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：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Cerebrovascular diseases in cancer patients significantly aggravate their condition and prognosis; therefore, prompt and accurate diagnosis and treatment are important. The purpose of this study was to investigate patient demographics, laboratory data, brain magnetic resonance imaging (MRI) findings, and prognosis among patients with stroke and cancer, especially cancer-associated ischemic stroke (CAIS). METHODS: We performed a retrospective, single-center study. We enrolled consecutive patients who had acute stroke and were admitted to our hospital between January 2011 and December 2021. We collected general demographic characteristics, cancer histopathological type, laboratory data, brain MRI findings, and prognosis data. RESULTS: Among 2040 patients with acute stroke, a total of 160 patients (7.8%) had active cancer. The types of strokes were cerebral infarction, cerebral hemorrhage, subarachnoid hemorrhage, and transient ischemic attack in 124, 25, 5, and 6 patients, respectively. Among the patients with ischemic stroke, there were 69 cases of CAIS. Pancreas and adenocarcinoma were the most frequent types of primary tumor and histopathology. Patients with adenocarcinoma and those with cerebral infarctions in both bilateral anterior and posterior cerebral circulation areas showed higher D-dimer levels. Pancreatic cancer and high plasma D-dimer levels were associated with poor survival rate. CONCLUSION: CAIS was seen more frequently in patients with pancreatic cancer and adenocarcinoma. Pancreatic cancer and high plasma D-dimer levels were potential factors of poor prognosis in patients with CAIS.

DOI： 10.1007/s10072-024-07332-y

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記述言語：英語  

DOI： 10.1007/s13760-023-02181-6

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: Antithrombotic medications pose a challenge for conducting surgical or invasive procedures, because their discontinuation is required to avoid postprocedural hemorrhagic complications but potentially increases the ischemic risk for the patient. This study aimed to estimate the increased risk of developing cerebral ischemic events during hospitalization requiring discontinuation of antithrombotic therapy. METHODS: This investigation was a single-center retrospective observational study. Clinical data in patients scheduled for admission between January 1, 2021, and December 31, 2022, were collected. Patients requiring discontinuation of antithrombotic therapy were identified by referring to the admission database. Patients who developed cerebral ischemia were identified by referring to the institution's stroke center database. RESULTS: Seven hundred ninety-six patients scheduled for nonneurosurgical procedures and 39 scheduled for neurosurgical procedures underwent discontinuation of antithrombotic therapy. Anticoagulation therapy was prescribed in 40.0%, and antiplatelet therapy was prescribed in 69.1% of the patients. A total of 9.2% of the entire cohort of patients were receiving both anticoagulation and antiplatelet therapy. Bridging therapy was administered in 20.9% of nonneurosurgical patients. No ischemic event was observed in the patients undergoing neurosurgical procedures. Among the entire cohort, 3 patients encountered some kind of thrombotic event-2 of which were cerebral ischemia-accounting for an incidence of 0.24%, which was significantly higher than incidental in-hospital stroke unrelated to discontinuation of antithrombotic therapy (p = 0.04). Patients undergoing both anticoagulation and antiplatelet therapy harbored a significantly higher risk for cerebral ischemia related to discontinuation of antithrombotic therapy (p < 0.0001). CONCLUSIONS: Discontinuing antithrombotic therapy during hospitalization for elective invasive procedures-including neurosurgical procedures-entailed a relatively small risk of developing cerebral ischemic events, but the risk was significantly higher compared to hospitalized patients without discontinuation of antithrombotic therapy.

DOI： 10.3171/2023.7.FOCUS23341

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

The patient, a 58-year-old man, experienced weakness of the proximal muscles in both lower extremities, and Lambert-Eaton myasthenic syndrome and small cell carcinoma of unknown primary origin were diagnosed. He received symptomatic treatment for myasthenia and radiochemotherapy for small cell carcinoma; once this regimen, the myasthenic symptoms improved. However, acute myocardial infarction occurred, after which type II respiratory failure developed, and the patient required ventilator management with tracheal intubation. Acute-phase treatment, such as plasma exchange, intravenous immune globulin therapy, and methylprednisolone pulse therapy, and intensification of symptomatic treatment allowed for extubation, and eventually the patient was able to walk independently. According to electrophysiological examination, compound muscle action potentials were larger at discharge than at the time of exacerbation.

DOI： 10.5692/clinicalneurol.cn-001849

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder that produces a broad spectrum of clinical conditions such as dementia, upper motor neuron involvement, extrapyramidal symptoms, and neuropathy. Some studies have reported ophthalmological conditions associated with the disease; however, the details of these conditions remain unclear. PATIENT CONCERNS: We report a 63-year-old Japanese female with cognitive decline, blurred vision, photophobia, and color blindness at 52 years of age who was diagnosed with cone dystrophy. She also had anxiety, insomnia, depression, delusions, hallucinations, a wide-based gait with short steps, and urinary incontinence. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Magnetic resonance imaging revealed diffuse cerebral white matter changes and subcortical hyperintensity on diffusion-weighted imaging. Skin biopsy showed p62-positive intranuclear inclusions in sweat glands. NOTCH2NLC gene analysis revealed abnormal GGC expansion; therefore, NIID was diagnosed. CONCLUSION: NOTCH2NLC mutation-positive NIID may be associated with retinal dystrophy. Brain magnetic resonance imaging and skin biopsy are helpful diagnostic clues, and gene analysis is crucial for accurate diagnosis and appropriate management.

DOI： 10.1097/MD.0000000000033789

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 63-year-old man with hepatitis C was treated with atezolizumab plus bevacizumab for unresectable diffuse hepatocellular carcinoma (HCC). After four cycles of atezolizumab plus bevacizumab, the diffuse HCC markedly shrank; however, he complained of general fatigue, loss of appetite, and slight loss of muscle strength in the lower legs. He was diagnosed with isolated adrenocorticotropic hormone deficiency (IAD), hypothyroidism, and myopathy, suggesting multisystem immune-related adverse events (irAEs). After administration of hydrocortisone, the clinical symptoms rapidly disappeared. Patients with multisystem irAEs can have favorable outcomes; thus, to continue immune-checkpoint inhibitors therapy, a correct diagnosis and management of multisystem irAEs are important.

DOI： 10.2169/internalmedicine.9393-22

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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：研究論文（学術雑誌）  

OBJECTIVE: To identify whether preoperative magnetic resonance imaging findings of the brain can predict postoperative delirium in patients who undergo arch replacement for aneurysms. METHODS: Overall, 193 patients who underwent aortic replacement for the first time at a single institution between April 2014 and September 2020 were enrolled in this retrospective study. After we excluded patients with acute aortic dissection, no preoperative magnetic resonance imaging findings of the brain, and postoperative cerebral infarction, 50 patients were included and divided into 2 groups, according to their confusion scale results: postoperative delirium (group D) and nonpostoperative delirium (group ND). Preoperative magnetic resonance imaging findings of the brain were classified into lacunar stroke, periventricular hyperintensity, and deep subcortical white matter hyperintensity groups; the latter 2 groups were further classified based on the Fazekas scale, grade 0 to 3. RESULTS: There were 23 patients (46%) in group D and 27 (54%) in group ND. The mean age was significantly greater in group D than in group ND (75 vs 70 years; P = .007). The mean operative time was significantly longer in group D than in group ND (447 vs 384 minutes; P = .024). As for preoperative magnetic resonance imaging findings of the brain, there were significantly more lacunar stroke cases in group D than in group ND (P = .027). In multivariable logistic regression with stepwise selection, high-grade periventricular hyperintensity was significantly related to postoperative delirium (odds ratio, 9.38; 95% confidence interval, 1.55-56.56; P = .015). CONCLUSIONS: Silent cerebral ischemia detected by preoperative magnetic resonance imaging of the brain was a significant risk factor for postoperative delirium.

DOI： 10.1016/j.xjon.2022.02.026

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: The left atrial volume index (LAVI) is considered to be the most accurate index to estimate the size of the left atrium (LA). In this study, we investigated the relationship between LA size measured by LAVI and the occurrence of large-vessel occlusion (LVO) in patients with cardiogenic cerebral infarction (CCI). METHODS: This retrospective single-center cohort study involved 118 patients with CCI within the internal carotid artery (ICA) or middle cerebral artery regions seen between January 2015 and July 2020. In all patients, the type of CCI was determined according to the Diffusion-Weighted Imaging-Alberta Stroke Program Early Computed Tomography Scores (TOAST) subtype diagnosis criteria. LVO was defined as positive when magnetic resonance imaging and computed tomography angiography showed ICA, M1, or M2 occlusion, with all others defined as non-LVO. Clinical characteristics, including LAVI, were evaluated in the records of several patients to investigate if they were risk factors for developing LVO. RESULTS: Seventy patients (59%) were diagnosed as having LVO infarction (ICA occlusion, n = 19 [16%]; M1 occlusion, n = 26 [22%]; and M2 occlusion, n = 25 [21%]). Echocardiography showed no difference between LVO and non-LVO in terms of the ejection fraction (P = 0.64), LA dimension (P = 0.93), and LA volume (P = 0.06). However, LAVI significantly differed between the LVO and non-LVO groups (P = 0.02). Multivariate logistic regression analysis showed larger LAVI as a significant risk factor for LVO (P = 0.01). CONCLUSIONS: Our findings suggest that a larger LAVI is a predictor of developing LVO in patients with CCI.

DOI： 10.1016/j.wneu.2021.12.003

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: This study examined the usefulness of cerebrospinal fluid (CSF) neuron-specific enolase (NSE) levels as a candidate biomarker of neurodegeneration in Alzheimer's disease (AD), Parkinson's disease (PD), PD with dementia (PDD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). METHODS: We performed a systematic search of PubMed, the Cochrane Library, Scopus, and Google Scholar to find studies that measured CSF NSE levels in AD, PD, DLB, and/or MSA. For each disease, we pooled all available data and performed a meta-analysis, and meta-regression analyses of age and sex were conducted if the main analysis found a significant association. RESULTS: Twenty studies were included (13 for AD, 8 for PD/PDD/DLB, and 4 for MSA). Significantly elevated CSF NSE levels were detected in AD (Hedges' g = 0.822, 95% confidence interval [95% CI] 0.332 to 1.311, p = 0.0010), but the data exhibited high heterogeneity (I2 = 88.43%, p < 0.001). The meta-regression analysis of AD showed that age (p < 0.001), but not sex, had a significant effect on CSF NSE levels. A meta-analysis of the pooled data for PD/PDD/DLB did not show any significant changes in the CSF NSE level, but a sub-group analysis of PDD/DLB revealed significantly elevated CSF NSE levels (Hedges' g = 0.507, 95% CI 0.020 to 0.993, p = 0.0412). No significant changes in CSF NSE levels were detected in MSA. CONCLUSIONS: The CSF NSE level may be a useful biomarker of neurodegeneration in AD and PDD/DLB. Age was found to affect the CSF NSE levels of AD patients.

DOI： 10.1186/s13195-021-00907-3

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Objective Fabry disease (FD) is a hereditary lysosomal storage disease that has been highlighted as a possible etiology of stroke at a young age and presents with other various neurological symptoms. Since FD is rare, limited information is currently available on the prevalence of neurological symptoms in Japanese patients with FD. Therefore, we examined the characteristics of neurological symptoms and brain magnetic resonance imaging (MRI) findings in adult Japanese patients with FD. Methods This was a retrospective, single-center study. We reviewed neurological symptoms and brain MRI findings in the medical records of 12 adult Japanese patients with FD diagnosed by a gene analysis of the α-galactosidase gene. Results Ten out of 12 patients with FD presented with the following neurological symptoms: acroparesthesia (n=6), headache (n=5) [migraine (n=4)], hypohidrosis (n=5), and cerebral infarction (n=3). Two and three of the patients with migraine were complicated by ischemic stroke and coronary spastic angina, respectively. Five and 10 patients presented with periventricular hyperintensity and deep white matter hyperintensity, respectively, on brain MRI. Two out of eight patients had cerebral microbleeds. Seven out of 11 patients had a dilated basilar artery diameter on magnetic resonance angiography. There were no patients with the pulvinar hyperintensity sign. Conclusion Patients with FD present with various neurological symptoms. Headache, particularly migraine, might be a major neurological symptom in patients with FD. Since migraine, ischemic stroke, and coronary spastic angina might occur together in FD, caution is needed when administering triptan to FD patients with migraine.

DOI： 10.2169/internalmedicine.6420-20

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 62-year-old Japanese man with swollen fingers and walking difficulty due to myalgia and muscle weakness in proximal limb muscles was admitted to our hospital. Serum creatine kinase was remarkably increased (7,380 U/l) and rapidly progressing interstitial pneumonia developed. Muscle biopsy showed necrotic and regenerating fibers without mononuclear infiltration and fibrosis. Anti-Th/To antibodies were detected in the serum, and anti-Th/To antibody-positive systemic sclerosis was diagnosed. Anti-Th/To antibody-positive sclerosis-associated myopathy has not yet been reported in the literature. The present case suggests that anti-Th/To antibody-positive systemic sclerosis can be accompanied by immune-mediated necrotizing myopathy and be effectively treated with immunotherapy comprising corticosteroids, tacrolimus and immunoglobulin.

DOI： 10.5692/clinicalneurol.cn-001509

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 45-year-old woman was referred to our hospital for the evaluation of proximal muscle weakness and serum creatine kinase elevation [corrected]. She had atrial fibrillation and left ventricular asynergy. She was diagnosed with myopathy, accompanied by cardiomyopathy of unknown etiology. She was treated with prednisolone. After long-term follow-up and a detailed examination, the patient was diagnosed with antimitochondrial antibody (AMA)-associated myopathy with cardiac involvement. Although the patient received medical treatment, including beta-blockers and prednisolone, her cardiac function deteriorated progressively. Physicians should consider AMA-associated myopathy when diagnosing myopathies of unknown etiology. The presence of cardiac involvement should be proactively investigated in AMA-associated myopathy.

DOI： 10.2169/internalmedicine.5600-20

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.2169/internalmedicine.E003-20

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVES: Multiple system atrophy (MSA) is a refractory neurodegenerative disease, but novel treatments are anticipated. An accurate natural history of MSA is important for clinical trials, but is insufficient. This regional registry was launched to complement clinical information on MSA. SETTING: Patient recruitment started in November 2014 and is ongoing at the time of submission. The number of participating facilities was 66. Postal surveys were sent to medical facilities and patients with MSA in Hokkaido, Japan. PARTICIPANTS: After obtaining written consent from 196 participants, 184 overview surveys and 115 detailed surveys were conducted. PRIMARY AND SECONDARY OUTCOME MEASURES: An overview survey evaluated conformity to diagnostic criteria and a detailed survey implemented an annual assessment based on the Unified Multiple System Atrophy Rating Scale (UMSARS). RESULTS: At the time of registration, 58.2% of patients were diagnosed with cerebellar symptoms predominant type MSA (MSA-C) and 29.9% were diagnosed with parkinsonism predominant type MSA (MSA-P). UMSARS Part Ⅳ score of 4 or 5 accounted for 53.8% of participants. The higher the UMSARS Part Ⅳ score, the higher the proportion of MSA-P. At baseline, levodopa was used by 69 patients (37.5%) and the average levodopa dose was 406.7 mg/day. The frequency of levodopa use increased over time. Eleven cases changed from MSA-C to MSA-P during the study, but the opposite was not observed. Information about survival and causes of death was collected on 54 cases. Half of deaths were respiratory-related. Sudden death was recorded even in the group with UMSARS Part Ⅳ score of 1. CONCLUSIONS: This study is the first large-scale prospective MSA cohort study in Asia. MSA-C was dominant, but the use of antiparkinsonian drugs increased over the study period. Changes from MSA-C to MSA-P occurred, but not vice versa.

DOI： 10.1136/bmjopen-2020-045100

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Unattended automated office blood pressure (AOBP) measurement has been endorsed as the preferred in-office measurement modality in recent Canadian and American clinical practice guidelines. However, the difference between AOBP and conventional office blood pressure (CBP) under the environment of a health checkup remains unclear. We aimed to identify the clinical significance of AOBP as compared to CBP under the environment of a health checkup. There were 491 participants (333 females, mean age of 62.5 years) who were at least 20 years old, including 179 participants who were previously diagnosed with hypertension. Mean AOBPs were 131.8 ± 20.9/76.6 ± 11.7 mm Hg, and CBPs were 135.6 ± 21.6/77.3 ± 11.5 mm Hg. There was a difference of 3.9 mm Hg in systolic blood pressure (SBP) and 0.8 mm Hg in diastolic BP between AOBP and CBP. In all participants, SBP and pulse pressure, as well as the white coat effect (WCE), increased with age. The cutoff value used was 140/90 mm Hg for CBP and 135/85 mm Hg for AOBP, and the prevalence of WCE and masked hypertension effect (MHE) was 12.4% and 14.1%, respectively. Even in a health checkup environment of the general population, there was a difference between the AOBP and CBP, and the WCE was observed more strongly in the elderly with a history of hypertension, suggesting that a combination of AOBP with CBP may be useful in detecting WCE and MHE in all clinical scenarios including health checkups, and help solve the "hypertension paradox" not only in Japan but in all over the world.

DOI： 10.1111/jch.14008

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Skeletal muscle has a capacity for muscular regeneration mediated by satellite cells (SCs) and non-SCs. Although it is proposed that non-SCs are attractive therapeutic targets for dystrophies, the biological properties of these cells remain unclear. We have recently identified novel multipotent pericytes (PCs), capillary stem cells (CapSCs) derived from the microvasculature. In the present study, we determined if CapSCs contributed to myogenic regeneration using muscular dystrophy mouse model. CapSCs were isolated as EphA7+NG2+PCs from the subcutaneous adipose tissues of GFP-transgenic mice. Co-culture with C2C12 myoblast cells showed that CapSCs effectively enhanced myogenesis as compared to controls including EphA7- PCs and adipose stromal cells (ASCs). CapSCs transplanted in cardiotoxin-injured gastrocnemius muscles were well differentiated into both muscle fibers and microvessels, as compared to controls. At three weeks after cell-transplantation into the limbs of the mdx/utrn-/-mouse, CapSCs increased the number of GFP+myofibers along with dystrophin expression and the area size of myofibers, and also enhanced the muscular mass and its performance, assessed by treadmill test as compared to controls. In conclusion, CapSCs have potent myogenic regeneration capacity and improved the pathological condition in a muscular dystrophy mouse. Thus, CapSCs are an attractive cellular source in regenerative therapy for muscular dystrophy.

DOI： 10.1016/j.scr.2020.101914

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記述言語：英語   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）  

Parkinson's disease (PD) is a common neurodegenerative disorder; however, well-established biochemical markers have not yet been identified. This review article covers several candidate cerebrospinal fluid (CSF) biomarkers for PD based on the recent literature and meta-analysis data. The decrease of α-synuclein in PD is supported by meta-analyses with modest reproducibility, and a decrease of amyloid β42 is seen as a prognostic marker for cognitive decline. Tau, phosphorylated tau (p-tau), and neurofilament light chains have been used to discriminate PD from other neurodegenerative disorders. This article also describes more hopeful biochemical markers, such as neurotransmitters, oxidative stress markers, and other candidate biomarkers.

DOI： 10.3390/brainsci10070466

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Spinocerebellar ataxia type 8 (SCA8) is a rare hereditary cerebellar ataxia showing mainly pure cerebellar ataxia. We herein report cases of SCA8 in Japanese monozygotic twins that presented with nystagmus, dysarthria, and limb and truncal ataxia. Their ATXN8OS CTA/CTG repeats were 25/97. They showed similar manifestations, clinical courses, and cerebellar atrophy on magnetic resonance imaging. Some of their pedigrees had nystagmus but not ataxia. These are the first monozygotic twins with SCA8 to be reported anywhere in the world. Although not all subjects with the ATXN8OS CTG expansion develop cerebellar ataxia, these cases suggest the pathogenesis of ATXN8OS repeat expansions in hereditary cerebellar ataxia.

DOI： 10.2169/internalmedicine.2905-19

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Parkinson's disease (PD) and dementia with Lewy bodies (DLB) are common neurodegenerative disorders, but no established biochemical markers for these diseases have been identified. We enrolled 78 subjects (27 patients with PD/DLB, 34 patients with non-PD/DLB neurodegenerative disorders [non-PD/DLB], and 17 controls). Cerebrospinal fluid (CSF) was collected via the standard lumbar puncture technique. The CSF levels of alpha-synuclein, amyloid β40, amyloid β42, tau, phosphorylated tau (p-tau), neuron-specific enolase (NSE), and hemoglobin were measured with enzyme-linked immunosorbent assays. Dopamine transporter imaging with 123I-ioflupane was also performed. The PD/DLB patients exhibited significantly lower CSF alpha-synuclein levels than non-PD/DLB group. Significantly elevated CSF levels of tau, p-tau, and NSE were detected in the non-PD/DLB group. Multivariate analysis revealed that the mini-mental state examination score was correlated with the CSF amyloid β42 level. The specific binding ratio on 123I-ioflupane imaging was decreased in the PD/DLB group, but it was not correlated with the CSF alpha-synuclein level. These results indicate that (1) the CSF alpha-synuclein level is a useful biomarker of PD/DLB; (2) the CSF levels of tau, p-tau, and NSE can be used to discriminate PD/DLB from non-PD/DLB; and (3) the CSF amyloid β42 level is an independent predictor of cognitive decline in neurological disorders.

DOI： 10.1016/j.neulet.2019.134564

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 69-year-old Japanese woman was admitted to our hospital with progressive muscle weakness and dysphagia. She was taking pitavastatin for dyslipidemia. Her serum creatine kinase was 6,300 U/L. Pitavastatin was stopped, but her symptoms deteriorated, and cardiac congestion appeared. A muscle biopsy showed necrotizing myopathy (NM), and anti-signal recognition particle (SRP) antibody was positive. 18F-fluorodeoxyglucose-positron emission tomography showed an abnormal uptake, and magnetic resonance imaging showed abnormal gadolinium enhancement in the left ventricular wall. An endomyocardial biopsy revealed inflammatory cardiomyopathy. Steroid, tacrolimus, and intravenous immunoglobulins were effective against the symptoms. This is the first case of biopsy-proven secondary cardiomyopathy due to anti-SRP-positive NM.

DOI： 10.2169/internalmedicine.2564-18

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by abnormalities in the α-galactosidase (Gal) A gene (GLA; MIM:300644). The reduced activity of the lysosomal enzyme, α-galactosidase A (α-Gal A) leads to classic early manifestations and vascular disease of the heart, kidneys, and brain. As a high-risk screening for symptomatic AFD using an enzymatic assay on dried blood spot samples, we enrolled 2325 individuals (803 females and 1522 males; median age: 66 years) with cardiac, renal, or neurological manifestations that met at least one of the following criteria: (a) family history of early-onset cardiovascular diseases; (b) typical classic manifestations, such as acroparesthesias, clustered angiokeratoma, cornea verticillata, and hypo-anhidrosis; (c) proteinuria; (d) receiving dialysis; (e) left ventricular hypertrophy on electrocardiography or echocardiography; or (f) history of stroke. Ninety-two patients displayed low α-Gal A activity. Four males and two females had different pathogenic GLA mutations (0.26%) including a novel mutation c.908-928del21. Four males (0.17%) harbored the GLA c.196G>C (p.E66Q) variant. This simple screening protocol using dried blood spot samples is useful for early diagnosis of AFD in high-risk and underdiagnosed patients suffering from various cardiac, renal, or neurological manifestations.

DOI： 10.1038/s10038-019-0633-1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Objective- Angiogenesis, entire step from endothelial cells (ECs) sprouts to vascular maturation, is a critical response to ischemia. To form functional mature vessels, interactions between ECs and pericytes are essential. Ninj1 (ninjurin1) is an adhesion molecule that contributes to the pathogenesis of neuroinflammation. We recently demonstrated that Ninj1 is expressed in pericytes during angiogenesis. However, the role of Ninj1 in angiogenesis under pathophysiological ischemic conditions has not yet been elucidated. Approach and Results- Ninj1 was detected in microvessels, and its expression was enhanced in ischemic tissues after mouse hindlimb ischemia. Knockdown of Ninj1 was performed by injection of biodegradable microspheres releasing Ninj1-small interfering RNA into muscle tissues. Alternatively, pericyte-specific Ninj1 knockout was induced by tamoxifen treatment of NG2-CreERT/Ninj1-flox mice. Ninj1 knockdown/knockout reduced the formation of blood-circulating functional vessels among total CD31+ microvessels within ischemic tissues and subsequently attenuated color Doppler-assessed blood flow recovery. Ninj1 overexpression enhanced expression of Anpt (angiopoietin) 1, whereas Ninj1 knockdown enhanced the endogenous Anpt1 antagonist, Anpt2 expression in pericytes and inhibited the association of pericytes with ECs and subsequent formation of capillary-like structure, that is, EC tube surrounded with pericytes in 3-dimensional gel culture. Conclusions- Our data demonstrate that Ninj1 is involved in the formation of functional matured vessels through the association between pericytes and ECs, resulting in blood flow recovery from ischemia. These findings further the current our understanding of vascular maturation and may support the development of therapeutics for ischemic diseases.

DOI： 10.1161/ATVBAHA.118.311375

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担当区分：筆頭著者   記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1111/cen3.12459

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 58-year-old female was admitted to our hospital because of recurrent multiple cranial neuropathy (right facial palsy followed by involvement of the left trigeminal, facial, acoustic, pharyngeal, and vagal nerves and the right abducens nerve). Brain MRI showed gadolinium enhancement of the right abducens, bilateral facial/acoustic, and left pharyngeal/vagal nerves, and 18F-Fluorodeoxyglucose (FDG)-positron emission tomography revealed abnormal FDG uptake in the right facial, acoustic, pharyngeal, and vagal nerves and the left cervical lymph nodes. Blood and biochemical analyses did not show any abnormalities, including in the patient's lactate dehydrogenase and soluble interleukin-2 receptor (sIL2R) levels. A cerebrospinal fluid (CSF) examination showed gradual increases in the patient's cell counts and protein, β2-microglobulin, and sIL2R levels, but no malignant cells were detected. A thorough investigation involving repeated CSF examinations, whole-body computed tomography, bone marrow aspiration, random skin biopsies, and cervical lymph node aspiration biopsy examinations did not result in any definitive conclusions. Steroid therapy was ineffective, and the patient developed deafness in her left ear. Therefore, we performed a biopsy examination of the left acoustic nerve, which resulted in the patient being diagnosed with diffuse large B-cell lymphoma. High-dose MTX following the intrathecal administration of MTX, cytarabine, and prednisolone partially improved her symptoms, but she died after several episodes of clinical recurrence. Acoustic nerve biopsy may help diagnose neurolymphomatosis in carefully selected cases.

DOI： 10.5692/clinicalneurol.cn-001080

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Immune checkpoint blockade is developed as standard treatment for non-small cell lung cancer. However immune-related adverse events (irAE) have still unknown complications. Here, we report a patient with lung squamous cell carcinoma who developed neuromyelitis optica spectrum disorder with nivolumab. CASE PRESENTATION: A 75-year-old Japanese man with lung squamous cell carcinoma was administered nivolumab as second-line treatment. Two months after treatment with nivolumab, he presented acute paralysis in the bilateral lower limbs, sensory loss. Spinal magnetic resonance imaging showed T2 hyperintense lesions between C5-6 and Th12-L1. He was diagnosed with neuromyelitis optica spectrum disorder (NMOSD) by anti-aquaporin-4 antibody-positive in the serum and other examinations. After treatment, steroid reactivity was poor. CONCLUSION: This is the first patient who developed anti-AQP4 antibody-positive NMOSD as a nivolumab-induced irAE. Clinicians should be aware of this kind of potential neurological complication by using immune check point inhibitor and start the treatment of this irAE as soon as possible.

DOI： 10.1186/s12885-018-3997-2

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND Carbon dioxide (CO2) is believed to be the safest gas for laparoscopic surgery, which is a standard procedure. We experienced severe cerebral infarction caused by paradoxical CO2 embolism during laparoscopic liver resection with injury of the hepatic vessels despite the absence of a right-to-left systemic shunt. CASE REPORT A 60-year-old man was diagnosed with hepatocellular carcinoma in the right hepatic lobe secondary to alcoholic liver disease. We planned the laparoscopy-assisted liver resection. During the surgery, the root of the right hepatic vein was injured. A 1.5-cm hole was accidentally made in the right hepatic vein, while mobilizing the right hepatic lobe laparoscopically. End-tidal CO2 dropped from 39 to 15.5 mmHg, and systemic blood pressure dropped from 121 to 45 mmHg, returning to normal with the administration of inotropes. The transesophageal echocardiography revealed numerous bubbles in the left atrium and ventricle. The Bispectral Index monitoring system showed low brain activity, suggesting cerebral infarction due to paradoxical gas embolism. The hepatectomy was completed by conversion to open laparotomy. The patient went into a coma and suffered quadriplegia after surgery, despite the cooling of his head and the administration of Thiamylal. Brain MRI revealed cerebral infarction in the broad area of the cerebral cortex right side predominantly, with poor blood flow confirmed by the brain perfusion single-photon emission CT. Rehabilitation was gradually achieved with Botox injections. CONCLUSIONS Cerebral infarction by paradoxical gas embolism is a rare complication in laparoscopic surgery, but it is important to be aware of the risk and to be prepared to treat it.

PubMed

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記述言語：英語  

A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. A cerebrospinal fluid examination revealed elevated protein levels and positive Epstein-Barr virus (EBV) DNA. Human immunodeficiency virus was negative. Brain biopsy showed perivascular lymphocytic infiltration in the parenchyma and meninx with EBV-encoded small RNA (EBER). Since this case did not fulfill the criteria for chronic active EBV infection (CAEBV), she was diagnosed with Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system. High-dose methylprednisolone, acyclovir, ganciclovir and foscarnet were not effective. Although EBV is a causative agent of infectious mononucleosis (IM), lymphomas and nasopharyngeal carcinomas, vasculitic pathology of the central nervous system with EBV reactivation in the elderly is rare. Immunosuppressive drugs such as steroids and MTX are widely used to treat autoimmune disorders, but may exacerbate the reactivation of EBV. This is the first case of biopsy-proven EBV-positive/HIV-negative vasculitis during the treatment of RA with MTX and steroids. This case indicates that EBV-associated vasculitis needs to be considered as a differential diagnosis of CNS vasculitis.

DOI： 10.1111/neup.12356

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 35-year-old male who had not previously suffered any major illnesses was admitted to our hospital because of general fatigue, fever, headache, vomiting, consciousness disturbance, and seizures. A neurological examination showed that he was in a semi-comatose state and exhibited neck stiffness. Brain magnetic resonance imaging detected high-intensity areas in the bilateral hippocampi and periventricular white matter. A cerebrospinal fluid examination revealed mononuclear pleocytosis, an elevated protein level, and positivity for human herpesvirus-7 (HHV-7) DNA. The patient's condition improved after the administration of methylprednisolone, intravenous immunoglobulins, and acyclovir. This is the first known case of limbic encephalitis associated with HHV-7 in an immunocompetent Japanese adult.

DOI： 10.2169/internalmedicine.56.8185

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Atrial fibrillation (AF) is a cardiac arrhythmia that frequently induces ischemic strokes. Nowadays, non-vitamin K antagonist oral anticoagulants (NOACs) have come into widespread use for cardiogenic embolism prevention in place of warfarin. Recently, cerebral microbleeds (CMBs) have been noticed for their potential implication in cerebral small vessel disease. We hypothesized that NOACs do not have an unfavorable influence over cerebral small vessels and investigated whether NOACs increase CMBs in AF patients in a prospective manner. METHODS: We performed baseline magnetic resonance imaging (MRI) examinations on the 69 enrolled AF patients and re-examined second round of MRI 1 year later. The enrolled patients continued the same anticoagulation therapy during the meantime. RESULTS: CMBs did not develop in the 23 patients with NOACs for 1 year. Nine patients with antiplatelets also did not develop CMBs. On the other hand, 3 of 21 patients continued on warfarin and 3 of 9 with warfarin and antiplatelets had CMBs. When divided into 2 groups according to whether the CMBs developed, significant differences in the incidence of using NOACs were observed between the 2 groups (P = .02). A multivariate regression analysis showed that warfarin was independently related to the new development of CMBs (hazard ratio, 10.75; 95% confidence interval, 1.22-94.99; P = .03). CONCLUSIONS: This is the first report to clarify that NOACs do not increase CMBs in AF patients longitudinally in 1 year. Further consideration will be continued with a much longer follow-up in large samples.

DOI： 10.1016/j.jstrokecerebrovasdis.2015.02.018

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Segmental craniocervical dystonia is characterized by blephalospasm and oromandibular dystonia and is also called Meige syndrome. The current treatment strategy including botulinum toxin (BTX) injections has not yet attained an acceptable level. We describe a long-term favorable response of a novel combination therapy with aripiprazole (ARP), trihexyphenidyl (THP), and BTX in three patients with segmental craniocervical dystonia. The symptoms of three patients responded promptly to the combination therapy with ARP 3-6 mg daily, THP 2-8 mg daily, and BTX. Although the patients were required to receive a BTX 50-100 IU injection every 3-6 months, their symptoms were kept in a satisfactory condition for up to 2 years without any adverse effects. ARP possesses the potential for dramatically improving dystonia. THP has the possibility to enhance the efficacy of ARP and prolong the effective period of BTX. It may be an important requisite to give all three agents together for a successful treatment. The combination therapy with ARP, THP, and BTX was well-tolerated and useful in controlling the symptoms of segmental craniocervical dystonia, however, the reason why this combination therapy succeeded is unknown. A further long-term follow-up is required to monitor the delayed neurological adverse effects.

DOI： 10.1007/s10072-014-1927-x

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Fabry disease can cause various neurological manifestations. We describe the case of a Japanese woman with Fabry disease who presented with ischemic stroke, aseptic meningitis, and psychiatric symptoms. The patient had a mutation in intron 4 of her α-galactosidase A gene, which was not detected in her family. This case suggests that Fabry disease should be considered in young patients who exhibit central nervous system symptoms such as ischemic stroke, even if there is no family history of the condition. The episodes of aseptic meningitis and stroke experienced by our patient suggest that persistent inflammation might be the mechanism underlying Fabry disease.

DOI： 10.2169/internalmedicine.54.4719

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A woman with Sjögren syndrome manifesting as aphasia with a left deep cerebral white matter lesion tested positive for anti-aquaporin 4 (AQP4) antibody. Open biopsy of the lesion revealed active demyelination with edematous changes and the preservation of most axons, indicating a non-necrotic demyelinating lesion. Immunostaining for AQP4 was diffusely lost, whereas the loss of glial fibrillary acidic protein immunostaining was limited but with highly degenerated astrocytic foot processes in perivascular areas. These results suggested neuromyelitis optica spectrum disorder (NMOSD) pathology rather than Sjögren-related vasculitis. Only cerebral cortical symptoms with a cerebral white matter lesion could be observed in NMOSDs.

DOI： 10.1177/1352458514540834

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Atrial fibrillation (AF) is a cardiac arrhythmia that does not infrequently induce ischemic strokes; however, little research has been reported on focal cerebral microangiopathic lesions in patients with AF. Recently cerebral microbleeds (CMBs) have been noticed for their potential implication in cerebral small vessel disease. Therefore, we had 2 goals in the present study: (1) to compare the prevalence of CMBs in patients with AF with that in patients without AF, and (2) to prove that CMBs could be a clinical predictive factor for the development of future cerebral microangiopathy in patients with AF without a history of symptomatic cerebral infarction in a prospective manner. METHODS: We performed yearly brain magnetic resonance imaging (MRI) assessments for a maximum of 5 years in 131 patients with AF and 112 control patients. Seventy-seven patients with AF underwent more than 3 yearly MRI scans. RESULTS: The Kaplan-Meier curve showed that the development of an asymptomatic cerebral infarction (ACI) was associated with the baseline presence of a CMB (P=.004). A multivariate Cox regression analysis revealed that the CMBs at baseline were significantly associated with an increment in not only the occurrence of ACIs (hazard ratio [HR], 5.414; 95% confidence interval [CI], 1.03-28.43; P=.046) but also in the consecutive development of CMBs (HR, 6.274; 95% CI, 1.43-27.56; P=.015). CONCLUSIONS: Patients with AF had a significantly higher prevalence of CMBs. The presence of CMBs in the baseline MRI may predict the consequent onset of an ACI and increase in CMBs in patients with AF.

DOI： 10.1016/j.jstrokecerebrovasdis.2014.01.005

PubMed

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記述言語：英語  

DOI： 10.1007/s10072-012-1239-y

PubMed

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

We report an elderly woman with sarcoidosis and rheumatoid arthritis who subsequently developed myasthenia gravis. She was given a diagnose of rheumatoid arthritis at the age of 65 years and sarcoidosis, proved by multiple lymphadenopathy with noncaseating granuloma at the age of 67. Prednisolone, methotrexate, and etanercept had been administrated for rheumatoid arthritis. She consulted our hospital because of bilateral ptosis with diurnal fluctuation at the age of 72. Myasthenia gravis was confirmed by an elevated serum anti-acetylcholine receptor antibody titer (1,100 nmol/l, normal <0.2) and a positive edrophonium test. A chest CT showed a small granular structure in the anterior mediastinum, suggesting thymic hyperplasia. This is the first reported case of myasthenia gravis complicated by sarcoidosis and rheumatoid arthritis. Administration of etanercept may be involved in the onset of myasthenia gravis.

PubMed

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: Patients with chronic kidney disease (CKD) are at a high risk for cardiovascular diseases including stroke. However, the characteristics of the stroke subtypes in patients with CKD remain to be determined. METHODS: We investigated the associations between stroke subtypes and estimated glomerular filtration rate (eGFR), and traditional risk factors in 451 (males, 239; mean age, 71.1 y) acute stroke patients at our hospital between 2006 and 2010. RESULTS: The stroke subtype was cardiogenic cerebral embolism in 129 (29%), cerebral hemorrhage in 104 (23%), unclassified cerebral infarction in 65 (14%), lacunar infarction in 65 (14%), subarachnoid hemorrhage in 41 (9%), atherothrombosis in 30 (7%), and transient ischemic attacks in 17 (4%). Among the 451 patients, 134 (30%) had CKD with eGFR <60 mL/min/1.73 m2. Compared with a group without CKD, mean age (75.9 vs. 69.0 years, p<0.05), the prevalence of atrial fibrillation (AF) (44% vs. 21%, p<0.01) and a history of cardiovascular disease (37% vs. 19%, p<0.01) were significantly higher in that with CKD. A comparison of stroke subtypes revealed a significantly higher incidence of cardiogenic cerebral embolism (36% vs. 26%, p<0.05) in the group with, than without CKD. CONCLUSION: We showed the prevalence of CKD in about 30% of acute stroke patients, and those patients were older, had a significantly higher prevalence of AF and a higher rate of cardiogenic cerebral embolism compared with patients without CKD. Thus, strict control of blood pressure and management of AF should be important to prevent stroke among patients with CKD.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1001/archneurol.2010.285

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Both the appearance of cytoplasmic inclusions containing phosphorylated TAR DNA-binding protein (TDP-43) and inefficient RNA editing at the GluR2 Q/R site are molecular abnormalities observed specifically in motor neurons of patients with sporadic amyotrophic lateral sclerosis (ALS). The purpose of this study is to determine whether a link exists between these two specific molecular changes in ALS spinal motor neurons. We immunohistochemically examined the expression of adenosine deaminase acting on RNA 2 (ADAR2), the enzyme that specifically catalyzes GluR2 Q/R site-editing, and the expression of phosphorylated and non-phosphorylated TDP-43 in the spinal motor neurons of patients with sporadic ALS. We found that all motor neurons were ADAR2-positive in the control cases, whereas more than half of them were ADAR2-negative in the ALS cases. All ADAR2-negative neurons had cytoplasmic inclusions that were immunoreactive to phosphorylated TDP-43, but lacked non-phosphorylated TDP-43 in the nucleus. Our results suggest a molecular link between reduced ADAR2 activity and TDP-43 pathology.

DOI： 10.1007/s00401-010-0678-x

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Marked reduction of RNA editing at the glutamine (Q)/arginine (R) site of the glutamate receptor subunit type 2 (GluR2) in motor neurons may be a contributory cause of neuronal death specifically in sporadic ALS. It has been shown that deregulation of RNA editing of several mRNAs plays a causative role in diseases of the central nervous system such as depression. We analyzed the effects of eight antidepressants on GluR2 Q/R site-RNA editing in a modified HeLa cell line that stably expresses half-edited GluR2 pre-mRNA. We also measured changes in RNA expression levels of adenosine deaminase acting on RNA type 2 (ADAR2), the specific RNA editing enzyme of the GluR2 Q/R site, and GluR2, in order to assess the molecular mechanism causing alteration of this site-editing. The editing efficiency at the GluR2 Q/R site was significantly increased after treatment with seven out of eight antidepressants at a concentration of no more than 10 microM for 24h. The relative abundance of ADAR2 mRNA to GluR2 pre-mRNA or to beta-actin mRNA was increased after treatment with six of the effective antidepressants, whereas it was unchanged after treatment with milnacipran. Our results suggest that antidepressants have the potency to enhance GluR2 Q/R site-editing by either upregulating the ADAR2 mRNA expression level or other unidentified mechanisms. It may be worth investigating the in vivo efficacy of antidepressants with a specific therapeutic strategy for sporadic ALS in view.

DOI： 10.1016/j.neures.2009.03.009

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Human herpesvirus-6 (HHV-6) is the main etiologic agent of exanthema subitum in young children. Central nervous system (CNS) infections in children due to HHV-6 have been described on many occasions. HHV-6 is also a common cause of infections in immunocompromised individuals. However, little is known concerning the impact of HHV-6 on the CNS in immunocompetent adults. We report the first case of relapsing HHV-6 encephalitis in a healthy 73-year-old female.

PubMed

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記述言語：英語   掲載種別：研究論文（国際会議プロシーディングス）  

Scopus

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記述言語：日本語   出版者・発行元：(一社)日本老年医学会  

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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   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：(公社)日本皮膚科学会  

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記述言語：日本語   出版者・発行元：(株)メディカルドゥ  

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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： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J01550&link_issn=&doc_id=20230612410004&doc_link_id=10.5692%2Fclinicalneurol.cn-001849&url=https%3A%2F%2Fdoi.org%2F10.5692%2Fclinicalneurol.cn-001849&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

記述言語：日本語   出版者・発行元：日本臨床皮膚科医会  

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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   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   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   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)日本病理学会  

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記述言語：日本語   出版者・発行元：金原出版(株)  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J00767&link_issn=&doc_id=20220209100023&doc_link_id=10.18888%2Fse.0000002043&url=https%3A%2F%2Fdoi.org%2F10.18888%2Fse.0000002043&type=%E5%8C%BB%E6%9B%B8.jp_%E3%82%AA%E3%83%BC%E3%83%AB%E3%82%A2%E3%82%AF%E3%82%BB%E3%82%B9&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

記述言語：日本語   出版者・発行元：(一社)日本認知症学会  

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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：(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：(株)へるす出版  

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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： https://search.jamas.or.jp/default/link?pub_year=2021&ichushi_jid=J01550&link_issn=&doc_id=20210414410002&doc_link_id=1390850711114165120&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390850711114165120&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

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   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)日本老年医学会  

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記述言語：日本語   出版者・発行元：(一社)日本老年医学会  

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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   出版者・発行元：(一社)日本臨床神経生理学会  

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記述言語：日本語   出版者・発行元：(一社)日本認知症学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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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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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2018&ichushi_jid=J01550&link_issn=&doc_id=20180213430003&doc_link_id=1390282680016996480&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390282680016996480&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

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：(NPO)日本肺癌学会  

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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   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   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   出版者・発行元：(一社)日本内科学会  

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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   出版者・発行元：(一社)日本心臓病学会  

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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：(株)東京医学社  

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記述言語：日本語   出版者・発行元：北海道医学会  

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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：旭川医科大学  

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記述言語：日本語   出版者・発行元：(株)南江堂  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2015&ichushi_jid=J00974&link_issn=&doc_id=20141225090032&doc_link_id=issn%3D0022-1961%26volume%3D115%26issue%3D1%26spage%3D171&url=http%3A%2F%2Fwww.pieronline.jp%2Fopenurl%3Fissn%3D0022-1961%26volume%3D115%26issue%3D1%26spage%3D171&type=PierOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00005_2.gif

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)日本頭痛学会  

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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)日本脳卒中学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2014&ichushi_jid=J01786&link_issn=&doc_id=20141003400001&doc_link_id=%2Fdh3strok%2F2014%2F003605%2F001%2F0327-0332%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdh3strok%2F2014%2F003605%2F001%2F0327-0332%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(一社)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：旭川医科大学  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2014&ichushi_jid=J03513&link_issn=&doc_id=20140403450031&doc_link_id=https%3A%2F%2Fasahikawa-med.repo.nii.ac.jp%2Frecords%2F4857&url=https%3A%2F%2Fasahikawa-med.repo.nii.ac.jp%2Frecords%2F4857&type=%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%EF%BC%9A%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%E5%AD%A6%E8%A1%93%E6%88%90%E6%9E%9C%E3%83%AA%E3%83%9D%E3%82%B8%E3%83%88%E3%83%AA_AMCoR&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F80003_3.gif

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：(株)日本臨床社  

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記述言語：日本語   出版者・発行元：(有)科学評論社  

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記述言語：日本語   出版者・発行元：(一社)日本臨床神経生理学会  

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記述言語：日本語   出版者・発行元：(一社)日本マグネシウム学会  

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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)日本老年医学会  

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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   出版者・発行元：(一社)日本神経学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2013&ichushi_jid=J01550&link_issn=&doc_id=20130516340003&doc_link_id=1390282680013132672&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390282680013132672&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

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：旭川医科大学  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2013&ichushi_jid=J03513&link_issn=&doc_id=20130312380029&doc_link_id=https%3A%2F%2Fasahikawa-med.repo.nii.ac.jp%2Frecords%2F4353&url=https%3A%2F%2Fasahikawa-med.repo.nii.ac.jp%2Frecords%2F4353&type=%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%EF%BC%9A%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%E5%AD%A6%E8%A1%93%E6%88%90%E6%9E%9C%E3%83%AA%E3%83%9D%E3%82%B8%E3%83%88%E3%83%AA_AMCoR&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F80003_3.gif

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   出版者・発行元：(一社)日本臨床神経生理学会  

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記述言語：日本語   出版者・発行元：(一社)日本内科学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2012&ichushi_jid=J01159&link_issn=&doc_id=20120815100019&doc_link_id=1390282681422521344&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390282681422521344&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

記述言語：日本語   出版者・発行元：(一社)日本脳卒中学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2012&ichushi_jid=J01786&link_issn=&doc_id=20120731030002&doc_link_id=%2Fdh3strok%2F2012%2F003404%2F002%2F0221-0227%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdh3strok%2F2012%2F003404%2F002%2F0221-0227%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(一社)日本医学教育学会  

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記述言語：日本語   出版者・発行元：(一社)日本医学教育学会  

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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：(公社)日本超音波医学会  

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記述言語：日本語   出版者・発行元：(公社)日本超音波医学会  

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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：旭川医科大学  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2012&ichushi_jid=J03513&link_issn=&doc_id=20120315450026&doc_link_id=https%3A%2F%2Fasahikawa-med.repo.nii.ac.jp%2Frecords%2F3784&url=https%3A%2F%2Fasahikawa-med.repo.nii.ac.jp%2Frecords%2F3784&type=%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%EF%BC%9A%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%E5%AD%A6%E8%A1%93%E6%88%90%E6%9E%9C%E3%83%AA%E3%83%9D%E3%82%B8%E3%83%88%E3%83%AA_AMCoR&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F80003_3.gif

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   出版者・発行元：(株)南江堂  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2011&ichushi_jid=J00974&link_issn=&doc_id=20111101160035&doc_link_id=issn%3D0022-1961%26volume%3D108%26issue%3D5%26spage%3D935&url=http%3A%2F%2Fwww.pieronline.jp%2Fopenurl%3Fissn%3D0022-1961%26volume%3D108%26issue%3D5%26spage%3D935&type=PierOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00005_2.gif

記述言語：日本語   出版者・発行元：(一社)日本遠隔医療学会  

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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：(一社)日本TDM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：(公社)日本超音波医学会  

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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：(公社)日本薬学会  

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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)日本内科学会  

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記述言語：日本語   出版者・発行元：(一社)日本脳卒中学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2011&ichushi_jid=J01786&link_issn=&doc_id=20110127110012&doc_link_id=%2Fdh3strok%2F2011%2F003301%2F012%2F0084-0088%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdh3strok%2F2011%2F003301%2F012%2F0084-0088%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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   出版者・発行元：(NPO)日本高血圧学会  

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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   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)日本脳卒中学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2010&ichushi_jid=J01786&link_issn=&doc_id=20100401160003&doc_link_id=%2Fdh3strok%2F2010%2F003202%2F003%2F0133-0137%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdh3strok%2F2010%2F003202%2F003%2F0133-0137%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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   出版者・発行元：(有)科学評論社  

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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   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   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)日本脳卒中学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2008&ichushi_jid=J01786&link_issn=&doc_id=20081007190004&doc_link_id=1390282679614251392&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390282679614251392&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

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)日本脳卒中学会  

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記述言語：日本語   出版者・発行元：(一社)日本脳卒中学会  

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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   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)日本脳卒中学会  

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記述言語：日本語   出版者・発行元：(一社)日本脳卒中学会  

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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   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   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：(有)科学評論社  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2002&ichushi_jid=J00695&link_issn=&doc_id=20020603280013&doc_link_id=http%3A%2F%2Famcor.asahikawa-med.ac.jp%2Fmodules%2Fxoonips%2Fdetail.php%3Fid%3D2002218490&url=http%3A%2F%2Famcor.asahikawa-med.ac.jp%2Fmodules%2Fxoonips%2Fdetail.php%3Fid%3D2002218490&type=%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%EF%BC%9A%E6%97%AD%E5%B7%9D%E5%8C%BB%E7%A7%91%E5%A4%A7%E5%AD%A6%E5%AD%A6%E8%A1%93%E6%88%90%E6%9E%9C%E3%83%AA%E3%83%9D%E3%82%B8%E3%83%88%E3%83%AA_AMCoR&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F80003_3.gif

記述言語：日本語   出版者・発行元：厚生労働省精神・神経疾患研究班  

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記述言語：日本語   出版者・発行元：厚生労働省精神・神経疾患研究班  

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記述言語：日本語   出版者・発行元：厚生労働省精神・神経疾患研究班  

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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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開催年月日： 2023年3月

記述言語：日本語   会議種別：ポスター発表  

開催地：パシフィコ横浜  

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開催年月日： 2022年5月

記述言語：英語   会議種別：ポスター発表  

[Objective] Fabry disease (FD) is a hereditary lysosomal storage disease that has been highlighted as a possible etiology of stroke at a young age, and it presents with other various neurological symptoms. Dolichoectasia of the basilar artery (BA) is a characteristic finding of FD; however, the characteristics of BA changes in FD remain unclear. Herein, we examined the characteristics of BA changes in adult Japanese patients with FD. [Methods] We reviewed brain MRI findings in the medical records of 14 adult Japanese patients (3 men and 11 women, mean age of all 14 patients was 49.3 ± 12.8 years) with FD diagnosed by a gene analysis of the α-galactosidase gene. BA mean diameter, curved length, ‘origin-to-end’ linear distance (linear length), and tortuosity index {(curved length ÷ linear length)-1} were retrospectively measured on MR angiography studies. [Results] The mean diameter of BA was 3.08 ± 0.50 mm. Ten out of 14 patients had a dilated BA (> 2.7 mm). The mean curved and linear lengths were 23.8 ± 3.96 mm and 26.2 ± 4.59 mm, respectively. The mean tortuosity index was 0.10 ± 0.08. Two patients showed high tortuosity index (> 0.2). There was no significant correlation between the BA diameter and the tortuosity index (r = 0.16). [Conclusions] The diameter of the BA in more than 50% of patients with FD was dilated; however, only two patients presented with high elongation of BA. This suggests that elongation of BA is not correlated with dilation of BA in patients with FD.

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開催年月日： 2022年3月

記述言語：日本語   会議種別：口頭発表（一般）  

開催地：大阪国際会議場  

【背景・目的】近年悪性腫瘍に対して免疫チェックポイント阻害薬(ICI)が使用される頻度が増え、悪性腫瘍に対する治療成績が向上している一方、ICIの使用に伴う種々の有害事象が報告されている。神経領域の有害事象として、自己免疫性末梢神経障害や重症筋無力症、炎症性筋疾患など自己免疫性疾患の報告が多かったが、近年血栓塞栓性脳梗塞についての報告もされている。今回当院の悪性腫瘍に対してICIによる治療中に脳卒中を発症した症例の臨床的特徴を解析することを目的とした。【対象・方法】2018年1月から2021年9月までの間に当院に入院した脳卒中症例のうち、悪性腫瘍に対してICIによる治療を実施中に発症した症例について、脳卒中や悪性腫瘍、ICIの種類などの臨床的背景について解析した。【結果】ICIによる治療中に脳卒中を発症した症例は5例で、そのうち脳梗塞が4例、脳出血が1例であり、脳梗塞の病型としては原因特定困難の脳梗塞、心原性梗塞、癌関連血栓症(Trousseau症候群)、空気塞栓症が各1例ずつであった。悪性腫瘍の種類は咽頭癌2例、肺癌1例、胃癌1例、膀胱癌1例であり、使用されたICIはニボルマブ2例、ベムブロマリズマブ2例、アテゾリズマブ1例であった。原因特定困難の脳梗塞については、ニボルマブによる治療中に発症していた。【考察】少数ではあるが、ニボルマブによる治療中に原因特定困難の脳梗塞を発症した症例があり、ニボルマブが発症に影響した可能性も否定できず、ICIによる治療には脳梗塞の発症に注意する必要が考慮された。【結論】ICIの副作用として脳梗塞が発症する可能性もあり、今後症例の蓄積による検討が必要と考えられた。

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開催年月日： 2021年10月

記述言語：日本語   会議種別：シンポジウム・ワークショップ パネル（指名）  

開催地：三重県  

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開催年月日： 2021年6月

記述言語：日本語   会議種別：口頭発表（一般）  

【目的】【方法】【結果】【結論】【キーワード】 ---------------------------------------------------------------------------------------------------- 【目的】ファブリー病は若年性脳梗塞や心病変、腎病変などをきたすライソゾーム病であり、脳梗塞などのために緊急入院が必要となる場合がある。当科において頻回の緊急入院を必要としたファブリー病の症例について報告する。【方法】若年性脳梗塞や四肢末端痛に加え、精神症状や無菌性髄膜炎をきたしたファブリー病の症例の詳細について記述する。【結果】症例は52歳女性。9歳頃から四肢末端痛が出現。27歳時出産後から幻覚や妄想が出現し、他院に緊急入院となり、統合失調症と診断された。その頃から間欠的に発熱が出現。39歳時妄想が悪化し、他院に緊急入院。入院後頭痛が出現し、精査の結果無菌性髄膜炎と診断された。42歳時右視床梗塞による左半身異常感覚のため当科に緊急入院。α-ガラクトシダーゼ(GLA)活性が低下しており、α-GLAの遺伝子検査ではイントロン4にFabry病の古典型変異部位があり、ファブリー病の診断となった。両親には本人と同様のα-GLA遺伝子変異を認めず、孤発例と考えられた。心病変として左室肥大を伴っていた一方、明らかな腎病変は認められなかった。46歳時左視床梗塞による右半身異常感覚のために当科に再入院。現在も当院への通院を継続している。【結論】ファブリー病に伴う神経疾患として若年性脳梗塞がよく知られているが、頻度は少ないものの精神症状や無菌性髄膜炎を合併することも報告されている。ファブリー病は多様な神経症状をきたしうることが考えられるため、神経救急において留意して診療に当たる必要がある。

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開催年月日： 2021年5月

記述言語：英語   会議種別：ポスター発表  

[Objective] Fabry disease (FD) is a hereditary lysosomal storage disease that has been highlighted as a possible etiology of stroke at a young age, and presents with other various neurological symptoms. Since FD is rare, limited information is currently available on the prevalence of neurological symptoms in Japanese patients with FD. Therefore, we herein examined the characteristics of neurological symptoms and brain MRI findings in adult Japanese patients with FD. [Methods] We reviewed neurological symptoms and brain MRI findings in the medical records of twelve adult Japanese patients with FD diagnosed by a gene analysis of the α-galactosidase gene. [Results] Ten out of twelve patients with FD presented with the following neurological symptoms: acroparesthesia (n=6), headache (n=5) [migraine (n=4)], hypohidrosis (n=5), cerebral infarction (n=3), psychiatric symptoms (n=2), and epilepsy (n=2). Two of the patients with migraine were complicated by ischemic stroke. Five and ten patients presented with periventricular hyperintensity and deep white matter hyperintensity, respectively, on brain MRI. Seven out of eleven patients had a dilated basilar artery diameter on MR angiography. There were no patients with the pulvinar hyperintensity sign. [Conclusions] Patients with FD may present with various neurological symptoms. Headache, particularly migraine, may be a major neurological symptom in patients with FD. FD needs to be considered in patients complicated by both migraine and ischemic stroke. The pulvinar sign on MRI may not necessarily be a sensitive marker for FD.

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開催年月日： 2021年3月

記述言語：日本語   会議種別：口頭発表（一般）  

【背景・目的】Fabry病は若年性脳梗塞の原因となるが、頻度がまれであり、病態や臨床的特徴について未解明な部分があることから、脳梗塞を合併したFabry病症例の臨床的特徴を解析することを目的とした。【対象・方法】2008年1月から2019年12月までの間、当院に入院したFabry病のうち、脳梗塞を合併した症例の臨床的背景やMRI所見について、脳梗塞非合併症例と比較を交えて解析した。【結果】Fabry病全症例12例(男性2名、女性10名、平均年齢53.4歳)のうち、3例で脳梗塞を合併しており、すべて女性だった。脳梗塞の病型について、2例は穿通枝梗塞、1例は心原性梗塞であった。脳梗塞を合併例ではすべてに頭痛を合併しており、うち2例は片頭痛であった。脳梗塞非合併例では2例において片頭痛を伴っていた。心病変はFabry病のすべての症例に合併しており、腎病変は脳梗塞合併例では1例、非合併例では6例でみられた。MRI所見では、脳梗塞の合併例と非合併例のそれぞれにおいて、大脳白質病変(periventricular hyperintensity, deep white matter hyperintensity)および椎骨動脈の拡張傾向が多くの割合でみられた。【考察】Fabry病症例全体の25%に脳梗塞が合併しており、穿通枝梗塞の割合が多かった。Fabry病のMRI所見の特徴とされる大脳白質病変や椎骨動脈の拡張は脳梗塞合併と非合併例の両方でみられ、脳梗塞との関連性は乏しかった。脳梗塞合併症例では頭痛を高頻度に伴っており、片頭痛の割合が高かった。脳梗塞と頭痛の両方を伴う女性症例では、Fabry病の可能性を考慮し、積極的に精査をする必要があると考えられた。【結論】Fabry病に伴う脳梗塞の病型としては穿通枝梗塞の割合が高く、頭痛を高頻度に合併しやすい可能性がある。

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開催年月日： 2021年2月

記述言語：日本語   会議種別：口頭発表（一般）  

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開催年月日： 2020年8月 - 2020年9月

記述言語：英語   会議種別：ポスター発表  

[Objective] Paraneoplastic neurological syndromes (PNS) comprise a group of disorders that can affect any part of the nervous system in patients with neoplasms and frequently develop as a result of autoimmune responses. Limited information is currently available on PNS because of their rarity. We report the clinical characteristics of patients with PNS in our hospital. [Methods] We presented a 10-year case series compiled in our Hospital between 2009 and 2019. [Results] We identified seven patients with a diagnosis of PNS. Median age was 54.1 years (range 31-71 and 57% of patients were male. PNS types were cerebellar ataxia (n=3), limbic encephalitis (n=2), peripheral neuropathy (n=1), and necrotizing myopathy (n=1), An anti-glutamate receptor antibody (limbic encephalitis: n=2), anti-CV2 antibody (cerebellar ataxia: n=1), and anti-Tr and recoverin antibody (cerebellar ataxia: n=1) were detected among antibody- positive cases. Neoplasms were lung carcinoma (n=2), ovary teratoma (n=2), esophageal carcinoma (n=1), lymphoma (n=1), and plasmacytoma (n=1). Immunotherapy used were steroids (n=6), and immunoglobulin (n=2), while neoplasms were treated with surgery (n=3) and chemotherapy (n=3). Neurological symptoms improved in six out of seven patients after immunotherapy and treatments for neoplasms, but remained unchained in the remaining patient. [Conclusions] Patients with PNS presented with various neurological symptoms. Neurological symptoms improved with immunotherapy and treatments for neoplasm. Therefore, patients with PNS need to be actively treated with these therapies.

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開催年月日： 2020年8月 - 2020年9月

記述言語：日本語   会議種別：口頭発表（一般）  

【背景・目的】悪性腫瘍に伴う脳卒中の原因の一つとして、化学療法の影響による血栓塞栓症や出血などの病態があるが、現在までに多数症例で検討した報告は少ないことから、悪性腫瘍に対する化学療法中に発症した脳卒中症例の臨床的特徴を解析することを目的とした。【方法】2011年1月から2018年12月までの間、当院に入院した脳卒中1394例のうち、134例で悪性腫瘍を合併しており、その中で悪性腫瘍に対する化学療法中に脳卒中を発症した34例を対象に、悪性腫瘍の原発臓器や使用された薬剤、脳卒中の病型などについて解析した。【結果】悪性腫瘍の原発臓器; 膵臓6例、肺・大腸各4例、脳・頭頸部・胃・前立腺各3例、乳房・肝臓各2例、胆管・卵巣・子宮各1例。薬剤(重複投与あり); ゲムシタビン7例、シスプラチン・テガフール各5例、フルオロウラシル4例、ゾレドロン・オキサリプラチン・トラスツズマブ・レトロゾール・カベシタビン各2例(他薬剤もあり)。脳卒中の病型; 脳梗塞25例(分類困難20例、心原性3例、アテローム血栓性・ラクナ各1例)、一過性脳虚血発作2例、脳出血7例。そのうち、脳卒中の発症に化学療法が関与した可能性を否定できない症例が16例あった。【考察】悪性腫瘍に伴う脳卒中の病態は多様であるが、その原因の1つとして化学療法の影響がある。化学療法に関連した虚血性脳卒中に関して、シスプラチン使用下での発症が比較的多かったとする過去の報告があるが、当院ではシスプラチン以外にゲムシタビンやテガフールの使用下での発症が比較的多かった。今後悪性腫瘍に対する新規治療薬剤が増加するにつれて、化学療法の影響によって発症する脳卒中が増加する可能性もあり、その知見を蓄積する必要がある。

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開催年月日： 2019年5月

記述言語：英語   会議種別：ポスター発表  

開催地：大阪狭山市  

[Objective] Fabry disease (FD) is a rare hereditary lysosomal storage disease that has been highlighted as a possible etiology of stroke at a young age. Cerebral white matter lesions, pulvinar sign and enlarged basilar artery diameters have been demonstrated in patients with FD. The aim of this study is to compare these findings of Japanese patients with FD to those of healthy controls. [Methods] Cerebral white matter lesions, T1-signal hyperintensity of the pulvinar thalami, and cerebral artery diameters were evaluated using magnetic resonance imaging (MRI) and angiography (MRA) in ten clinically affected patients with FD and ten age-and gender-matched controls. White matter lesions were rated according to periventricular hyperintensity (PVH), and deep and subcortical white matter hyperintensity (DSWMH) grading. Diameters of the following cerebral arteries were measured using SYNAPSE VINCENT software. [Results] DSWMH grading of patients with FD were significantly higher than controls. PVH grading did not differ between patients with FD and controls. Pulvinar hyperintensity was present in neither patients with FD nor controls. Compared to controls, patients with FD had significantly enlarged diameters of rostal part of basilar artery. The diameters at the other parts of basilar artery, and the other cerebral arteries did not differ significantly between patients with FD and the controls. [Conclusions] Evaluation of DSWMH and basilar artery diameters could be a screening tool to detect FD. Pulvinar sign on MRI may not necessarily be sensitive marker in FD patients.

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開催年月日： 2019年3月

記述言語：日本語   会議種別：ポスター発表  

開催地：横浜市  

【背景・目的】癌関連血栓症による脳卒中の病態や治療については未解明な部分が多いことから、抗凝固療法中に発症した癌併発脳卒中症例を検討することで、その特徴を解析することを目的とした。【対象・方法】抗凝固療法中に脳卒中を発症した癌症例の臨床的背景を後ろ向きに解析した。【結果】2011年1月から2018年9月までの脳卒中症例(1338例)のうち、129例で悪性腫瘍を併発していた。その中で18例が抗凝固療法中の発症であり、臨床的背景は以下のようであった。癌原発臓器; 膵臓4例、肺・血液・前立腺各3例、食道・胃・大腸・肝臓・卵巣各1例。抗凝固療法対象疾患; 心房細動7例、深部静脈血栓症 6例、その他5例。脳卒中の病型; 脳梗塞17例(癌関連血栓症[Trousseau症候群]6例、心原性・アテローム血栓性各4例、その他4例)、くも膜下出血1例。使用抗凝固薬; エドキサバン6例、ワルファリン5例、アピキサバン3例、未分画ヘパリン2例、リバロキサバン・アルガトロバン各1例。治療(重複あり); エダラボン15例、未分画ヘパリン10例、血管内治療3例、アルガトロバン2例、その他6例。転帰; 転院8例、死亡6例、生存4例。癌関連血栓症のうち、脳梗塞発症後に経口抗凝固薬(エドキサバン3例、リバロキサバン1例)から未分画ヘパリン皮下注射に変更した症例では脳梗塞の再発なく経過した。【考察】癌症例では癌に関連した過凝固状態の影響で、抗凝固療法下においても脳梗塞を発症しやすい傾向にあることが考えられた。癌関連血栓症の再発予防治療として、経口抗凝固薬よりもヘパリン皮下注射の方が優れている可能性がある。

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開催年月日： 2018年9月

記述言語：日本語   会議種別：ポスター発表  

開催地：旭川市  

【目的】 日本人Fabry病患者の高血圧合併の有無による頭部MRIの大脳白質病変所見について比較し、その特徴を明らかにする。 【方法】 当院のFabry病患者の頭部MRIにおける脳室周囲病変(PVH)や深部皮質下白質病変(DSWMH)を、脳ドックのガイドライン2014で採用されている分類に準じて評価し、高血圧合併の有無による相違について比較検討した。 【成績】 10名のFabry病患者のうち、高血圧合併は2名(女性2名、平均年齢55歳、古典型1名、心亜型1名)、非合併は8名(男性2名、女性6名、平均年齢52.1歳、古典型6名、心亜型2名)だった。高血圧合併群では、2名ともPVHとDSWMHが認められた。一方、高血圧非合併群では、2名でPVH、7名でDSWMHが認められた。また、DSWMHは大脳白質病変の危険因子を有さない1例でも認められた。 【結論】 Fabry病ではMRIにおける大脳白質病変の出現頻度が高いことが知られているが、日本人における多数症例での検討はされていない。大脳白質病変の最大の危険因子は高血圧であるが、高血圧を合併していないFabry病患者でも高頻度に大脳白質病変を有しており、なかでも深部皮質下白質に多く、Fabry病による細動脈病変を反映している可能性が考えられた。

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開催年月日： 2018年5月

記述言語：英語   会議種別：ポスター発表  

開催地：札幌市  

[Objective] Fabry disease is a rare hereditary lysosomal Storage disease that has been highlighted as a possible etiology of stroke at a young age. Enlarged basilar artery diameters have been demonstrated in patients with Fabry disease abroad. The aim of this study is to compare basilar artery diameters of Japanese patients with Fabry disease to those of healthy age-matched controls. [Methods] Eight clinically affected patients with Fabry disease (age 54.4 ± 11.2) and eight age- and gender-matched controls were measured cerebral artery diameters using magnetic resonance angiography (MRA). Diameters of the cerebral arteries were measured within the time-of-flight sequences using SYNAPSE VINCENT software. The basilar artery diameters were measured at the three points: 1) caudal, 2) intermediate, and 3) rostal. Besides the basilar artery, diameters of the following arteries were also measured; anterior cerebral artery, middle cerebral artery, posterior cerebral artery, and internal carotid artery. [Results] Compared to the controls, the patients with Fabry disease had significantly enlarged diameters of rostal part of basilar artery (p = 0.045). The diameters at the other parts of basilar artery, and the other cerebral arteries did not differ significantly between the patients with Fabry disease and the controls. [Conclusions]Enlarged diameters of rostal part of basilar artery were able to detect Fabry disease within a cohort of Fabry disease and healthy controls. Basilar artery measurement using MRA could be a screening marker to detect Fabry disease in Japanese.

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開催年月日： 2017年9月

記述言語：英語   会議種別：ポスター発表  

開催地：京都市  

Background: Anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab) is positive in some patients with anti-aquaporin 4 antibody (AQP4-Ab) -negative central nervous system (CNS) lesions; however, their clinical diversity remains unclear. Objective: To elucidate the clinical features of patients with CNS lesions who tested positive for MOG-Ab or AQP4-Ab. Methods: We herein described the clinical features of 22 adult patients with CNS lesions who tested positive for MOG-Ab or AQP4-Ab. Results: Seven MOG-Ab-positive and 15 AQP4-Ab-positive patients were identified. MOG-Ab-positive patients were more likely to be male, younger at disease onset, and achieved better functional recovery after the administration of corticosteroid therapy for attacks than AQP4-Ab-positive patients. Three MOG-Ab-positive patients presented with only brain lesions including the cerebral gray and white matters, brain stem, and cerebellum and had fewer relapses. Two of these 3 patients also had meningoencephalitis. Among MOG-Ab-positive patients, optic neuritis was detected more frequently and relapses were more likely in females. None of the MOG-Ab-positive patients had spinal cord lesions. Four AQP4-Ab-positive patients also had autoimmune diseases including Sjögren’s syndrome, systemic lupus erythematosus, and rheumatoid arthritis, whereas MOG-Ab-positive patients did not have these diseases. Conclusions: The clinical features of MOG-Ab-positive patients with CNS lesions, including a male predominance and good responses to corticosteroids, differ from those of AQP4-Ab-positive patients with CNS lesions. Patients with MOG-Ab may present with only brain lesions, including the gray matter, and mimic meningoencephalitis. Among MOG-Ab-positive patients with CNS lesions, clinical features may differ according to gender, because optic neuritis was more common and relapses occurred more frequently in female patients.

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開催年月日： 2016年5月

記述言語：日本語   会議種別：ポスター発表  

開催地：兵庫県神戸市  

【目的】 近年抗aquaporin-4(AQP4)抗体陰性のneuromyelitis optica spectrum disorders(NMOSD)で、抗myelin-oligodendrocyte glycoprotein(MOG)抗体陽性である症例が報告されているが、その病態や臨床的特徴については未解明の部分が多い。当院における抗MOG抗体および抗AQP4抗体陽性のNMOSD症例を解析することにより、その特徴を明らかにすることを目的とした。 【方法】 当院の抗MOG抗体および抗AQP4抗体陽性のNMOSD症例の臨床的評価項目について、それぞれ解析した。 【結果】 抗MOG抗体陽性例は1例(女性)、抗AQP4抗体陽性例は13例(女性11、男性2)であった。抗MOG抗体陽性例は27歳で発症し、8回視神経炎のみを起こしたが、急性期のステロイド投与で軽快し、免疫維持療法は実施されなかった。抗AQP4抗体陽性例に関して、発症平均年齢は51.2歳、平均再発回数は3.3回であり、病変部位について視神経炎は8例、脊髄炎は11例(病変長が3椎体以上:9例、1椎体:2例)、大脳病変は9例で認められた。大脳と脳幹に病変を有し、視神経炎と脊髄炎が認められない症例が1例あった。ステロイドや免疫吸着療法による急性期治療に反応を示したものが多かったが、ステロイドによる再発予防治療実施下で、再発を繰り返し重篤な後遺症を残したものが2例あった。 【結論】 当院における抗MOG抗体陽性例は、抗AQP4抗体陽性例と比べて少数で、発症年齢は低く、再発回数が多かったが、ステロイドの投与で軽快した。また既報の抗MOG抗体陽性症例と比べても、再発回数が多かった。抗MOG抗体陽性NMOSDの病態や治療については未解明の部分が多く、今後症例の蓄積による検討が必要と考えられた。

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開催年月日： 2014年5月

記述言語：英語   会議種別：ポスター発表  

【目的】孤発性筋萎縮性側索硬化症(ALS)の脊髄運動ニューロンでは、グルタミン酸受容体のうちAMPA受容体のサブユニットであるGluA2のグルタミン(Q)/アルギニン(R)部位のRNA編集率が疾患特異的・部位選択的に低下し、ALSの運動ニューロン細胞死の病態に深く関与している。同部位のRNA編集率を回復する薬剤はALSの治療に使用しうると考え、薬剤のスクリーニングを行った。【方法】In vitroでの薬剤負荷による GluA2 Q/R部位のRNA編集率の変化について検討するために、同部位の編集率を人為的に50%程度に調節したTet-HeLa G2m細胞にUS drug collectionに収載されている薬剤を24時間負荷した後、そのRNA編集率を測定した。【結果】複数の薬剤がGlu2 Q/R部位のRNA編集率の有意に上昇させることを見出した。【結論】市販薬剤の中にはGluA2 Q/R部位の編集率を上昇させるものがあり、孤発性ALSの特異的治療に応用できる可能性が示唆された。

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開催年月日： 2013年5月

記述言語：英語   会議種別：ポスター発表  

【目的】孤発性筋萎縮性側索硬化症(ALS)の脊髄運動ニューロンでは、グルタミン酸受容体のうちAMPA受容体のサブユニットであるGluA2のグルタミン(Q)/アルギニン(R)部位のRNA編集率が疾患特異的・部位選択的に低下し、ALSの運動ニューロン細胞死の病態に深く関与している。同部位のRNA編集率を回復する薬剤はALSの治療に使用しうると考え、薬剤のスクリーニングを行った。【方法】In vitroでの薬剤負荷による GluA2 Q/R部位のRNA編集率の変化について検討するために、同部位の編集率を人為的に50%程度に調節したTet-HeLa G2m細胞にUS drug collectionに収載されている薬剤を24時間負荷した後、そのRNA編集率を測定した。【結果】複数の薬剤がGlu2 Q/R部位のRNA編集率の有意に上昇させることを見出した。【結論】市販薬剤の中にはGluA2 Q/R部位の編集率を上昇させるものがあり、孤発性ALSの特異的治療に応用できる可能性が示唆された。

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開催年月日： 2013年3月

記述言語：日本語   会議種別：ポスター発表  

開催地：東京都  

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開催年月日： 2012年5月

記述言語：日本語   会議種別：ポスター発表  

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開催年月日： 2011年5月

記述言語：日本語   会議種別：ポスター発表  

【目的】孤発性筋萎縮性側索硬化症(ALS)の脊髄運動ニューロンでは、グルタミン酸受容体のうちAMPA受容体のサブユニットであるGluA2のグルタミン(Q)/アルギニン(R)部位のRNA編集率が疾患特異的・部位選択的に低下し、ALSの運動ニューロン細胞死の病態に深く関与している。同部位のRNA編集率を回復する薬剤はALSの治療に使用しうると考え、薬剤のスクリーニングを行った。【方法】In vitroでの薬剤負荷による GluA2 Q/R部位のRNA編集率の変化について検討するために、同部位の編集率を人為的に50%程度に調節したTet-HeLa G2m細胞にUS drug collectionに収載されている薬剤を24時間負荷した後、そのRNA編集率を測定した。【結果】複数の薬剤がGlu2 Q/R部位のRNA編集率の有意に上昇させることを見出した。【結論】市販薬剤の中にはGluA2 Q/R部位の編集率を上昇させるものがあり、孤発性ALSの特異的治療に応用できる可能性が示唆された。

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開催年月日： 2010年5月

記述言語：日本語   会議種別：ポスター発表  

【目的】孤発性筋萎縮性側索硬化症(ALS)の脊髄運動ニューロンでは、グルタミン酸受容体のうちAMPA受容体のサブユニットであるGluA2のグルタミン(Q)/アルギニン(R)部位のRNA編集率が疾患特異的・部位選択的に低下し、ALSの運動ニューロン細胞死の病態に深く関与している。同部位のRNA編集率を回復する薬剤はALSの治療に使用しうると考え、薬剤のスクリーニングを行った。【方法】In vitroでの薬剤負荷による GluA2 Q/R部位のRNA編集率の変化について検討するために、同部位の編集率を人為的に50%程度に調節したTet-HeLa G2m細胞にUS drug collectionに収載されている薬剤を24時間負荷した後、そのRNA編集率を測定した。【結果】複数の薬剤がGlu2 Q/R部位のRNA編集率の有意に上昇させることを見出した。【結論】市販薬剤の中にはGluA2 Q/R部位の編集率を上昇させるものがあり、孤発性ALSの特異的治療に応用できる可能性が示唆された。

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開催年月日： 2009年5月

記述言語：日本語   会議種別：ポスター発表  

【目的】孤発性筋萎縮性側索硬化症(ALS)の脊髄運動ニューロンでは、グルタミン酸受容体のうちAMPA受容体のサブユニットであるGluA2のグルタミン(Q)/アルギニン(R)部位のRNA編集率が疾患特異的・部位選択的に低下し、ALSの運動ニューロン細胞死の病態に深く関与している。同部位のRNA編集率を回復する薬剤はALSの治療に使用しうると考え、薬剤のスクリーニングを行った。【方法】In vitroでの薬剤負荷による GluA2 Q/R部位のRNA編集率の変化について検討するために、同部位の編集率を人為的に50%程度に調節したTet-HeLa G2m細胞にUS drug collectionに収載されている薬剤を24時間負荷した後、そのRNA編集率を測定した。【結果】複数の薬剤がGlu2 Q/R部位のRNA編集率の有意に上昇させることを見出した。【結論】市販薬剤の中にはGluA2 Q/R部位の編集率を上昇させるものがあり、孤発性ALSの特異的治療に応用できる可能性が示唆された。

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開催年月日： 2008年5月

記述言語：日本語   会議種別：ポスター発表  

【目的】孤発性筋萎縮性側索硬化症(ALS)の脊髄運動ニューロンでは、グルタミン酸受容体のうちAMPA受容体のサブユニットであるGluA2のグルタミン(Q)/アルギニン(R)部位のRNA編集率が疾患特異的・部位選択的に低下し、ALSの運動ニューロン細胞死の病態に深く関与している。同部位のRNA編集率を回復する薬剤はALSの治療に使用しうると考え、薬剤のスクリーニングを行った。【方法】In vitroでの薬剤負荷による GluA2 Q/R部位のRNA編集率の変化について検討するために、同部位の編集率を人為的に50%程度に調節したTet-HeLa G2m細胞にUS drug collectionに収載されている薬剤を24時間負荷した後、そのRNA編集率を測定した。【結果】複数の薬剤がGlu2 Q/R部位のRNA編集率の有意に上昇させることを見出した。【結論】市販薬剤の中にはGluA2 Q/R部位の編集率を上昇させるものがあり、孤発性ALSの特異的治療に応用できる可能性が示唆された。

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開催年月日： 2007年5月

記述言語：日本語   会議種別：ポスター発表  

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開催年月日： 2006年5月

記述言語：日本語   会議種別：ポスター発表  

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開催年月日： 2005年5月

記述言語：日本語   会議種別：ポスター発表  

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富良野地域における神経難病医療に携わる医療関係者向けの講演

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北海道における神経難病患者のネットワーク事業に関する会議

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富良野地域における神経難病患者及び家族の在宅療養支援のための多職種会議

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