually no AQP content in the CSF. Information on the vascular risk factors (available for 61 patients) confirmed some type of vascular risk factor in 86% of the patients with possible iNPH and 58% of the patients with AD. In conclusion, the ELISA analysis showed insufficient sensitivity to detect the presence of AQP1 and AQP4 in CSF, ruling out the possible use of these proteins as biomarkers for diagnosing iNPH.


Assuntos
Doença de Alzheimer/diagnóstico , Aquaporina 1/líquido cefalorraquidiano , Aquaporina 4/líquido cefalorraquidiano , Diagnóstico Diferencial , Hidrocefalia de Pressão Normal/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/líquido cefalorraquidiano , Doença de Alzheimer/genética , Doença de Alzheimer/patologia , Peptídeos beta-Amiloides/genética , Biomarcadores/líquido cefalorraquidiano , Feminino , Humanos , Hidrocefalia de Pressão Normal/líquido cefalorraquidiano , Hidrocefalia de Pressão Normal/genética , Hidrocefalia de Pressão Normal/patologia , Masculino , Pessoa de Meia-Idade , Fragmentos de Peptídeos/líquido cefalorraquidiano , Proteínas tau/genética
10.
Int J Mol Sci ; 22(18)2021 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-34575909

RESUMO

Aquaporin-4 (AQP4) is the principal water channel in the brain being expressed in astrocytes and ependymal cells. AQP4 plays an important role in cerebrospinal fluid (CSF) homeostasis, and alterations in its expression have been associated with hydrocephalus. AQP4 contributes to the development of hydrocephalus by hypoxia in aged mice, reproducing such principal characteristics of the disease. Here, we explore whether these alterations associated with the hydrocephalic state are permanent or can be reverted by reexposure to normoxia. Alterations such as ventriculomegaly, elevated intracranial pressure, and cognitive deficits were reversed, whereas deficits in CSF outflow and ventricular distensibility were not recovered, remaining impaired even one month after reestablishment of normoxia. Interestingly, in AQP4-/- mice, the impairment in CSF drainage and ventricular distensibility was completely reverted by re-normoxia, indicating that AQP4 has a structural role in the chronification of those alterations. Finally, we show that aged mice subjected to two hypoxic episodes experience permanent ventriculomegaly. These data reveal that repetitive hypoxic events in aged cerebral tissue promote the permanent alterations involved in hydrocephalic pathophysiology, which are dependent on AQP4 expression.


Assuntos
Aquaporina 4/genética , Suscetibilidade a Doenças , Hidrocefalia/etiologia , Hidrocefalia/metabolismo , Hipóxia/genética , Hipóxia/metabolismo , Fatores Etários , Animais , Aquaporina 4/líquido cefalorraquidiano , Aquaporina 4/metabolismo , Biomarcadores , Encéfalo/metabolismo , Encéfalo/patologia , Encéfalo/fisiopatologia , Modelos Animais de Doenças , Hidrocefalia/diagnóstico , Hidrocefalia/patologia , Imuno-Histoquímica , Imageamento por Ressonância Magnética , Camundongos , Fenótipo
11.
Medicine (Baltimore) ; 100(38): e27207, 2021 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-34559109

RESUMO

RATIONALE: Neuromyelitis optica spectrum disorder (NMOSD) associated with active replication of hepatitis B virus (HBV) is rare. High-dose corticosteroids are the mainstay treatment of NMOSD; however, these may cause reactivation of viral replication in patients with stable HBV which may lead to liver damage. Therefore, care should be placed in corticosteroid use in patients with NMOSD and HBV infection. PATIENT CONCERNS: Herein, we report the case of a 31-year-old woman with NMOSD and HBV infection who was seropositive for anti-aquaporin-4 antibody. The stable and HBV carrier status of the patient led to the deferment of antiviral and hepatoprotective agents in early treatment. However, liver function impairment was detected during follow-up, with an improvement in the best-corrected visual acuity. DIAGNOSES: The patient was diagnosed with NMOSD with active replication of HBV and seropositive anti-aquaporin-4 antibody considering the medical history and ancillary examinations. INTERVENTIONS: To manage NMOSD, intravenous high-dose methylprednisolone (20 mg/kg d) was administered for 5 days which was gradually tapered to oral steroids. However, liver function impairment was observed during follow-up; therefore, anti-HBV drugs (entecavir) and hepatoprotective drugs (bicyclol or polyunsaturated phosphatidylcholine) were administered. OUTCOMES: A marked improvement was observed in the patient's best-corrected visual acuity after 4 weeks of treatment. However, follow-up examinations revealed liver function damage which necessitated administration of antiviral and hepatoprotective drugs. Liver function normalized after 1 month. LESSON: This case underscores the importance of preventive treatment of liver protection in patients with HBV infection prior to or simultaneous with glucocorticoid therapy and furthermore, there is an urgent need to develop authoritative guidelines regulating corticosteroid use in the treatment of patients with HBV infection.


Assuntos
Corticosteroides/uso terapêutico , Aquaporina 4/imunologia , Hepatite B Crônica/complicações , Metilprednisolona/uso terapêutico , Neuromielite Óptica/diagnóstico , Corticosteroides/administração & dosagem , Corticosteroides/efeitos adversos , Adulto , Autoanticorpos/sangue , Diagnóstico Diferencial , Feminino , Humanos , Infusões Intravenosas , Metilprednisolona/administração & dosagem , Metilprednisolona/efeitos adversos , Neuromielite Óptica/complicações , Neuromielite Óptica/tratamento farmacológico
12.
Arch Microbiol ; 203(10): 6329-6335, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34562144

RESUMO

The pathology of streptococcal meningitis is poorly understood, even though streptococcal infection induces meningitis. The aim of this study was to clarify the relationship between streptococcal meningitis and aquaporin 4 (AQP4) in the mouse brain. After Streptococcus suis infection, the streptococcal number was calculated, and AQP4 mRNA expression in the brain was quantified at 2 and 7 days after infection. At 7-day post-infection, mice with neurological symptoms showed significantly higher S. suis levels in the brain than mice without neurological symptoms. AQP4 expression was significantly decreased in mice with neurological symptoms than in mice without neurological symptoms. Image analysis demonstrated that S. suis progressed to invade the white matter. Pathological analysis revealed that infected mouse brains had higher inflammation and neurological damage scores than uninfected mouse brains. Therefore, mice with neurological symptoms caused by streptococcal meningitis had high S. suis levels in the brain and reduced AQP4 expression.


Assuntos
Meningites Bacterianas , Meningite , Infecções Estreptocócicas , Streptococcus suis , Animais , Aquaporina 4/genética , Encéfalo , Modelos Animais de Doenças , Camundongos , Streptococcus suis/genética
13.
J Med Case Rep ; 15(1): 391, 2021 Aug 06.
Artigo em Inglês | MEDLINE | ID: mdl-34353370

RESUMO

BACKGROUND: Concomitant diagnosis of neuromyelitis optica spectrum disease and pulmonary tuberculosis has rarely been reported. CASE REPORT: We report a case involving a young Tunisian male patient who developed dry cough followed, 2 months later, by weakness in the lower limbs. The findings of central nervous system imaging and anti-aquaporin-4 antibody positivity were compatible with the diagnosis of neuromyelitis optica spectrum disease. Constellation of the clinical and the typical radiological pulmonary findings in our patient, coming from an endemic region, allowed the diagnosis of pulmonary tuberculosis, although sputum smear examination for acid-fast bacilli and cultures was negative. The patient received anti-tuberculous polytherapy associated with immunomodulation, consisting of methylprednisolone and intravenous immunoglobulins. Pulmonary infection symptoms initially improved but with no motor recovery. The patient suddenly died at home 4 months after the onset of the first symptoms. Current data regarding the clinical presentation of this underreported concomitant or associated condition, the possible pathophysiological mechanisms, and the therapeutic options were reviewed. CONCLUSIONS: This case underscores the necessity to understand the exact mechanism of these coincident entities and to clarify the best immunomodulatory choice since immunosuppression targeting neuromyelitis optica spectrum disease can lead to dissemination of pulmonary tuberculosis.


Assuntos
Neuromielite Óptica , Tuberculose Pulmonar , Aquaporina 4 , Autoanticorpos , Humanos , Masculino , Metilprednisolona/uso terapêutico , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/tratamento farmacológico , Tuberculose Pulmonar/complicações , Tuberculose Pulmonar/diagnóstico , Tuberculose Pulmonar/tratamento farmacológico
14.
Artigo em Russo | MEDLINE | ID: mdl-34460164

RESUMO

OBJECTIVE: To present clinical and epidemiological aspects of neuromyelitis optica spectrum disorders (NMOSD) in the Russian Federation. MATERIAL AND METHODS: We studied 142 patients who met diagnostic criteria of 2015 for NMOSD. Sex, age at disease onset, presence or absence of aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), mail clinical symptoms, oligoclonal IgG, therapy for the treatment of exacerbations and prevention of exacerbations, compliance with 2006 diagnostic criteria were assessed. RESULTS: The prevalence of women is 4.26:1, the most frequent age at disease onset is 18-29 years (36% of cases). The laboratory aspects of the disease are characterized and approaches to the treatment and prevention of exacerbations of NMOSD in patients of the Russian population are evaluated. Approaches to diagnostics are compared depending on the applied diagnostic criteria (34% of patients do not meet neuromyelitis optica 2006 diagnostic criteria). A prognosis for the prevalence of NMOSD in the Russian population has been proposed: 0.45-4.21/100000. CONCLUSION: This is the first published data on clinical and epidemiological characteristics of NMOSD in the Russian Federation.


Assuntos
Aquaporina 4 , Neuromielite Óptica , Autoanticorpos , Feminino , Humanos , Glicoproteína Mielina-Oligodendrócito , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/epidemiologia , Federação Russa/epidemiologia
15.
Brain Nerve ; 73(9): 983-989, 2021 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-34462369

RESUMO

Cerebral edema is a major contributor to the mortality associated with ischemic stroke and traumatic brain injuries; however, limited therapeutic strategies are available for cerebral edema. Aquaporin-4 (AQP4), the main water channel in the brain plays a key role in water homeostasis and edema formation in the central nervous system. Therefore, regulation of AQP4 function or expression is considered a possible target for treatment of edema. Despite extensive research over several decades, AQP4 inhibitors have not been approved for the treatment of edema in humans. Further studies are warranted to gain a deeper understanding of the exact properties and functions of AQP4, to facilitate the development of newer therapeutic approaches for cerebral edema.


Assuntos
Edema Encefálico , Aquaporina 4 , Encéfalo/metabolismo , Sistema Nervoso Central , Humanos , Água/metabolismo
16.
Zh Nevrol Psikhiatr Im S S Korsakova ; 121(7. Vyp. 2): 5-12, 2021.
Artigo em Russo | MEDLINE | ID: mdl-34387440

RESUMO

Neuromyelitis optica spectrum disorder (NMOSD) is a group of rare and mostly severe autoimmune demyelinating central nervous system disorders which prevalence is 0.7-1 per 100.000 population and incidence is 0.037-0.73 per 100.000 person-years. NMOSD may present as a combination of uni- or bilateral optic neuritis, transverse myelitis or lesions of brain stem and other brain regions. The symptoms are mostly relapsing (up to 97.5%) and progressive. Occurrence of relapses is associated with seropositivity for aquaporin-4 (up to 80% of NMOSD patients) and bears a less favorable prognosis (mortality up to 32%). Women seropositive for aquaporin 4 constitute 90% of NMOSD patients. Compared to other demyelinating disorders, NMOSD is characterized by late onset (mean age is about 39 years) and association with other autoimmune disorders, including systemic lupus erythematosus, myasthenia gravis and Sjogren's syndrome. A genetic predisposition was found among Blacks and Asians, with HLA-DRB1*03:01 gene associated with higher risk of NMOSD in Asians. The course of the disease tends to be more severe in Blacks. There are clusters of an increased incidence of NMOSD in the Carribeans and in the Far East. Continued increase of prevalence and incidence of NMOSD worldwide compels continued epidemiological research in order to provide early diagnosis and treatment for this disorder.


Assuntos
Miastenia Gravis , Neuromielite Óptica , Neurite Óptica , Adulto , Aquaporina 4 , Feminino , Humanos , Recidiva Local de Neoplasia , Neuromielite Óptica/epidemiologia
17.
Int J Mol Sci ; 22(16)2021 Aug 11.
Artigo em Inglês | MEDLINE | ID: mdl-34445343

RESUMO

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune central nervous system (CNS) inflammatory disorder that can lead to serious disability and mortality. Females are predominantly affected, including those within the reproductive age. Most patients develop relapsing attacks of optic neuritis; longitudinally extensive transverse myelitis; and encephalitis, especially brainstem encephalitis. The majority of NMOSD patients are seropositive for IgG autoantibodies against the water channel protein aquaporin-4 (AQP4-IgG), reflecting underlying aquaporin-4 autoimmunity. Histological findings of the affected CNS tissues of patients from in-vitro and in-vivo studies support that AQP4-IgG is directly pathogenic in NMOSD. It is believed that the binding of AQP4-IgG to CNS aquaporin-4 (abundantly expressed at the endfoot processes of astrocytes) triggers astrocytopathy and neuroinflammation, resulting in acute attacks. These attacks of neuroinflammation can lead to pathologies, including aquaporin-4 loss, astrocytic activation, injury and loss, glutamate excitotoxicity, microglial activation, neuroinflammation, demyelination, and neuronal injury, via both complement-dependent and complement-independent pathophysiological mechanisms. With the increased understanding of these mechanisms underlying this serious autoimmune astrocytopathy, effective treatments for both active attacks and long-term immunosuppression to prevent relapses in NMOSD are increasingly available based on the evidence from retrospective observational data and prospective clinical trials. Knowledge on the indications and potential side effects of these medications are essential for a clear evaluation of the potential benefits and risks to NMOSD patients in a personalized manner. Special issues such as pregnancy and the coexistence of other autoimmune diseases require additional concern and meticulous care. Future directions include the identification of clinically useful biomarkers for the prediction of relapse and monitoring of the therapeutic response, as well as the development of effective medications with minimal side effects, especially opportunistic infections complicated by long-term immunosuppression.


Assuntos
Neuromielite Óptica/terapia , Aquaporina 4/imunologia , Autoanticorpos/fisiologia , Biomarcadores/análise , Biomarcadores/sangue , Humanos , Imunossupressão/métodos , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/etiologia , Neuromielite Óptica/patologia
18.
Front Immunol ; 12: 650782, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34367127

RESUMO

The changes in the serum levels of aquaporin-4-IgG (AQP4-IgG), immunoglobulins, and inflammatory mediators in neuromyelitis optica spectrum disorder (NMOSD) cases treated with immunoadsorption have been rarely described in detail. Here we report a 29-year-old steroid-resistant NMOSD female with a severe disability (bilateral blindness and paraplegia) who received protein-A immunoadsorption as a rescue treatment. During the total 5 sessions, the circulating level of AQP4-IgG, immunoglobulins, and complement proteins (C3 and C4) showed a rapid and sawtooth-like decrease, and the serum AQP4-IgG titer declined from 1:320 to below the detectable limit at the end of the 3rd procedure. Of all the antibodies, IgG had the biggest removal rate (>96.1%), followed by IgM (>66.7%) and IgA (53%), while complement C3 and C4 also dropped by 73% and 65%, respectively. The reduced pro-inflammatory cytokines (interleukin-8 and tumor necrosis factor-α) and marked increased lymphocyte (T and B cell) counts were also observed. The improvement of symptoms initiated after the last session, with a low AQP4-IgG titer (1:32) persisting thereafter. Accordingly, protein-A immunoadsorption treatment could be one of the potential rescue therapies for steroid-resistant NMOSD patients with a severe disability.


Assuntos
Aquaporina 4/imunologia , Biomarcadores/sangue , Imunoglobulina G/imunologia , Neuromielite Óptica/terapia , Plasmaferese/métodos , Proteína Estafilocócica A/imunologia , Adulto , Complemento C3/imunologia , Complemento C4/imunologia , Feminino , Humanos , Imunoglobulina G/sangue , Interleucina-8 , Contagem de Linfócitos , Neuromielite Óptica/sangue , Neuromielite Óptica/imunologia , Fator de Necrose Tumoral alfa/sangue , Fator de Necrose Tumoral alfa/imunologia
19.
Orphanet J Rare Dis ; 16(1): 369, 2021 08 30.
Artigo em Inglês | MEDLINE | ID: mdl-34461943

RESUMO

BACKGROUND: Neuromyelitis Optica Spectrum Disorders (NMOSD) are rare inflammatory diseases of the central nervous system that cause transverse myelitis and optic neuritis. Steroids are commonly administered in NMOSD patients. The use of steroids may lead to osteonecrosis, which makes some of the NMOSD patients candidate for total hip arthroplasty (THA). To date, the clinical outcome of THA in NMOSD patients have not been investigated. AIM: Investigate the patient reported outcome measures (PROM), radiographic outcome and complication in NMOSD patients after THA, compared with that of non-NMOSD patients. METHODS: Patients from Jan. 2016 to October. 2020 were identified in our database. 12 NMOSD cases which met the inclusion criteria were matched to non-NMOSD cases in a ratio of 1:2 based on age, sex, Charlson Comorbidity Index (CCI) and surgical date. Relevant outcome were analyzed and compared between the two groups. RESULTS: There was a significantly increased risk of dislocation in NMOSD patients. Post-operative HOOS score was similar between the two groups even though the pre-operative HOOS score is significantly higher in the non-NMOSD group. NMOSD patients had poor performance in EQ-5D and EQ-VAS. The cups were placed more anteverted in NMOSD cases (P = 0.01). CONCLUSION: There is a significantly increased risk of dislocation after THA in NMOSD patients. However, satisfactory improvement in functional outcome of the hip was achieved. Due to the natural process of NMOSD, rehabilitation and hip precaution should be patient-specific and time-specific.


Assuntos
Artroplastia de Quadril , Neuromielite Óptica , Aquaporina 4 , Artroplastia de Quadril/efeitos adversos , Estudos de Casos e Controles , Humanos , Estudos Retrospectivos
20.
Front Immunol ; 12: 677190, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34335576

RESUMO

Background: Neuromyelitis optica spectrum disorders (NMOSDs) are severe inflammatory diseases mediated mainly by humoral and cellular immunity. Circulating follicular helper T (Tfh) cells are thought to be involved in the pathogenesis of NMOSD, and serum C-X-C motif ligand 13 (CXCL13) levels reflect the effects of Tfh cells on B-cell-mediated humoral immunity. Immune cell and cytokine changes during the dynamic relapsing and remitting processes in NMOSD require further exploration. Patients and methods: Blood samples were collected from 36 patients in acute and recovery phases of NMOSD, 20 patients with other noninflammatory neurological diseases (ONND) and 20 age- and sex-matched healthy volunteers. CD4+CXCR5+PD-1+ Tfh cells were detected by flow cytometry, and serum CXCL13 levels were assessed by enzyme-linked immunosorbent assay (ELISA). Results: The percentage of CD4+CXCR5+PD-1+ Tfh cells was significantly higher during the acute phase than during the recovery phase, and serum CXCL13 levels were significantly higher in patients in the acute and recovery phases of NMOSD than in the ONND and control groups. The Tfh cell percentage was positively correlated with CXCL13 levels, and both were positively correlated with Expanded Disability Status Scale (EDSS) scores and cerebrospinal fluid protein levels in patients with acute NMOSD. Conclusion: Circulating Tfh cells level has the potential to be a biomarker of disease severity.


Assuntos
Quimiocina CXCL13/sangue , Neuromielite Óptica/sangue , Neuromielite Óptica/imunologia , Índice de Gravidade de Doença , Células T Auxiliares Foliculares/imunologia , Adulto , Anticorpos/sangue , Anticorpos/imunologia , Aquaporina 4/imunologia , Biomarcadores/sangue , Antígenos CD4/metabolismo , Estudos de Casos e Controles , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Imunidade Celular , Masculino , Pessoa de Meia-Idade , Receptor de Morte Celular Programada 1/metabolismo , Receptores CXCR5/metabolismo
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...