RESUMO
BACKGROUND: Optic neuritis (ON) prognosis is influenced by various factors including attack severity, underlying aetiologies, treatments and consequences of previous episodes. This study, conducted on a large cohort of first ON episodes, aimed to identify unique prognostic factors for each ON subtype, while excluding any potential influence from pre-existing sequelae. METHODS: Patients experiencing their first ON episodes, with complete aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibody testing, and clinical data for applying multiple sclerosis (MS) diagnostic criteria, were enrolled. 427 eyes from 355 patients from 10 hospitals were categorised into four subgroups: neuromyelitis optica with AQP4 IgG (NMOSD-ON), MOG antibody-associated disease (MOGAD-ON), ON in MS (MS-ON) or idiopathic ON (ION). Prognostic factors linked to complete recovery (regaining 20/20 visual acuity (VA)) or moderate recovery (regaining 20/40 VA) were assessed through multivariable Cox regression analysis. RESULTS: VA at nadir emerged as a robust prognostic factor for both complete and moderate recovery, spanning all ON subtypes. Early intravenous methylprednisolone (IVMP) was associated with enhanced complete recovery in NMOSD-ON and MOGAD-ON, but not in MS-ON or ION. Interestingly, in NMOSD-ON, even a slight IVMP delay in IVMP by >3 days had a significant negative impact, whereas a moderate delay up to 7-9 days was permissible in MOGAD-ON. Female sex predicted poor recovery in MOGAD-ON, while older age hindered moderate recovery in NMOSD-ON and ION. CONCLUSION: This comprehensive multicentre analysis on first-onset ON unveils subtype-specific prognostic factors. These insights will assist tailored treatment strategies and patient counselling for ON.
Assuntos
Autoanticorpos , Metilprednisolona , Glicoproteína Mielina-Oligodendrócito , Neurite Óptica , Humanos , Masculino , Feminino , Prognóstico , Adulto , Neurite Óptica/diagnóstico , Neurite Óptica/imunologia , Glicoproteína Mielina-Oligodendrócito/imunologia , Pessoa de Meia-Idade , Autoanticorpos/sangue , Metilprednisolona/uso terapêutico , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/imunologia , Aquaporina 4/imunologia , Acuidade Visual/fisiologia , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/imunologia , Adulto Jovem , Adolescente , IdosoRESUMO
A clinical scale fully dedicated to evaluating ocular motor abnormalities is required for now. We investigated the utility of a recently developed Scale for Ocular motor Disorders in Ataxia (SODA) in patients with multiple system atrophy (MSA). We prospectively assessed SODA in consecutive patients with MSA between August 2021 and August 2023 at the Korea University Medical Center. The results of the clinical exam-based SODA were compared with those measured using video-oculography (VOG-guided SODA). We also compared the findings with other established clinical scales targeting patients with MSA, including the Unified Multiple System Atrophy Rating Scale (UMSARS) I-II, Movement Disorder Society-Unified Parkinson's Disease Rating Scale motor part (UPDRS-III), Scale for Assessment of Rating of Ataxia (SARA), Composite Autonomic Symptom Score-31 (COMPASS-31), and Composite Autonomic Severity Score (CASS). Twenty patients were enrolled in our study (17 with cerebellar-type MSA and three with Parkinson-type MSA). Scores ranged from 1 to 14 (median [interquartile range (IQR)] = 8 [5-10]). Among the subscales, saccades had a median score of 2.5 (IQR = 1-3), followed by ocular pursuit (1 [0-1]), nystagmus (1 [0-2]), saccadic intrusions (1 [0-1]), vestibulo-ocular reflex (VOR) (0.5 [0-1]), ocular alignment (0 [0-1]), and VOR cancellation (1 [0-1]). The clinical-exam-based SODA (p = 0.020) and VOG-guided SODA (p = 0.034) positively correlated with disease duration. No correlation was found between clinical exam-based SODA and other scales. Skew deviation, gaze-evoked nystagmus, VOR cancellation, and smooth pursuit had the highest precision among the items. Ocular misalignment and spontaneous and positional nystagmus were frequently false positive and were poorly detected with clinical exam-based SODA. Six patients with repeated evaluation exhibited higher scores, along with deterioration documented on other clinical scales. The SODA can reliably predict neurodegeneration as an additional clinical surrogate in MSA.
Assuntos
Ataxia , Medições dos Movimentos Oculares , Atrofia de Múltiplos Sistemas , Transtornos da Motilidade Ocular , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Ataxia/complicações , Medições dos Movimentos Oculares/normas , Reações Falso-Positivas , Seguimentos , Atrofia de Múltiplos Sistemas/complicações , Nistagmo Fisiológico , Transtornos da Motilidade Ocular/complicações , Transtornos da Motilidade Ocular/diagnóstico , Transtornos da Motilidade Ocular/fisiopatologia , Acompanhamento Ocular Uniforme , Reflexo Vestíbulo-Ocular , Reprodutibilidade dos Testes , Movimentos Sacádicos , Sensibilidade e EspecificidadeRESUMO
PURPOSE: The specific characteristics of autonomic involvement in patients with early Parkinson's disease (PD) are unclear. This study aimed to evaluate the characteristics of autonomic dysfunction in drug-naïve patients with early-stage PD without orthostatic hypotension (OH) by analyzing Valsalva maneuver (VM) parameters. METHODS: We retrospectively analyzed drug-naïve patients without orthostatic hypotension (n = 61) and controls (n = 20). The patients were subcategorized into early PD (n = 35) and mid-PD (n = 26) groups on the basis of the Hoehn and Yahr staging. VM parameters, including changes in systolic blood pressure at late phase 2 (∆SBPVM2), ∆HRVM3, Valsalva ratio (VR), pressure recovery time, adrenergic baroreflex sensitivity, and vagal baroreflex sensitivity, were assessed. RESULTS: In the early PD group, ∆SBPVM2, a marker of sympathetic function, was significantly lower compared with that in controls (risk ratio = 0.95, P = 0.027). Receiver operating characteristic (ROC) curve analysis showed an optimal cut-off value of -10 mmHg for ∆SBPVM2 [P = 0.002, area under the curve (AUC): 0.737]. VR exhibited an inverse relationship with Unified Parkinson's Disease Rating Scale Part 3 scores in the multivariable regression analysis (VR: P = 0.038, ß = -28.61), whereas age showed a positive relationship (age: P = 0.027, ß = 0.35). CONCLUSION: The ∆BPVM2 parameter of the VM may help detect autonomic nervous system involvement in early-PD without OH. Our results suggest that sympathetic dysfunction is an early manifestation of autonomic dysfunction in patients with PD.
Assuntos
Doenças do Sistema Nervoso Autônomo , Barorreflexo , Doença de Parkinson , Manobra de Valsalva , Humanos , Doença de Parkinson/fisiopatologia , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico , Masculino , Feminino , Estudos Retrospectivos , Idoso , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Autônomo/fisiopatologia , Doenças do Sistema Nervoso Autônomo/diagnóstico , Doenças do Sistema Nervoso Autônomo/etiologia , Manobra de Valsalva/fisiologia , Barorreflexo/fisiologia , Sistema Nervoso Simpático/fisiopatologia , Pressão Sanguínea/fisiologiaRESUMO
BACKGROUND: During the coronavirus disease 2019 (COVID-19) pandemic, patients with myasthenia gravis (MG) were more susceptible to poor outcomes owing to respiratory muscle weakness and immunotherapy. Several studies conducted in the early stages of the COVID-19 pandemic reported higher mortality in patients with MG compared to the general population. This study aimed to investigate the clinical course and prognosis of COVID-19 in patients with MG and to compare these parameters between vaccinated and unvaccinated patients in South Korea. METHODS: This multicenter, retrospective study, which was conducted at 14 tertiary hospitals in South Korea, reviewed the medical records and identified MG patients who contracted COVID-19 between February 2022 and April 2022. The demographic and clinical characteristics associated with MG and vaccination status were collected. The clinical outcomes of COVID-19 infection and MG were investigated and compared between the vaccinated and unvaccinated patients. RESULTS: Ninety-two patients with MG contracted COVID-19 during the study. Nine (9.8%) patients required hospitalization, 4 (4.3%) of whom were admitted to the intensive care unit. Seventy-five of 92 patients were vaccinated before contracting COVID-19 infection, and 17 were not. During the COVID-19 infection, 6 of 17 (35.3%) unvaccinated patients were hospitalized, whereas 3 of 75 (4.0%) vaccinated patients were hospitalized (P < 0.001). The frequencies of ICU admission and mechanical ventilation were significantly lower in the vaccinated patients than in the unvaccinated patients (P = 0.019 and P = 0.032, respectively). The rate of MG deterioration was significantly lower in the vaccinated patients than in the unvaccinated patients (P = 0.041). Logistic regression after weighting revealed that the risk of hospitalization and MG deterioration after COVID-19 infection was significantly lower in the vaccinated patients than in the unvaccinated patients. CONCLUSION: This study suggests that the clinical course and prognosis of patients with MG who contracted COVID-19 during the dominance of the omicron variant of COVID-19 may be milder than those at the early phase of the COVID-19 pandemic when vaccination was unavailable. Vaccination may reduce the morbidity of COVID-19 in patients with MG and effectively prevent MG deterioration induced by COVID-19 infection.
Assuntos
Vacinas contra COVID-19 , COVID-19 , Hospitalização , Miastenia Gravis , SARS-CoV-2 , Vacinação , Humanos , COVID-19/epidemiologia , COVID-19/prevenção & controle , COVID-19/complicações , Estudos Retrospectivos , Masculino , Feminino , Pessoa de Meia-Idade , República da Coreia/epidemiologia , Idoso , SARS-CoV-2/isolamento & purificação , Adulto , Prognóstico , Unidades de Terapia Intensiva , Respiração ArtificialRESUMO
PURPOSE: To delineate the association between otolith function and changes in mean orthostatic blood pressure (BP) and heart rate (HR) in patients with postural orthostatic tachycardia syndrome (POTS). METHODS: Forty-nine patients with POTS were prospectively recruited. We analyzed the results of ocular vestibular-evoked myogenic potentials (oVEMPs) and cervical vestibular-evoked myogenic potentials (cVEMPs), as well as head-up tilt table tests using a Finometer. The oVEMP and cVEMP responses were obtained using tapping stimuli and 110 dB tone-burst sounds, respectively. We measured maximal changes in 5-s averaged systolic BP (SBP), diastolic BP (DBP), and heart rate (HR) within 15 s and during 10 min after tilting. We compared the results with those of 20 age- and sex-matched healthy participants. RESULTS: The n1-p1 amplitude of oVEMPs was larger in patients with POTS than in healthy participants (p = 0.001), whereas the n1 latency (p = 0.280) and interaural difference (p = 0.199) did not differ between the two. The n1-p1 amplitude was a positive predictor for POTS (odds ratio 1.07, 95% confidence interval 1.01-1.13, p = 0.025). Body weight (p = 0.007) and n1-p1 amplitude of oVEMP (p = 0.019) were positive predictors for ΔSBP15s in POTS, whereas aging was a negative predictor (p = 0.005). These findings were not observed in healthy participants. CONCLUSIONS: Augmented utricular inputs may be associated with a relative predominance of sympathetic over vagal control of BP and HR, especially for an early response during orthostasis in patients with POTS. Overt sympathoexcitation due to exaggerated utricular input and lack of readaptation may be associated with the pathomechanism of POTS.
Assuntos
Síndrome da Taquicardia Postural Ortostática , Potenciais Evocados Miogênicos Vestibulares , Humanos , Síndrome da Taquicardia Postural Ortostática/diagnóstico , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Frequência Cardíaca , Envelhecimento , Pressão SanguíneaRESUMO
The need for developing a simple and effective assessment tool for muscle mass has been increasing in a rapidly aging society. This study aimed to evaluate the feasibility of the surface electromyography (sEMG) parameters for estimating muscle mass. Overall, 212 healthy volunteers participated in this study. Maximal voluntary contraction (MVC) strength and root mean square (RMS) values of motor unit potentials from surface electrodes on each muscle (biceps brachii, triceps brachii, biceps femoris, rectus femoris) during isometric exercises of elbow flexion (EF), elbow extension (EE), knee flexion (KF), knee extension (KE) were acquired. New variables (MeanRMS, MaxRMS, and RatioRMS) were calculated from RMS values according to each exercise. Bioimpedance analysis (BIA) was performed to determine the segmental lean mass (SLM), segmental fat mass (SFM), and appendicular skeletal muscle mass (ASM). Muscle thicknesses were measured using ultrasonography (US). sEMG parameters showed positive correlations with MVC strength, SLM, ASM, and muscle thickness measured by US, but showed negative correlations with SFM. An equation was developed for ASM: ASM = -26.04 + 20.345 × Height + 0.178 × weight - 2.065 × (1, if female; 0, if male) + 0.327 × RatioRMS(KF) + 0.965 × MeanRMS(EE) (SEE = 1.167, adjusted R2 = 0.934). sEMG parameters in controlled conditions may represent overall muscle strength and muscle mass in healthy individuals.
Assuntos
Articulação do Cotovelo , Músculo Esquelético , Humanos , Masculino , Feminino , Eletromiografia , Músculo Esquelético/diagnóstico por imagem , Músculo Esquelético/fisiologia , Cotovelo/fisiologia , Braço , Força Muscular , Contração Isométrica/fisiologia , Contração Muscular/fisiologiaRESUMO
PURPOSE: To delineate the association between otolithic dysfunction and orthostatic hypotension (OH). METHODS: We retrospectively reviewed the medical records of 382 patients who presented with orthostatic dizziness at a tertiary dizziness center between July 2017 and December 2021. Patients were included for analyses when they had completed ocular (oVEMP) and/or cervical vestibular-evoked myogenic potentials (cVEMP), and head-up tilt table test with a Finometer (n = 155). We compared the results between the patients with OH (n = 38) and those with NOI (normal head-up tilt table test despite orthostatic intolerance, n = 117). RESULTS: Thirty-eight patients with OH were further categorized as either classic (n = 30), delayed (n = 7), or initial (n = 1) types. Multivariable logistic regression showed that OH was associated with high baseline systolic BP (p = 0.046), presence of heart failure (p = 0.016), and unilateral oVEMP abnormalities (p = 0.016). n1 latency of oVEMP were negatively correlated with the maximal changes of systolic blood pressure (BP) in 15 s ([Formula: see text]SBP15s, p = 0.013), 3 min ([Formula: see text]SBP3min, p = 0.005) and 10 min ([Formula: see text]SBP10min, p = 0.002). In contrast, the n1-p1 amplitude was positively correlated with [Formula: see text]SBP15s (p = 0.029). Meanwhile, p13 latency of cVEMP was negatively correlated with [Formula: see text]SBP10min (p = 0.018). CONCLUSIONS: Our study provides evidence of utricular dysfunction related to OH.
Assuntos
Doenças do Sistema Nervoso Autônomo , Hipotensão Ortostática , Potenciais Evocados Miogênicos Vestibulares , Humanos , Hipotensão Ortostática/diagnóstico , Tontura , Estudos Retrospectivos , Potenciais Evocados Miogênicos Vestibulares/fisiologia , Teste da Mesa InclinadaRESUMO
BACKGROUND: Handgrip strength (HGS), an indicator of overall muscle strength, is a key component in sarcopenia diagnosis. Although exercise is an effective strategy to prevent sarcopenia, the most appropriate exercise type targeting sarcopenia needs to be established. This study aimed to investigate the relationship between the physical activity (PA) patterns and HGS. METHODS: This was a cross-sectional study using the data from the 7th Korea National Health and Nutrition Examination Survey (2016-2018). The study population included 12,814 adults aged ≥ 40 years. According to the World Health Organization PA guidelines for public health, both aerobic (moderate to vigorous PA ≥ 150 min/week) and resistance exercises (≥ 2 sessions/week) are recommended. Study participants were categorized into one of the four groups depending on their adherence to each of two exercise guidelines ("neither," "aerobic only," "resistance only," and "combined"). By defining normal HGS cutoff values as the lowest quartile of HGS from the population aged 20 years and above, we classified participants as "preserved" HGS group if their HGS was equal to or above the cutoff values. A Poisson regression model was used to calculate adjusted prevalence ratios (APRs) for preserved HGS across the four PA guideline adherences stratified by age and sex groups. RESULTS: In middle-aged adults, the "combined" exercise group was independently associated with the preserved HGS (male, age 50-59 years, APR = 1.072; male, age 60-69 years, APR = 1.180; female, age 50-59 years, APR = 1.112; female, age 60-69 years, APR = 1.188). For adults aged ≥ 70 years, meeting only aerobic or resistance exercise guidelines showed a positive association with HGS before adjusting for other health-related variables. In males of ≥ 70 years, the APR of preserved HGS was highest in the "combined" exercise group ("resistance only," APR = 1.459, "combined," APR = 1.664), while in women aged ≥ 70 years, the significance was lost after adjusting for covariates. CONCLUSIONS: Adults meeting both aerobic and resistance exercise guidelines were associated with the highest prevalence of preserved HGS. Performing both types of exercise might be the most effective way to prevent sarcopenia that should be investigated in future clinical trials.
Assuntos
Força da Mão , Sarcopenia , Idoso , Estudos Transversais , Exercício Físico/fisiologia , Feminino , Força da Mão/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Inquéritos Nutricionais , República da Coreia/epidemiologia , Sarcopenia/diagnóstico , Sarcopenia/epidemiologia , Sarcopenia/prevenção & controleRESUMO
Step length is a critical hallmark of health status. However, few studies have investigated the modifiable factors that may affect step length. An exploratory, cross-sectional study was performed to evaluate the surface electromyography (sEMG) and body impedance analysis (BIA) parameters, combined with individual demographic data, to predict the individual step length using the GAITRite® system. Healthy participants aged 40−80 years were prospectively recruited, and three models were built to predict individual step length. The first model was the best-fit model (R2 = 0.244, p < 0.001); the root mean square (RMS) values at maximal knee flexion and height were included as significant variables. The second model used all candidate variables, except sEMG variables, and revealed that age, height, and body fat mass (BFM) were significant variables for predicting the average step length (R2 = 0.198, p < 0.001). The third model, which was used to predict step length without sEMG and BIA, showed that only age and height remained significant (R2 = 0.158, p < 0.001). This study revealed that the RMS value at maximal strength knee flexion, height, age, and BFM are important predictors for individual step length, and possibly suggesting that strengthening knee flexor function and reducing BFM may help improve step length.
Assuntos
Nível de Saúde , Articulação do Joelho , Composição Corporal , Estudos Transversais , Impedância Elétrica , Eletromiografia , HumanosRESUMO
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.
Assuntos
Aquaporina 4 , Doenças do Sistema Nervoso Central , Adulto , Humanos , Glicoproteína Mielina-Oligodendrócito , República da Coreia/epidemiologia , Estudos SoroepidemiológicosRESUMO
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.
Assuntos
Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Biomarcadores , Humanos , Glicoproteína Mielina-Oligodendrócito , Neuromielite Óptica/diagnósticoRESUMO
BACKGROUND: Although neuromyelitis optica spectrum disorder (NMOSD) is known to be a rare disease, its prevalence and incidence have not yet been studied in Korea. We performed a population-based study to examine the prevalence and incidence of NMOSD in Korea using data from the Korean National Health Insurance (NHI) claims database. METHODS: Data from 2013 to 2017 were obtained, with a washout period set as 2013 and 2014. The prevalence and incidence of NMOSD in 2016 and 2017 were calculated using population census data. Subjects were divided into 5 groups at 15-year intervals, depending on the age at which the diagnostic code was entered. The relative risk (RR) for each age group was compared with the oldest (≥ 60 years) age group. RESULTS: The overall prevalence was estimated to be 3.36 and 3.56 per 100,000 individuals, with an incidence of 0.41 and 0.65 per 100,000 individuals-year in 2016 and 2017, respectively. The mean age was 43.08 (standard deviation, 14.56) years, and the ratio of male to females was 1:4.7. The incidence was higher in female individuals aged between 30 and 59 years (RR, 2.8-3.05; P < 0.05). CONCLUSION: Nationwide prevalence of NMOSD in Korea was 3.36 and 3.56/100,000 and its incidence was 0.41 and 0.65/100,000-year in 2016 and 2017 respectively.
Assuntos
Neuromielite Óptica/diagnóstico , Adolescente , Adulto , Fatores Etários , Azatioprina/uso terapêutico , Criança , Bases de Dados Factuais , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/tratamento farmacológico , Neuromielite Óptica/epidemiologia , Prevalência , República da Coreia/epidemiologia , Fatores de Risco , Adulto JovemRESUMO
OBJECTIVES: To evaluate the validity of the revised 2017 McDonald criteria for multiple sclerosis (MS) compared with the 2010 McDonald criteria to predict conversion to clinically definite multiple sclerosis (CDMS) in patients with clinically isolated syndrome (CIS). METHODS: A total of 163 patients from seven referral hospitals in Korea, who experienced a first clinical event suggestive of MS between 2006 and 2017, were enrolled. Patients were stratified into two groups according to outcome at the last visit: CDMS converters who experienced a second clinical event and non-converters. RESULTS: Of the 163 patients with a mean follow-up of 63 months, 60% converted to CDMS. The sensitivity, specificity, positive and negative predictive values and accuracy were, respectively, 88.8%, 43.1%, 70.2%, 71.8% and 70.6% for the 2017 McDonald criteria and 53.1%, 69.2%, 72.2%, 49.5% and 59.5% for the 2010 McDonald criteria. After exclusion of 82 patients who received disease-modifying agents before the second attack, the specificity of the 2017 and 2010 McDonald criteria increased to 85.0% and 95.0%, but sensitivity decreased to 83.6% and 47.5%, respectively. CONCLUSION: The 2017 McDonald criteria afforded higher sensitivity and accuracy but lower specificity compared with the 2010 McDonald criteria for prediction of conversion to CDMS in Korean CIS patients.
Assuntos
Doenças Desmielinizantes/diagnóstico por imagem , Esclerose Múltipla/diagnóstico , Adolescente , Adulto , Crotonatos/uso terapêutico , Doenças Desmielinizantes/líquido cefalorraquidiano , Doenças Desmielinizantes/tratamento farmacológico , Progressão da Doença , Feminino , Cloridrato de Fingolimode/uso terapêutico , Acetato de Glatiramer/uso terapêutico , Humanos , Hidroxibutiratos , Fatores Imunológicos/uso terapêutico , Imunossupressores/uso terapêutico , Interferon beta/uso terapêutico , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/líquido cefalorraquidiano , Esclerose Múltipla/diagnóstico por imagem , Nitrilas , Bandas Oligoclonais/líquido cefalorraquidiano , Neurite Óptica/líquido cefalorraquidiano , Neurite Óptica/diagnóstico por imagem , Quinolonas/uso terapêutico , Reprodutibilidade dos Testes , República da Coreia , Sensibilidade e Especificidade , Fatores de Tempo , Toluidinas/uso terapêutico , Adulto JovemRESUMO
OBJECTIVE: The objective of this study was to investigate the usefulness of muscle ultrasound in evaluating dissociated small hand muscle atrophy, termed 'split hand', and its feasibility in the diagnosis of amyotrophic lateral sclerosis (ALS). METHODS: Forty-four patients with ALS, 18 normal subjects and 9 patients with other neuromuscular disorders were included in this study. The hand muscles were divided into three regions, the median-innervated lateral hand muscle group (ML), the ulnar-innervated lateral hand muscle (UL) and the ulnar-innervated medial hand muscle (UM), and the muscle echo intensity (EI) and compound muscle action potential (CMAP) were measured. We calculated the split hand index (SHI) using muscle EI (SHImEI) and CMAP (SHICMAP) for comparison among groups. The SHI was derived by dividing muscle EI (or CMAP) measured at the ML and UL by that measured at the UM. RESULTS: The SHImEI was significantly higher in patients with ALS (51.7±28.3) than in normal controls (29.7±9.9) and disease controls with other neuromuscular disorders (36.5±7.3; P<0.001), particularly in upper limb-onset ALS (66.5±34.0; P<0.001). Receiver operating characteristic curve analysis indicated that the SHImEI had significantly better diagnostic accuracy than the SHICMAP. CONCLUSIONS: The SHImEI was more sensitive in evaluating dissociated small hand muscle atrophy compared with the SHICMAP and may be a reliable diagnostic marker for differentiating ALS from other neuromuscular disorders and healthy controls.
Assuntos
Esclerose Lateral Amiotrófica/diagnóstico , Mãos , Músculo Esquelético/diagnóstico por imagem , Músculo Esquelético/fisiopatologia , Atrofia Muscular/diagnóstico por imagem , Atrofia Muscular/fisiopatologia , Potenciais de Ação/fisiologia , Idoso , Esclerose Lateral Amiotrófica/complicações , Esclerose Lateral Amiotrófica/fisiopatologia , Estudos de Casos e Controles , Diagnóstico Diferencial , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Atrofia Muscular/etiologia , Valor Preditivo dos Testes , Curva ROC , Reprodutibilidade dos Testes , UltrassonografiaRESUMO
OBJECTIVES: We compared validity of 2010 McDonald and newly proposed 2016 Magnetic Resonance Imaging in Multiple Sclerosis (MAGNIMS) criteria for dissemination in space (DIS) in predicting the conversion to clinically definite multiple sclerosis (CDMS) in patients with clinically isolated syndrome (CIS). METHODS: Between 2006 and 2016, we enrolled 170 patients who had a first clinical event suggestive of multiple sclerosis (MS) from seven referral hospitals in Korea. Patients were classified into two groups based on the main outcome at the last follow-up: CDMS converters, who experienced a second attack, and non-converters. RESULTS: Of 170 patients with mean follow-up duration of 54 months, 51% converted to CDMS. The sensitivity, specificity, accuracy, and positive and negative predictive values of 2010 McDonald criteria were 70.9%, 63.1%, 67.1%, 66.3%, and 67.9%, and those for 2016 MAGNIMS criteria were 88.4%, 46.4%, 67.7%, 62.8%, and 79.6%, respectively. When we excluded 80 patients who underwent disease-modifying therapy before the second clinical event, the specificity increased to 92.3% and 84.6%, but the sensitivity decreased to 58.8% and 82.4% for 2010 McDonald and 2016 MAGNIMS criteria, respectively. CONCLUSION: 2016 MAGNIMS magnetic resonance imaging (MRI) criteria for DIS showed higher sensitivity but lower specificity than 2010 McDonald criteria in predicting conversion to CDMS in CIS patients.
Assuntos
Doenças Desmielinizantes/diagnóstico por imagem , Doenças Desmielinizantes/patologia , Imageamento por Ressonância Magnética/métodos , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/patologia , Adolescente , Adulto , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , República da Coreia , Sensibilidade e Especificidade , Adulto JovemRESUMO
BACKGROUND: There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD). OBJECTIVE: We aimed to describe the characteristic features of patients with LO-NMOSD in Korea. METHODS: Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (<50 years of age at onset). Clinical, laboratory, and magnetic resonance imaging (MRI) parameters were investigated. RESULTS: Among a total of 147 patients (125 female; age of onset, 39.4 ± 15.2 years), 45 patients (30.6%) had an age of onset of more than 50 years. Compared to patients with EO-NMOSD, patients with LO-NMOSD had more frequent isolated spinal cord involvement at onset (64.4% vs 37.2%, p = 0.002), less frequent involvement of the optic nerve (40.0% vs 67.7%, p = 0.002), and less frequent brain MRI lesions (31.1% vs 50.0%, p = 0.034). Furthermore, there was a significant positive correlation between age of onset and Expanded Disability Status Scale (EDSS) score at last follow-up ( r = 0.246, p = 0.003). CONCLUSION: Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.
Assuntos
Aquaporina 4/imunologia , Autoanticorpos/sangue , Neuromielite Óptica , Índice de Gravidade de Doença , Adulto , Idade de Início , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/sangue , Neuromielite Óptica/epidemiologia , Neuromielite Óptica/patologia , Neuromielite Óptica/fisiopatologia , República da Coreia/epidemiologia , Estudos RetrospectivosRESUMO
OBJECTIVE: To determine the most reliable method to measure the length of the radial nerve during a nerve conduction study (NCS). DESIGN: Cadaveric investigation. SETTING: A practical anatomy research laboratory in a university. PARTICIPANTS: Fresh cadavers (N=10), with 1 cadaver for study design and 9 for data. INTERVENTIONS: Design of measurement methods using cadaver dissection and comparison of the measured values to the true length in 18 arms of 9 cadavers. MAIN OUTCOME MEASURES: Four points (A, B, C, D) were determined: (A) proximal stimulation point in NCS; (B) point at the elbow crease; (C) point in the midforearm; and (D) distal stimulation point 5cm above the extensor indicis. The true length of the radial nerve between the stimulus points (points A and D) in NCS was compared with the measured values by summation of the straight line segments between those points with various combinations. The difference in root mean square error (RMSE) of the distance measured by each method compared with the true length was calculated to determine the best measurement method. RESULTS: The closest distance to the true length (28.7±2.8cm) in the cadaveric investigation was obtained using the summation of straight line segments between points A, B, and D (A-B-D, RMSE=.72cm), followed by the A-B-C-D distance (RMSE=.87cm) and the A-D distance (RMSE=1.38cm) methods, in sequence. The former 2 distance measurements were relatively closer to the true length than the latter measurement method. CONCLUSIONS: Multiple segmentation measurement methods reflected the course of the radial nerve better than a single linear measurement method. We suggest that the distance measured using a stopover point near the lateral epicondyle between 2 stimulus points (A-B-D distance) is closer to the true length of the nerve.
Assuntos
Condução Nervosa/fisiologia , Nervo Radial/anatomia & histologia , Braço/inervação , Cadáver , HumanosRESUMO
BACKGROUND: The purpose of this study was to identify whether the distribution of Hounsfield Unit (HU) values across the intracranial area in computed tomography (CT) images can be used as an effective diagnostic tool for determining the severity of cerebral edema in pediatric traumatic brain injury (TBI) patients. METHODS: CT images, medical records and radiology reports on 70 pediatric patients were collected. Based on radiology reports and the Marshall classification, the patients were grouped as mild edema patients (n=37) or severe edema patients (n=33). Automated quantitative analysis using unenhanced CT images was applied to eliminate artifacts and identify the difference in HU value distribution across the intracranial area between these groups. RESULTS: The proportion of pixels with HU=17 to 24 was highly correlated with the existence of severe cerebral edema (P<0.01). This proportion was also able to differentiate patients who developed delayed cerebral edema from mild TBI patients. A significant difference between deceased patients and surviving patients in terms of the HU distribution came from the proportion of pixels with HU=19 to HU=23 (P<0.01). CONCLUSIONS: The proportion of pixels with an HU value of 17 to 24 in the entire cerebral area of a non-enhanced CT image can be an effective basis for evaluating the severity of cerebral edema. Based on this result, we propose a novel approach for the early detection of severe cerebral edema.
Assuntos
Edema Encefálico/diagnóstico por imagem , Lesões Encefálicas/diagnóstico por imagem , Edema Encefálico/complicações , Lesões Encefálicas/complicações , Criança , Pré-Escolar , Diagnóstico Precoce , Feminino , Humanos , Lactente , Escala de Gravidade do Ferimento , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Although neuromyelitis optica (NMO) is a central nervous system (CNS) autoimmune disease distinct from multiple sclerosis (MS). NMO and NMO spectrum disorder (NMOSD) sometimes show asymptomatic lesions on brain magnetic resonance imaging (MRI) at onset, and even present with symptomatic brain involvement. OBJECTIVES: We investigated whether brain MRI at onset can be helpful for the differentiation of MS and NMOSD. METHODS: We retrospectively analyzed initial brain MRIs, performed within three months of onset, in patients with MS (n = 51) and anti-aquaporin4-antibody-positive patients with NMOSD (n = 67). RESULTS: NMOSD patients met the Paty (37%) and Barkhof (13%) criteria, and the criteria of the European Magnetic Imaging in MS (MAGNIMS) study group (9%), for MS. Ovoid lesions perpendicular to the lateral ventricle, isolated juxtacortical lesions in U-fibers and isolated ovoid/round cortical lesions were found only in MS patients, whereas longitudinal corticospinal tract lesions, extensive hemispheric lesions, periependymal lesions surrounding the lateral ventricle and cervicomedullary lesions were found only in NMOSD patients. CONCLUSIONS: Our study suggests that it is difficult to differentiate MS from NMOSD by the fulfillment of the MRI criteria for MS on brain MRI at onset; however, the characteristic morphology of brain lesions is highly useful for the early differentiation of the two disorders.
Assuntos
Encéfalo/patologia , Diagnóstico Diferencial , Imageamento por Ressonância Magnética , Esclerose Múltipla/patologia , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/patologia , Adulto , Aquaporina 4/imunologia , Autoanticorpos/metabolismo , Encéfalo/imunologia , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
We investigated the validity of the 2005 and 2010 McDonald magnetic resonance imaging (MRI) criteria for dissemination in space (DIS) to predict the conversion of clinically definite multiple sclerosis (CDMS) in 94 Korean patients with clinically isolated syndromes (CIS). The sensitivity, specificity, accuracy, positive and negative predictive values of the 2005, 2010 McDonald DIS criteria were comparable to those observed in the Caucasian population. This finding suggests that after careful exclusion of alternative explanations, particularly neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD), both the 2005 and 2010 McDonald DIS criteria are also useful to predict conversion to CDMS in Korean patients with CIS.