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1.
Artigo em Inglês | MEDLINE | ID: mdl-33881652

RESUMO

PURPOSE: The purpose of this study was to verify whether carotid ultrasonography (CUS) findings could be associated with the occurrence of perioperative stroke after thoracic aortic aneurysm (TAA) treatment. METHODS: Patients with TAAs who were treated by either total arch replacement or thoracic endovascular aortic repair (TEVAR) were retrospectively enrolled. Left subclavian artery (LSA) embolization and bypass surgery of the left common carotid artery (CCA) to the LSA before TEVAR were additionally performed for some patients. CUS was performed before TAA treatment to evaluate carotid atherosclerosis and flow velocities of bilateral cervical arteries. After dividing patients into those with and without perioperative stroke, their background, atherosclerotic risk factors, history of stroke, TAA location and size, treatment procedures, and CUS parameters were compared between the two groups. RESULTS: Of the 60 patients (18 women, 42 men; mean age 73.5 ± 10.2 years) with TAA, four (7.5%) developed perioperative stroke. There were no significant differences in the patients' characteristics and their TAAs between those with and without perioperative stroke. For the CUS parameters, end-diastolic velocity (EDV) of bilateral CCAs was significantly decreased in perioperative stroke patients (with vs without stroke; right: 9.2 ± 1.8 vs. 14.5 ± 4.6 cm/s, P = 0.025, left: 9.1 ± 0.3 vs. 15.0 ± 4.5 cm/s, P = 0.012), whereas the resistance index (RI) of bilateral CCAs was significantly elevated (right: 0.76 vs. 0.87, P = 0.008, left: 0.76 vs. 0.87, P < 0.001). CONCLUSIONS: Lower EDV and higher RI of bilateral CCAs were significantly associated with perioperative stroke after TAA treatment. Thus, CUS findings may help predict the occurrence of perioperative stroke.

2.
Int J Mol Sci ; 22(8)2021 Apr 13.
Artigo em Inglês | MEDLINE | ID: mdl-33924373

RESUMO

A common pathological hallmark of several neurodegenerative diseases, including amyotrophic lateral sclerosis, is cytoplasmic mislocalization and aggregation of nuclear RNA-binding protein TDP-43. Perry disease, which displays inherited atypical parkinsonism, is a type of TDP-43 proteinopathy. The causative gene DCTN1 encodes the largest subunit of the dynactin complex. Dynactin associates with the microtubule-based motor cytoplasmic dynein and is required for dynein-mediated long-distance retrograde transport. Perry disease-linked missense mutations (e.g., p.G71A) reside within the CAP-Gly domain and impair the microtubule-binding abilities of DCTN1. However, molecular mechanisms by which such DCTN1 mutations cause TDP-43 proteinopathy remain unclear. We found that DCTN1 bound to TDP-43. Biochemical analysis using a panel of truncated mutants revealed that the DCTN1 CAP-Gly-basic supradomain, dynactin domain, and C-terminal region interacted with TDP-43, preferentially through its C-terminal region. Remarkably, the p.G71A mutation affected the TDP-43-interacting ability of DCTN1. Overexpression of DCTN1G71A, the dynactin-domain fragment, or C-terminal fragment, but not the CAP-Gly-basic fragment, induced cytoplasmic mislocalization and aggregation of TDP-43, suggesting functional modularity among TDP-43-interacting domains of DCTN1. We thus identified DCTN1 as a new player in TDP-43 cytoplasmic-nuclear transport, and showed that dysregulation of DCTN1-TDP-43 interactions triggers mislocalization and aggregation of TDP-43, thus providing insights into the pathological mechanisms of Perry disease and other TDP-43 proteinopathies.

3.
Rinsho Shinkeigaku ; 61(4): 219-227, 2021 Apr 21.
Artigo em Japonês | MEDLINE | ID: mdl-33762500

RESUMO

A questionnaire survey was conducted on 8,402 members of the Japanese Neurological Society to examine the current status and countermeasures for physician burnout, and 1,261 respondents (15.0%) responded. In this paper, we report the results of a comparison between male and female physicians. There was a significant difference in working and living conditions only for married people. It was confirmed that men work under stricter conditions in terms of working hours, and that the burden on women is heavier in the division of housework. Analysis using the Japanese Burnout Scale revealed no gender differences in overall scores, but as for factors related to burnout, in addition to factors common to both men and women, factors specific to men or women were clarified.

4.
Clin Neurol Neurosurg ; 204: 106595, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33752143

RESUMO

OBJECTIVE: Young-onset stroke has a greater social impact than does stroke in older persons, indicating the importance of its prevention. Although there have been studies comparing stroke risk factors in young versus older individuals, no definition of young-onset ischemic stroke has been established. Large extracranial and intracranial atheroma, small vessel disease and atrial fibrillation have a major role in cases of stroke in the elderly, while these disorders are much less frequent in young adults. The purpose of this study was to determine the optimal cut-off point for defining young-onset ischemic stroke according to its cause. METHODS: We identified 203 patients aged 65 years or less who had been admitted to our hospital between 2010 and 2017 with ischemic stroke, and we divided them into two groups according to the causes of the stroke. We allocated patients with strokes caused by small vessel occlusion, large artery atherosclerosis, atrial fibrillation, or aortic atheroma to Group A and those with strokes of other causes to Group B which included dissection, Trousseau syndrome and cerebral sinus thrombosis. We then used receiver operating characteristics curve analysis by the above groups and by sex to determine the cut-off age for defining young-onset. RESULTS: Group A comprised 131 patients (58 ± 7 years, 92 men, 39 women) and Group B 72 (45 ± 15 years, 47 men, 25 women). Receiver operating characteristics curve analysis to differentiate Group B from Group A in all participants indicated a cut-off value of 53 years of age (area under curve: 0.78 [0.71-0.85], P < 0.001), which we therefore considered should define young-onset ischemic stroke. After dividing all patients by their sex, ROC analyses identified a cut-off for age of between 53 and 54 years for men (AUC: 0.75, 95% CI: 0.65-0.85, P < 0.001). In comparison, ≤ 48 years was the cut-off for young ischemic stroke in women (AUC: 0.83, 95% CI: 0.71-0.94, P < 0.001). CONCLUSIONS: The age of 53 years may be the optimal cut-off point for young-onset ischemic stroke. Of note, the cut-off point between young- and non-young-onset stroke was 48 years for women, whereas it was 53 years for men. It is therefore important to carefully examine and treat female patients with this sex difference in mind.

5.
J Neural Transm (Vienna) ; 128(3): 337-344, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33630140

RESUMO

The double-blind part of the COMFORT-PD (COMt-inhibitor Findings from Opicapone Repeated Treatment for Parkinson's Disease) study in Japanese levodopa-treated patients with Parkinson's disease and motor fluctuations found that both opicapone 25 and 50 mg were significantly more effective than placebo. This 52-week open-label extension study evaluated the long-term safety and efficacy of opicapone 50 mg tablets in patients who completed the double-blind part of the COMFORT-PD study. Safety was monitored via adverse events, laboratory testing, and physical, cardiovascular and neurological examinations. Efficacy was primarily assessed by change in OFF-time. Secondary efficacy measures included: ON-time, percentage of OFF/ON-time responders, other outcomes from the double-blind part. 391/437 patients were transferred to the open-label extension period and included in the safety analysis set (full analysis set, n = 387; open-label completers, n = 316). Adverse events were frequently reported (n = 338, 86.4%), but < 50% were considered drug-related (39.9%) and few were considered serious (2.6%) or led to discontinuation (2.8%). Decreased OFF-time was consistently observed over the open-label period regardless of initial randomization. Change [LSM (SE)] in OFF-time from the open-label baseline to the last visit showed a persistent effect in patients initially randomized to opicapone 25 mg [- 0.37 (0.20) h, P = 0.0689] and opicapone 50 mg [- 0.07 (0.21) h, P = 0.6913] whereas opicapone 50 mg led to a statistically significant reduction in the previous placebo group [- 1.26 (0.19) h, P < 0.05]. Once-daily opicapone 50 mg was generally well tolerated and consistently reduced OFF-time over 52 weeks in Japanese levodopa-treated patients with motor fluctuations.Trial registration JapicCTI-153112; date of registration: December 25, 2015.

6.
Brain Nerve ; 73(2): 179-182, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561832

RESUMO

An 80-year-old man was diagnosed with prostate cancer in April 2014 and underwent anticancer treatment. His serum prostate-specific antigen (PSA) level was abruptly increased on December 26, 2014. He was admitted to the neurological department of our hospital on January 14, 2015, because of the appearance of staggering gait and diplopia. Neurological examination revealed marked opsoclonus, limb ataxia and ataxic gait. The patient was diagnosed with paraneoplastic opsoclonus and ataxia caused by prostate cancer relapse. Steroid pulse therapy was initiated and his symptoms, including opsoclonus and ataxia, markedly improved. Although most cases of paraneoplastic opsoclonus precede the discovery of cancer, our case developed symptoms simultaneously with relapse and acute progression of prostate cancer. Paraneoplastic opsoclonus with prostate cancer is rare. Additionally, our case showed excellent response of opsoclonus to steroid therapy without treatment of the underlying disease. (Received June 1, 2020; Accepted September 18, 2020; Published February 1, 2021).


Assuntos
Ataxia Cerebelar , Transtornos da Motilidade Ocular , Neoplasias da Próstata , Idoso de 80 Anos ou mais , Ataxia/tratamento farmacológico , Ataxia/etiologia , Humanos , Masculino , Recidiva Local de Neoplasia , Neoplasias da Próstata/complicações , Neoplasias da Próstata/tratamento farmacológico
7.
Brain Nerve ; 73(2): 183-187, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561833

RESUMO

A 66-year-old woman visited our hospital complaining of shortness of breath during exertion and progressive weakness in all her limb muscles. On admission, we noted muscle weakness in her trunk and in her proximal limb muscles, although, her muscle MRI showed no remarkable findings. However, her serum CK level (2,747U/L) was above the normal range. Histopathological examination of muscle biopsy, performed from the left biceps brachii muscle, revealed immune-mediated necrotizing myopathy (IMNM). Her serum samples were negative for myositis-associated autoantibodies (MAAs), anti-SRP, and HMGCR antibodies. However, as the anti-SS-A antibody level in her serum was high (53.2U/mL), we conducted the salivary gland biopsy and the Schirmer test on her eyes. We found lymphocytes infiltration in her salivary gland tissue, and thus, she was diagnosed with primary Sjögren syndrome (SjS). We also observed necrotizing myopathy associated with the SjS. Following her treatment with oral steroids, her symptoms and CK level improved. Although, inflammatory myositis frequently occurs in association with general collagen diseases, necrotizing myopathy has rarely been observed secondary to SjS. We report here this rare case study along with the review of the relevant literature. (Received June 24, 2020; Accepted October 12, 2020; Published February 1, 2021).


Assuntos
Doenças Autoimunes , Doenças Musculares , Miosite , Síndrome de Sjogren , Idoso , Autoanticorpos , Feminino , Humanos , Doenças Musculares/etiologia , Miosite/complicações , Síndrome de Sjogren/complicações
8.
Rinsho Shinkeigaku ; 61(2): 89-102, 2021 Feb 23.
Artigo em Japonês | MEDLINE | ID: mdl-33504753

RESUMO

To identify factors associated with burnout among Japanese physician and to use them in future measures, the Japanese Society of Neurology conducted a survey of neurologists on burnout using a web-based questionnaire in October 2019. A total of 1,261 respondents, 15.0% of the 8,402 members, responded to the survey. The mean of the subscales of the Japanese Burnout Scale was 2.86/5 points for emotional exhaustion, 2.21/5 points for depersonalization, and 3.17/5 points for lack of personal accomplishment. In addition, the burnout of our country's neurologists is not related to workloads such as working hours and the number of patients in charge, but also to a decreased meaningfulness and professional accomplishment. Therefore, it is necessary to take comprehensive measures to improve these issues at the individual, hospital, academic and national levels.

9.
Parkinsonism Relat Disord ; 83: 49-53, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33476877

RESUMO

INTRODUCTION: Perry disease (Perry syndrome), a hereditary TAR DNA-binding protein 43 (TDP-43) proteinopathy, is caused by dynactin subunit 1 (DCNT1) mutations and is characterized by rapidly progressive parkinsonism accompanied by depression, apathy, unexpected weight loss, and respiratory symptoms including central hypoventilation and central sleep apnea. Meta-iodobenzylguanidine (MIBG) myocardial scintigraphy is considered a diagnostic biomarker for Lewy body disease (LBD), as denervation of cardiac sympathetic nerves is a pathological feature in LBD. However, our previous studies have reported a decreased cardiac uptake of MIBG in patients with Perry disease. In this study, we aimed to correlate the MIBG myocardial scintigraphy findings with clinical features in Perry disease. METHODS: We evaluated data obtained from a multicenter survey of patients of Japanese origin with suspected Perry disease, who visited neurology departments in Japan from January 2010 to December 2018. We screened each patient's DNA for the DCTN1 mutation using Sanger sequencing and obtained the clinical details of all patients including findings from their MIBG myocardial scintigraphy. RESULTS: We identified two novel mutations, p.G71V and p.K68E, in DCTN1 in patients from two different families. The majority of patients (7/8, 87.5%) showed a decrease in cardiac uptake (heart to mediastinum ratio) in MIBG myocardial scintigraphy. These patients commonly presented with symptoms related to autonomic dysfunction: constipation, fecal incontinence, urinary disturbance, and orthostatic hypotension. CONCLUSIONS: MIBG myocardial scintigraphy may be a useful biomarker of autonomic dysfunction in Perry disease.

10.
Ultrasound Med Biol ; 47(4): 928-931, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33408050

RESUMO

The iPlaque software package can use integrated backscatter (IB) values of carotid plaque to extract information on tissue composition. The aim of this study was to evaluate the association between the plaque histologic classification and IB values evaluated by iPlaque. In 49 patients undergoing carotid endarterectomy, IB values of whole carotid plaque were measured using iPlaque from the long-axis ultrasonographic image. Histologic findings of resected plaques were defined using the classification of the American Heart Association. The average IB values were statistically compared with the classification. Plaque samples from 49 patients were categorized into V, VI and VII, (13, 32 and 4 cases, respectively). Both the average and standard deviation of the IB values in each plaque sample significantly differed among the three classifications (p = 0.001). The IB of carotid plaque obtained by iPlaque analysis was associated with its histologic characteristics.

11.
mSystems ; 5(6)2020 Dec 08.
Artigo em Inglês | MEDLINE | ID: mdl-33293403

RESUMO

Gut dysbiosis has been repeatedly reported in Parkinson's disease (PD) but only once in idiopathic rapid-eye-movement sleep behavior disorder (iRBD) from Germany. Abnormal aggregation of α-synuclein fibrils causing PD possibly starts from the intestine, although this is still currently under debate. iRBD patients frequently develop PD. Early-stage gut dysbiosis that is causally associated with PD is thus expected to be observed in iRBD. We analyzed gut microbiota in 26 iRBD patients and 137 controls by 16S rRNA sequencing (16S rRNA-seq). Our iRBD data set was meta-analyzed with the German iRBD data set and was compared with gut microbiota in 223 PD patients. Unsupervised clustering of gut microbiota by LIGER, a topic model-based tool for single-cell RNA sequencing (RNA-seq) analysis, revealed four enterotypes in controls, iRBD, and PD. Short-chain fatty acid (SCFA)-producing bacteria were conserved in an enterotype observed in controls and iRBD, whereas they were less conserved in enterotypes observed in PD. Genus Akkermansia and family Akkermansiaceae were consistently increased in both iRBD in two countries and PD in five countries. Short-chain fatty acid (SCFA)-producing bacteria were not significantly decreased in iRBD in two countries. In contrast, we previously reported that recognized or putative SCFA-producing genera Faecalibacterium, Roseburia, and Lachnospiraceae ND3007 group were consistently decreased in PD in five countries. In α-synucleinopathy, increase of mucin-layer-degrading genus Akkermansia is observed at the stage of iRBD, whereas decrease of SCFA-producing genera becomes obvious with development of PD.IMPORTANCE Twenty studies on gut microbiota in PD have been reported, whereas only one study has been reported on iRBD from Germany. iRBD has the highest likelihood ratio to develop PD. Our meta-analysis of iRBD in Japan and Germany revealed increased mucin-layer-degrading genus Akkermansia in iRBD. Genus Akkermansia may increase the intestinal permeability, as we previously observed in PD patients, and may make the intestinal neural plexus exposed to oxidative stress, which can lead to abnormal aggregation of prion-like α-synuclein fibrils in the intestine. In contrast to PD, SCFA-producing bacteria were not decreased in iRBD. As SCFA induces regulatory T (Treg) cells, a decrease of SCFA-producing bacteria may be a prerequisite for the development of PD. We propose that prebiotic and/or probiotic therapeutic strategies to increase the intestinal mucin layer and to increase intestinal SCFA potentially retard the development of iRBD and PD.

12.
J Neurol Sci ; 419: 117172, 2020 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-33065494

RESUMO

INTRODUCTION: Parkinson's disease (PD) is characterized by a range of classic motor symptoms and heterogeneous nonmotor symptoms that affect patients' quality of life (QoL). Studies have individually reported the effect of either motor or nonmotor symptoms on patients' QoL; however, a thorough assessment of the symptoms that have the greatest influence on QoL is limited. This JAQPAD study examined the effect of both motor and nonmotor symptoms and patient demographics on QoL in a large population of patients with PD in Japan. METHODS: All members of the Japan Parkinson's Disease Association were invited to participate in the study. Questionnaires assessing wearing-off symptoms (the 9-item Wearing-Off Questionnaire [WOQ-9]), nonmotor symptoms (Non-Motor Symptoms Questionnaire [NMSQ]) and QoL (the 8-item Parkinson's Disease Questionnaire [PDQ-8]) were included. Multiple regression analyses assessed the effect of clinical factors on the PDQ-8 Summary Index (PDQ-8 SI). Spearman rank correlation coefficient (r) estimated the correlation between each subdomain score of nine NMSQ domains and the PDQ-8 SI. RESULTS: A total of 3022 patients were included in the analysis. The PDQ-8 SI score correlated with off-time, age, duration of PD, work status, and the NMSQ total score and subdomain scores. Memory problems correlated most strongly with the PDQ-8 SI score (r = 0.4419), followed by mood (r = 0.4387) and digestive problems (r = 0.4341; p < 0.0001). CONCLUSIONS: Physicians tend to focus on motor symptoms, while nonmotor symptoms often go under-recognized in clinical practice. This JAQPAD study highlights the importance of recognition and management of both motor and nonmotor symptoms, which together significantly affect patient QoL.

13.
Mov Disord ; 2020 Oct 19.
Artigo em Inglês | MEDLINE | ID: mdl-33073879

RESUMO

OBJECTIVES: This placebo-controlled, randomized study evaluated the efficacy and safety of opicapone 25-mg and 50-mg tablets in Japanese levodopa-treated patients with Parkinson's disease and motor fluctuations. METHODS: Japanese adults (n = 437, age 39-83 years) with Parkinson's disease (United Kingdom Parkinson's Disease Society criteria) received opicapone 25-mg (n = 145), opicapone 50-mg (n = 145), or placebo (n = 147) tablets over the double-blind treatment period (14-15 weeks). The primary efficacy assessment was change in OFF-time; secondary efficacy assessments included OFF/ON-time responders (≥1 hour change from baseline), total ON-time, ON-time with and without troublesome dyskinesia, and Unified Parkinson's Disease Rating Scale. RESULTS: The least squares mean (standard error) change in OFF-time from baseline to the last visit was -0.42 (0.21) hour for the placebo group, -1.16 (0.22) hour for the opicapone 25 mg group, and -1.04 (0.21) hour for the opicapone 50 mg group. The percentage of ON-time responders, changes in total ON-time/ON-time without troublesome dyskinesia, and Unified Parkinson's Disease Rating Scale II (at OFF) all showed statistically significant improvements versus placebo for both opicapone tablet doses (P < 0.05). Unified Parkinson's Disease Rating Scale III (at ON) was improved versus placebo in patients who received opicapone 50 mg (P < 0.05). Adverse events were more common in patients treated with opicapone 25 mg (60.0%) or opicapone 50 mg (54.5%) versus placebo (48.3%). The most commonly reported adverse event was dyskinesia (placebo, 2.7%; opicapone 25 mg, 9.0%; opicapone 50 mg, 12.4%). CONCLUSIONS: In Japanese patients, both opicapone 25 and 50 mg were significantly more effective than placebo with no dose-dependent difference in efficacy, and both doses were well tolerated. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC. on behalf of International Parkinson and Movement Disorder Society.

14.
Clin Neurol Neurosurg ; 199: 106266, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33059317

RESUMO

OBJECTIVE: Ways of introducing a rotigotine patch to Parkinson disease (PD) patients include initial induction for dopamine agonist (DA)-free patients and overnight switch (OS), cross-titration (CT), and add-on (AO) for patients already taking oral DAs. We investigated whether or not the introductions method affects the continuation rate of rotigotine patch. METHODS: The subjects were 188 PD patients who started using a rotigotine patch at the Department of Neurology, Fukuoka University Hospital. The rate of successful continuation of rotigotine patch for one year after initiation and the reasons for discontinuation were investigated; for the patients who discontinued due to poor efficacy, the DA dose before and after the start of rotigotine patch treatment was determined. RESULTS: The 1-year continuation rates were 38.5 % (20/52) in the OS group, 61.5 % (8/13) in the CT group, 35.3 % (6/17) in the AO group, and 50.9 % (54/106) in the de novo group. The most common reason for discontinuation in all groups was skin reactions. Compared with the de novo group, only the OS group had a significantly higher discontinuation rate due to poor efficacy (3.8 % vs. 21.2 %, P <  0.001). However, in the OS group, the continuation rate in the subjects with an increased total DA dose, after rotigotine was introduced, was significantly higher than that in the subjects with a decreased total DA dose (p = 0.031). CONCLUSION: The use of a rotigotine patch with an equivalent dose should be considered when switching from oral DAs, and appropriate care should be administered for any skin reactions. The present findings suggested that not the introduction method but the use of an equivalent dose between DA formulations might affect the continuation rate of rotigotine patch.

15.
Clin Neurol Neurosurg ; 198: 106196, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32980799

RESUMO

INTRODUCTION: Patients with neurological and neuromuscular disorders (NNMD) frequently experience swallowing disorders that increase aspiration pneumonia risk and therefore require specialized diets or tube feeding. Diet type level usually is assessed by video fluoroscopic swallowing study (VFSS). To identify a simpler assessment method, we examined the association between diet type (based on the Functional Oral Intake Scale [FOIS]) diet type and maximum tongue pressure (MTP). METHODS: From 2011-2020, FOIS diet type level and MTP were assessed in a sample of 927 patients. Of these patients, 186 had Parkinson's disease (PD), 69 had Parkinson-related disease (PRD), 61 had multiple system atrophy (MSA), 42 had spinocerebellar degeneration (SCD), 147 had amyotrophic lateral sclerosis (ALS), 180 had myotonic dystrophy type 1 (DM1), and 242 had Duchenne muscular dystrophy (DMD). VFSS was conducted while patients swallowed water and foods containing barium. MTP measurements were collected the same day. Participants' diet type level was adjusted based on the VFSS, with some participants requiring multiple examinations. Relationships between diet type level and MTP were tested using univariate and Spearman rank correlation analyses. RESULTS: Mean MTP for the entire NNMD group (25.5 ± 13.1 kPa) was lower than that of healthy elderly individuals, as determined in previous reports. The highest MTP was found in the MSA group (32.2 ± 15.7 kPa) and the lowest in the DM1 group (19.1 ± 9.0 kPa). Diet type level was highest in the MSA group (5.8 ± 1.4) and lowest in the DMD group (5.2 ± 1.7). A significant correlation was observed between diet type level and MTP (R = 0.384, p < 0.001). The optimum MTP cutoff values-detected using ROC curves to predict a requirement to change to a dysphagia diet-was highest in the DMD group (29.0 kPa) and lowest in the ALS group (12.3 kPa). CONCLUSIONS: The decision to change NNMD patients to a dysphagia diet can be made based on MTP. Modifying a patient's oral diet (FOIS level ≤ 5) should be considered for those with a MTP of 10-25 kPa, with the cutoff value varying by disease.

16.
Jpn J Radiol ; 2020 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-32939741

RESUMO

PURPOSE: The Cingulate Island Sign score (CIScore) by rCBF SPECT is used in the differentiation between Dementia with Lewy bodies (DLB) and Alzheimer's disease (AD) but has some false-positive AD cases. To resolve the problem, we developed new differential diagnosing method incorporating occipital lobe and para-hippocampal rCBF. MATERIALS AND METHODS: In 27 DLB and 31 AD cases undertaken Tc-99 m-ECD SPECT, we evaluated the mean Z score in the bilateral superior, middle, inferior occipital gyri, cuneus, amygdala, hippocampus, and para-hippocampus. One criterion of DLB was defined as the case with CIScore lower than 0.27. The other criteria were the cases of following either or both two conditions were satisfied. (1) The number of occipital gyri with mean Z score higher than 1 is three or more. (2) The number of hippocampal regions with mean Z score higher than 1 is one or less. We compared the differential diagnostic ability among these four criterions. RESULTS: The diagnostic accuracy by CIscore was 69% and that of the occipital gyri analysis 84%, para-hippocampal regions analysis 76% and combined occipital gyri and para-hippocampal regions analysis 93%. CONCLUSION: The new method by combined rCBF analysis of occipital gyri and para-hippocampal regions showed best diagnostic ability in differentiating DLB from AD.

17.
J Mov Disord ; 2020 Sep 21.
Artigo em Inglês | MEDLINE | ID: mdl-32942840

RESUMO

Perry disease is a hereditary neurodegenerative disease with autosomal dominant inheritance. It is characterized by parkinsonism, psychiatric symptoms, unexpected weight loss, central hypoventilation, and transactive-response DNA-binding protein of 43kD (TDP-43) aggregation in the brain. In 2009, Perry disease was found to be caused by dynactin I gene (DCTN1), which encodes dynactin subunit p150 on chromosome 2p, in patients with the disease. The dynactin complex is a motor protein that is associated with axonal transport. Presently, at least 8 mutations and 22 families have been reported; other than the "classic" syndrome, distinct phenotypes are recognized. The neuropathology of Perry disease reveals severe degeneration in the substantia nigra and TDP-43 inclusions in the basal ganglia and brain stem. How dysfunction of the dynactin molecule is related to TDP-43 pathology in Perry disease is important to elucidate the pathological mechanism and develop new treatment.

18.
Medicine (Baltimore) ; 99(38): e22370, 2020 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-32957414

RESUMO

BACKGROUND: Dementia among the Japanese aged 65 years or over population is estimated to approach about 700 million cases by 2025, and a corresponding rapid increase in Alzheimer disease (AD) can also be expected. The ballooning number of dementia patients, including AD, is creating major medical and social challenges. At present, only 3 drugs are recognized for the treatment of mild AD, and these are only used to alleviate symptoms. Although new therapies are needed to treat mild AD, insufficient development of disease-modifying drugs is being done. METHODS/DESIGN: The aim of this exploratory, open standard, treatment-controlled, randomized allocation, multicenter trial is to determine the efficacy of the traditional Japanese Kampo medicine hachimijiogan (HJG) on the cognitive dysfunction of mild AD.Eighty-six patients with AD diagnosed according to the Diagnostic and Statistical Manual of Mental Disorders (DSM)-5 and as mild AD according to the Mini Mental State Examination (MMSE ≥21) will be included. All will already have been taking the same dose of Donepezil, Galantamine, or Rivastigmine for more than 3 months. The patients will be randomly assigned to receive additional treatment with HJG or to continue mild AD treatment without additional HJG. The primary endpoint is the change from baseline of the Alzheimer's Disease Assessment Scale-cognitive component- Japanese version (ADAS-Jcog). ADAS-Jcog is a useful index for detecting change over time that investigates memory and visuospatial cognition injury from the early stage. The secondary endpoints are the changes from baseline of the Instrumental Activity of Daily Life, Apathy scale, and Nueropsychiatric Inventory scores. In this protocol, we will examine the Geriatric depression scale and do Metabolome analysis as exploratory endpoints. The recruitment period will be from August 2019 to July 2021. DISCUSSION: This is the first trial of Kampo medicine designed to examine the efficacy of HJG for the cognitive dysfunction of patients with mild AD. TRIAL REGISTRATION: This trial was registered on the Japan Registry of Clinical trials on 2 August 2, 2019 (jRCTs 071190018).


Assuntos
Doença de Alzheimer/tratamento farmacológico , Medicamentos de Ervas Chinesas/uso terapêutico , Idoso , Cognição/efeitos dos fármacos , Feminino , Humanos , Masculino , Medicina Kampo/métodos , Estudos Multicêntricos como Assunto , Ensaios Clínicos Controlados Aleatórios como Assunto
19.
Neurobiol Aging ; 2020 Jul 13.
Artigo em Inglês | MEDLINE | ID: mdl-32771225

RESUMO

This study aimed to evaluate genotype-phenotype correlations of Parkinson's disease (PD) patients with phospholipase A2 group V (PLA2G6) variants. We analyzed the DNA of 798 patients with PD, including 78 PD patients reported previously, and 336 in-house controls. We screened the exons and exon-intron boundaries of PLA2G6 using the Ion Torrent system and Sanger method. We identified 21 patients with 18 rare variants, such that 1, 9, and 11 patients were homozygous, heterozygous, and compound heterozygous, respectively, with respect to PLA2G6 variants. The allele frequency was approximately equal between patients with familial PD and those with sporadic PD. The PLA2G6 variants detected frequently were identified in the early-onset sporadic PD group. Patients who were homozygous for a variant showed more severe symptoms than those who were heterozygous for the variant. The most common variant was p.R635Q in our cohort, which was considered a risk variant for PD. Thus, the variants of PLA2G6 may play a role in familial PD and early-onset sporadic PD.

20.
Intern Med ; 2020 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-32713917

RESUMO

A 47-year-old woman, who was diagnosed to have systemic lupus erythematosus (SLE), was admitted because she suffered a severe ischemic stroke three weeks after experiencing a transient attack of aphasia. Diffusion-weighted MR imaging revealed high intensity at the borderzone of the middle cerebral artery (MCA), while the proximal portion of the left MCA was occluded with its vascular wall enhanced by gadolinium. Intravenous methylprednisolone and heparin were administrated without any symptomatic benefit. She developed severe right hemiparesis with aphasia. Isolated cerebral vasculitis in the large vessel has been rarely reported in SLE patients. The presence of an enhanced vascular wall in the MR imaging with gadolinium could support the diagnosis.

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