著者

渡辺 恭良

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.36, no.6, pp.S153, 2019 (Released:2019-10-25)

著者

飯塚 高浩

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.36, no.4, pp.388-394, 2019 (Released:2020-04-24)

参考文献数

29

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After the discovery of new class of autoantibodies against neuronal cell surface antigens or synaptic proteins (NSA), the concept of acute encephalitis has dramatically changed. Encephalitis can be divided into infectious and autoimmune encephalitis (AE). AE may have autoantibodies against intracellular onconeuronal antigens (e.g. Hu, Yo, Ri, CRMP5, Ma2), intracellular synaptic proteins (e.g. amphiphysin, GAD65) or NSA (e.g. NMDAR, AMAPAR, GABAaR, GABAbR, LGI1, Caspr2, DPPX).Most of the classic paraneoplastic anti–neuronal antibodies are less likely pathogenic but IgG NSA antibodies are more likely pathogenic, and the presence of the NSA antibodies implies that patients may respond to immunotherapy. Although early initiation of immunotherapy is often emphasized in AE, antibody testing is not readily accessible in most situation, thus initiation of immunotherapy may be delayed. In 2016, a practical diagnostic approach to AE was published as a position paper in Lancet Neurology to achieve prompt immunotherapy at 3 levels of evidence for AE (possible, probable, and definite) with several new diagnostic criteria including possible AE, probable AE, probable and definite anti–NMDAR encephalitis, autoimmune limbic encephalitis, ADEM, and Hashimoto encephalopathy.In this lecture, I focus on recent progress in antibody–mediated encephalitis, stiff–person spectrum disorder, and cryptogenic new–onset refractory status epilepticus (C–NORSE) with development of a clinically–based score that predicts C–NORSE.

著者

平田 幸一

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.33, no.3, pp.357-361, 2016 (Released:2016-11-10)

参考文献数

11

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Making the center of the refractory migraine is chronic migraine (CM) and medication overuse headache.CM is a disabling neurological condition affecting 0.5–2% of the population.In the current third edition of the International Classification of Headache Disorders, medication overuse is no longer an exclusion criterion and CM is diagnosed in patients suffering from at least 15 headache days per month of which at least eight are related to migraine.CM is difficult to treat, and preventive treatment options are limited.

著者

辰巳 寛 山本 正彦

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.33, no.3, pp.362-367, 2016 (Released:2016-11-10)

参考文献数

43

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Aphasia greatly influences a patient's quality of life (QOL). Recovery from aphasia requires a long time, and improvements in anterior language function can be difficult to achieve. Aphasia is a major obstacle for returning to work and thus can have serious economic consequences.The diagnosis of aphasia requires a qualitative assessment (classification of type of aphasia) and a quantitative judgment (severity assessment). Importantly, the various symptoms that constitute the pathology of aphasia must be understood when making a qualitative assessment. Quantitative assessments must also be evaluable using a systematic objective test.The mainstream of aphasia treatment is the classical stimulus–facilitation method. Recently, a cognitive neuropsychological approach, a pragmatic approach, and Melodic Intonation Therapy have also attracted attention as possible therapies. Furthermore, studies on pharmacotherapy and noninvasive therapy for aphasia are proceeding.The psychosocial study of aphasic persons and their families has become a very important theme. Lately, a depression rating scale and a QOL rating scale for people with aphasia, a family's communication burden scale, and a communication self–efficacy scale have been developed. Clinically, it is important to provide hospitable support to both people with aphasia and their families.

著者

犬塚 貴 木村 暁夫 林 祐一

出版者

日本神経治療学会

雑誌

神経治療学

巻号頁・発行日

vol.33, no.2, pp.94-98, 2016

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<p>Several autoantibodies are associated with autoimmune encephalitis. Some of these antibodies are directed against intracellular neuronal antigens such as Hu and Ma2, which are strongly associated with paraneoplatic syndrome. In the past 10 years, various antibodies were identified that recognize neuronal cell–surface or synaptic proteins in patients associated with or without malignancy. Some of these antibodies are able to directly access receptors of neurotransmitters or channels and are responsible for causing neurological syndromes. Autoimmune encephalopathy with these antibodies generally responds to immunotherapies, such as steroids, plasmapheresis, and intravenous immunoglobulin as well as immunosuppressant and anti–cancer treatments in cases of paraneoplastic syndrome.</p><p>Patients with N–methyl–D–aspartate (NMDA) receptor antibodies, which are the most common in autoimmune encephalopathy, often cause psychiatric manifestation, memory impairment, seizures, dyskinesia, catatonia, autonomic instability and respiratory failures. Although 86% of patients become worse at the stage of mRS5, almost 80% of all patients recover to the stage of less than mRS2 with immunomodulatory therapy and careful management for their general condition. Detection of those antibodies in both serum and CSF using cell–based assays is important for definite diagnosis. Availability of screening systems of antibodies and covering health insurance for immunomodulatory therapy for autoimmune encephalitis are highly expected.</p>

著者

冨本 秀和

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.34, no.3, pp.188-192, 2017 (Released:2017-10-14)

参考文献数

19

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In recent years, outstanding progress has emerged in neuroimaging research of Alzheimer's disease (AD), which enables us to diagnose patients even in preclinical stages. However, it still remains uncertain to develop disease modifying therapy for AD. There are new concepts on the mechanism of tau and amyloid β accumulation, and its understanding may open an efficient way for therapeutic strategies for AD. This review focuses on the recent developments on the diagnosis and treatment of dementia.

著者

目崎 高広

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.34, no.4, pp.359-362, 2018 (Released:2018-02-20)

参考文献数

20

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Botulinum toxin has been widely applied to therapeutic and cosmetic uses since 1980's. In Japan two formulations, one type A and one type B, are available under strict official regulations. Off–label use is prohibited, and the reimbursement of national insurance is too much restrictive to encourage clinicians of the next generation to launch the practice of this highly effective treatment. Moreover, many of the doctors in Japan are too busy to learn and perform this relatively time–consuming but financially unrewarded procedure. These hardships may regrettably result in the future decline, both quantitative and qualitative, of practice in the clinical setting, as far as Japan is concerned.For injection, the author prefers 27–30 gauge thin needles to thicker ones, to minimize patient's agony, and whenever possible tries to accurately identify target muscles under the monitoring with electromyography, electrical stimulation, or ultrasonography, for the treatment of the limb or deep cervical muscles. Especially for the obliquus capitis inferior and semispinalis cervicis muscles, the ultrasound is superior to other monitoring techniques.In spasticity many patients have anticoagulant or antiplatelet drugs for the secondary prevention of cardio– or cerebro–vascular diseases, and special care should be taken to avoid massive intramuscular bleeding in these patients. The consensus about the adequate use of these drugs still remains to be confirmed.Antitoxin antibody may abolish the toxin effect, but significant number of patients may show apparent secondary resistance to the therapy due to another reasons than neutralizing antibody. Target sites, dose, and technical issues should be re–assessed before abandoning treatment whenever the toxin effect appears to have diminished.

著者

相澤 仁志

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.35, no.6, pp.S156, 2018 (Released:2018-11-12)

著者

岩田 淳

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.34, no.3, pp.283-287, 2017 (Released:2017-10-14)

参考文献数

17

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Although enormous efforts have been made to develop anti–amyloid therapy against mild to moderate Alzheimer's disease (AD) dementia, no single drug has succeeded in clinical trials. These failures could be attributed to 1) inaccurate clinical diagnosis, and 2) inappropriate timing for intervention. The clinical diagnosis used in previous trials are clinical criteria with low sensitivity and specificity. Thus new criteria that incorporated biomarker results has been established. In addition, various longitudinal studies revealed that Alzheimer's disease develops dementia long after it's pathological starting point where almost 15 years of asymptomatic stage precedes mild cognitive impairment (MCI) due to AD or prodromal AD. This asymptomatic stage is now called “Preclinical AD” and thought to be a promising intervention period for anti–amyloid therapy. We started an observational study in Japan which is funded by the Japan Agency for Medical Research and Development (AMED) recruiting 500 individuals in total, that follows cognition, biomarker samples, and multimodal images for 3 years. Moreover, in the US, there are multiple drug intervention studies for preclinical AD subjects. Hopefully, these efforts could help developing AD prevention.

著者

髙嶋 博

出版者

日本神経治療学会

雑誌

神経治療学

巻号頁・発行日

vol.34, no.4, pp.472-473, 2018

著者

近藤 直英 佐橋 健太郎 飯田 円 藤内 玄規 祖父江 元 勝野 雅央

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.34, no.2, pp.101-106, 2017 (Released:2017-07-25)

参考文献数

50

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Spinal and bulbar muscular atrophy (SBMA), also known as Kennedy disease, is an adult–onset neuromuscular disease caused by an expansion of a CAG triplet–repeat within the first exon of the androgen receptor gene. Recent studies have indicated that SBMA is not a pure neurodegenerative disease, but affecting various non–neuronal tissues, including skeletal muscle. Furthermore, impairment in the interaction between motor neurons and skeletal muscles may be the key mechanism underlying the pathophysiology of SBMA. In the presence of testosterone, a disease–causing abnormal AR protein accumulates in the brainstem, spinal anterior horn, skeletal muscle, and several peripheral organs ; this induces a variety of symptoms, such as bulbar and muscular atrophy, liver dysfunction, and arrhythmia, in patients with SBMA. Studies using animal models have resulted in the development of promising therapeutic strategies for SBMA, including 1) testosterone suppression, 2) mutant androgen receptor gene silencing, 3) activation of protein quality control, and 4) rescuing of mitochondrial function. Efficacy of physical therapy has also been tested in small–scale clinical trials. In this review, we describe the clinical features and pathogenesis of SBMA, and summarize the therapeutic drug targets developed on the basis of results from recent studies using the well–established mouse models for this neuromuscular disease. Strategies for the development of disease–modifying therapy supported by basic experiments as well as well–planned clinical trials may lead to a breakthrough in the therapy for SBMA.

著者

近藤 達也

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.33, no.2, pp.92, 2016 (Released:2016-08-10)

著者

松本 理器

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.38, no.4, pp.437-440, 2021 (Released:2022-04-28)

参考文献数

9

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In the super–aging society like Japan, neurologists are highly expected to treat patients with epilepsy, which incident and prevalence has bimodal peaks in the young and elderly population. In year 2020, various old and new generation anti–epileptic drugs (AEDs) become available. Monotherapy is recommended, but rational polypharmacy is the second choice when a few monotherapy trials fail. Medical treatment should be tailored to each patient by taking account of epilepsy/seizure classification, comorbidity such as cognitive impairment, psychiatric symptoms and visceral impairment, drug–interaction profile, side–effect profile, and medical cost, in reference to the available guidelines. When the patient is considered medically intractable, neurologists should refer the patient to epileptologists in the epilepsy center for the re–evaluation of the diagnosis and the work–up for epilepsy surgery using the state–of–the art examinations such as the long–term video–EEG monitoring.

著者

冨本 秀和

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.39, no.3, pp.113-116, 2022 (Released:2022-11-22)

参考文献数

20

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Amyloid β (Aβ) is produced in the neurons and transported into the vessel walls and perivascular space through the extracellular space. These pathways are designated as intramural periarterial drainage (IPAD) pathway or glymphatic system in the brain. Overproduction of Aβ or alternatively, stagnation of Aβ transportation may lead to accumulation of amyloid fibrils in the vessel, thereby causing cerebral amyloid angiopathy (CAA). Spontaneous antibody production against Aβ occurs in patients with CAA, and induce CAA–related inflammation (CAA–ri). In terms of cellular mechanism and symptomatology, this entity is very close to amyloid–related imaging abnormalities (ARIA) which is often encountered in the patients during the course of amyloid vaccination treatment. This review overviews mechanisms of Aβ clearance in the brain and discuss on the Aβ antibody–related pathological conditions, including CAA–ri and ARIA.

著者

勝野 雅央 土方 靖浩 橋詰 淳 祖父江 元

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.35, no.4, pp.427-431, 2018 (Released:2019-04-22)

参考文献数

18

被引用文献数

1

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Spinal and bulbar muscular atrophy (SBMA) is a hereditary neuromuscular degenerative disease characterized by slowly progressive muscle weakness and atrophy. The cause of SBMA is the expansion of a trinucleotide CAG repeat encoding a polyglutamine tract within the first exon of the androgen receptor (AR) gene. The ligand–dependent nuclear accumulation of the polyglutamine–expanded AR protein is central to the gender–specific pathogenesis of SBMA. The pathogenic AR–mediated neurodegeneration is suppressed by androgen inactivation, the efficacy of which has been tested in randomized controlled trials. Decreases in serum creatinine, a biomarker of SBMA, occurred approximately 15 years before the onset of subjective muscle weakness. The mean serum creatinine concentration is ∼0.6mg/dl and ∼0.8mg/dl at the onset of weakness and hand tremor, respectively. The low serum creatinine concentration in subjects with SBMA is caused by impaired muscle uptake of creatine due to the pathogenic AR–mediated down–regulation of creatine transporter SLC6A8, in addition to being caused by neurogenic atrophy.

著者

高井 良樹 三須 建郎 藤原 一男 青木 正志

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.39, no.3, pp.282-288, 2022 (Released:2022-11-22)

参考文献数

51

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MOG (Myelin oligodendrocyte glycoprotein) is a myelin protein expressed exclusively in the central nervous system (CNS). MOG has long been studied as a target antigen for autoimmune inflammatory demyelinating diseases of the CNS because of its distribution in the outermost layer of the myelin sheath and its structural features as a member of the immunoglobulin superfamily. Recently, the detection of conformation–sensitive MOG antibodies has led to the establishment of the clinical concept of MOG–antibody associated disease (MOGAD) as an independent autoimmune demyelinating disease. The clinical phenotypes include acute/multiphasic disseminated encephalomyelitis, optic neuritis, myelitis and brain stem and cerebral cortical encphalitis, whereas MOG antibodies are rarely detected in typical multiple sclerosis.The treatment of MOGAD is divided into acute and chronic phases. The acute treatment is mainly provided by high dose methylprednisolone. The response to the treatment is generally good, and most cases recover without severe sequelae. On the other hand, there is still no established treatment to relapse prevention. The currently accepted approach is to treat the acute phase of the disease followed by 3–6 months of oral steroid therapy, reassessment of MOG antibodies, and continued maintenance treatment in patients with persistent positive MOG antibodies. For patients who become negative for MOG antibodies, it may be appropriate to discontinue maintenance therapy and follow–up. However, the pathogenesis of MOGAD is complex, with variability in response to B–cell removal therapy between cases. We hoped that the pathophysiology of MOGAD will be further elucidated in the future.

著者

清水 俊夫

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.40, no.4, pp.580-584, 2023 (Released:2023-11-27)

参考文献数

52

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Weight loss is frequently observed in early–stage amyotrophic lateral sclerosis (ALS) and is considered an independent predictor of survival. Weight loss observed in ALS is associated with multifactorial etiology, including muscle wasting and dysphagia. Recent studies have implicated disease–specific hypermetabolism as one of causes of weight loss in ALS. Involvement of the hypothalamus in ALS has been the other topic in metabolic dysfunction in ALS. TDP–43 protein aggregates detected in the hypothalamic subnuclei may be associated with weight loss or abnormalities of eating behavior in patients with ALS. Nutritional intervention to maintain body weight could become one of disease–modifying therapies, and recent studies have reported that slowing of weight reduction rate after diagnosis was associated with better survival and that a high–calorie fat diet improved survival in patients with rapidly progressive disease. Nutritional education regarding a high–calorie diet, weight control, and early gastric tube placement is required at the time of diagnosis. Formulas to estimate the recommended daily energy intake for patients with early–stage ALS were reported from USA, Europe and Japan. Multidisciplinary team approach and rehabilitation is necessary to support patients with swallowing disturbance. Surgical intervention to prevent aspiration is often needed for patients who frequently develop aspiration pneumonia.

著者

仙石 錬平

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.40, no.4, pp.463-466, 2023 (Released:2023-11-27)

参考文献数

14

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One of the clinical characteristics of elderly Parkinson disease (PD) is an increased incidence of postural reflex impairment, falls, and freezing of gait compared to other age groups with PD. Aging is considered a risk factor for PD, in addition to genetic and environmental factors. Abnormal protein accumulation associated with aging has been observed in several proteins, including tau, amyloid beta, argyrophilic grains, and TDP–43, all of which show an increased incidence with age. However, the presence of Lewy pathology, a hallmark of PD, is reported to decrease after the age of 80, and may be absent in cases over 100 years old. Thus, it may be said that the pathological feature of PD in the elderly is characterized by the absence of Lewy pathology in cases over 100 years old.

著者

上野 真一 波田野 琢 服部 信孝

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.40, no.4, pp.455-457, 2023 (Released:2023-11-27)

参考文献数

15

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Parkinson disease (PD) is a neurodegenerative disease with various motor and non–motor symptoms such as bradykinesia, rigidity, tremor, constipation, sleep disturbance, cognitive dysfunction, and depression. Although multiple risk factors including aging, genetic predisposition, and environmental factors are involved in the pathogenesis of PD, the number of patients with PD has been increasing in recent years in the world. Previous reports have shown that the severity of PD is high in elderly patients from the early stage of the disease and that a particularly high percentage of them have symptoms such as impaired postural instability, freezing of gait, hallucination, and cognitive dysfunction that reduce their quality of life (QOL). In addition, it is necessary to take into account factors that may reduce motor function, such as frailty, cerebrovascular disease, and spinal disease, as well as the effects of multiple medications for comorbid diseases and the environment surrounding the patient, such as elderly caregivers. In this section, we discuss future issues, focusing on the epidemiology of PD.

著者

齊藤 勇二

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.40, no.4, pp.458-462, 2023 (Released:2023-11-27)

参考文献数

14

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The average life expectancy of Japanese people in 2021 is 81.47 years for men and 87.57 years for women. Because of recent improvements in nutrition and sanitation, as well as the advances in medical care, it is predicted that the average life expectancy will reach 100 years, so–called the era of 100–year–life. Although the lifespan is getting longer, very few people will be free from disorder throughout their lives, and the majority will live with various diseases, especially chronic diseases.Parkinson disease (PD) is the second most common, progressive neurodegenerative disorder, and its global incidence is on the rise, due to the aging of the world's population. “Elderly PD patients” are divided into two groups : patients with PD those who have been living with PD for a long time and those who develop PD at an old age. In the clinical practice for the elderly with PD, it is indispensable for clinicians to keep complex physical conditions, such as pharmacokinetics, comorbidities, and polypharmacy in “the elderly” in mind, as well as “PD” itself. In addition, reduced physical activity in the elderly could make it difficult to attend distant medical facilities; therefore, clinicians should provide PD care in their lives locally. In other words, the essence of clinical practice for the elderly with PD is not only to provide specialized “PD” treatment but also to treat and care for “the elderly”.In general, it is difficult to establish evidence in the medical field for the elderly, because each elderly has a wide variety of background factors, including comorbidities or polypharmacy, which could be biases in the clinical research. Japan is one of the first countries in the world to face a super–aging society, and we are in a position to establish evidence about the clinical practice for “the elderly patients with PD”. We need to discuss what we can and should do for the elderly patients with PD and to provide evidence, preparing for the world's PD pandemic.