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INTRODUCTION: The purpose of this study was to identify course of the corticobulbar tract and factors associated with the occurrence of facial paresis (FP) in lateral medullary infarction (LMI). METHODS: Patients diagnosed with LMI who were admitted to tertiary hospital were retrospectively investigated and divided into two groups based on the presence of FP. FP was defined as grade 2 or more by the House-Brackmann scale. Differences between the two groups were analyzed with respect to anatomical location of the lesions, demographic data (age, sex), risk factors (diabetes, hypertension, smoking, prior stroke, atrial fibrillation, and other cardiac risk factors for stroke), large vessel involvement on magnetic resonance angiography, other symptoms and signs (sensory symptoms, gait ataxia, limb ataxia, dizziness, Horner syndrome, hoarseness, dysphagia, dysarthria, nystagmus, nausea/vomiting, headache, neck pain, diplopia, and hiccup). RESULTS: Among 44 LMI patients, 15 patients (34%) had FP, and all of them had ipsilesional central-type FP. The FP group tended to involve upper (p < 0.0001) and relative ventral (p = 0.019) part of the lateral medulla. Horizontally large lesion was also related to the presence of FP (p = 0.044). Dysphagia (p = 0.001), dysarthria (p = 0.003), and hiccups (p = 0.034) were more likely to be accompanied by FP. Otherwise, there were no significant differences. CONCLUSION: The results of present study indicate that the corticobulbar fibers innervating the lower face decussate at the upper level of the medulla and ascend through the dorsolateral medulla, where the concentration of the fibers is densest near the nucleus ambiguus.
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Trastornos de Deglución , Parálisis Facial , Síndrome Medular Lateral , Accidente Cerebrovascular , Humanos , Parálisis Facial/diagnóstico por imagen , Parálisis Facial/etiología , Disartria/complicaciones , Disartria/patología , Estudios Retrospectivos , Imagen por Resonancia Magnética/efectos adversos , Bulbo Raquídeo/diagnóstico por imagen , Infarto , Síndrome Medular Lateral/complicaciones , Síndrome Medular Lateral/diagnóstico por imagenRESUMEN
BACKGROUND: A culturally validated Korean version of the PainDETECT Questionnaire (PD-Q) was used to identify neuropathic pain components (NeP) in patients suffering from chronic pain. The purpose of this study was to determine if the Korean PD-Q can be used to subgroup patients with peripheral NeP according to sensory symptom profiles. METHODS: This study included 400 Korean patients with peripheral neuropathic pain diagnosed as probable or definite NeP. The total scores and subscores for each item in PD-Q were transformed into a Z-score for standardization. Hierarchical cluster analysis was performed to identify clusters of subjects by PD-Q scores. RESULTS: The mean total PD-Q score of the study participants was 14.57 ± 6.46. A hierarchical cluster analysis identified 5 clusters with distinct pain characteristic profiles. Cluster 1 had relatively severe burning and tingling sensations. The mean total PD-Q score for cluster 2 was the lowest of the 5 clusters. Cluster 3 tended to be vulnerable to pain in response to cold/heat stimulation. Cluster 4 showed relatively severe pain induced by physical stimuli, such as light touch or slight pressure. Cluster 5 had high scores for all NeP symptoms. CONCLUSION: This study demonstrates the ability of patients to cluster by symptoms using the Korean PD-Q. Subgrouping of peripheral neuropathic pain by sensory symptom profile may be useful in making effective drug treatment decisions.
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Dimensión del Dolor/instrumentación , Enfermedades del Sistema Nervioso Periférico/complicaciones , Trastornos de la Sensación/etiología , Anciano , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Manejo del Dolor/métodos , Manejo del Dolor/estadística & datos numéricos , Dimensión del Dolor/normas , Dimensión del Dolor/estadística & datos numéricos , Enfermedades del Sistema Nervioso Periférico/epidemiología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , República de Corea/epidemiología , Trastornos de la Sensación/epidemiología , Trastornos de la Sensación/fisiopatología , Encuestas y CuestionariosRESUMEN
INTRODUCTION: To develop a new method to quantify the density of nerves, vessels, and the neurovascular contacts, we studied skin biopsies in diabetes and control subjects. METHODS: Skin biopsies with dual immunofluorescent staining were used to visualize nerves and blood vessels. The density of nerves, vessels, and their neurovascular contacts were quantified with unbiased stereology. Results were compared with examination findings, validated questionnaires, and autonomic function. RESULTS: In tissue from 19 controls and 20 patients with diabetes, inter-rater and intra-rater intraclass correlation coefficients were high (>0.85; P < .001) for all quantitative methods. In diabetes, the nerve densities (P < .05), vessel densities (P < .01), and the neurovascular densities (P < .01) were lower compared with 20 controls. Results correlated with autonomic function, examination and symptom scores. DISCUSSION: We report an unbiased, stereological method to quantify the cutaneous nerve, vessel and neurovascular density and offer new avenues of investigation into cutaneous neurovascular innervation in health and disease.
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Enfermedades del Sistema Nervioso Autónomo/patología , Neuropatías Diabéticas/patología , Microvasos/patología , Nervios Periféricos/patología , Piel/patología , Neuropatía de Fibras Pequeñas/patología , Adulto , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Capilares/inervación , Capilares/patología , Estudios de Casos y Controles , Diabetes Mellitus Tipo 2/complicaciones , Neuropatías Diabéticas/etiología , Neuropatías Diabéticas/fisiopatología , Femenino , Humanos , Masculino , Microscopía Confocal , Microscopía Fluorescente , Microvasos/inervación , Persona de Mediana Edad , Piel/irrigación sanguínea , Piel/inervación , Neuropatía de Fibras Pequeñas/fisiopatologíaRESUMEN
Guillain-Barré syndrome (GBS) is the most common immune-mediated polyradiculoneuropathy and it is also the most commonly reported severe adverse event following immunization in adults. To evaluate the results of clinical and laboratory features of GBS after vaccination in Korea, we analyzed the claims-based data from 2002 to 2014 using materials collected for the Advisory Committee Vaccination Injury Compensation (ACVIC) meeting including, clinical features, nerve conduction studies (NCSs), cerebrospinal fluid (CSF) profiles, treatment, and outcomes. Forty-eight compensated GBS cases (median age, 15 years; interquartile range [IQR], 13-51; male:female ratio, 1:1) of 68 suspected GBS were found following immunization and all of them with influenza immunizations with either monovalent (n = 35) or trivalent (n = 13). Among them, 30 cases fulfilled the Brighton criteria level 1-3 (62.5%). The median duration between the onset of symptoms to nadir, duration of the nadir, and total admission period were 3 (IQR, 2-7 days), 2 (IQR, 1-5 days), and 14 (IQR, 6-33 days) days, respectively. The most frequently reported symptom was quadriparesis which was present in 36 cases (75%) at nadir. CSF examination revealed albuminocytologic dissociation in 25.0% and NCS was abnormal in 61.8%. After treatment, most of them showed improvement. Clinical features were similar to typical post-infectious GBS and there were both demyelinating and axonal forms suggesting heterogeneous pathogenic mechanism. In order to improve the diagnostic certainty of post-vaccination GBS, careful documentation of clinical features and timely diagnostic work-up with follow-up studies are needed.
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Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/patología , Vacunación/efectos adversos , Adolescente , Adulto , Niño , Preescolar , Femenino , Hospitalización , Humanos , Lactante , Masculino , Persona de Mediana Edad , Cuadriplejía/complicaciones , República de Corea , Vacunas/efectos adversos , Adulto JovenAsunto(s)
Enfermedades Desmielinizantes/etiología , Enfermedades Desmielinizantes/terapia , Neoplasias Cardíacas/complicaciones , Mixoma/complicaciones , Polineuropatía Paraneoplásica/etiología , Polineuropatía Paraneoplásica/terapia , Anciano , Femenino , Neoplasias Cardíacas/cirugía , Humanos , Mixoma/cirugía , Examen Neurológico , Cuadriplejía/etiología , Resultado del TratamientoRESUMEN
BACKGROUND: Low 25-hydroxyvitamin D (25(OH)D) concentrations have been shown to predict risk of cardiovascular disease and all-cause mortality. Although the prevalence of 25(OH)D deficiency is high in patients with acute stroke, the prognostic value of 25(OH)D in stroke has not been clearly established. The purpose of this study was to determine whether the baseline serum 25(OH)D level was associated with the functional outcome in patients with acute ischemic stroke. METHODS: From June 2011 to January 2014, consecutive patients with acute ischemic stroke within 7 days of symptom onset were enrolled in this study from a prospectively maintained stroke registry. Serum 25(OH)D level was measured at admission. Clinical and laboratory data including stroke severity using the National Institute of Health Stroke Scale (NIHSS) score were collected during admission, and the functional outcome at 3 months was assessed by modified Rankin scale (mRS). The association between the baseline 25(OH)D level and a good functional outcome (mRS 0-2) at 3 months was analyzed by multiple logistic regression models. RESULTS: A total of 818 patients were enrolled in this study. Mean age was 66.2 (±12.9) years, and 40.5% were female. The mean 25(OH)D level was 47.2 ± 31.7 nmol/l, and the majority of patients met vitamin D deficient status (<50 nmol/l; 68.8%), while an optimal vitamin D level (≥75 nmol/l) was present in only 13.6% of the patients, and 436 (53.3%) patients showed good functional outcome at 3 months. Serum 25(OH)D levels in patients with good outcomes were significantly higher than those with poor outcome (50.2 ± 32.7 vs. 43.9 ± 30.0 nmol/l, p = 0.007). The 3-month functional outcome was significantly associated with month-specific 25(OH)D quartiles in multivariable logistic regression analysis. After adjustment for age and sex, the highest 25(OH)D quartile group had higher tendency for good functional outcome at 3 months (odds ratio (OR) = 1.68, 95% confidence interval (CI) = 1.13-2.51). After fully adjusting for other potential confounders, such as stroke severity and vascular risk factors, the association was further strengthened with an OR (95% CI) of 1.90 (1.14-3.16). Other factors associated with good functional outcome in multivariable analysis were younger age, lower initial NIHSS score and absence of diabetes. CONCLUSIONS: This study suggests that serum 25(OH)D level is an independent predictor of functional outcome in patients with acute ischemic stroke. Further studies are required to determine whether vitamin D supplementation could improve functional outcome in patients with ischemic stroke.
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Isquemia Encefálica/complicaciones , Accidente Cerebrovascular/complicaciones , Deficiencia de Vitamina D/complicaciones , Vitamina D/análogos & derivados , Adulto , Factores de Edad , Anciano , Biomarcadores/sangre , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/fisiopatología , Isquemia Encefálica/terapia , Distribución de Chi-Cuadrado , Evaluación de la Discapacidad , Femenino , Humanos , Modelos Logísticos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Oportunidad Relativa , Pronóstico , Sistema de Registros , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Accidente Cerebrovascular/diagnóstico , Accidente Cerebrovascular/fisiopatología , Accidente Cerebrovascular/terapia , Factores de Tiempo , Vitamina D/sangre , Deficiencia de Vitamina D/sangre , Deficiencia de Vitamina D/diagnósticoRESUMEN
It is well known that patients with peripheral neuropathy along with autonomic involvement can also exhibit autonomic hyperactivity. There are rare cases in which these patients developed posterior reversible encephalopathy syndrome (PRES). Patients with primary Sjögren's syndrome (pSS) may be more likely to exhibit autonomic hypofunction rather than autonomic hyperfunction, which is a rare event. In the present work, we report the first known case of PRES as an initial neurological manifestation of pSS.
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Síndrome de Leucoencefalopatía Posterior/complicaciones , Síndrome de Leucoencefalopatía Posterior/diagnóstico , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Adulto , Diagnóstico Diferencial , Femenino , HumanosRESUMEN
OBJECTIVES: We attempted to determine the propensity for sidedness of cardiogenic emboli associated with atrial fibrillation (AF) by comparing the sides on which microembolic signals (MES) were detected via transcranial Doppler (TCD) monitoring and the location of infarcts on magnetic resonance imaging. DESIGN: Patients with AF on Holter monitoring and MES on TCD monitoring were selected from an ischemic stroke registry. Patients with prosthetic valves or cerebral/carotid artery stenosis were excluded. RESULTS: By TCD monitoring of 30 patients, 78 MES were detected: 47 on the right and 31 on the left side (60.3% vs. 39.7%, p < 0.01, chi-square test). Among 21 patients who had middle or anterior cerebral artery (MCA/ACA) territory infarcts, 16 had right-side-dominant infarcts and 5 patients had left-side-dominant infarcts (76.2% vs. 23.8%, p < 0.01, chi-square test). The median infarct volume on the right side was 16.2 (3.18-75.4) ml, while that of left side was 1.2 (0.25-5.05) ml (p < 0.01, Mann-Whitney U test). CONCLUSION: This study demonstrated the existence of a right-side propensity of cardiogenic emboli and the larger infarct volume of right-side MCA/ACA stroke in patients with AF. These results can be attributed to anatomical differences between the innominate and the left common carotid artery.
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Fibrilación Atrial/complicaciones , Infarto Encefálico , Cerebro/patología , Embolia Intracraneal , Anciano , Fibrilación Atrial/diagnóstico , Infarto Encefálico/diagnóstico , Infarto Encefálico/etiología , Infarto Encefálico/fisiopatología , Electrocardiografía Ambulatoria/métodos , Femenino , Humanos , Embolia Intracraneal/diagnóstico , Embolia Intracraneal/etiología , Embolia Intracraneal/fisiopatología , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Sistema de Registros , República de Corea , Índice de Severidad de la Enfermedad , Ultrasonografía Doppler Transcraneal/métodosRESUMEN
PURPOSE: Nerve conduction study (NCS) is essential for subclassifying Guillain-Barré syndrome (GBS). It is well known that the GBS subclassification can change through serial NCSs. However, the usefulness of serial NCSs is debatable, especially in patients with early stage GBS. METHODS: Follow-up NCS data within 3 weeks (early followed NCS, EFN) and within 3 to 10 weeks (late-followed NCS, LFN) were collected from 60 patients with GBS who underwent their first NCS (FN) within 10 days after symptom onset. Each NCS was classified into five subtypes (normal, demyelinating, axonal, inexcitable, and equivocal), according to Hadden's and Rajabally's criteria. We analyzed the frequency of significant changes in classification (SCCs) comprising electrodiagnostic aggravation and subtype shifts between demyelinating and axonal types according to follow-up timing. RESULTS: Between FN and EFN, 33.3% of patients with Hadden's criteria and 18.3% with Rajabally's criteria showed SCCs. Between FN and LFN, 23.3% of patients with Hadden's criteria and 21.7% with Rajabally's criteria showed SCCs, of which 71.4% (Hadden's criteria) and 46.2% (Rajabally's criteria) already showed SCCs from the EFN. The conditions of delayed SCCs between EFN and LFN were very early FN, mild symptoms at the FN, or persistent electrophysiological deterioration 3 weeks after symptom onset. CONCLUSIONS: A substantial proportion of patients with GBS showed significant changes in neurophysiological classification at the early stage. Serial NCS may be helpful for precise neurophysiological classification. This study suggests that follow-up NCSs should be performed within 3 weeks of symptom onset in patients with GBS in whom FN was performed within 10 days of symptom onset.
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Síndrome de Guillain-Barré , Cinostatina , Humanos , Síndrome de Guillain-Barré/diagnóstico , Estudios de Conducción Nerviosa , NeurofisiologíaRESUMEN
BACKGROUND: It is difficult to clarify whether small deep infarction is caused by cardioembolism or intrinsic small vessel disease in patients with atrial fibrillation (AF). The purpose of this study was to determine whether preexisting small vessel disease would differ according to the presenting infarct pattern and to determine the factors associated with acute single small deep infarction in stroke patients with AF. METHODS: Between January 2008 and August 2012, 1,592 consecutive patients with acute ischemic stroke presenting within 7 days of symptom onset were entered in a prospectively maintained stroke registry. For the present study, 231 stroke patients with AF were enrolled irrespective of the stroke subtype. We divided these patients into 2 groups (lacunar infarct pattern, n = 20, vs. nonlacunar infarct pattern, n = 211) according to the acute infarct pattern on diffusion-weighted imaging. Patients with acute single small deep infarction on diffusion-weighted imaging were assigned to the lacunar infarct pattern group. We assessed the severity of preexisting small vessel disease by grading white matter lesions (WMLs) according to the Fazekas scale (periventricular WML score ranging from 0 to 3 and deep WML score ranging from 0 to 3 were added to give a total WML score ranging from 0 to 6), multilacunar state (number of chronic lacunes ≥2) and the presence of microbleeds. Demographic characteristics, vascular risk factors and neuroimaging data were compared between the two groups. RESULTS: Patients with a lacunar infarct pattern showed more severe WMLs than those with a nonlacunar pattern [median total WML score 2.5 (range 2-4) vs. 1.0 (0-2); p < 0.001]. A multilacunar state was more prevalent in the lacunar infarct pattern group compared with the nonlacunar pattern group (65 vs. 28.9%; p = 0.001). However, the presence of microbleeds did not differ between the groups. Multiple logistic regression analyses revealed periventricular WMLs [odds ratio (OR) 4.12, 95% confidence interval (CI) 2.14-7.92], deep WMLs (OR 3.42, 95% CI 1.75-6.66) and multilacunar state (OR 7.85, 95% CI 2.45-25.6) as the predictors of a lacunar infarct pattern. CONCLUSIONS: The severity of WMLs and chronic lacunes were independent predictors of the incident infarct pattern, which suggested that acute single small deep infarction might be caused by intrinsic small vessel disease despite the presence of concomitant AF.
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Fibrilación Atrial/patología , Infarto Encefálico/patología , Anciano , Anciano de 80 o más Años , Fibrilación Atrial/complicaciones , Encéfalo/irrigación sanguínea , Encéfalo/patología , Infarto Encefálico/complicaciones , Imagen de Difusión por Resonancia Magnética/métodos , Femenino , Humanos , Masculino , Factores de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND/AIMS: An increase in the pulsatility index (PI) has been suggested to reflect distal vascular resistance. The purpose of the present study was to investigate the association between intracranial arterial calcification and intracranial PIs. METHODS: Consecutive patients with acute ischemic stroke or transient ischemic attack were included. The PIs of both middle cerebral arteries (MCAs) were measured by transcranial Doppler ultrasonography. Intracranial carotid artery calcification (ICAC) was assessed on computed tomography angiography, and then compared with the mean PI of both MCAs. Patients with internal carotid artery steno-occlusion were excluded from this study. RESULTS: A total of 156 patients were finally enrolled. The prevalence of diabetes increased as the PI value increased (p for trends; p = 0.025). PI was correlated with ICAC score (r = 0.413, p < 0.001) and age (r = 0.507, p < 0.001). Multiple linear regression analysis indicated that aging and ICAC were independent determinants of the PI of MCA after adjusting for sex, systolic blood pressure, smoking, and the presence of diabetes. CONCLUSIONS: This study shows that an increase in PI was correlated with the severity of ICAC, which suggests calcification-related vascular resistance might have a role in the elevation of PI.
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Isquemia Encefálica/diagnóstico por imagen , Calcinosis/diagnóstico por imagen , Enfermedades de las Arterias Carótidas/diagnóstico por imagen , Accidente Cerebrovascular/diagnóstico por imagen , Adulto , Anciano , Anciano de 80 o más Años , Isquemia Encefálica/complicaciones , Isquemia Encefálica/fisiopatología , Calcinosis/complicaciones , Calcinosis/fisiopatología , Enfermedades de las Arterias Carótidas/complicaciones , Enfermedades de las Arterias Carótidas/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Arteria Cerebral Media/diagnóstico por imagen , Arteria Cerebral Media/fisiopatología , Análisis de la Onda del Pulso , Factores de Riesgo , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/fisiopatología , Ultrasonografía Doppler Transcraneal , Resistencia Vascular/fisiologíaRESUMEN
BACKGROUND AND PURPOSE: The description of pain is the most-important indicator leading to the adequate treatment of patients with neuropathic pain (NeP). The purpose of this study was to identify and characterize the unique features of Korean verbal descriptions in patients with peripheral NeP. METHODS: This study included 400 patients (167 males and 233 females) and their 1,387 pain-description responses. Patients with peripheral NeP freely described their symptoms in Korean. Collected verbal descriptions were grouped according to terminologies with similar meanings. Participants completed validated patient-reported outcome scales including the neuropathic pain symptom inventory (NPSI) and painDETECT questionnaire (PD-Q). The frequencies of each verbal pain descriptor were compared between the NPSI and PD-Q scores. RESULTS: 'Jeorim' (tingling) was the most common among 17 types of organized verbal pain descriptors, and the 'Sirim' (cold) symptom had a significantly higher rate of use in the 2 high-severity groups when participants were classified by their total scores on the NPSI and PD-Q. CONCLUSIONS: Korean verbal NeP descriptors were significantly diverse. The Jeorim (tingling) and Sirim (cold) descriptors can be utilized in evaluations of Korean patients with NeP.
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To explore the clinical significance of anti-cytosolic 5'-nucleoditase 1A (NT5c1A) antibody seropositivity in inflammatory myopathies, we measured anti-NT5c1A antibodies and analyzed their clinical features. Anti-NT5c1A antibodies were measured in the sera of 103 patients with inflammatory myopathies using an enzyme-linked immunosorbent assay. Positivity for anti-NT5c1A antibody was found in 13 (12.6%) of 103 patients with inflammatory myopathy. Anti-NT5c1A antibody was most frequently identified in patients with inclusion body myositis (IBM) (8/20, 40%), followed by dermatomyositis (2/13, 15.4%), immune-mediated necrotizing myopathy (2/28, 7.1%), and polymyositis (1/42, 2.4%). In eight patients with the anti-NT5c1A antibody-seropositive IBM, the median age at symptom onset was 54 years (interquartile range [IQR]: 48-57 years), and the median disease duration was 34 months (IQR: 24-50 months]. Knee extension weakness was greater than or equal to hip flexion weakness in eight (100%) patients, and finger flexion strength was less than shoulder abduction in three (38%) patients. Dysphagia symptoms were found in three (38%) patients. The median serum CK level was 581 IU/l (IQR: 434-868 IU/L]. Clinically significant differences were not found between anti-NT5c1A antibody-seropositive and seronegative IBM groups with respect to gender, age at symptom onset, age at diagnosis, disease duration, serum CK values, presence of other autoantibodies, dysphagia, and the pattern of muscle impairment. Although anti-NT5c1A antibody is known to be associated with IBM, seropositivity has also been noted in non-IBM inflammatory myopathies, and is insufficient to have clinical significance by itself. These findings have important implications for interpreting anti-NT5c1A antibody test results as the first study in Korea.
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Trastornos de Deglución , Miositis por Cuerpos de Inclusión , Miositis , Humanos , Persona de Mediana Edad , Autoanticuerpos , Relevancia Clínica , República de CoreaRESUMEN
BACKGROUND AND PURPOSE: To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured anti-HMGCR antibodies and analyzed the clinical, radiological, and pathological features of patients with anti-HMGCR myopathy. METHODS: We measured titers of anti-HMGCR antibodies in the sera of 99 patients with inflammatory myopathy, 36 patients with genetic myopathy, and 63 healthy subjects using an enzyme-linked immunosorbent assay. We tested 16 myositis-specific autoantibodies (MSAs) in all patients with anti-HMGCR myopathy. RESULTS: Positivity for the anti-HMGCR antibody was observed in 17 (4 males and 13 females) of 99 patients with inflammatory myopathy. The median age at symptom onset was 60 years. Ten (59%) of the patients with anti-HMGCR positivity had taken statins. The titer of anti-HMGCR antibodies was significantly higher in the statin-naïve group (median=230 U/mL, interquartile range=170-443 U/mL) than in the statin-exposed group (median=178 U/mL, interquartile range=105-210 U/mL, p=0.045). The most common symptom was proximal muscle weakness in 15 patients (88%), followed by myalgia in 9 (53%), neck weakness in 4 (24%), dysphagia in 3 (18%), and skin lesions in 2 (12%). The median titer of anti-HMGCR antibody was 202 U/mL. We found eight different MSAs in nine (53%) patients. The median disease duration from symptom onset to diagnosis was significantly shorter in the MSA-positive group than in the MSA-negative group (p=0.027). CONCLUSIONS: Our study was the first to measure anti-HMGCR antibodies in inflammatory myopathy. It has provided new findings, including the suggestion of the coexistence of other MSAs in Korean patients.
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OBJECTIVES: Reportedly, hippocampal neuronal degeneration by kainic acid (KA)-induced seizures in rats <14 days old was enhanced by lipopolysaccharide (LPS). This study was to test the hypothesis that cytokines such as interleukin (IL)-1ß, IL-6 and tumor necrosis factor-α are associated with aggravated neuronal damage. MATERIALS AND METHODS: Sixty male Sprague-Dawley, 14-day-old rats were used. Experiments were conducted in saline, LPS + saline, saline + KA and LPS + KA groups. Intraperitoneal LPS injections (0.04 mg/kg) were administered 3 h prior to KA injection (3 mg/kg). RESULTS: The LPS + KA group showed a tendency toward shorter latency to seizure onset (p = 0.086) and significantly longer seizure duration (p < 0.05) compared with the KA group. Induction of the proconvulsant cytokine IL-1ß in rat pup brains was significantly greater in the LPS + KA group compared to the KA group (38.8 ± 5.5 vs. 9.2 ± 1.0 pg/µg; p < 0.05); however, IL-6 levels were higher in the KA group than in the LPS + KA group (108.7 ± 6.8 vs. 60.9 ± 4.7 pg/µg; p < 0.05). The difference in tumor necrosis factor-α between the LPS + KA group and the KA group was insignificant (12.1 ± 0.6 vs. 10.9 ± 2.3 pg/µg; p = 0.64). CONCLUSIONS: Our results showed an increase in the proconvulsant cytokine IL-1ß and a decrease in a potentially neuroprotective cytokine, IL-6, in rat pups treated with LPS + KA. These results warrant further investigation into the possible role of IL-1ß induction and IL-6 suppression in LPS-promoted neuronal damage.
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Lesiones Encefálicas/etiología , Lesiones Encefálicas/prevención & control , Citocinas/metabolismo , Lipopolisacáridos/administración & dosificación , Fármacos Neuroprotectores/administración & dosificación , Convulsiones/complicaciones , Animales , Animales Recién Nacidos , Temperatura Corporal/efectos de los fármacos , Modelos Animales de Enfermedad , Agonistas de Aminoácidos Excitadores/toxicidad , Femenino , Hipocampo/efectos de los fármacos , Hipocampo/metabolismo , Hipocampo/patología , Interleucina-1beta/metabolismo , Interleucina-6/metabolismo , Ácido Kaínico/toxicidad , Masculino , Ratas , Ratas Sprague-Dawley , Tiempo de Reacción/efectos de los fármacos , Convulsiones/inducido químicamente , Convulsiones/tratamiento farmacológico , Estadísticas no Paramétricas , Estado Epiléptico/inducido químicamente , Estado Epiléptico/complicaciones , Factores de Tiempo , Factor de Necrosis Tumoral alfa/metabolismoRESUMEN
Dysferlinopathy is caused by mutations in the DYSF gene. To characterize the clinical spectrum, we investigated the characteristics of 31 Korean dysferlinopathy patients confirmed by immunohistochemistry. The mean age of symptom onset was 22.23 ± 7.34 yr. The serum creatine kinase (CK) was highly increased (4- to 101-fold above normal). The pathological findings of muscle specimens showed nonspecific dystrophic features and frequent inflammatory cell infiltration. Muscle imaging studies showed fatty atrophic changes dominantly in the posterolateral muscles of the lower limb. The patients with dysferlinopathy were classified by initial muscle weakness: fifteen patients with Miyoshi myopathy phenotype (MM), thirteen patients with limb girdle muscular dystrophy 2B phenotype (LGMD2B), two patients with proximodistal phenotype, and one asymptomatic patient. There were no differences between LGMD2B and MM groups in terms of onset age, serum CK levels and pathological findings. Dysferlinopathy patients usually have young adult onset and high serum CK levels. However, heterogeneity of clinical presentations and pathologic findings upon routine staining makes it difficult to diagnose dysferlinopathy. These limitations make immunohistochemistry currently the most important method for the diagnosis of dysferlinopathy.
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Distrofia Muscular de Cinturas/diagnóstico , Adolescente , Adulto , Edad de Inicio , Creatina Quinasa/sangre , Miopatías Distales/patología , Disferlina , Femenino , Humanos , Inmunohistoquímica , Masculino , Proteínas de la Membrana/genética , Persona de Mediana Edad , Proteínas Musculares/genética , Atrofia Muscular/patología , Distrofia Muscular de Cinturas/genética , Distrofia Muscular de Cinturas/patología , Mutación , Fenotipo , República de Corea , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
BACKGROUND: Previous diffusion-weighted MRI (DWI) studies have indicated that 10-40% of patients have silent embolism during neurointerventional procedures. However, lesion patterns of the embolisms have not been adequately investigated. METHODS: DWI was taken within 7 days before and 48 h after cerebral angioplasty and stent procedures. New lesions on the follow-up DWI were analyzed in the non-treated arterial territories. Based on the arterial territories, supratentorial lesions were classified into cortical lesions and subcortical lesions. Cortical lesions were subdivided into cortical border zone and cortical proper lesions. Subcortical lesions were divided into deep perforator and internal border zone lesions. Infratentorial lesions were divided into brainstem and cerebellar lesions. RESULTS: 72 patients were included in this study. There were 223 new DWI lesions (1-23 lesions) in the non-treated arterial territories of 37 patients. There were 154 cortical lesions, 45 cerebellar lesions, 21 subcortical lesions and 3 brainstem lesions. Analysis of the distribution pattern of cortical lesions showed that 88 of 154 lesions were located at the cortical border zone. Of the subcortical lesions, 13 of 21 lesions were located at the internal border zone area, within the corona radiata and centrum ovale. Only 4 lesions were located at the deep perforator territory. Infratentorial lesions were mostly located at the cerebellar hemisphere (45/48). Most lesions were tiny infarcts (<5 mm diameter); 7 of 223 lesions were >10 mm in diameter. CONCLUSIONS: Interventional-angiography-related microembolisms mostly lodge in the cerebral cortical border zone area and cerebellar hemisphere. Microembolisms to the deep perforating artery territory are distinctly rare.
Asunto(s)
Angioplastia/efectos adversos , Enfermedades de las Arterias Carótidas/terapia , Infarto Cerebral/etiología , Trastornos Cerebrovasculares/terapia , Embolia Intracraneal/etiología , Anciano , Angioplastia/instrumentación , Enfermedades de las Arterias Carótidas/diagnóstico , Infarto Cerebral/diagnóstico , Trastornos Cerebrovasculares/diagnóstico , Distribución de Chi-Cuadrado , Imagen de Difusión por Resonancia Magnética , Femenino , Humanos , Embolia Intracraneal/diagnóstico , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , República de Corea , Estudios Retrospectivos , Stents , Factores de TiempoRESUMEN
Although progressive hemiparesis occurs frequently in acute ischemic stroke, the topography and mechanisms associated with progressive motor deficit (PMD) remain unclear. The aim of this study was to identify the differences in PMD according to lesion location and the presumed underlying pathogenesis in patients with lacunar motor syndrome. Consecutive patients experiencing acute lacunar motor syndrome within 24 hours of stroke onset were included. Topographic patterns, risk factors, and presumed stroke mechanisms were compared between patients with PMD and those without PMD. Of the 168 patients in the study group, 47 (28.0%) had PMD. Baseline National Institutes of Health Stroke Scale score (P = .034) and female sex (P = .005) were associated with PMD on univariate analysis. Deep perforating artery infarct was more frequently associated with PMD (35.8%) compared with large artery disease (27.3%) and cardioembolism (5.3%). Multiple logistic analysis found that deep perforating artery infarct was independently associated with PMD (odds ratio, 2.87; 95% confidence interval, 1.26-6.5; P = .012). Deep perforating artery infarct is the major cause of PMD. In patients with lacunar syndrome, the pattern of PMD varies according to the location and etiology of stroke.
Asunto(s)
Arterias Cerebrales/patología , Actividad Motora , Paresia/etiología , Accidente Cerebrovascular/clasificación , Adulto , Anciano , Anciano de 80 o más Años , Distribución de Chi-Cuadrado , Imagen de Difusión por Resonancia Magnética , Progresión de la Enfermedad , Femenino , Humanos , Modelos Logísticos , Angiografía por Resonancia Magnética , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Paresia/patología , Paresia/fisiopatología , Sistema de Registros , República de Corea , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Índice de Severidad de la Enfermedad , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/patología , Accidente Cerebrovascular/fisiopatología , Factores de TiempoRESUMEN
Piriformis syndrome (PS) is a rare condition characterized by pain and paresthesia of the buttock, often radiating to the posterior thigh. A patient with sciatica that was clinically suspicious for PS, underwent diagnostic work-up. A diagnosis of diffuse large B-cell lymphoma with neurolymphomatosis (NL) was made. To our knowledge, this is the first report of NL presenting as PS. NL is a possible cause of secondary PS.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Linfoma de Células B Grandes Difuso/complicaciones , Linfoma de Células B Grandes Difuso/diagnóstico , Síndrome del Músculo Piriforme/etiología , Ciática/etiología , Anciano , Anticuerpos Monoclonales/administración & dosificación , Anticuerpos Monoclonales de Origen Murino , Ciclofosfamida/administración & dosificación , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Humanos , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Imagen por Resonancia Magnética , Masculino , Conducción Nerviosa/fisiología , Rituximab , Resultado del Tratamiento , Vincristina/administración & dosificaciónRESUMEN
BACKGROUND AND AIMS: Leukoaraiosis (LA) is associated with aging and vascular risk factors, and is a risk factor of intracerebral hemorrhage (ICH) after treatment with warfarin or thrombolytic treatment for ischemic stroke. In this study, we sought to examine whether LA is a predictor of outcome after spontaneous ICH. METHODS: We retrospectively analyzed 238 consecutive patients with spontaneous supratentorial ICH identified by a database search. Patients were divided into two groups according to neurological outcome at 90 days: patients with good outcomes (Glasgow Outcome Scale ≥4) and patients with poor outcomes. Demographic features, ICH characteristics, and LA severity as assessed by van Swieten score on brain CT were compared between the two groups. RESULTS: Overall, 105 (44.1%) of the patients analyzed had poor outcomes. In univariate analysis, LA severity, ICH volume on initial brain CT, initial Glasgow Coma Scale (GCS), presence of intraventricular hemorrhage (IVH), old age, surgical treatment, and higher admission serum glucose level were associated with poor outcome. Multiple logistic regression analysis showed that severity of LA, initial GCS score, hematoma volume, presence of IVH, and surgical treatment were independent predictors of poor outcome. CONCLUSION: LA is an independent predictor of poor neurological outcome in patients with spontaneous supratentorial ICH.