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1.
Neuromuscul Disord ; 33(9): 74-80, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37652755

RESUMEN

Anti-mitochondrial M2 antibody (AMA-M2)-positive myositis is an idiopathic inflammatory myopathy (IIM). Of all patients with myositis, 2.5-19.5% have AMA-M2 antibodies. However, the detailed distribution of muscles affected in AMA-positive myositis is unknown. Therefore, we examined lower muscle magnetic resonance imaging (MRI) findings of patients with AMA-positive myositis. Among the 63 patients with IIM at our institute, 5 (7.9%) were positive for AMA-M2 antibodies. However, one was also positive for anti-Jo1 antibodies; therefore, four patients were finally participated in this study. All patients had high-intensity MRI signals in the proximal muscles, including the gluteus maximus and iliopsoas muscles, and in the thigh muscles, including the vastus lateralis, vastus medialis, adductor magnus, and semimembranosus muscles. Lower leg muscles were relatively spared. Fascial edema was observed in all patients and was also present in the lower leg muscles. Subcutaneous edema was observed, particularly in the proximal portion of the lower limbs. In AMA-positive myositis, proximal muscles, including the gluteus maximus, vastus lateralis, adductor magnus, and the semimembranosus, were markedly affected, while the lower leg muscles were relatively preserved. Additionally, fascial edema was evident even in lower leg muscles. Therefore, muscle MRI can be a useful diagnostic aid for AMA-positive myositis.


Asunto(s)
Extremidad Inferior , Miositis , Humanos , Extremidad Inferior/diagnóstico por imagen , Miositis/diagnóstico por imagen , Pierna , Músculo Cuádriceps , Anticuerpos , Imagen por Resonancia Magnética
2.
Intern Med ; 61(18): 2793-2796, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36104177

RESUMEN

We herein report a 61-year-old woman who was genetically diagnosed with spinocerebellar ataxia type 31 whose symptoms were modified by anti-amino terminal of alpha-enolase (NAE) antibodies, known as a biomarker of Hashimoto's encephalopathy (HE), and ultimately responded to immunotherapy. The relative titers of anti-NAE antibodies increased when her cerebellar ataxia showed acute deterioration and decreased after immunotherapy. This is the first report of cerebellar ataxia associated with genetic spinocerebellar ataxia with concomitant cerebellar type HE. Physicians should be mindful of measuring anti-NAE antibodies to prevent overlooking patients with genetic spinocerebellar ataxia with treatable simultaneous ataxic diseases.


Asunto(s)
Ataxia Cerebelosa , Ataxias Espinocerebelosas , Autoanticuerpos , Ataxia Cerebelosa/diagnóstico , Encefalitis , Femenino , Enfermedad de Hashimoto , Humanos , Persona de Mediana Edad , Fosfopiruvato Hidratasa
3.
Drug Discov Ther ; 16(1): 37-42, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35264473

RESUMEN

Persistence of urinary incontinence (UI) after robot-assisted radical prostatectomy (RARP) is a bothersome problem because of its negative effect on the patient's quality of life (QOL). This study aimed to evaluate the effect of transperineal ultrasound (TPUS)-guided pelvic floor muscle training (PFMT) on prolonged UI after RARP. Thirty men with stress UI persisting for > 1 year after RARP underwent biofeedback PFMT using TPUS once every 2-3 weeks for 3 months. The frequency and duration of sustaining pelvic floor muscle (PFM) contractions were assessed using ultrasound imaging. The severity of UI and UI-related QOL were evaluated using a 24-hour pad test and the incontinence quality of life (I-QOL) questionnaire. Twenty-four men (mean age, 72.2 years) completed the TPUS-guided PFMT. The mean duration from RARP to PFMT was 1,228.9 days. The mean cumulative session and the total duration of TPUS-guided PFMT were 4.6 times and 73.3 days, respectively. Compared with the data before TPUS-guided PFMT, the frequency of PFM contractions and duration of sustaining contraction significantly improved after TPUS-guided PFMT (p < 0.05). Additionally, the total amount of urinary leakage after TPUS-guided PFMT was reduced significantly (248.6 ± 280.6 g vs. 397.0 ± 427.0 g, p = 0.024). The I-QOL score was significantly increased after TPUS-guided PFMT (72.1 ± 16.8 vs. 61.0 ± 19.0, p < 0.001). TPUS-guided PFMT may be effective in improving prolonged UI occurring > 1 year after RARP.


Asunto(s)
Robótica , Incontinencia Urinaria , Anciano , Terapia por Ejercicio/métodos , Humanos , Masculino , Diafragma Pélvico/diagnóstico por imagen , Prostatectomía/efectos adversos , Calidad de Vida , Resultado del Tratamiento , Ultrasonografía , Ultrasonografía Intervencional/efectos adversos , Incontinencia Urinaria/diagnóstico por imagen , Incontinencia Urinaria/etiología , Incontinencia Urinaria/terapia
4.
Prog Rehabil Med ; 7: 20220064, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36591556

RESUMEN

Background: To prevent aspiration, patients with irreversible dysphagia may undergo surgeries that separate the esophagus and trachea. Such interventions result in loss of vocal function and require alternative communication methods. We report a patient with amyotrophic lateral sclerosis (ALS) who used esophageal speech after receiving a central-part laryngectomy (CPL) to prevent aspiration. Case: A 64-year-old woman with ALS was admitted to our hospital. The patient maintained good cognitive and oral function and presented with mild dysarthria and dysphagia. Faced with rapidly worsening respiratory distress, saliva aspiration, and excessive sputum, she underwent a tracheostomy on the premise of invasive ventilation. Subsequently, the patient began using a voice-generating application for communication. Given the patient's sincere hope to prevent aspiration and aspiration pneumonia, achieve safe oral intake, and decrease caregiver burden for frequent suctioning, the patient underwent a CPL. Following surgery, belching was observed during meals, and the patient could phonate when she belched. This finding led to four speech therapy sessions to practice esophageal speech, allowing the patient to use the pseudo-speech technique for short conversations. Removal of the entire cricoid cartilage in the CPL decreases the upper esophageal sphincter (UES) pressure, thereby allowing air to easily pass through the UES. Therefore, the patient could use the air as a sound source for esophageal speech without extensive training. Discussion: Esophageal speech may be an alternative to oral communication in patients undergoing CPL. Further research is warranted to generalize these findings to patients undergoing CPL.

5.
Brain Nerve ; 73(5): 544-551, 2021 May.
Artículo en Japonés | MEDLINE | ID: mdl-34006687

RESUMEN

Hashimoto encephalopathy (HE) has been recognized as a new clinical disease based on an autoimmune mechanism associated with Hashimoto's thyroiditis. HE is successfully treated with steroids. In 2005, we discovered serum autoantibodies against the NH2-terminal of α-enolase (NAE) as a highly specific diagnostic biomarker for HE. We analyzed the serum anti-NAE autoantibodies and clinical features in many cases of HE from institutions in Japan and other countries. Approximately half of the patients with HE had anti-NAE antibodies. In our study, HE was widely distributed in patients aged 50-60 years. Most patients with HE were in euthyroid states, and all patients had anti-thyroid antibodies. The common neuropsychiatric features of these patients were consciousness disturbance and psychosis, followed by cognitive dysfunction, involuntary movements, seizures, and ataxia. Abnormalities in EEG and decreased cerebral blood flow on the brain SPECT were common findings. In contrast, abnormalities on the brain MRI were rare except for diffuse subcortical lesions and limbic lesions. Patients with HE had varied clinical phenotypes, including acute encephalopathy, chronic psychiatric form, and other particular clinical presentations, such as limbic encephalitis, progressive cerebellar ataxia, and Creutzfeldt-Jakob disease (CJD)-mimic form. The cerebellar ataxic form of HE clinically mimics spinocerebellar degeneration (SCD) and is characterized by the absence of nystagmus, absent or mild cerebellar atrophy, and lazy background activities on electroencephalography (EEG). Taken together, these features should indicate the possibility of encephalopathy associated with thyroid disorders.


Asunto(s)
Encefalopatías , Encefalitis , Enfermedad de Hashimoto , Autoanticuerpos , Encefalopatías/tratamiento farmacológico , Encefalitis/diagnóstico , Enfermedad de Hashimoto/diagnóstico , Humanos , Japón , Persona de Mediana Edad
6.
J Neurol ; 268(11): 4291-4295, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-33856546

RESUMEN

BACKGROUND: Hashimoto's encephalopathy with serum anti-NH2-terminal of α-enolase (NAE) antibodies occasionally displays clinical symptoms such as cerebellar ataxia and parkinsonism. We studied the frequency of anti-NAE antibodies in patients with Parkinson-plus syndrome. METHODS: We examined the positive rates of anti-NAE antibodies in 47 patients with multiple system atrophy (MSA), 29 patients with Parkinson's disease (PD), eight patients with corticobasal syndrome (CBS), and 18 patients with progressive supranuclear palsy (PSP) using conventional immunoblot analysis. RESULTS: Positive anti-NAE antibody rates of 31.9%, 10.3%, 50.0%, and 11.1% were reported in the MSA, PD, CBS, and PSP patients, respectively. The duration from onset to a wheelchair-bound state in seropositive MSA patients tended to be shorter than that in seronegative MSA patients. CONCLUSIONS: Anti-NAE antibodies are detected in some patients clinically diagnosed with MSA and CBS. Although its pathophysiological significance remains uncertain, serum anti-NAE antibodies might represent a prognostic marker in the clinical course of MSA.


Asunto(s)
Atrofia de Múltiples Sistemas , Enfermedad de Parkinson , Parálisis Supranuclear Progresiva , Humanos , Atrofia de Múltiples Sistemas/epidemiología , Enfermedad de Parkinson/epidemiología , Fosfopiruvato Hidratasa , Prevalencia , Parálisis Supranuclear Progresiva/epidemiología
7.
Nutrients ; 12(10)2020 Oct 14.
Artículo en Inglés | MEDLINE | ID: mdl-33066591

RESUMEN

Although folate deficiency was reported to be associated with hyperhomocysteinemia, influence of folate supplementation on cognition remains controversial. Therefore, we explored the effects of folate supplementation on the cognition and Homocysteine (Hcy) level in relatively short periods in patients with folate deficiency and cognitive impairment. Enrolled 45 patients (mean age of 79.7 ± 7.9 years old) with folate deficiency (<3.6 ng/mL) with cognitive impairment underwent Mini-Mental State Examination (MMSE), and laboratory examinations, including folate, vitamin B12, and Hcy. The degree of hippocampal atrophy in MRI was estimated using a voxel-based specific regional analysis system for Alzheimer's disease (VSRAD). Patients were administrated folate (5 mg/day), then Hcy, and MMSE score were re-examined after 28 to 63 days. Mean Hcy significantly decreased from 25.0 ± 18.0 to 11.0 ± 4.3 nmol/mL (p < 0.001). Average MMSE scores also significantly changed from 20.1 ± 4.7 to 22.2 ± 4.3 (p < 0.001). The degree of change in the MMSE score and basic Hcy or Hcy change was significantly positively correlated, while degree of hippocampal atrophy in MRI did not. Although several factors should be taken into account, folate supplementation ameliorated cognitive impairment, at least for a short period, in patients with folate deficiency.


Asunto(s)
Cognición , Disfunción Cognitiva/sangre , Disfunción Cognitiva/psicología , Suplementos Dietéticos , Deficiencia de Ácido Fólico/psicología , Ácido Fólico/administración & dosificación , Ácido Fólico/farmacología , Homocisteína/sangre , Anciano , Anciano de 80 o más Años , Atrofia , Disfunción Cognitiva/diagnóstico , Disfunción Cognitiva/tratamiento farmacológico , Femenino , Deficiencia de Ácido Fólico/sangre , Deficiencia de Ácido Fólico/dietoterapia , Hipocampo/patología , Humanos , Masculino , Pruebas de Estado Mental y Demencia , Factores de Tiempo , Resultado del Tratamiento
8.
J Neurol Sci ; 406: 116453, 2019 Nov 15.
Artículo en Inglés | MEDLINE | ID: mdl-31525528

RESUMEN

PURPOSE: Hashimoto encephalopathy (HE) is an autoimmune-mediated encephalopathy associated with anti-thyroid antibodies. We previously discovered serum autoantibodies against the NH2-terminal of α-enolase (NAE), which serve as a specific diagnostic biomarker for HE and may be involved in the autoimmune pathophysiology of HE, including vasculitis. Although the common findings of brain magnetic resonance imaging (MRI) in HE have been recognized as normal or non-specific white matter lesions, serial MRI changes have been less well studied. The aim of this study was to clarify detailed and longitudinal MRI changes in HE associated with anti-NAE antibodies. METHODS: We investigated serial brain MR images in 12 Japanese patients with HE who had serum anti-NAE antibodies. RESULTS: Brain MRI showed diffuse white matter abnormalities and/or multiple small subcortical lesions in 10 patients. These lesions were apparently non-specific; however, in 7 of these patients we observed expanding and diminishing white matter lesions, emerging subcortical high-intensity spots on diffusion-weighted images, or reversible limbic lesions, which worsened at relapse and improved after recovery following immunotherapies. CONCLUSION: MRI lesions that fluctuate according to the disease condition were frequently observed in HE patients with anti-NAE antibodies, which suggests that these fluctuation may be associated with the autoimmune pathophysiology of HE.


Asunto(s)
Autoanticuerpos/sangre , Encéfalo/diagnóstico por imagen , Encefalitis/sangre , Encefalitis/diagnóstico por imagen , Enfermedad de Hashimoto/sangre , Enfermedad de Hashimoto/diagnóstico por imagen , Imagen por Resonancia Magnética/tendencias , Fosfopiruvato Hidratasa/sangre , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad
9.
Neurourol Urodyn ; 38(4): 1067-1075, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30995359

RESUMEN

AIMS: To clarify longitudinal change of lower urinary tract symptoms (LUTS) and various types of urinary incontinence following robot-assisted radical prostatectomy (RARP) using validated questionnaires. MATERIALS AND METHODS: The core lower urinary tract symptom score (CLSS) and the International Consultation on Incontinence Questionnaire (ICIQ)-Short Form (SF) questionnaires were administered to 607 consecutive, treatment-naïve men receiving RARP before and after surgery. The time course of comprehensive LUTS and various types of urinary incontinence, including stress-, urgency-, and urinary incontinence with no obvious reason, were evaluated. Continence recovery rates were compared for the different types of incontinence using Cox hazard regression analysis. RESULTS: After surgery, stress urinary incontinence (SUI) was reported most frequently (32% of cases) as the chief complaint with the most impact on daily life, as assessed by the CLSS questionnaire, followed by urgency urinary incontinence (UUI; 27% of cases). The rates of continence recovery differed among the different types of urinary incontinence, such as after urinating, when dressed, when asleep, when physically active or exercising, when coughing or sneezing, before reaching the toilet, and for no obvious reason. Incontinence for no obvious reason at 1 month after RARP was a strongest prognostic factor of delayed continence recovery (hazard ratio, 0.61; P < 0.0001), whereas patients reporting SUI and UUI gradually regained continence. CONCLUSIONS: Further time course on continent recovery after RARP would be more precisely predictable based on the incontinence status at one month postoperatively. Especially, incontinence with no obvious reason would be a significant factor for delayed recovery.


Asunto(s)
Síntomas del Sistema Urinario Inferior/etiología , Próstata/cirugía , Prostatectomía , Neoplasias de la Próstata/cirugía , Procedimientos Quirúrgicos Robotizados/efectos adversos , Incontinencia Urinaria/etiología , Anciano , Anciano de 80 o más Años , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Periodo Posoperatorio , Encuestas y Cuestionarios , Incontinencia Urinaria/diagnóstico , Micción
10.
Neurourol Urodyn ; 38(1): 158-164, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30375062

RESUMEN

AIMS: The efficacy of perioperative pelvic floor muscle training (PFMT) for continence recovery after robot-assisted radical prostatectomy (RARP) remains unclear. Visualization of the bladder neck and urethra using transperineal ultrasound (US) may promote self-recognition of urethral closure during PFM contraction. This study aimed to examine whether transperineal US-guided PFMT promotes early recovery of post-RARP incontinence. METHODS: This prospective cohort study included 116 men undergoing RARP. All men were offered to undergo transperineal US-guided PFMT, and 36 men agreed. The protocol consisted of biofeedback PFMT using transperineal US before RARP and 1-month after RARP with verbal instruction of PFMT immediately after urethral catheter removal. The remaining 80 patients received verbal instruction for PFMT alone. Continence recovery was defined as the number of days requiring a small pad (20 g) per day by self-report. RESULTS: No differences were observed in demographic or peri-operative parameters between the two groups except the longer operative time in the US-guided PFMT group. The mean time until continence recovery was significantly shorter in the US-guided PFMT group (75.6 ± 100.0 days) than in the verbal-PFMT group (121.8 ± 132.0 days, P = 0.037). Continence recovery rates within 30 days were 52.8% (19/36) and 35.4% (28/80) in the US-guided PFMT and verbal-PFMT groups, respectively (P = 0.081). US-guided PFMT was associated with better postoperative continence status (adjusted hazard ratio [95% confidence interval]: 0.550 [0.336-0.900], P = 0.017). CONCLUSIONS: The results showed that transperineal US-guided PFMT perioperatively was associated with early recovery of urinary continence after RARP.


Asunto(s)
Terapia por Ejercicio/métodos , Diafragma Pélvico/diagnóstico por imagen , Complicaciones Posoperatorias/terapia , Prostatectomía/efectos adversos , Procedimientos Quirúrgicos Robotizados/efectos adversos , Incontinencia Urinaria/etiología , Incontinencia Urinaria/terapia , Anciano , Biorretroalimentación Psicológica , Estudios de Cohortes , Humanos , Masculino , Persona de Mediana Edad , Atención Perioperativa , Estudios Prospectivos , Recuperación de la Función , Resultado del Tratamiento , Ultrasonografía
11.
J Neurol Sci ; 394: 58-62, 2018 11 15.
Artículo en Inglés | MEDLINE | ID: mdl-30216759

RESUMEN

PURPOSE: The aim of this study was to evaluate the clinical utility of arterial spin labeling (ASL) magnetic resonance imaging (MRI) for the detection of cerebellar hypoperfusion in patients with spinocerebellar degeneration (SCD). METHODS: Regional cerebral blood flow (CBF) were obtained from ASL and 123I-IMP single-photon emission computed tomography (SPECT) images by volume-of-interest analysis in patients with SCD (n = 16). Regional CBF were also measured by ASL in age-matched controls (n = 19) and by SPECT in separate controls (n = 17). The cerebellar CBF values were normalized to the CBF values for the whole gray matter (nCBF) in ASL and SPECT. RESULTS: The mean cerebellar nCBF measured by ASL was lower in patients with SCD (0.70 ±â€¯0.09) than in the controls (0.91 ±â€¯0.05) (p < 0.001), which was consistent with the comparison using SPECT (0.82 ±â€¯0.05 vs. 0.98 ±â€¯0.05, p < 0.001). The cerebellar nCBF measured by ASL significantly correlated with that determined by SPECT in patients (r = 0.56, p < 0.001). CONCLUSIONS: ASL imaging showed decreased cerebellar blood flow, which correlated with that measured by SPECT, in patients with SCD. These findings suggest the clinical utility of noninvasive MRI with ASL for detecting cerebellar hypoperfusion in addition to atrophy, which would aid the diagnosis of SCD.


Asunto(s)
Cerebelo/diagnóstico por imagen , Circulación Cerebrovascular/fisiología , Imagen por Resonancia Magnética , Degeneraciones Espinocerebelosas/diagnóstico por imagen , Degeneraciones Espinocerebelosas/fisiopatología , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Marcadores de Spin , Tomografía Computarizada de Emisión de Fotón Único
12.
J Sci Food Agric ; 98(15): 5666-5676, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-29722013

RESUMEN

BACKGROUND: Use of covering cultivation to shade tea (Camellia sinensis L.) trees to produce high-quality, high-priced green tea has recently increased in Japan. Knowledge of shading effects on morphological and color traits and on chemical components of new tea shoots is important for product quality and productivity. We assessed these traits of tea shoots and their relationships under covering cultivation of various radiation intensities. RESULTS: Leaf thickness, leaf mass per area, and leaf density of new tea leaves were smaller under covering culture than under open-field culture. SPAD values and chlorophyll contents were larger under covering culture than under open culture. The derived exponential equation for estimating chlorophyll contents from SPAD values was improved by considering leaf thickness. Covering culture decreased epicatechin and epigallocatechin contents, and increased theanine and caffeine contents. Principal component analysis on shoot and leaf traits indicated that leaf mass per area, chlorophyll, epicatechin, and epigallocatechin contents were strongly associated with shading effects. CONCLUSION: The morphological traits, color traits, and chemical components of new tea shoots and leaves varied depending on radiation intensity, shoot growth, and cropping season. These findings are useful for covering cultivation with high quality and high productivity in tea gardens. © 2018 Society of Chemical Industry.


Asunto(s)
Camellia sinensis/química , Camellia sinensis/crecimiento & desarrollo , Producción de Cultivos/métodos , Cafeína/análisis , Catequina/análogos & derivados , Catequina/análisis , Clorofila/análisis , Color , Producción de Cultivos/instrumentación , Glutamatos/análisis , Japón , Fenotipo , Hojas de la Planta/química , Hojas de la Planta/crecimiento & desarrollo , Estaciones del Año , Té/química
13.
eNeurologicalSci ; 7: 15-17, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-29260019

RESUMEN

BACKGROUND: Rhabdomyolysis with influenza infection is rarely reported in adults. We report here influenza A induced rhabdomyolysis and anterior compartment syndrome (ACS). CASE REPORT: This case report describes a 43-year-old woman exhibiting influenza A induced rhabdomyolysis. High levels of creatine kinase (97,000 IU/L) and high titer of anti-influenza A virus antibody (H3N2) (320 ×) with negative anti-influenza B virus antibody were observed. T2 fat suppression muscle MRI imaging showed high-intensity signals in rectus femoris, vastus lateralis, adductor magnus, and semimembranosus (SM) muscles. The existence of ACS was suspected out. Muscle biopsy showed that fiber size variations exist without infiltration of inflammatory cells. The symptoms and muscle MRI findings of T2 fat suppression imaging was markedly improved. CONCLUSIONS: Muscle MRI T2 fat suppression imaging is a useful method to monitor influenza A induced rhabdomyolysis. We should keep in mind the possibilities of rhabdomyolysis and ACS in patients with influenza A infection presenting serious muscle pain.

14.
Medicine (Baltimore) ; 96(10): e6181, 2017 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-28272206

RESUMEN

Several types of autoantibodies have been reported in autoimmune limbic encephalitis (LE), such as antibodies against the voltage-gated potassium channel (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). We recently reported a patient with autoimmune LE and serum anti-NH2-terminal of α-enolase (NAE) antibodies, a specific diagnostic marker for Hashimoto encephalopathy (HE), who was diagnosed with HE based on the presence of antithyroid antibodies and responsiveness to immunotherapy. This case suggests that LE patients with antibodies to both the thyroid and NAE could be diagnosed with HE and respond to immunotherapy. The aim of this study was to clarify the clinicoimmunological features and efficacy of immunotherapy in LE associated with anti-NAE antibodies to determine whether the LE is a clinical subtype of HE.We examined serum anti-NAE antibodies in 78 LE patients with limbic abnormality on magnetic resonance imaging and suspected HE based on positivity for antithyroid antibodies. Nineteen of the 78 patients had anti-NAE antibodies; however, 5 were excluded because they were double positive for antibodies to the VGKC complex including LGI1. No antibodies against the N-methyl-D-aspartate receptor (NMDAR), contactin-associated protein 2 (Caspr2), γ-aminobutyric acid-B receptor (GABABR), or α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptor (AMPAR) were detected in the 19 patients. Among the remaining 14 who were positive only for anti-NAE antibodies, the median age was 62.5 (20-83) years, 9 (64%) were women, and 8 (57%) showed acute onset, with less than 2 weeks between onset and admission. Consciousness disturbance (71%) and memory disturbance (64%) were frequently observed, followed by psychiatric symptoms (50%) and seizures (43%). The frequency of these symptoms significantly differed between the acute- and subacute-onset groups. Abnormalities in cerebrospinal fluid and electroencephalogram were commonly observed (92% for both). Tumors were not identified in any cases. All patients responded to immunotherapy or spontaneously remitted, thereby fulfilling the criteria of HE.This study demonstrated that LE associated with anti-NAE antibodies is a nonparaneoplastic LE and various limbic symptoms that depend on the onset type. Favorable therapeutic efficacy suggests that this LE can be considered a clinical subtype of HE and that anti-NAE antibodies may be a promising indicator of the need for immunotherapy.


Asunto(s)
Autoanticuerpos , Encefalitis/inmunología , Enfermedad de Hashimoto/inmunología , Encefalitis Límbica/inmunología , Fosfopiruvato Hidratasa/inmunología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven
15.
Rinsho Shinkeigaku ; 57(3): 118-123, 2017 03 28.
Artículo en Japonés | MEDLINE | ID: mdl-28228619

RESUMEN

A 49-year-old woman presented with progressive muscle weakness of the limbs and dysphagia. Her past and family medical history were unremarkable and she did not take statins or any other medications. Laboratory tests showed that serum levels of creatine kinase were elevated (13,565 IU/l) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies were detected in the serum. Other autoantibodies to the nuclear (ANA), RNP, aminoacyl-tRNA synthetases (ARS), and signal recognition particle (SRP) were negative. Pathological analysis of the left biceps muscle revealed minimal lymphocytic infiltration into the muscle fibers together with many necrotic and regenerated fibers, which corresponded to necrotizing myopathy. Abdominal CT and upper gastrointestinal endoscopy showed an advanced gastric cancer with lymph node metastasis. The patient was subsequently diagnosed with anti-HMGCR antibody-positive paraneoplastic necrotizing myopathy associated with advanced gastric cancer. The patient underwent radical surgery to remove the cancer and was initially treated with oral prednisolone and intravenous methylprednisolone pulse therapy; however, her symptoms worsened and she became bedridden. After an additional treatment with intravenous immunoglobulin (IVIg), she showed noticeable improvements in muscle strength and dysphagia and became ambulatory. This case and recent case-series studies suggest that anti-HMGCR antibody-positive necrotizing myopathy may be included in paraneoplastic syndrome and that physicians should screen for malignant tumors in patients with anti-HMGCR antibody-positive necrotizing myopathy. Moreover, IVIg can be a useful therapy in patients with anti-HMGCR antibody-positive paraneoplastic necrotizing myopathy who show refractoriness to tumor resection and corticosteroid therapies.


Asunto(s)
Autoanticuerpos/sangre , Hidroximetilglutaril-CoA Reductasas/inmunología , Inmunoglobulinas Intravenosas/administración & dosificación , Enfermedades Musculares/etiología , Enfermedades Musculares/terapia , Síndromes Paraneoplásicos/etiología , Síndromes Paraneoplásicos/terapia , Neoplasias Gástricas/complicaciones , Biomarcadores/sangre , Femenino , Humanos , Persona de Mediana Edad , Enfermedades Musculares/diagnóstico , Enfermedades Musculares/inmunología , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/inmunología , Neoplasias Gástricas/cirugía , Resultado del Tratamiento
16.
Biochem Biophys Res Commun ; 478(3): 1035-42, 2016 09 23.
Artículo en Inglés | MEDLINE | ID: mdl-27543203

RESUMEN

Tau aggregation and amyloid ß protein (Aß) deposition are the main causes of Alzheimer's disease (AD). Peroxisome proliferator-activated receptor γ (PPARγ) activation modulates Aß production. To test whether the PPARγ agonist pioglitazone (PIO) is also effective in preventing tau aggregation in AD, we used a cellular model in which wild-type tau protein (4R0N) is overexpressed (M1C cells) (Hamano et al., 2012) as well as primary neuronal cultures. PIO reduced both phosphorylated and total tau levels, and inactivated glycogen synthase kinase 3ß, a major tau kinase, associated with activation of Akt. In addition, PIO decreased cleaved caspase3 and C-terminal truncated tau species by caspase, which is expected to decrease tau aggregation. A fractionation study showed that PIO reduced high molecular-weight (120 kDa), oligomeric tau species in Tris Insoluble, sarkosyl-soluble fractions. Tau decrease was reversed by adding GW9662, a PPARγ antagonist. Together, our current results support the idea that PPARγ agonists may be useful therapeutic agents for AD.


Asunto(s)
Multimerización de Proteína/efectos de los fármacos , Tiazolidinedionas/farmacología , Proteínas tau/metabolismo , Animales , Muerte Celular/efectos de los fármacos , Línea Celular Tumoral , Supervivencia Celular/efectos de los fármacos , Células Cultivadas , Humanos , Ratones Endogámicos ICR , Neuronas/citología , Neuronas/efectos de los fármacos , Neuronas/metabolismo , Neuroprotección/efectos de los fármacos , PPAR gamma/antagonistas & inhibidores , PPAR gamma/metabolismo , Fosforilación/efectos de los fármacos , Pioglitazona
17.
Eur Neurol ; 76(1-2): 87-94, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27466802

RESUMEN

BACKGROUND: The purpose of this study was to explore the relation between muscle MRI findings and weakness of the upper extremity muscles in patients with myotonic dystrophy type 1 (DM1). METHODS: Nineteen DM1 patients from 15 families were enrolled in this study. Muscle weakness was evaluated using the modified Medical Research Council scale. Subjects also underwent a genetic study and muscle MRI of the upper extremities. RESULTS: In patients with DM1, the flexor digitorum profundus (FDP), flexor pollicis longus, flexor digitorum superficialis (FDS), extensor pollicis, abductor pollicis longus (APL), lateral head of triceps brachii and infraspinatus (INF) muscles were frequently and severely affected. Muscle strength was significantly correlated with the severity of muscle MRI findings in the FDP, short head of biceps brachii (SBB), and medial head of triceps brachii muscles. Disease duration was correlated significantly with MRI findings in the FDP, FDS, long head of biceps brachii, INF, APL, and SBB muscles. Unexpectedly, the degree of trinucleotide expansion of myotonin protein kinase was not correlated with muscle MRI findings. CONCLUSION: Muscle MRI of the upper extremity is useful to detect affected muscles in DM1 patients.


Asunto(s)
Imagen por Resonancia Magnética , Debilidad Muscular/diagnóstico por imagen , Músculo Esquelético/diagnóstico por imagen , Distrofia Miotónica/diagnóstico por imagen , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Extremidad Superior/diagnóstico por imagen
18.
Brain Nerve ; 68(4): 399-405, 2016 Apr.
Artículo en Japonés | MEDLINE | ID: mdl-27056858

RESUMEN

Endocrine diseases affecting various organs, such as the pituitary gland, the thyroid, the parathyroid, the adrenal glands and the pancreas, occasionally cause dementia. While Alzheimer's disease (AD) is the main cause of dementia in the elderly and is untreatable, dementia caused by endocrine diseases is treatable in most cases. However, patients with dementia associated with endocrine diseases show memory impairments similar to those found in AD, often leading to misdiagnoses. Patients with endocrine diseases often present with other characteristic systemic and neuropsychiatric symptoms caused by altered hormone levels. Such neuropsychiatric symptoms include involuntary movements, depression, seizures, and muscle weakness. In these cases, abnormalities in imaging and blood or urine tests are helpful in making a differential diagnosis. As delays in the diagnosis and treatment of these patients may cause irreversible brain damage, it is imperative for clinicians to carefully exclude the possibility of latent endocrine diseases when treating patients with dementia.


Asunto(s)
Demencia/complicaciones , Demencia/diagnóstico , Enfermedades del Sistema Endocrino/complicaciones , Enfermedades del Sistema Endocrino/diagnóstico , Trastornos de la Memoria/complicaciones , Demencia/terapia , Trastorno Depresivo/complicaciones , Trastorno Depresivo/diagnóstico , Trastorno Depresivo/terapia , Diagnóstico Diferencial , Enfermedades del Sistema Endocrino/terapia , Humanos , Trastornos de la Memoria/diagnóstico , Trastornos de la Memoria/terapia , Pruebas Neuropsicológicas
19.
Biosci Biotechnol Biochem ; 80(2): 360-2, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-26364623

RESUMEN

To examine the possible benefits of tea flavonols, we compared anti-atherogenic effects between common and flavonol-rich tea cultivars. The tea infusion made from a flavonol-rich cultivar, but not a common cultivar, significantly decreased the plasma oxidized low-density lipoprotein level in mice fed a high-cholesterol diet. The result suggests that tea flavonols have the potential to protect against cardiovascular diseases.


Asunto(s)
Camellia sinensis/química , Colesterol en la Dieta/efectos adversos , Dieta Alta en Grasa , Flavonoles/farmacología , Hipercolesterolemia/dietoterapia , Té/química , Animales , Hipercolesterolemia/sangre , Hipercolesterolemia/etiología , Hipercolesterolemia/patología , Lipoproteínas LDL/sangre , Masculino , Ratones , Ratones Endogámicos ICR
20.
Cerebellum ; 15(2): 213-32, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25823827

RESUMEN

In the last few years, a lot of publications suggested that disabling cerebellar ataxias may develop through immune-mediated mechanisms. In this consensus paper, we discuss the clinical features of the main described immune-mediated cerebellar ataxias and address their presumed pathogenesis. Immune-mediated cerebellar ataxias include cerebellar ataxia associated with anti-GAD antibodies, the cerebellar type of Hashimoto's encephalopathy, primary autoimmune cerebellar ataxia, gluten ataxia, Miller Fisher syndrome, ataxia associated with systemic lupus erythematosus, and paraneoplastic cerebellar degeneration. Humoral mechanisms, cell-mediated immunity, inflammation, and vascular injuries contribute to the cerebellar deficits in immune-mediated cerebellar ataxias.


Asunto(s)
Ataxia Cerebelosa/fisiopatología , Cerebelo/fisiopatología , Consenso , Encefalitis/fisiopatología , Enfermedad de Hashimoto/fisiopatología , Neuroinmunomodulación/fisiología , Animales , Ataxia Cerebelosa/diagnóstico , Glútenes/metabolismo , Humanos
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