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BACKGROUND: In cases of immune-mediated neurological disorders (IMND), different syndromes are associated with antibodies against neuronal surface antigens, intra-neuronal antigens, astrocytic aquaporin, and gangliosides. These autoantibodies can be pathogenic or connected to neuroinflammation and resulting neuronal injuries. This study aims to identify a blood biomarker that can detect neuronal damage in individuals with IMND. To this end, we use immunomagnetic reduction (IMR) nanobead technology to measure plasma neurofilament light chain (NfL). METHODS: The patients with IMND were enrolled in the Chang Gung Memorial Hospital at Keelung from 2018 to 2023. Seronegative patients were excluded based on the results of antibody tests. The healthy controls (HC) were community-dwelling adults from the Northeastern Taiwan Community Medicine Research Cohort (NTCMRC) conducted by the Community Medicine Research Center of the Keelung CGMH from 2020 to 2022. IMR technique detects magnetic susceptibility via measuring magnetic signal reduction caused by antigen-antibody immunocomplex formation on magnetic nanobeads. The plasma level of NfL was determined by the magnetic susceptibility changes in IMR. RESULTS: The study enrolled 57 IMND patients from the hospital and 73 HC participants from the communities. The plasma NfL was significantly higher in the IMND than in the HC (11.022 ± 2.637 vs. 9.664 ± 2.610 pg/mL, p = 0.004), regardless of age effects on plasma NfL in an analysis of covariance (ANCOVA) (F = 0.720, p = 0.950). In the receiver of operation curve analysis, the area under curve for plasma NfL to discriminate IMND and HC was 0.664 (95% CI = 0.549 to 0.739, p = 0.005). The subgroup analysis of plasma NfL in the IMND patients showed no difference between peripheral immune-mediated neuropathy (IMN) and central immune-mediated encephalomyelitis (IMEM) (11.331 ± 2.895 vs. 10.627 ± 2.260 pg/mL, p = 0.322), nor between tumor and non-tumor IMND (10.784 ± 3.446 vs. 11.093 ± 2.391 pg/mL, p = 0.714). Additionally, the antibody class of ganglioside antibodies in IMN did not have an impact on plasma NfL level (p = 0.857). CONCLUSION: Plasma NfL measurement is a reliable indicator of axonal injuries in patients with IMND. It is equally effective in detecting nerve injuries in inflammatory peripheral neuropathies and central neuroinflammation. The IMR nanobead technology offers a feasible method of detecting plasma NfL, which helps identify axonal injuries in IMND.
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Doenças do Sistema Nervoso Periférico , Adulto , Humanos , Axônios , Biomarcadores , Filamentos Intermediários , Proteínas de Neurofilamentos , Doenças Neuroinflamatórias , NeurôniosRESUMO
PURPOSE: Early distal muscle weakness and myotonia are typical clinical presentations in type I myotonic dystrophy (DM1). We present a DM1 case with unusual predominant proximal weakness without action myotonia. CASE REPORT: The chief complaint of this 48-year-old female was difficulty in raising her arms and frequent falling in recent years. On neurological examination, proximal muscle weakness was more pronounced than the distal muscle groups, in addition to facial involvement. Although she did not experience any action myotonia throughout her life, hand and tongue myotonia were readily inducible by percussion during neurological examination. The diagnosis of DM1 was later supported by electromyography and neuropathological studies, and confirmed by molecular testing. The pathological findings in this patient and the characteristic features in typical DM1 patients were briefly reviewed. CONCLUSION: The unusual presentation of this DM1 patient suggests the importance of comprehensive neurological examination including percussion of thenar and tongue muscles, even in a patient with atypical distribution of muscle weakness and without a clear personal and family history of myotonia. In addition to molecular testing, muscle biopsy remains supportive in making the diagnosis.
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Miotonia , Distrofia Miotônica , Eletromiografia , Feminino , Humanos , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Músculo Esquelético , Miotonia/diagnóstico , Distrofia Miotônica/complicações , Distrofia Miotônica/diagnóstico , Distrofia Miotônica/genéticaRESUMO
This case series reported a group of patients with Guillain-Barré syndrome (GBS) and their plasma cytokine changes before and after immunotherapy. We aimed to understand GBS's pathogenesis and pathophysiology through observing the interval differences of the representative cytokines, which were the thymus and activation regulated chemokine (TARC) for T-cell chemotaxis, CD40 ligand (CD40L) for cosimulation of B and T cells, activated complement component C5/C5a, and brain-derived neurotrophic factor (BDNF) for survival and regenerative responses to nerve injuries. The fluorescence magnetic bead-based multiplexing immunoassay simultaneously quantified the five cytokines in a single sample. From June 2018 to December 2019, we enrolled five GBS patients who had completed before-after blood cytokine measurements. One patient was diagnosed with paraneoplastic GBS and excluded from the following cytokine analysis. The BDNF level decreased consistently in all the patients and made it a potential biomarker for the acute stage of GBS. Interval changes of the other four cytokines were relatively inconsistent and possibly related to interindividual differences in the immune response to GBS triggers, types of GBS variants, and classes of antiganglioside antibodies. In summary, utilizing the multiplexing immunoassay helps in understanding the complex immune mechanisms of GBS and the variation of immune responses in GBS subtypes; this method is feasible for identifying potential biomarkers of GBS.
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Recognizing the cause is essential for the management of meralgia paresthetica (MP), also known as lateral femoral cutaneous neuropathy. The aim of this study was to investigate the etiologies of MP and their influence on each other. This retrospective study enrolled referral patients with electromyographic studies who fulfilled the clinical and electrodiagnostic criteria of MP from January 2003 to December 2013. Data including age, gender, body weight, body height, occupation, and relevant medical history were collected. The etiological analysis was based on age and gender. A total of 50 patients (30 males and 20 females) were enrolled. The average age (±standard deviation) at diagnosis was 49.8±12.8years. Risk factors were identified in 29 cases (58.0%). More patients younger than 50years of age were male (73.1%, p=0.049). Peaks of age occurred between 41-50years in men and 51-60years in women. More males had a body mass index≥24kg/m2 (69.2% vs. 31.6%, p=0.012) and ≥27kg/m2 (34.6% vs. 0.0%, p=0.006). Overweight and obese patients were more vulnerable to occupational factors (50.0% vs. 19.0%, p=0.030). Only one case had diabetes mellitus (2%). Male middle-aged patients with a higher body mass index and certain occupations had an increased risk of MP. In contrast to the peak age distribution of the male patients, the frequency of developing MP was relatively even among the women at all ages. The cause was often obscure.
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Síndromes de Compressão Nervosa/epidemiologia , Adulto , Feminino , Neuropatia Femoral , Hospitais/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Taiwan/epidemiologiaRESUMO
Importance: The current assessment of visual field loss in diseases such as glaucoma is affected by the subjectivity of patient responses and the lack of portability of standard perimeters. Objective: To describe the development and initial validation of a portable brain-computer interface (BCI) for objectively assessing visual function loss. Design, Setting, and Participants: This case-control study involved 62 eyes of 33 patients with glaucoma and 30 eyes of 17 healthy participants. Glaucoma was diagnosed based on a masked grading of optic disc stereophotographs. All participants underwent testing with a BCI device and standard automated perimetry (SAP) within 3 months. The BCI device integrates wearable, wireless, dry electroencephalogram and electrooculogram systems and a cellphone-based head-mounted display to enable the detection of multifocal steady state visual-evoked potentials associated with visual field stimulation. The performances of global and sectoral multifocal steady state visual-evoked potentials metrics to discriminate glaucomatous from healthy eyes were compared with global and sectoral SAP parameters. The repeatability of the BCI device measurements was assessed by collecting results of repeated testing in 20 eyes of 10 participants with glaucoma for 3 sessions of measurements separated by weekly intervals. Main Outcomes and Measures: Receiver operating characteristic curves summarizing diagnostic accuracy. Intraclass correlation coefficients and coefficients of variation for assessing repeatability. Results: Among the 33 participants with glaucoma, 19 (58%) were white, 12 (36%) were black, and 2 (6%) were Asian, while among the 17 participants with healthy eyes, 9 (53%) were white, 8 (47%) were black, and none were Asian. The receiver operating characteristic curve area for the global BCI multifocal steady state visual-evoked potentials parameter was 0.92 (95% CI, 0.86-0.96), which was larger than for SAP mean deviation (area under the curve, 0.81; 95% CI, 0.72-0.90), SAP mean sensitivity (area under the curve, 0.80; 95% CI, 0.69-0.88; P = .03), and SAP pattern standard deviation (area under the curve, 0.77; 95% CI, 0.66-0.87; P = .01). No statistically significant differences were seen for the sectoral measurements between the BCI and SAP. Intraclass coefficients for global and sectoral parameters ranged from 0.74 to 0.92, and mean coefficients of variation ranged from 3.03% to 7.45%. Conclusions and Relevance: The BCI device may be useful for assessing the electrical brain responses associated with visual field stimulation. The device discriminated eyes with glaucomatous neuropathy from healthy eyes in a clinically based setting. Further studies should investigate the feasibility of the BCI device for home-based testing as well as for detecting visual function loss over time.
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Cegueira/diagnóstico , Interfaces Cérebro-Computador , Potenciais Evocados Visuais/fisiologia , Glaucoma/diagnóstico , Campos Visuais/fisiologia , Idoso , Cegueira/etiologia , Cegueira/fisiopatologia , Desenho de Equipamento , Feminino , Seguimentos , Glaucoma/complicações , Glaucoma/fisiopatologia , Humanos , Pressão Intraocular , Masculino , Estudos Prospectivos , Curva ROCRESUMO
OBJECTIVE: Interactive displays armed with natural user interfaces (NUIs) will likely lead the next breakthrough in consumer electronics, and brain-computer interfaces (BCIs) are often regarded as the ultimate NUI-enabling machines to respond to human emotions and mental states. Steady-state visual evoked potentials (SSVEPs) are a commonly used BCI modality due to the ease of detection and high information transfer rates. However, the presence of flickering stimuli may cause user discomfort and can even induce migraines and seizures. With the aim of designing visual stimuli that can be embedded into video images, this study developed a novel approach to induce detectable SSVEPs using a composition of red/green/blue flickering lights. APPROACH: Based on the opponent theory of colour vision, this study used 32 Hz/40 Hz rectangular red-green or red-blue LED light pulses with a 50% duty cycle, balanced/equal luminance and 0°/180° phase shifts as the stimulating light sources and tested their efficacy in producing SSVEP responses with high signal-to-noise ratios (SNRs) while reducing the perceived flickering sensation. MAIN RESULTS: The empirical results from ten healthy subjects showed that dual-colour lights flickering at 32 Hz/40 Hz with a 50% duty cycle and 180° phase shift achieved a greater than 90% detection accuracy with little or no flickering sensation. SIGNIFICANCE: As a first step in developing an embedded SSVEP stimulus in commercial displays, this study provides a foundation for developing a combination of three primary colour flickering backlights with adjustable luminance proportions to create a subtle flickering polychromatic light that can elicit SSVEPs at the basic flickering frequency.
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Interfaces Cérebro-Computador , Visão de Cores/fisiologia , Cor , Potenciais Evocados Visuais/fisiologia , Fusão Flicker/fisiologia , Estimulação Luminosa/métodos , Interface Usuário-Computador , Adulto , Feminino , Humanos , MasculinoRESUMO
BACKGROUND: Diabetes mellitus (DM) is a risk factor for atrial fibrillation (AF) and is known to be an important risk factor for death from stroke. The influence of AF on long-term outcomes in patients with ischemic stroke remains controversial. To clarify the exact influence of AF on stroke outcome and exclude the effect from DM, we investigated the influence of AF on the 3-year outcomes of nondiabetic patients with acute first-ever ischemic stroke. METHODS: Five-hundred seventy-four nondiabetic patients with acute first-ever ischemic stroke were enrolled and had been followed for 3 years. Patients were divided into 2 groups according to whether AF was diagnosed or not. Clinical presentations, risk factors for stroke, laboratory data, comorbidities, and outcomes were recorded. RESULTS: A total of 107 patients (18.6%) had AF. The age was significantly older in patients with AF. Total anterior circulation syndrome occurred more frequently among patients with AF (P < .001). The mean length of stay in the acute ward was significantly higher in patients with AF (P < .001). Furthermore, dependent functional status following discharge was higher in patients with AF (P < .001). Multivariate Cox regression revealed that AF is a significant predictor of 3-year all-cause mortality (hazard ratio = 1.98, 95% confidence interval = 1.07-3.67, P = .022). CONCLUSIONS: AF is associated with increased risk of 3-year mortality in nondiabetic patients with acute first-ever ischemic stroke. Careful cardiac evaluation and treatment are essential in patients with AF and stroke.
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Fibrilação Atrial/mortalidade , Isquemia Encefálica/mortalidade , Acidente Vascular Cerebral/mortalidade , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/terapia , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/terapia , Distribuição de Qui-Quadrado , Comorbidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Tempo de Internação , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prevalência , Modelos de Riscos Proporcionais , Medição de Risco , Fatores de Risco , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/terapia , Taiwan/epidemiologia , Fatores de TempoRESUMO
The influence of pneumonia in acute stroke stage on the clinical presentation and long-term outcomes of patients with acute ischemic stroke is still controversial. We investigate the influence of pneumonia in acute stroke stage on the 3-year outcomes of patients with acute first-ever ischemic stroke. Nine-hundred and thirty-four patients with acute first-ever ischemic stroke were enrolled and had been followed for 3years. Patients were divided into two groups according to whether pneumonia occurred during acute stroke stage or not. Clinical presentations, risk factors for stroke, laboratory data, co-morbidities, and outcomes were recorded. The result showed that a total of 100 patients (10.7%) had pneumonia in acute stroke stage. The prevalence of older age, atrial fibrillation was significantly higher in patients with pneumonia in acute stroke stage. Total anterior circulation syndrome and posterior circulation syndrome occurred more frequently among patients with pneumonia in acute stroke stage (P<0.001 and P=0.009, respectively). Multivariate Cox regression revealed that pneumonia in acute stroke stage is a significant predictor of 3-year mortality (hazard ratio=6.39, 95% confidence interval=4.03-10.11, P<0.001). In conclusion, pneumonia during the acute stroke stage is associated with increased risk of 3-year mortality. Interventions to prevent pneumonia in acute stroke stage might improve ischemic stroke outcome.
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Isquemia Encefálica/complicações , Isquemia Encefálica/mortalidade , Pneumonia/complicações , Pneumonia/mortalidade , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/mortalidade , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/complicações , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prevalência , Fatores de RiscoRESUMO
OBJECTIVE: The influence of renal dysfunction on the clinical presentation and outcomes of patients with acute ischemic stroke is still controversial. We investigate the influence of renal dysfunction on the outcomes of patients with acute first-ever ischemic stroke. METHODS: Nine-hundred thirty-four patients with acute first-ever ischemic stroke were enrolled and followed for 3 years. Renal function was assessed using the equation of the Modification Diet for Renal Disease for estimated glomerular filtration rate (eGFR). Serum creatinine levels were obtained within 3 days of acute stroke onset. Reduced eGFR was defined as eGFR<60ml/min/1.73m(2). Clinical presentation, risk factors for stroke, laboratory data, co-morbidities, and outcomes were recorded. RESULTS: Total 264 patients (28.3%) had a reduced eGFR. The prevalence of older age, hypertension, and atrial fibrillation was significantly higher in patients with a reduced eGFR. Total anterior circulation syndrome occurred more frequently among patients with a reduced eGFR (P=0.010). Multivariate Cox regression revealed that a reduced eGFR is a significant predictor of 3-year mortality (HR=1.67, 95% CI=1.06-2.62, P=0.026). CONCLUSION: Reduced eGFR during the acute stroke stage is associated with increased risk of 3-year mortality. Furthermore, risk of acute complications and poor functional outcomes following discharge was significantly higher in patients with a reduced eGFR.
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Fibrilação Atrial/complicações , Isquemia Encefálica/complicações , Taxa de Filtração Glomerular/fisiologia , Nefropatias/complicações , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/mortalidade , Feminino , Humanos , Nefropatias/mortalidade , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Fatores de TempoRESUMO
In this paper, we used the Hilbert-Huang transform (HHT) analysis method to examine the time-frequency characteristics of spike waves for detecting epilepsy symptoms. We obtained a sample of spike waves and nonspike waves for HHT decomposition by using numerous intrinsic mode functions (IMFs) of the Hilbert transform (HT) to determine the instantaneous, marginal, and Hilbert energy spectra. The Pearson correlation coefficients of the IMFs, and energy-IMF distributions for the electroencephalogram (EEG) signal without spike waves, Spike I, Spike II and Spike III sample waves were determined. The analysis results showed that the ratios of the referred wave and Spike III wave to the referred total energy for IMF1, IMF2, and the residual function exceeded 10%. Furthermore, the energy ratios for IMF1, IMF2, IMF3 and the residual function of Spike I, Spike II to their total energy exceeded 10%. The Pearson correlation coefficients of the IMF3 of the EEG signal without spike waves and Spike I wave, EEG signal without spike waves and Spike II wave, EEG signal without spike waves and Spike III wave, Spike I and II waves, Spike I and III waves, and Spike II and III waves were 0.002, 0.06, 0.01, 0.17, 0.03, and 0.3, respectively. The energy ratios of IMF3 in the δ band to its referred total energy for the EEG signal without spike waves, and of the Spike I, II, and III waves were 4.72, 6.75, 5.41, and 5.55%, respectively. The weighted average frequency of the IMF1, IMF2, and IMF3 of the EEG signal without spike waves was lower than that of the IMF1, IMF2, and IMF3 of the spike waves, respectively. The weighted average magnitude of the IMF3, IMF4, and IMF5 of the EEG signal without spike waves was lower than that of the IMF1, IMF2, and IMF3 of spike waves, respectively.
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Eletroencefalografia/métodos , Epilepsia/diagnóstico , Epilepsia/fisiopatologia , Processamento de Sinais Assistido por Computador/instrumentação , Algoritmos , HumanosRESUMO
In an attempt to develop safe and robust methods for monitoring migraineurs' brain states, we explores the feasibility of using white, red, green and blue LED lights flickering around their critical flicker fusion (CFF) frequencies as foveal visual stimuli for inducing steady-state visual evoked potentials (SSVEP) and causing discernible habituation trends. After comparing the habituation indices, the multi-scale entropies and the time dependent intrinsic correlations of their SSVEP signals, we reached a tentative conclusion that sharp red and white light pulses flickering barely above their CFF frequencies can replace commonly used 13Hz stimuli to effectively cause SSVEP habituation among normal subjects. Empirical results showed that consecutive short bursts of light can produce more consistent responses than a single prolonged stimulation. Since these high frequency stimuli do not run the risk of triggering migraine or seizure attacks, further tests of these stimuli on migraine patients are warranted in order to verify their effectiveness.
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Potenciais Evocados Visuais/efeitos da radiação , Fóvea Central/fisiologia , Fóvea Central/efeitos da radiação , Habituação Psicofisiológica/efeitos da radiação , Luz , Estimulação Luminosa , Adulto , Feminino , Humanos , Masculino , Processamento de Sinais Assistido por Computador , Adulto JovemRESUMO
OBJECTIVES: We aimed to investigate the effect of hypercholesterolemia on recovery after acute ischemic stroke. METHODS: Data of 3048 patients admitted for acute ischemic stroke from January to December 2009 were collected from the Stroke Registry in the Chang Gung Healthcare System. Baseline characteristics of patients with and without hypercholesterolemia were compared. The association of hypercholesterolemia with neurological severity and recovery was analyzed using multivariate logistic regression. The patients were then divided on the basis of age for subgroup analysis. RESULTS: The number of patients with and without a history of hypercholesterolemia was 474 (15.6%) and 2574 (84.4%), respectively. Univariate analysis showed that patients with hypercholesterolemia had a lower National Institutes of Health Stroke Scale (NIHSS) score on admission (p=0.004). However, during hospitalization, these patients displayed less improvement in their NIHSS score (p=0.002). These results remained significant in multivariate logistic regression analysis (p<0.001 and p=0.002, respectively). Subgroup analysis showed a similar association for hypercholesterolemia in both younger (age<70) and older (age≥70) age groups. CONCLUSIONS: Acute ischemic stroke in patients with hypercholesterolemia was correlated with reduced severity on admission and less favorable recovery during hospitalization, regardless of age.
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Hipercolesterolemia/fisiopatologia , Recuperação de Função Fisiológica/fisiologia , Acidente Vascular Cerebral/fisiopatologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Hipercolesterolemia/complicações , Lipídeos/sangue , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/etiologia , Doenças do Sistema Nervoso/fisiopatologia , Prognóstico , Sistema de Registros , Fatores de Risco , Acidente Vascular Cerebral/complicações , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Hyponatremia is the most common electrolyte disorder in hospitalized patients, and is frequently a marker of a significant underlying disease. The prognostic value of hyponatremia in patients with acute first-ever ischemic stroke is not known. We aimed to analyze whether hyponatremia in the acute stroke stage contributed to the risk of mortality or recurrent stroke in these patients. METHODS: We studied 925 patients presenting with acute first-ever ischemic stroke between 2002 and 2004. Sodium levels were obtained on arrival at the emergency room within 3 days of acute stroke onset. Hyponatremia was defined as a serum sodium concentration of 134 mmol/l or less. Clinical presentation, stroke risk factors, associated medical disease, and outcome were recorded. All patients were followed for 3 years for survival analysis. A multivariate Cox proportional hazards model was used to identify risk factors for 3-year mortality in these patients. We also constructed Kaplan-Meier survival curves, and compared groups with hyponatremia and normonatremia by means of log rank tests for significant differences. RESULTS: Among the patients with acute first-ever ischemic stroke, 107 (11.6%) were hyponatremic. Among stroke risk factors, the prevalence of diabetes mellitus was significantly higher among hyponatremic patients (p < 0.001). Prevalence of chronic renal insufficiency was also higher in the hyponatremic group (p = 0.002). Clinical presentations, such as the length of acute ward stay, initial impaired consciousness, and clinical course in acute stroke were similar among normo- and hyponatremic patients. Among the complications, pneumonia and urinary tract infection were significantly higher in hyponatremic than in normonatremic patients. After multivariate logistic regression analysis, diabetes mellitus and chronic renal insufficiency were associated with hyponatremia in these patients. Kaplan-Meier analysis indicated that the survival rate was significantly lower in hyponatremic patients than in normonatremic patients (log rank test; p value <0.001). After multivariate Cox proportional hazards model analysis, hyponatremia was a significant predictor of 3-year mortality in these patients after adjustment for related variables (p value = 0.003, hazard ratio = 2.23, 95% confidence interval: 1.30-3.82). CONCLUSION: Hyponatremia in the acute stroke stage is a predictor of 3-year mortality in patients with acute first-ever ischemic stroke that is independent of other clinical predictors of adverse outcome.
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Isquemia Encefálica/complicações , Hiponatremia/sangue , Hiponatremia/mortalidade , Acidente Vascular Cerebral/mortalidade , Idoso , Idoso de 80 Anos ou mais , Isquemia Encefálica/sangue , Feminino , Humanos , Hiponatremia/complicações , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Fatores de Risco , Sódio/sangue , Acidente Vascular Cerebral/complicações , Taxa de SobrevidaRESUMO
BACKGROUND/PURPOSE: Most reports about oculopharyngeal muscular dystrophy (OPMD) have been contributed by occidental countries, and most of the victims of this disease are racially white. In contrast, this disorder is rarely seen in Asians and has only one African report. Consequently, OPMD has been regarded as a disease of the Western world. The purpose of this paper is to challenge the accuracy of this concept. METHODS: In a Chinese immigrant family, 3 patients manifesting signs related to OPMD were examined. Electromyography, nerve conduction studies, muscle biopsy and genetic analysis were performed on the proband. All the 322 papers about OPMD were reviewed and their country of origin was labeled to perceive the approximate prevalence of OPMD. Countries were categorized into groups according to the continents to which they belonged. RESULTS: The proband's muscle histopathology showed small angulated fiber with rimmed vacuoles, ultrastructural pathology exposed filamentous intranuclear inclusions, and genetic analysis of the polyadenylate binding protein nuclear 1(PABPN1) gene revealed 13 GCG trinucleotide repeats in one allele (GCG)13 while being normal in the other. The survey of the country of origin of OPMD reports showed that 80% of these papers were contributed by occidental countries and that the number of publications of OPMD among countries of Americas and Asia were unequal, when compared to those of European countries, which were fairly proportioned. An epidemiologic review of the literature is presented and the prevalence of OPMD is discussed. CONCLUSION: This is a China-born Chinese patient with both morphologically and genetically proven of OPMD. The very low OPMD report rate in developing countries of East Asia is due to the unfamiliarity of medical workers to OPMD and the unavailability of medical supplies to confirm the diagnosis. In addition, the present and previous reports provide clear evidence that OPMD in these areas is underdiagnosed.
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Distrofia Muscular Oculofaríngea/epidemiologia , Proteína II de Ligação a Poli(A)/genética , Proteína I de Ligação a Poli(A)/genética , Adulto , Idoso , América/epidemiologia , Ásia/epidemiologia , China/epidemiologia , Eletromiografia , Europa (Continente)/epidemiologia , Feminino , Técnicas de Genotipagem , Humanos , Masculino , Pessoa de Meia-Idade , Distrofia Muscular Oculofaríngea/diagnóstico , Distrofia Muscular Oculofaríngea/genética , Condução Nervosa/fisiologia , Prevalência , Repetições de Trinucleotídeos/genéticaRESUMO
The relationship between the number of myelinated nerve fibers from the hand that are reinnervating the distal glabrous skin of the toe and the number of myelinating nerve fibers originally innervating that toe target was investigated utilizing the toe-to-hand microvascular transplantation model. Digital nerve biopsy specimens suitable for morphometric analysis were obtained from the hand and toe at the time of surgery from 15 patients. Patients received sensory reeducation in the postoperative period to standardize rehabilitation. At a mean of 10.8 months after surgery, the degree of sensory recovery was determined by the hand therapist. Morphometric analysis was done by a neurologist blinded as to final sensory outcome. There was a statistically significant relationship between recovered moving two-point discrimination and ratio of the total number of reinnervating (neurotizing) finger axons compared with the original toe axons (r = 0.703, p < 0.003). A similar relationship was observed for static two-point discrimination (r = 0.802, p < 0.001). These results suggest that degree of sensory recovery in toe-to-hand transfer may be improved by increasing the number of reinnervating myelinated nerve fibers from the hand that neurotizes the free tissue transfer.
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Dedos/inervação , Transferência de Nervo , Recuperação de Função Fisiológica , Transtornos de Sensação/reabilitação , Dedos do Pé/inervação , Dedos do Pé/transplante , Adolescente , Adulto , Axônios , Humanos , Pessoa de Meia-Idade , Fibras Nervosas Mielinizadas , Adulto JovemRESUMO
Although toe-to-hand microvascular transplantation is now an accepted and successful reconstructive strategy for the thumb, there remains varying success in recovery of sensibility. The purpose of the present study was to provide an anatomic basis for analyzing neurotization of the great toe or the second toe with the digital nerves of the fingers and hand. Axon counts were obtained from donor and recipient nerves during reconstructive procedures. Sixty-seven upper- and eighty lower-extremity nerves were analyzed. No statistical difference was found between the mean number of axons between the thumb and the index finger; however, the thumb had significantly more (p < 0.001) axons than the middle or the ring finger. The great toe had significantly more axons than the second toe (p < 0.001). The common plantar digital nerve had significantly more axons than a digital nerve (p < 0.001). The thumb had significantly more axons than the great toe (p < 0.001). The thumb had significantly more axons than the second toe (p < 0.001). Numerical data are now available for strategic planning of toe transplantation, and direct neurotization from the dorsum of the hand to the digits.
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Axônios , Dedos/cirurgia , Nervos Periféricos/anatomia & histologia , Dedos do Pé/transplante , Dedos/inervação , Humanos , Microcirurgia , Procedimentos Neurocirúrgicos , Dedos do Pé/inervaçãoRESUMO
Leukocyte count predicted the risk of first-time myocardial infarction and ischemic stroke. The aim of this study was to determine the role of elevated leukocyte count in non-diabetic patients admitted for acute first-ever ischemic stroke on clinical presentation and 3-year mortality. We studied 462 patients with acute first-ever ischemic stroke without diabetes mellitus or active infection at admission. Patients were classified into 2 groups according to their leukocyte count. A white blood cell (WBC) count ≥ 10,000/µL was defined as an elevated leukocyte count, otherwise as normal. Clinical presentation, risk factors for stroke, laboratory data, co-morbidities, and outcomes were recorded. 64 patients (13.9%) had elevated leukocytes. Multivariate logistic regression showed that an elevated platelet count was positively associated with the elevated leukocyte count, while a low serum sodium level was negatively associated with an elevated leukocyte count (P=0.008, P=0.003, respectively). An elevated leukocyte count was associated with a higher risk of a stroke in evolution (P=0.021). Multivariate Cox regression analysis revealed that an elevated leukocyte count is a significant predictor of 3-year mortality [P=0.010, HR=3.26 (1.33-7.98)]. In conclusion, higher leukocyte counts during the acute stroke stage are associated with increased risk of 3-year mortality in patients with acute, first-ever ischemic stroke.
Assuntos
Isquemia Encefálica/sangue , Isquemia Encefálica/mortalidade , Diabetes Mellitus , Contagem de Leucócitos/tendências , Acidente Vascular Cerebral/sangue , Acidente Vascular Cerebral/mortalidade , Idoso , Idoso de 80 Anos ou mais , Diabetes Mellitus/sangue , Diabetes Mellitus/mortalidade , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Taxa de Sobrevida/tendênciasRESUMO
With brain-computer interface (BCI) applications in mind, we analyzed the amplitudes and the signal-to-noise ratios (SNR) of steady-state visual evoked potentials (SSVEP) induced in the foveal and extra-foveal regions of human retina. Eight subjects (age 20-55) have been exposed to 2° circular and 16°-18° annular visual stimulation produced by white LED lights flickering between 5Hz and 65Hz in 5Hz increments. Their EEG signals were recorded using a 64-channel NeuroScan system and analyzed using non-parametric spectral and canonical convolution techniques. Subjects' perception of flickering and their levels of comfort towards the visual stimulation were also noted. Almost all subjects showed distinctively higher SNR in their foveal SSVEP responses between 25Hz and 45Hz. They also noticed less flickering and felt more comfortable with the visual stimulation between 30Hz and 45Hz. These empirical evidences suggest that lights flashing above the critical flicker fusion rates (CFF) of human vision may be used as effective and comfortable stimuli in SSVEP BCI applications.
Assuntos
Potenciais Evocados Visuais/fisiologia , Fóvea Central/fisiologia , Razão Sinal-Ruído , Adulto , Feminino , Análise de Fourier , Humanos , Masculino , Pessoa de Meia-Idade , Estimulação Luminosa , Células Fotorreceptoras Retinianas Cones/fisiologia , Células Fotorreceptoras Retinianas Bastonetes/fisiologia , Processamento de Sinais Assistido por Computador , Percepção Visual/fisiologia , Adulto JovemRESUMO
BACKGROUND/PURPOSE: Despite effective vaccine programs, tetanus is occasionally observed in adults. We reviewed clinical presentation data for adult patients with tetanus in the post-vaccine era in Taiwan. METHODS: We retrospectively reviewed the medical records of all adult patients (age >18 years) discharged from Chang-Gung Memorial Hospital at Lin-Ko (CGMHLK) after treatment for tetanus between January 1996 and July 2005. Data regarding demographic characteristics, clinical manifestation, treatment, and outcome were collected. To assess the features for different age groups, patients were divided into those aged ≥65 years and those aged <65 years. To identify risk factors for respiratory failure, the patients were classified as those with and without respiratory failure. RESULTS: Twenty-three patients with tetanus, 11 (48%) women and 12 (52%) men, were included in the study. The average age was 57 ± 18 years (range 18-84 years). Eighteen (78%) patients had a history of acute injury. The average incubation period was 8 ± 5 days. The most common clinical presentation at onset was trismus (78%). Thirteen (57%) patients developed respiratory failure and underwent endotracheal intubation. The most common complication was pneumonia (30%). All the patients survived and recovered. Age ≥65 years was significantly associated with trismus, dysphagia, dysarthria, and pneumonia. Generalized tetanus subtype and pneumonia were significant risk factors for respiratory failure. CONCLUSION: This study revealed several characteristics of adult tetanus cases in the post-vaccine era in Taiwan. Further serological studies and improved tetanus vaccinations may be needed to ensure better protection, especially for high-risk populations. The exceptionally good prognosis for our patients confirms that appropriate treatment, including wound care, early diagnosis, proper medication, and prevention of complications, is essential in managing this traditional curable disease.
Assuntos
Tétano/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taiwan/epidemiologia , Tétano/tratamento farmacológico , Tétano/epidemiologia , Resultado do TratamentoRESUMO
OBJECTIVE: Primary quadriceps weakness/atrophy is a rare disorder with variable etiologies; therefore, this disorder has been regarded as a clinical syndrome rather than a distinct entity. However, three affected patients of a Taiwanese family demonstrate a uniform pattern of quadriceps weakness and atrophy, their clinical manifestations and pattern of inheritance may suggest a new disease entity. PATIENTS AND METHODS: Three patients in a Taiwanese kindred with selective quadriceps weakness and atrophy, which began after age 40 years, were examined. To disclose the confines of this disorder, muscle CT scans, electromyography, nerve conduction studies and muscle biopsies were performed; and to unravel and better understand the nature of this disorder, histopathological, ultrastructural, immunocytochemical and genetic studies were carried out. RESULTS: In two patients with long-standing disease, muscle imaging showed marked atrophy and fat replacement of the anterior thigh muscles and electromyography showed a mixture of myopathic and neuropathic changes. Muscle histopathology on the mildly affected tibialis anterior showed myopathic changes with myofibrillar degeneration and secondary neurogenic alterations. Immunocytochemical staining was not diagnostic but excluded the dystrophinopathies and other well-known muscular dystrophies. CONCLUSION: All previously identified diseases resulting in quadriceps weakness and atrophy have been ruled out and the present disorder appears to be a new disease entity of autosomal dominant late onset quadriceps myopathy.