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We aimed to compare seroprevalence of anti-myelin oligodendrocyte glycoprotein (MOG) and anti-aquaporin-4 (AQP4) antibodies in Korean adults with inflammatory demyelinating diseases (IDDs) of the central nervous system (CNS), based on a multicenter nationwide database. Sera were analyzed using a live cell-based assay for MOG and AQP4 antibodies. Of 586 Korean adults with IDDs of the CNS, 36 (6.1%) and 185 (31.6%) tested positive for MOG and AQP4 antibodies, respectively. No participant showed double positivity. Seroprevalence of MOG antibodies was about five times lower than that of AQP4 antibodies in a large cohort of Korean adults with IDDs of the CNS.
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Aquaporina 4 , Doenças do Sistema Nervoso Central , Adulto , Humanos , Glicoproteína Mielina-Oligodendrócito , República da Coreia/epidemiologia , Estudos SoroepidemiológicosRESUMO
Colony-stimulating factor 1 receptor (CSF1R)-related leukoencephalopathy is one of the most common causes of adult-onset leukodystrophy and is caused by mutation of the CSF1R gene. Brain magnetic resonance imaging (MRI) findings in asymptomatic patients have not been well recognized. We report on the case of a patient with CSF1R-related leukoencephalopathy who had a novel missense variant of the CSF1R gene with a family history of early onset dementia. This is a representative case of CSF1R-related leukoencephalopathy, which shows the progression of brain MRI and cognitive decline from an asymptomatic state.
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Leucoencefalopatias , Adulto , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Humanos , Leucoencefalopatias/diagnóstico por imagem , Leucoencefalopatias/genética , Leucoencefalopatias/patologia , Imageamento por Ressonância Magnética , Mutação , Neuroimagem/métodos , Testes NeuropsicológicosRESUMO
BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) targets astrocytes and elevates the levels of astrocyte-injury markers during attacks. FAM19A5, involved in reactive gliosis, is secreted by reactive astrocytes following central nervous system (CNS) damage. OBJECTIVE: To investigate the significance of serum FAM19A5 in patients with NMOSD. METHODS: We collected clinical data and sera of 199 patients from 11 hospitals over 21 months. FAM19A5 levels were compared among three groups: NMOSD with positive anti-aquaporin-4 antibody (NMOSD-AQP4), other CNS demyelinating disease, and healthy controls. RESULTS: The median serum FAM19A5 level was higher in the NMOSD-AQP4 (4.90 ng/mL (3.95, 5.79)) than in the other CNS demyelinating (2.35 ng/mL (1.83, 4.07), p < 0.001) or healthy control (1.02 ng/mL (0.92, 1.14), p < 0.001) groups. There were significant differences in the median serum FAM19A5 levels between the attack and remission periods (5.89 ng/mL (5.18, 6.98); 4.40 ng/mL (2.72, 5.13), p < 0.001) in the NMOSD-AQP4 group. Sampling during an attack (p < 0.001) and number of past attacks (p = 0.010) were independently associated with increased serum FAM19A5. CONCLUSION: Serum FAM19A5 was higher in patients with NMOSD-AQP4 and correlated with clinical characteristics. Thus, serum FAM19A5 may be a novel clinical biomarker for NMOSD-AQP4.
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Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Biomarcadores , Humanos , Glicoproteína Mielina-Oligodendrócito , Neuromielite Óptica/diagnósticoRESUMO
PURPOSE: Few studies have assessed pre-surgery cognitive impairment or the impact of pre-surgery cognitive impairment on quality of life. The purpose of this study was to assess changes in perceived cognitive function from pre-surgery to 1 month post-surgery and to determine whether cognitive function predicted health-related quality of life in women who awaited adjuvant treatment for breast cancer. METHODS: This study used a descriptive pre-post design to assess women newly diagnosed with breast cancer prior to any treatment (N = 132). Cognition was assessed using the Attentional Function Index (AFI) and health-related quality of life was assessed using the Functional Assessment of Cancer Therapy-General (FACT-G). Statistical methods included descriptive, comparative and regression analyses. Covariates assessed and controlled for in analyses included depressed mood, fatigue, disturbed sleep, surgery-related symptoms (lymphedema/decreased mobility), and cultural tendency. RESULTS: Perceived attention and memory function decreased from pre-surgery to 1 month post-surgery alongside alterations in arm function and a decrease in depressed mood (p < 0.05). Regression analysis indicated that, after controlling for covariates, poorer perceived attention and memory function, surgery-specific symptoms, and a greater tendency toward collectivism predicted poorer quality of life. CONCLUSION: Perceived function on tasks requiring attention and working memory 1 month post-surgery was poorer compared to pre-surgery suggesting that the mental and physical demands of a new diagnosis of breast cancer and surgery may effect cognitive function. Additionally, changes in perceived cognitive function significantly predicted perceived quality of life in women awaiting adjuvant treatment for breast cancer. Findings suggest that breast cancer patients are at risk for an early decline in cognitive function and that interventions aimed at supporting and optimizing function may improve quality of life early in the disease trajectory.
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Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/psicologia , Neoplasias da Mama/cirurgia , Quimioterapia Adjuvante/psicologia , Disfunção Cognitiva/psicologia , Qualidade de Vida/psicologia , Estresse Psicológico/complicações , Adulto , Feminino , Humanos , Pessoa de Meia-Idade , Período Pós-Operatório , República da Coreia , Tempo para o TratamentoRESUMO
Neuromyelitis optica spectrum disorders (NMOSD) can cause various ocular motor disorders in addition to optic neuritis. Ocular motor findings associated with NMOSD include spontaneous vertical and gaze-evoked nystagmus, wall-eyed bilateral internuclear ophthalmoplegia, and trochlear nerve palsy. The association between dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity has not been reported. Here, we report a patient displaying typical dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity.
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Encefalopatias/fisiopatologia , Mesencéfalo/patologia , Neuromielite Óptica/fisiopatologia , Aquaporina 4/imunologia , Autoanticorpos/sangue , Autoanticorpos/imunologia , Autoantígenos/imunologia , Encefalopatias/imunologia , Feminino , Humanos , Pessoa de Meia-Idade , Neuromielite Óptica/imunologia , SíndromeRESUMO
BACKGROUND: Chemotherapy-induced peripheral neuropathy (CIPN) is a major symptom of distress among chemotherapy-treated cancer survivors. Although various interventions have been attempted, there is no criterion-standard treatment. OBJECTIVE: The aim of this study was to examine the efficacy and safety of auricular acupressure (AA) in improving peripheral neuropathy symptoms in breast cancer patients undergoing taxane-based treatment. METHODS: A total of 51 eligible participants were enrolled and randomly allocated (1:1) to AA or sham control groups. The intervention was performed weekly for 3 weeks using different ear points, depending on the group. The Total Neuropathy Score was used to measure CIPN objectively. The Numerical Rating Scale and the European Organization for Research and Treatment of Cancer Quality of Life Chemotherapy-Induced Peripheral Neuropathy-20 were used to measure the subjective symptoms of CIPN. Outcomes were compared between groups by time interaction using generalized estimating equations based on the intention-to-treat principle. RESULTS: Only 1 participant dropped out because of nausea and mild fever. The results of generalized estimating equations showed significant improvement in Numerical Rating Scale scores on the hands and feet and total Chemotherapy-Induced Peripheral Neuropathy-20 in the experimental group compared with the sham control group (all Ps < .05). Although the experimental group showed a greater reduction in Total Neuropathy Score scores than the sham control group, no significant differences were found. CONCLUSION: Auricular acupressure is an effective and safe nurse-led intervention for managing CIPN symptoms in breast cancer patients. IMPLICATIONS FOR PRACTICE: The findings help nurses to integrate AA easily and usefully into nursing care, contributing to managing symptoms of CIPN in cancer patients and survivors.
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A 49-year-old man presented with progressive asymmetric weakness and pain. Electrodiagnostic tests and nerve biopsy suggested chronic demyelinating polyneuropathy refractory to immune-modulating therapy. The patient's symptoms were aggravated, and he was finally diagnosed with T-cell lymphoma based on the findings of the second 18F-2 fluoro-2-deoxy-glucose positron emission tomography/CT performed 16 months after symptom onset. The patient received intravenous chemotherapy, but died 2 months later because of lymphoma progression. A clinical suspicion of neurolymphomatosis and early diagnosis are important for proper management.
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Doença Enxerto-Hospedeiro , Linfoma de Células T , Neurolinfomatose , Polineuropatia Paraneoplásica , Doença Enxerto-Hospedeiro/complicações , Humanos , Linfoma de Células T/complicações , Masculino , Pessoa de Meia-Idade , Polineuropatia Paraneoplásica/diagnóstico , Polineuropatia Paraneoplásica/etiologia , Tomografia por Emissão de Pósitrons , Tomografia Computadorizada por Raios X/efeitos adversosRESUMO
BACKGROUND AND OBJECTIVE: To investigate the clinical relevance of CSF myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) testing in a large multicenter cohort. METHODS: In this multicenter cohort study, paired serum-CSF samples from 474 patients with suspected inflammatory demyelinating disease (IDD) from 11 referral hospitals were included. After serum screening, patients were grouped into seropositive myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD, 31), aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG + NMOSD, 60), other IDDs (217), multiple sclerosis (MS, 45), and non-IDDs (121). We then screened CSF for MOG-IgG and compared the clinical and serologic characteristics of patients uniquely positive for MOG-IgG in the CSF to seropositive patients with MOGAD. RESULTS: Nineteen patients with seropositive MOGAD (61.3%), 9 with other IDDs (CSF MOG + IDD, 4.1%), 4 with MS (8.9%), but none with AQP4-IgG + NMOSD nor with non-IDDs tested positive in the CSF for MOG-IgG. The clinical, pathologic, and prognostic features of patients uniquely positive for CSF MOG-IgG, with a non-MS phenotype, were comparable with those of seropositive MOGAD. Intrathecal MOG-IgG synthesis, observed from the onset of disease, was shown in 12 patients: 4 of 28 who were seropositive and 8 who were uniquely CSF positive, all of whom had involvement of either brain or spinal cord. Both CSF MOG-IgG titer and corrected CSF/serum MOG-IgG index, but not serum MOG-IgG titer, were associated with disability, CSF pleocytosis, and level of CSF proteins. DISCUSSION: CSF MOG-IgG is found in IDD other than MS and also in MS. In IDD other than MS, the CSF MOG-IgG positivity can support the diagnosis of MOGAD. The synthesis of MOG-IgG in the CNS of patients with MOGAD can be detected from the onset of the disease and is associated with the severity of the disease. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that the presence of CSF MOG-IgG can improve the diagnosis of MOGAD in the absence of an MS phenotype, and intrathecal synthesis of MOG-IgG was associated with increased disability.
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Autoanticorpos/líquido cefalorraquidiano , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/líquido cefalorraquidiano , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/diagnóstico , Glicoproteína Mielina-Oligodendrócito/imunologia , Adolescente , Adulto , Autoanticorpos/sangue , Biomarcadores/líquido cefalorraquidiano , Estudos de Coortes , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/sangue , Pessoas com Deficiência , Feminino , Humanos , Imunoglobulina G , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
Background Many chemotherapeutic agents, especially taxanes, can induce peripheral neuropathy. Aim To evaluate the clinical characteristics of taxane-induced neuropathy (TIN) and determine the proper assessment tool for TIN in patients with breast cancer. Setting and Design Single-center, observational, prospective study. Methods and Material Forty-three patients with breast cancer treated with taxanes were prospectively enrolled. The reduced version of the Total Neuropathy Score (TNSr) was performed at baseline and 3 months after enrollment. TIN was diagnosed if the difference between the baseline and 3-month TNSr was greater than 1. In patients with TIN, the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire- Chemotherapy-Induced Peripheral Neuropathy (20-item scale (EORTC-CIPN20) was also assessed 3 months after enrollment. Results Thirty-seven out of 43 (86.0%) patients were diagnosed with TIN. Sensory symptoms (64.9%) were the most frequent abnormality, followed by autonomic symptoms (54.1%). No patients reported motor symptoms or motor weakness. The TNSr sensory symptom score positively correlated with that of the EORTC-CIPN20. Nerve conduction studies showed reduced nerve conduction velocities and amplitudes after taxane treatment compared to those before chemotherapy in all tested nerves; however, only three (8.1%) patients had sural sensory nerve action potential amplitude outside normal limits. Conclusions TIN was predominantly sensory with normal nerve conduction studies which is the main feature of small fiber neuropathy. A combination scale comprising of a clinician-based scale and a patient-reported questionnaire and specialized tests for small nerve fibers should be considered as proper assessment tools to evaluate TIN.
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BACKGROUND AND PURPOSE: The Boston Autonomic Symptom Questionnaire (BASQ) is a quantitative tool using a numeric rating scale to assess the symptoms of systemic dysautonomia, including cardiovascular, gastrointestinal, urinary, sudomotor, vasomotor, and sexual functions. The aim of this study was to validate the Korean version of the BASQ (KBASQ). METHODS: Prospectively enrolled subjects who submitted to autonomic function tests, including tests for cardiovagal, adrenergic, and sudomotor functions, also completed the KBASQ and the Korean version of the Orthostatic Grading Scale (KOGS), a validated questionnaire or assessing orthostatic symptoms.Twenty-eight subjects completed the KBASQ twice to assess test-retest reliability. We classified the subjects to dysautonomia or normal control group according to dysautonomic symptoms and the results of autonomic function tests. RESULTS: This study enrolled 225 subjects aged 54.0±18.1 years (mean±standard deviation), with a male/female ratio of 1/1.03. The internal validity of the KBASQ was excellent (Cronbach's α=0.922), and that of each of its subscales ranged from excellent to acceptable (Cronbach's α=0.709-0.952). The test-retest reliability was good, with correlation coefficients ranging from 0.354 to 0.917. The subcategory scores for the KBASQ were significantly higher in the dysautonomia group than in the normal control group. There were significant correlations among the items in the KBASQ and KOGS. There was also a significant correlation between KBASQ scores and the results of the autonomic function tests. CONCLUSIONS: The internal validity and reliability of the KBASQ were good, indicating that it may be a useful screening tool for the systematic evaluation of autonomic symptoms in patients with dysautonomia.
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BACKGROUND: Chemotherapy-induced peripheral neuropathy (CIPN) is a frequently-reported distress symptom in breast carcinoma patients under chemotherapy. Although previous studies emphasized lack of ideal neuroprotective or therapeutic agents for CIPN, there are no strongly recommended treatments. Nevertheless, auricular acupressure (AA) is a novel remedy for controlling symptoms in many healthcare settings. However, therapeutic effects of AA among patients with CIPN have not yet been elucidated fully. Therefore, we designed a trial to examine the effectiveness and safety of AA in breast cancer patients. METHODS: This randomized, double-blind, sham-controlled trial will assess 120 breast cancer survivors. After enrollment, the participants will be stratified depending on administration of medications prescribed for CIPN treatment, and then assigned randomly to the experimental or control groups in an allocation ratio of 1:1. For experimental groups, AA will be applied on four points, namely, shemen, liver, spleen, and finger/toe, while for the control groups, sham AA will be exerted on other four points that are remote from the treatment points and unrelated to neuropathic symptoms. All participants will undergo the same acupressure procedure, for two minutes each time, thrice daily for 3 weeks, and CIPN symptoms and health-related quality of life will be assessed by a blinded research assistant and a physician before, during, immediately after, and 4 weeks after AA. Occurrence, type, and severity of safety issues will be routinely monitored to confirm the non-toxic nature of AA. Repeated measures analysis of variance will be used to examine the changing pattern of CIPN symptoms and based on sensitivity analysis. DISCUSSION: It is expected that the rationale and design of this protocol will offer knowledge regarding a standardized process to guide current and future studies and accumulates clinical experiences in applying non-pharmacological intervention. The present trial is the first to examine the therapeutic effects of AA in breast cancer patients with CIPN. The findings of the study may provide convincing evidence regarding the effectiveness of CIPN symptoms. TRIAL REGISTRAION: Clinical Research Information Service, Republic of Korea, ID: KCT0004930. Registered retrospectively on April 14, 2020.
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Acupressão , Antineoplásicos , Neoplasias da Mama , Doenças do Sistema Nervoso Periférico , Antineoplásicos/efeitos adversos , Neoplasias da Mama/tratamento farmacológico , Humanos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/terapia , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Background: This study examined the effect of a portable low-frequency electrostimulation (ES) device on patients diagnosed with chemotherapy-induced peripheral neuropathy (CIPN) immediately after chemotherapy for breast cancer. Methods: A single-center, randomized, placebo-controlled trial was conducted. A total of 72 patients newly diagnosed with CIPN were enrolled and randomly placed into the ES (n = 36) or the sham ES group (SES; n = 36). Duloxetine or pregabalin was prescribed to all participants from the initial assessment. The devices for 14 days, at least twice a day, for at least 120 minutes. The primary outcomes were the overall intensities of the CIPN symptoms as assessed using Numerical Rating Scale (NRS). Secondary outcomes included Total Neuropathy Score (TNS), European Organization for Research and Treatment of Cancer Quality of Life (EORTC-QLQ), Chemotherapy-Induced Peripheral Neuropathy 20 (CIPN20), Functional Assessment of Cancer Therapy-Breast (FACT-B), and Instrument on Pattern Identification and Evaluation for CIPN (IPIE-CIPN). Results: No differences in NRS scores were found between the patients in the ES and the SES group (P = 0.267). Patients in both groups showed significantly reduced CIPN intensities (ES P < .001; SES P < .001). No significant differences between the groups were found in TNS, EORTC-QLQ, CIPN20, and FACT-B. The general symptoms of CIPN diagnosed as cold arthralgia showed significance only in the ES group (P = .006). Conclusion: Compared with a placebo, the effectiveness of the low-frequency ES device with pharmacological intervention was not significantly different, but a therapeutic effect was possible.
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Antineoplásicos , Neoplasias da Mama , Terapia por Estimulação Elétrica , Doenças do Sistema Nervoso Periférico , Antineoplásicos/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica , Neoplasias da Mama/tratamento farmacológico , Feminino , Humanos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/terapia , Qualidade de VidaRESUMO
OBJECTIVE: Anti-transcriptional intermediary factor 1 (TIF1) antibody is associated with idiopathic inflammatory myopathies (IIMs). The aim of this study was to investigate the expression of TIF1s in IIMs. METHOD: TIF1α, ß or γ expression in the skin and muscle of patients and controls was studied by immunohistochemistry. Serum myositis-specific autoantibodies were detected by immunoblot. RESULTS: TIF1α was expressed in the skin of most dermatomyositis (DM) patients but not in the controls (80% vs 0%, P < 0.001). TIF1ß was expressed in all tissues of patients and controls. TIF1γ was expressed in the muscle of DM patients compared to controls (42.1% vs 0%, P = 0.039) and associated with tubuloreticular bodies (P = 0.003). Anti-TIF1γ was related with TIF1γ expression in the muscle (P = 0.042). CONCLUSION: TIF1α expression in the skin and TIF1γ in the muscle of DM was increased compared to controls. TIF1γ expression in the muscle of IIMs was related to anti-TIF1γ antibody.
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Autoanticorpos/imunologia , Músculo Esquelético/imunologia , Miosite/imunologia , Pele/imunologia , Fatores de Transcrição/imunologia , Adulto , Idoso , Autoanticorpos/sangue , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miosite/sangue , Miosite/diagnóstico , Proteínas Nucleares/análise , Fatores de Transcrição/análise , Proteína 28 com Motivo Tripartido/análiseRESUMO
BACKGROUND: The environmental risks of multiple sclerosis (MS), including adolescent obesity and vitamin D deficiency, are increasing in Korea. We aimed to determine whether the patterns and/or severity of MS in Korea can change according to the year of birth or disease onset. METHODS: Two hundred and sixty-six patients with adult-onset MS, including 164 with an available baseline magnetic resonance imaging (MRI), were retrospectively included from 17 nationwide referral hospitals in Korea. The demographics, MRI T2 lesion burden at disease onset, cerebrospinal fluid markers, and prognosis were assessed. RESULTS: The birth year, time from disease onset to first MRI, and female sex were associated with a higher number of baseline MRI T2 lesions. The birth year was also associated with the presence of oligoclonal band in the cerebrospinal fluid and high immunoglobin G index. An increased female/male ratio was observed among those with a more recent year of birth and/or disease onset. CONCLUSIONS: In Korea, the disease pattern of adult-onset MS may be changing toward a more baseline T2 MRI lesions, intrathecal humoral immune responses, and also higher female ratio.
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Encéfalo/diagnóstico por imagem , Imunidade Humoral/fisiologia , Esclerose Múltipla/diagnóstico por imagem , Bandas Oligoclonais/líquido cefalorraquidiano , Adulto , Biomarcadores/líquido cefalorraquidiano , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/líquido cefalorraquidiano , Esclerose Múltipla/imunologia , Extratos Vegetais , Prognóstico , República da Coreia , Estudos Retrospectivos , Fatores Sexuais , Fatores de TempoRESUMO
We have identified a compound heterozygous mutation of PYGM in a Korean patient with McArdle disease, which is composed of a novel single codon deletion (p.779delE) and a common nonsense mutation (p.R50X). Our study also showed an evidence of nonsense-mediated mRNA decay (NMD) caused by p.R50X mutation, supporting the importance of RNA processing defects in the molecular pathology of McArdle disease.
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Códon sem Sentido , Glicogênio Fosforilase Muscular/genética , Doença de Depósito de Glicogênio Tipo V/genética , Deleção de Sequência , Adolescente , Doença de Depósito de Glicogênio Tipo V/diagnóstico , Doença de Depósito de Glicogênio Tipo V/fisiopatologia , Humanos , Coreia (Geográfico) , Masculino , Estabilidade de RNA/genética , Estabilidade de RNA/fisiologia , Reação em Cadeia da Polimerase Via Transcriptase ReversaRESUMO
OBJECTIVES: The aims of this study were to obtain data on the frequency with which Korean patients with autoimmune myasthenia gravis (MG) present solely with ocular disturbances and progress to develop generalized disease and to identify the prognostic factors associated with secondary generalization. METHODS: We conducted a retrospective multicenter survey in which a total of 376 adult patients who were newly diagnosed with MG from 2000 through 2005 were reviewed for analysis. Patients with ocular MG at the time of symptom presentation (n=202, 53.7%) were divided into two subgroups according to their prognosis: the patients whose disease remained ocular throughout the follow-ups were placed in the OMG-R group, and the patients who progressed to develop generalized disease were placed in the OMG-G group. Clinical characteristics and laboratory findings were compared between the two subgroups. RESULTS: Secondary generalization developed in 47 (23.3%) of the 202 study subjects, mostly within the first 6 months after symptom presentation, while the disease remained ocular throughout the follow-up duration (median 11.8 months) in the remaining 155 patients (76.7%). AChR antibody, abnormal repetitive nerve stimulation tests (RNST) and thymoma were more frequently observed in the patients in the OMG-G group than in those in the OMG-R group (p<0.01 in all). In seropositive cases, the titers of AChR antibody were also significantly higher in the OMG-G group than in the OMG-R group (median, 3.8 nM vs. 6.4 nM; p<0.05). Cox proportional hazards regression analyses showed that early oral prednisolone treatment significantly reduced the risk of secondary generalization (HR, 0.24; 95% CI, 0.11-0.56), whereas abnormal AChR antibody (HR, 5.34; 95% CI, 1.60-17.8) and thymoma (HR, 2.32; 95% CI, 1.21-4.45) were predictive of the development of secondary generalization. CONCLUSIONS: Our findings suggest that several factors, including the AChR antibody, thymoma, early corticosteroid treatment, and possibly latent neuromuscular abnormality revealed by RNST, may have an impact on the risk of developing generalized disease in Korean patients presenting with ocular myasthenia.
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Envelhecimento/patologia , Miastenia Gravis/diagnóstico , Miastenia Gravis/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Feminino , Humanos , Coreia (Geográfico)/epidemiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Receptores Colinérgicos/imunologia , Receptores Colinérgicos/fisiologia , Estudos Retrospectivos , Estatísticas não ParamétricasRESUMO
Cerebral microbleeds (CMBs) are increasingly recognized neuroimaging findings, occurring with cerebrovascular disease, dementia, and aging. CMBs are associated with subsequent hemorrhagic and ischemic stroke, and also with an increased risk of cognitive deterioration and dementia. They occur in the setting of impaired small vessel integrity due to hypertension or cerebral amyloid angiopathy. This review summarizes the concepts, cause or risk factors, histopathological mechanisms, and clinical consequences of CMBs.
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BACKGROUND: Chemotherapy-induced peripheral neuropathy (CIPN) is a progressive, enduring, and sometimes irreversible neurotoxic symptom that occurs in 30-40% of chemotherapy-treated cancer patients. CIPN negatively affects both the patient's abilities to perform daily activities and their health-related quality of life (HRQOL) after chemotherapy treatment. Although this neuropathy has been treated with duloxetine and/or gabapentin, limited therapeutic benefits have been reported, thereby necessitating the development of an integrated approach that combines pharmacological management and complementary methods such as acupuncture and electric nerve stimulation. Therefore, this study is designed to examine the effect of a portable, low-frequency electrostimulation (ES) device on CIPN symptoms and HRQOL of female patients diagnosed with CIPN immediately after chemotherapy for breast cancer. METHODS: This study is a single-center, randomized, placebo-controlled trial with two parallel groups and a 2-week follow-up. We will enroll 80 breast cancer patients who are newly diagnosed with CIPN after chemotherapy. Duloxetine or pregabalin will be prescribed to all participants from the initial assessment. Half of the patients will be assigned into the experimental group and the other half to the control group. The CarebandR (Piomed Inc., Seoul, Korea), a wearable wristband that generates low-frequency electrostimulation, will be administered only to the experimental group. Electrostimulation will be administered on the unilateral PC6 acupoint. A numerical rating scale will be used to assess the overall intensity of CIPN symptoms. The key secondary outcome variables include patient-reported CIPN symptom distress tested by a self-rated questionnaire, physician-rated symptom severity assessed by the Total Neuropathy Score, and HRQOL. DISCUSSION: It is expected that the combination of a low-frequency electrostimulation device and pharmacological intervention (duloxetine or pregabalin) will produce synergistic effects in breast cancer patients with CIPN after treatment. To our knowledge, this is the first study to investigate the beneficial effect of a new integrated approach for CIPN management after breast cancer treatment. The study findings can expand our knowledge and understanding of the occurrence of CIPN and the efficacy of integrated intervention efforts to ameliorate CIPN symptoms. TRIAL REGISTRATION: Clinical Research Information Service (CRIS), Republic of Korea, ID: KCT0002357 . Registered retrospectively on 13 June 2017.
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Antineoplásicos/efeitos adversos , Neoplasias da Mama/tratamento farmacológico , Terapia por Estimulação Elétrica/métodos , Doenças do Sistema Nervoso Periférico/terapia , Qualidade de Vida , Atividades Cotidianas , Analgésicos/uso terapêutico , Neoplasias da Mama/patologia , Terapia Combinada , Cloridrato de Duloxetina/uso terapêutico , Terapia por Estimulação Elétrica/instrumentação , Feminino , Humanos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/fisiopatologia , Doenças do Sistema Nervoso Periférico/psicologia , Pregabalina/uso terapêutico , Ensaios Clínicos Controlados Aleatórios como Assunto , República da Coreia , Fatores de Tempo , Resultado do TratamentoRESUMO
[This corrects the article DOI: 10.1371/journal.pone.0193723.].
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Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA). We also included 8 patients with ocular MG, 3 with Lambert-Eaton myasthenic syndrome, 5 with motor neuron disease, and 9 with other diagnoses as comparators for the serological testing. Antibodies were identified in 25/62 (40.3%) patients: 7 had antibodies to clustered AChR, 17 to MuSK, and 2 to LRP4. Three patients were double seropositive: 1 for MuSK and LRP4, and 2 for MuSK and clustered AChR. The patients with MuSK antibodies were mostly female (88.2%) and characterized by predominantly bulbar involvement (70%) and frequent myasthenic crises (58.3%). The patients with antibodies to clustered AChR, including 2 with ocular MG, tended to have a mild phenotype and good prognosis.