記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL  

BACKGROUND AND PURPOSE: Presence of an intimal flap is a critical imaging finding in diagnosing intracranial artery dissection (ICAD). Recent reports showed that high-resolution magnetic resonance imaging (MRI) was better at identifying intimal flaps as compared with routine MRI techniques used in clinical settings. However, no current standardized sequence for high-resolution MRI without gadolinium enhancement produces images of satisfactory quality with clinically tolerable scanning times. This study evaluated a nonenhanced high-resolution fast spin echo (HR-FSE) MRI sequence for visualizing intimal flaps in patients with ICAD.
SUBJECTS AND METHODS: Three patients with ICAD underwent plain MRI examination using a 2-dimensional T2-weighted FSE imaging sequence optimized for our 3T system (in-plane pixel size, .23 mm x .23 mm; slice thickness 3 mm with no interslice gap), as well as scanning with conventional modalities, including CT angiography, magnetic resonance angiography, and digital subtraction angiography. We assessed whether these imaging methods could visualize an intimal flap and/or double lumen sign in the participants and compared the results between HR-FSE and the other modalities.
RESULTS: HR-FSE images clearly showed intimal flaps and double lumen signs in all 3 patients, whereas the conventional modalities identified a double lumen sign in only 2 of the 3 patients.
CONCLUSIONS: The present method of optimized HR-FSE imaging with a 3T system improved visualization of intimal flaps and should thus be considered for assessing patients with suspected ICAD that cannot be definitively diagnosed by conventional imaging modalities.

DOI： 10.1111/jon.12380

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：JAPAN SOC INTERNAL MEDICINE  

A 65-year-old Japanese man with advanced chronic kidney disease (CKD) developed acute-onset type 1 diabetes mellitus (T1D) that was associated with severe acute kidney injury and was manifested by generalized tonic-clonic status epilepticus. His seizures resolved without recurrence after correcting the diabetic ketoacidosis. Although hyperglycemia is an important cause of acute symptomatic seizure (ASS), patients with ketotic hyperglycemia develop ASS less frequently. In this T1D case with CKD, severe hyperglycemia in conjunction with other metabolic insults, such as uremia, hyponatremia, and hypocalcemia, probably provoked his seizure despite the severe ketonemia.

DOI： 10.2169/internalmedicine.56.8304

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCIENCE BV  

Laryngeal stridor is recognized as a characteristic clinical manifestation in patients with multiple system atrophy (MSA). However, the pathogenic mechanisms underlying this symptom are controversial. Neurogenic atrophy of the posterior cricoarytenoid muscle has been identified in cases of MSA, suggesting that laryngeal abductor weakness contributes to laryngeal stridor. However, dystonia in the laryngeal adductor muscles has also been reported to cause laryngeal stridor. Depletion of serotonergic neurons in the medullary raphe nuclei, which exert tonic drive to activate the posterior cricoarytenoid muscle, has recently been identified in MSA cases. This adds weight to the possibility that laryngeal abductor weakness underlies laryngeal stridor in MSA. Continuous positive airway pressure therapy is currently used in the treatment of laryngeal stridor, but should be used with caution in patients showing contraindications. Current knowledge of the clinical and neuropathological features of laryngeal stridor is summarized in this paper, and the hypothesized causes and possible therapeutic options for this symptom are discussed. (C) 2016 Elsevier B.V. All rights reserved.

DOI： 10.1016/j.jns.2016.01.007

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCIENCE BV  

Objective: Fifty percent of patients with amyotrophic lateral sclerosis (ALS) have cervical spondylotic myelopathy (CSM) as a complication. Because patients with ALS do not develop bulbar signs and symptoms at onset, differentiating them from patients with CSM is sometimes difficult. We aimed to determine whether the apparent diffusion coefficients (ADCs) of intracranial corticospinal tracts can be used to distinguish between patients with ALS and those with CSM.
Methods: We evaluated 19 consecutive patients with ALS who did not have CSM by cervical MRI, 16 patients with CSM, and 11 healthy controls. We examined the mean ADCs in the precentral gyrus, the corona radiata, the posterior limbs of the internal capsule (PLIC), and the cerebral peduncle by 3T magnetic resonance imaging (MRI). The mean ADCs in the intracranial corticospinal tracts in patients with ALS were compared with those in patients with CSM.
Results: The mean ADCs in the intracranial corticospinal tracts in patients with ALS were compared with those in patients with CSM (p<0.05). Additionally, the mean ADCs in the precentral gyrus, the PLIC, and the cerebral peduncle in the patients with ALS, including the patients who were initially diagnosed as having clinically possible ALS on the basis of the revised El Escorial criteria and did not develop bulbar symptoms at onset, were also higher than those in patients with CSM (p<0.05).
Conclusions: Elevated ADCs in the intracranial corticospinal tracts might be useful for distinguishing ALS from CSM in the early stage of the disease. (C) 2015 Elsevier B.V. All rights reserved.

DOI： 10.1016/j.clineuro.2015.02.009

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：KARGER  

Patients with myotonic dystrophy type 1 (DM1) (n = 14) were compared with healthy controls (n = 13) using 3.0 T proton magnetic resonance spectroscopy (H-1-MRS) to investigate brain pathophysiology. H-1-MRS imaging revealed reduced N-acetylaspartate to creatine ratio (NAA/Cr) in multiple brain regions (average 24%), suggesting diffuse brain abnormalities among patients with DM1. Single-voxel H-1-MRS among patients with DM1 showed (1) reduced NAA in both the frontal cortex (23%) and frontal white matter (31%) and unaltered myo-inositol, suggesting neuronal abnormalities without significant gliosis; and (2) elevated glutamine in the frontal cortex (36%) and reduced glutamate in the frontal white matter (20%) among patients with DM1, suggesting abnormalities in the glutamatergic system in the brain of patients with DM1. We consider that these results reflect brain abnormalities that cannot be detected by neuropathological assessment in patients with DM1. (C) 2015 S. Karger AG, Basel

DOI： 10.1159/000371575

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Background: Combination therapy with infliximab (IFX) and azathioprine (AZA) is significantly more effective for treatment of active Crohn's disease (CD) than IFX monotherapy. However, AZA is associated with an increased risk of lymphoma in patients with inflammatory bowel disease. Aim: To evaluate the cost-effectiveness of combination therapy with IFX plus AZA for drug-refractory CD. Methods: A decision analysis model is constructed to compare, over a time horizon of 1. year, the cost-effectiveness of combination therapy with IFX plus AZA and that of IFX monotherapy for CD patients refractory to conventional non-anti-TNF-α therapy. The treatment efficacy, adverse effects, quality-of-life scores, and treatment costs are derived from published data. One-way and probabilistic sensitivity analyses are performed to estimate the uncertainty in the results. Results: The incremental cost-effectiveness ratio (ICER) of combination therapy with IFX plus AZA is 24,917 GBP/QALY when compared with IFX monotherapy. The sensitivity analyses reveal that the utility score of nonresponding active disease has the strongest influence on the cost-effectiveness, with ICERs ranging from 17,147 to 45,564 GBP/QALY. Assuming that policy makers are willing to pay 30,000 GBP/QALY, the probability that combination therapy with IFX plus AZA is cost-effective is 0.750. Conclusions: Combination therapy with IFX plus AZA appears to be a cost-effective treatment for drug-refractory CD when compared with IFX monotherapy. Furthermore, the additional lymphoma risk of combination therapy has little significance on its cost-effectiveness. © 2012 European Crohn's and Colitis Organisation.

DOI： 10.1016/j.crohns.2012.04.007

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.crohns.2012.04.007

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：KARGER  

DOI： 10.1159/000353873

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TOHOKU UNIV MEDICAL PRESS  

The incidence of esophageal squamous cell carcinoma (ESCC), which is the eighth most common malignancy worldwide, is highest in China. The purpose of this study was to investigate the association between nitrogen compounds in drinking water with the incidence of ESCC by geographical spatial analysis. The incidence of ESCC is high in Shexian county, China, and environmental factors, particularly nitrogen-contaminated drinking water, are the main suspected risk factors. This study focuses on three nitrogen compounds in drinking water, namely, nitrates, nitrites, and ammonia, all of which are derived mainly from domestic garbage and agricultural fertilizer. The study surveyed 48 villages in the Shexian area with a total population of 54,716 (661 adults with ESCC and 54,055 non-cancer subjects). Hot-spot analysis was used to identify spatial clusters with a high incidence of ESCC and a high concentration of nitrogen compounds. Logistic regression analysis was used to detect risk factors for ESCC incidence. Most areas with high concentrations of nitrate nitrogen in drinking water had a high incidence of ESCC. Correlation analysis revealed a significant positive relationship between nitrate concentration and ESCC (P = 0.01). Logistic regression analysis also confirmed that nitrate nitrogen has a significantly higher odds ratio. The results indicate that nitrate nitrogen is associated with ESCC incidence in Shexian county. In conclusion, high concentrations of nitrate nitrogen in drinking water may be a significant risk factor for the incidence of ESCC.

DOI： 10.1620/tjem.226.11

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記述言語：日本語  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: The aim of this study was to find biomarkers of disease severity in multiple system atrophy of cerebellar type by imaging disease specific regions using proton magnetic resonance spectroscopy on a 3.0 T system. METHODS: We performed proton magnetic resonance spectroscopy separately in the pons and medulla on 12 multiple system atrophy of cerebellar type patients and 12 age and gender matched control subjects. The metabolite concentrations were estimated from single-voxel proton magnetic resonance spectra measured by point resolved spectroscopy, which were then correlated with clinical severity using Part I, II, and IV of the unified multiple system atrophy rating scale. RESULTS: Proton magnetic resonance spectroscopy showed that myo-inositol concentrations in both the pons and medulla were significantly higher in multiple system atrophy of cerebellar type patients compared to those of the control subjects (P < 0.05). By contrast, total N-acetylaspartate (the sum of N-acetylaspartate and N-acetylaspartylglutamate) and total choline compounds concentrations in both the pons and medulla were significantly lower in multiple system atrophy of cerebellar type patients compared to control subjects (P < 0.05). Creatine concentration in the pons was significantly higher in multiple system atrophy of cerebellar type patients compared to the control subjects (P < 0.05). Furthermore, a significant correlation was found between the myo-inositol/creatine ratio in the pons and clinical severity, defined by the sum score of unified multiple system atrophy rating scale (I+II+IV) (r = 0.76, P < 0.01). CONCLUSION: Proton magnetic resonance spectroscopy, in conjunction with a 3.0 T system, can be feasible to detect part of pathological changes in the brainstem, such as gliosis and neuronal cell loss, and the metabolites can be used as biomarkers of clinical severity in multiple system atrophy of cerebellar type patients.

DOI： 10.1002/mds.23550

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DOI： 10.1002/mds.23550

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.jtbi.2010.07.036

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Neuronal growth cones migrate directionally under the control of axon guidance molecules, thereby forming synapses in the developing brain. The signal transduction system by which a growth cone detects surrounding guidance molecules, analyzes the detected signals, and then determines the overall behavior remains undetermined. In this study, we describe a novel stochastic model of this behavior that utilizes multiple sensors on filopodia to respond to guidance molecules. Overall growth cone behavior is determined by using only the concentration gradients of guidance molecules in the immediate vicinity of each sensor. The detected signal at each sensor, which is treated as a vector quantity, is sent to the growth cone center and then integrated to determine axonal growth in the next step by means of a simple vector operation. We compared the results of computer simulations of axonal growth with observations of actual axonal growth from co-culture experiments using olfactory bulb and septum. The probabilistic distributions of axonal growth generated by the computer simulation were consistent with those obtained from the culture experiments, indicating that our model accurately simulates growth cone behavior. We believe that this model will be useful for elucidating the as yet unknown mechanisms responsible for axonal growth in vivo. © 2010 Elsevier Ltd.

DOI： 10.1016/j.jtbi.2010.07.036

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：新潟医学会  

静脈血栓塞栓症(VTE)は、発症しても臨床症状に乏しく早期診断が困難であること、また重篤な場合は死に至ることから予防が重要とされている。本研究の目的は、人工股関節置換術(THA)と人工膝関節置換術(TKA)を受けた入院患者を対象とし、予防の実施状況、VTEの発症率、および、理学的・薬物的予防効果を推定することである。本院において2005年1月から2006年3月までに、THAおよびTKAを受けた97例を対象とした。解析に用いたデータ項目は、診察所見、検査、手術に関するデータの13項目である。理学的予防、薬物的予防の実施率は、それぞれ100%、19.6%であった。97例におけるVTE発症率は23.7%であったが、VTE既往歴の有無で層別したときの発症率は、それぞれ、42.3%、16.9%であった。ロジスティック回帰分析の結果、VTE発症に関与する危険因子はVTE既往歴のみが選択された。VTEの既往歴で層別したそれぞれの層において、薬物的予防実施群と非実施群のVTE発症率を比較した。その結果、いずれの層でも実施群のVTE発症率が非実施群に比べて低かった。また合併症指標として薬物的予防実施群と非実施群でのヘモグロビン値と血小板数を比較した結果、有意な差は認められなかった(P=0.864、P=0.255)。本研究において、理学的・薬物的予防がVTEの発症率を下げることを示唆した。これらの予防効果に関する探索的な推定値を使い、確証的な予防効果を得るための臨床試験を行うことが必要である。(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND AND PURPOSE Three-dimensional anisotropy contrast (3DAC) based on a periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) sequence on a 3.0T system is a new magnetic resonance imaging technique capable of providing images with significantly high anatomical resolution. The purpose of this study was to confirm whether this technique can characterize the degenerative processes in the brainstem of patients with spinocerebellar degeneration (SCD). METHODS 3DAC images of 13 patients with multiple system atrophy with predominant cerebellar symptoms (MSA-C) and seven International Cooperative Ataxia Rating Scale (ICARS) score-matched patients with Machado-Joseph disease (MJD) were created using a diffusion-weighted PROPELLER sequence on a 3.0T system. The section of the middle pons was chosen for morphometric and diffusivity analyses. RESULTS The above analyses showed that atrophy and increased diffusivity of the ventral portion of the pons indicated MSA-C, whereas atrophy and increased diffusivity of the pontine tegmentum indicated MJD. Furthermore, ICARS scores significantly correlated with both the severities of the pontine atrophy and the mean diffusivity values of the ventral pontocerebellar tracts. CONCLUSIONS This study demonstrated that 3DAC PROPELLER on a 3.0T system enables in vivo "tract by tract" quantitative analysis of pontine degeneration in SCD. J Neuroimaging 2009
19:220-226. © 2008 by the American Society of Neuroimaging.

DOI： 10.1111/j.1552-6569.2008.00273.x

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DOI： 10.1111/j.1552-6569.2008.00273.x

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記述言語：日本語   出版者・発行元：(一社)日本血栓止血学会  

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DOI： 10.1620/tjem.215.33

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

In Japan, the national screening for the hepatitis C virus (HCV) has been started for both the general population and the high-risk groups. Our cost-effectiveness analysis was based on the result of the screening program including 99,001 people among the general population and 42,538 people among the high risk group from 2003 to 2006. The screening was performed using the three steps of the semi-quantitative HCV antibody test, the HCV core antigen test and the HCV-PCR test. A Markov model for HCV infected patients was constructed to estimate the future clinical benefits and the lifetime cost and the cost-effectiveness analysis was performed considering the recent treatment with peginterferon plus ribatvirin. In the cost-effectiveness analysis, the cohort, in which the screening was implemented (= screening strategy), was compared with the similar cohort without the screening (= no-screening strategy) in both the general population and the high-risk group, stratified by age. The infection rates of the general population and the high-risk group were 0.36% and 0.81%, respectively. The incremental cost-effectiveness ratio (ICER), a measure of cost-effectiveness, of the general population and the high-risk group was calculated to be from 848 to 4,825 and - 749 to 2,297 $/life expectancy gained, respectively. The treatment effectiveness, transition probabilities and the infection rate varied in the one-way sensitivity analyses, but the superiority of the screening strategy regarding the cost-effectiveness was unchanged. In conclusion, the screening strategy in both the general population and the high-risk group therefore appears to be more cost-effective than a no-screening strategy. © 2008 Tohoku University Medical Press.

DOI： 10.1620/tjem.215.33

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：KARGER  

To elucidate the still unclear mechanism of delayed encephalopathy after carbon monoxide ( CO) poisoning, we serially performed single-voxel proton magnetic resonance spectroscopy (H-1-MRS) and diffusion tensor imaging (DTI) on a 3.0T system and intelligence tests in 2 patients, while they were undergoing hyperbaric oxygenation therapy. The chronological changes in H-1-MRS- and DTI-derived parameters indicated the following: ( 1) White matter demyelination, aerobic metabolism inhibition, and cytotoxic edema persisted for at least 3 months even after starting the hyperbaric oxygenation therapy; ( 2) the axonal function and structural integrity of the white matter were initially severely impaired and then gradually and partially improved for 5 months, showing changes similar to those in the scores of the intelligence tests. The results demonstrated that brain damage after CO poisoning may persist longer than expected, and that the H-1-MRS- and DTI-derived parameters are good indicators of the clinical progress of a patient. The combination of H-1-MRS and DTI on a 3.0T system is useful for monitoring the changes in brain damage and the clinical symptoms of patients with delayed encephalopathy after CO poisoning. Copyright (C) 2007 S. Karger AG, Basel.

DOI： 10.1159/000109262

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ACADEMIC PRESS INC ELSEVIER SCIENCE  

There have been several methods proposed so far using diffusion tensor imaging (DTI) for the assessment of normal-appearing brain tissue (NABT) injury in multiple sclerosis (MS). However, for these methods, the analyses of the NABT injury at the cellular level, wherein histological examinations can be used, still present challenging problems. We developed a method of segregating NABT into the following anatomical structures using lambda chart analysis associated with a two-dimensional Gaussian deconvolution of diffusion characteristic functions: 1) structures primarily composed of small neurons and glia; 2) structures primarily composed of large neurons; 3) structures primarily composed of short axons; and 4) structures primarily composed of long axons. Each segregated structure that had a distinctive diffusion characteristic was subjected to the statistical inference of DTI-derived parameters for 14 patients with conventional relapsing-remitting MS (RRMS) and 20 age-matched healthy volunteers. In all of the structures, the trace values were significantly higher and the fractional anisotropy values were significantly lower in the RRMS patients than in the healthy volunteers. Furthermore, the volume fractions of the structures primarily composed of short axons markedly decreased, whereas those of the structures primarily composed of small neurons and glia markedly increased. These results suggest that axonal loss and glial proliferation predominantly occurred in the subcortical white matter and adjacent deep cortical layer, namely, the juxtacortical region. This cell-oriented analysis of NABT injury using DTI confirmed in vivo the histological observation that the juxtacortical region is the most vulnerable site in MS. (C) 2007 Elsevier Inc. All rights reserved.

DOI： 10.1016/j.neuroimage.2007.06.026

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER MEDICAL ASSOC  

Background: The mechanism underlying nocturnal sudden death in patients with MSA remains unclear. It may be explained by upper airway obstruction, such as vocal cord abductor paralysis; an impairment of the respiratory center, such as Cheyne- Stokes respiration; or an impaired hypoxemic ventilatory response.
Objective: To investigate the mechanism of sleep-disordered breathing in multiple system atrophy ( MSA).
Design: We recruited 21 patients with probable MSA who were admitted sequentially to our hospital, and performed daytime blood gas analysis, pulmonary function tests, polysomnography, and fiberoptic laryngoscopy during wakefulness and with the patient under anesthesia.
Results: A decrease in arterial oxygen pressure and an increase in alveolar- arterial oxygen gradient significantly correlated with disease duration ( P=. 045 and.046, respectively). Polysomnography demonstrated CheyneStokes respiration in 3 ( 15%) of 20 patients. Fiberoptic laryngoscopy during wakefulness showed that 3 ( 14%) of the 21 patients exhibited vocal cord abductor paralysis, and laryngoscopy under anesthesia showed that 9 ( 45%) of 20 patients exhibited vocal cord abductor paralysis. Laryngoscopy under anesthesia also revealed that 11 ( 55%) of 20 patients showed upper airway obstruction in places other than the vocal cords, including obstruction at the base of the tongue or soft palate. In addition, it demonstrated novel laryngopharyngeal findings, such as floppy epiglottis and airway obstruction at the arytenoid.
Conclusions: We observed daytime hypoxemia with an increased alveolar- arterial oxygen gradient, CheyneStokes respiration, and novel abnormal laryngopharyngeal movements in patients with MSA. We also found that laryngoscopy under anesthesia might be useful for evaluating upper airway obstruction. The significance of these findings to the mechanism of sudden death in those with MSA needs to be examined.

DOI： 10.1001/archneur.64.6.856

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER MEDICAL ASSOC  

Background: Multiple system atrophy (MSA) has been considered a sporadic disease, without patterns of inheritance.
Objective: To describe the clinical features of 4 multiplex families with MSA, including clinical genetic aspects.
Design: Clinical and genetic study. Setting: Four departments of neurology in Japan.
Patients: Eight patients in 4 families with parkinsonism, cerebellar ataxia, and autonomic failure with age at onset ranging from 58 to 72 years. Two siblings in each family were affected with these conditions.
Main Outcome Measures: Clinical evaluation was performed according to criteria by Gilman et al. Trinucleotide repeat expansion in the responsible genes for the spinocerebellar ataxia (SCA) series and for dentatorubral-pallidoluysian atrophy (DRPLA) was evaluated by polymerase chain reaction. Direct sequence analysis of coding regions in the alpha-synuclein gene was performed.
Results: Consanguineous marriage was observed in 1 of 4 families. Among 8 patients, 1 had definite MSA, 5 had probable MSA, and 2 had possible MSA. The most frequent phenotype was MSA with predominant parkinsonism, observed in 5 patients. Six patients showed pontine atrophy with cross sign or slitlike signal change at the posterolateral putaminal margin or both on brain magnetic resonance imaging. Possibilities of hereditary ataxias, including SCA1 (ataxin 1, ATXN1), SCA2 (ATXN2), Machado-Joseph disease/SCA3 (ATXN1), SCA6 (ATXN1), SCA7 (ATXN7), SCA12 (protein phosphatase 2, regulatory subunit B, beta isoform; PP2R2B), SCA17 (TATA box binding protein, TBP) and DRPLA (atrophin 1; ATN1), were excluded, and no mutations in the alpha-synuclein gene were found.
Conclusions: Findings in these multiplex families suggest the presence of familial MSA with autosomal recessive inheritance and a genetic predisposition to MSA. Molecular genetic approaches focusing on familial MSA are expected to provide clues to the pathogenesis of MSA.

DOI： 10.1001/archneur.64.4.545

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER  

Behcet disease (BD) is a chronic relapsing multisystem disorder of unknown etiology, which preferentially affects the oral and genital mucous membranes, skin, and eyes. Neurological involvement is one of the most serious manifestations of BD, known as neuro-Behcet disease (NBD). We here describe clinical, radiological, and neuropathological findings for two patients with a possible variant of NBD, who manifested progressive ataxia in the absence of mucocutaneo-ocular signs characteristic for BD. Both patients presented a slowly progressive cerebellar phenotype, accompanied by behavioral changes and sphincter disturbance. Brain MRI scan revealed mild atrophy in pons and cerebellum. Both patients showed a mild CSF pleocytosis, and were positive for HLA-B51. The post-mortem examination performed in one patient, showed widespread foci of chronic encephalitis, consistent with the diagnosis of NBD. Steroid pulse therapy was effective in one patient. Identifying the progressive ataxia phenotype of NBD without mucocutaneo-ocular symptoms is important, because these patients may benefit from early steroid therapy.

DOI： 10.1007/s00296-006-0171-y

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：DR DIETRICH STEINKOPFF VERLAG  

In multiple system atrophy (MSA), symptoms associated with dysfunctions of the brainstem and autonomic nervous system are important prognostic factors. We investigated brainstem involvement in 12 patients with MSA with predominant cerebellar symptoms (MSA-C) (mean age, 56.3 +/- 9.9 years, median disease duration, 3 years), and 11 controls (57.6 +/- 12.0 years) matched for age using diffusion-weighted MR imaging (DWI). We demonstrated that apparent diffusion coefficients (ADCs) in the pons and middle cerebellar peduncle of MSA-C patients are significantly higher than those of normal controls even though the patients are in the early stage of the disease. Furthermore, we demonstrated that increased ADC values correlated well with the disease duration. The current study demonstrated that DWI is a useful noninvasive method for the quantitative evaluation of the brainstem involvement in MSA-C patients.

DOI： 10.1007/s00415-004-0494-0

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

We report of a woman aged 52 years born to consanguineous parents and seeking treatment for progressive dementia and delusion. Neurologic examination revealed dementia and emotional instability, indifference, and confabulation. There was also mild spasticity of the bilateral lower limbs. MRI revealed diffuse white matter hyperintensity on T2-weighted images accompanied by hypointense areas on fluid-attenuated inversion recovery images. A homozygous missense mutation was identified in EIF2B5.

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A new algorithm for tractography based on diffusion tensor analysis (DTA) is presented. This method, called EZ-tracing, is based on a new algorithm for analyzing DTA data, lambda chart analysis (LCA), and effectively overcomes the main shortcomings of previous methods for tractography. EZ-tracing is written in MATLAB scripting language (MathWorks, Natick, MA, USA) and can be implemented on any of the common operating systems, such as Microsoft Windows, UNIX, and LINUX. The program is available in public at coe@bri.niigata-u.ac.jp. © 2002 Elsevier Science B.V. All rights reserved.

DOI： 10.1016/S0165-0270(02)00039-0

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCIENCE BV  

A new algorithm for tractography based on diffusion tensor analysis (DTA) is presented. This method, called EZ-tracing, is based on a new algorithm for analyzing DTA data, lambda chart analysis (LCA), and effectively overcomes the main shortcomings of previous methods for tractography. EZ-tracing is written in MATLAB scripting language (MathWorks, Natick, MA, USA) and can be implemented on any of the common operating systems, such as Microsoft Windows, UNIX, and LINUX. The program is available in public at coe@bri.niigata-u.ac.jp. (C) 2002 Elsevier Science B.V. All rights reserved.

DOI： 10.1016/S0165-0270(02)00039-0

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：KARGER  

DOI： 10.1159/000050795

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   掲載種別：記事・総説・解説・論説等（学術雑誌）   出版者・発行元：新潟県医師会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   掲載種別：記事・総説・解説・論説等（学術雑誌）  

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記述言語：日本語   出版者・発行元：新潟医学会  

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記述言語：日本語  

J-GLOBAL

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記述言語：日本語  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   掲載種別：記事・総説・解説・論説等（学術雑誌）   出版者・発行元：科学評論社  

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記述言語：日本語   出版者・発行元：日本神経治療学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：日本神経治療学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：日本神経治療学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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その他リンク： http://search.jamas.or.jp/link/ui/2004055415

記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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記述言語：日本語   出版者・発行元：(一社)日本神経学会  

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