臨床神経学
Online ISSN : 1882-0654
Print ISSN : 0009-918X
ISSN-L : 0009-918X
50 巻 , 11 号
選択された号の論文の144件中1~50を表示しています
第51回日本神経学会総会(2010 年)
会長講演
  • 辻 省次
    2010 年 50 巻 11 号 p. 771-777
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    The 51st Annual Meeting of the Japanese Society of Neurology was held in Tokyo (Tokyo International Forum) from Thursday, May 20 to Saturday, May 22, 2010 with as many as 5,471 attendants. Our Society has been celebrating its 50th anniversary during the period from 2009 through 2010. At the 51st Annual Meeting in 2010, we looked toward the future, as we celebrate our 50th anniversary together with distinguished guests closely related to our Society.
    The theme for the 51st Annual Meeting was set as "Future of Neurology-Breakthrough to the next stage-." As represented in the theme, I hope that the Annual Meeting provided an excellent opportunity for all of us to look ahead to the future of Neurology and our Society in the next half-century.
    We have achieved tremendous advances in better understanding neurological diseases and developing more efficacious treatment over the last half century. Great strides have been made in all areas, of which diagnostic imaging, molecular genetics, immunology and physiology are just a few examples, and understanding of diseases has similarly taken a great leap forward.
    In Japan, the aging of society coupled with the declining birthrate has placed ever-increasing expectations on neurologists to provide better care for dementia, cerebrovascular disorders and neurodegenerative diseases. Given this situation our Society is required to provide outstanding education in both the pre- and post-graduate context, and, furthermore, to ensure that excellent training programs are available for young neurologists preparing for Board certification.
    Looking towards the future of neurology, we should continue to anticipate new, ground-breaking achievements for better understanding neurological diseases and establishing more effective treatment through our ongoing endeavors.
Plenary Lecture
2009年度受賞講演
楢林賞受賞講演
  • 村田 美穂
    2010 年 50 巻 11 号 p. 780-782
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    要旨:臨床経験をもとに抗パーキンソン病薬ゾニサミド(ZNS)を開発した.2000年に痙攣発作を併発したパーキンソン病患者にZNSを投与したことをきっかけに,臨床研究,大規模治験を通して,ZNSが抗てんかん薬としての常用量よりもきわめて少ない,25mg 1日1回投与で進行期パーキンソン病患者の運動症状を著明に改善し,長期的にも効果を維持することを明らかにし,ZNSは2009年3月抗パーキンソン病薬として承認された.抗パーキンソン効果の作用機序として,チロシン水酸化酵素mRNA発現増加をともなうドパミン合成亢進と中等度のモノアミン酸化酵素阻害作用を明らかにし,T型Caチャネル阻害作用の関与を示唆した.一方,ZNSはグリアおよびニューロンを標的に複数の機序で各種パーキンソンモデルで神経保護効果を呈することを示した.今後,ゾニサミドの神経保護作用について臨床的に検証すること,SNP検索などをもちいて抗パーキンソン効果の個人差の機序を明らかにしていく必要がある.
Education Program 1
  • 木村 和美
    2010 年 50 巻 11 号 p. 783-786
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    Recently, diagnosis and treatment for transient ischemic attack (TIA) and acute stroke is greatly changing in Japan. Now, TIA is closed up because it has been clarified that TIA attack is very high risk for following stroke. Therefore, TIA patients should be immediately evaluated TIA etiology and be treated after TIA attack as soon as possible in order to prevent following stroke. Medical equipment for stroke such as ultrasound and MRI is improving. In particular, development of MRI study including DWI, T2*, FLAIR, and MRA resulted in accurate diagnosis and etiology of super acute ischemic stroke, and paradoxical embolism, arterial dissection, and aortogenic embolism can be diagnosed in acute stroke patients. t-PA therapy has been approved by Japanese government since 1995, October. t-PA therapy can improve patient outcome due to early recanalization of occluded brain artery. The early recanalization rate was approximately 50% of major artery occlusion. We reported that early recanalization depended on time from stroke onset to IV-t-PA administration. Furthermore, we shown that the large ischemic lesions on diffusion-weighted imaging done before-PA infusion and presence of M1 susceptibility vessel sign were predictor for no-early recanalization and poor outcome. Stroke unit consisting stroke doctors, stroke nurse, and rehabilitation staff can improve patient outcome. In this way, management for acute stroke is greatly changing in Japan.
Education Program 2
Education Program 3
  • 吉良 潤一
    2010 年 50 巻 11 号 p. 788-793
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    視神経脊髄炎(neuromyelitis optica,NMO)は,NMO-IgGの発見により多発性硬化症(multiple sclerosis,MS)とは病態機序が異なる独立した疾患とする説が有力となっている.NMO-IgGが認識するaquaporin-4(AQP4)がアストロサイトの足突起に存在する水チャネル分子であることから,NMO-IgG(抗AQP4抗体)が,アストロサイトの足突起上のAQP4に結合し補体を活性化することでアストロサイト死を誘導するとされる.他方,Baló病は脱髄層と非脱髄層が交互に同心円状に分布する特徴的な病理像を示す.このような同心円状病巣は,MSやNMOでもみられることがある.私たちは,Baló病巣では脱髄巣も非脱髄巣もふくめて広汎にAQP4が脱落していることをみいだした.Baló病では,血管周囲性に抗体や補体の沈着はみられず,血清抗AQP4抗体も陰性であることから,抗AQP4抗体非依存性アストロサイトパチーが,オリゴデンドロサイトパチーを誘導して,脱髄をひきおこすとの新しい説を提唱している.同様な血管周囲性の抗体や補体の沈着をともなわないAQP4の脱落は,MSやNMOの病巣でもみられることがあり,自己抗体非依存性アストロサイトパチーは広く脱髄性疾患に共通するメカニズムである可能性が高い.
Education Program 4
  • 桑原 聡
    2010 年 50 巻 11 号 p. 794-796
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    Crow-Fukase syndrome, also called POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome, is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan involvement. The pathogenesis of Crow-Fukase syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably mediated by monoclonal proliferation of plasma cells, is likely to be responsible for most of the characteristic symptoms. There is no established treatment regimen. In appropriate candidates, high-dose chemotherapies with autologous peripheral blood stem cell transplantation is highly recommended, because this treatment could result in obvious improvement in neuropathy as well as other symptoms, with a significant decrease in serum VEGF levels. Indication of this treatment has not yet been established, and long-term prognosis is unclear at present. Thalidomide should be considered for patients who are not indicated for transplantation therapy. Treatments that should be considered as future therapy include lenalidomide, bortezomib, and anti-VEGF monoclonal antibody (bevacizumab).
Education Program 5
  • 水澤 英洋
    2010 年 50 巻 11 号 p. 797-802
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    Human prion diseases are classified into 3 categories according to etiologies: idiopathic of unknown cause, acquired of infectious origin, and genetic by PRNP mutation. The surveillance committee have analyzed 2,494 cases and identified 1,402 as prion diseases. Most of them are idiopathic, namely sporadic CJD (77%) with less genetic and acquired prion diseases (17% and 5%, respectively). The number of patients identified by the surveillance committee in these years is about 120 which are less than the number of annual death of prion disease. The difference might be due to partly the fact our surveillance need the consent from patients' family and is not complete. The mean age at onset of prion disease is late 60s while the range is fairly wide. Brain MRIs and increase of CSF 14-3-3 and tau protein levels are very characteristic. Classical sporadic CJD could show completely normal T1WI with patchy high signals in the cerebral cortex and basal ganglia on DWI. In Japan, classical sporadic CJD (MM1) is most popular but there are some rare atypical subtypes. Among them, MM2-thalamic CJD is hardest to diagnose because it shows no high intensity signals on DWI, in addition to frequent absence of CSF and EEG characteristics. In this case, CBF decrease in the thalamus on SPECT is very helpful. Genetic prion diseases in Japan are quite distinct from those in Europe. V180I and M232R mutations are unique to Japan and show sporadic CJD phenotype. Dura graft-associated CJD (dCJD) are composed of 67% of classical sporadic CJD phenotype and 33% of atypical phenotype showing slower progression with amyloid plaques. Trace-back experiments suggested the PrPsc of the atypical dCJD was likely to be modified from infection of abnormal VV2 protein. Although there are some atypical forms of prion diseases as mentioned above, almost all prion cases could be diagnosed with EEG, MRI, genetic test, CSF test and SPECT. We also have some incidents in which brain surgery was done before the diagnosis of prion disease and many other patients were operated using the same operating instruments before their sterilization against prion disease had been done. The explanation of possibility of prion disease infection to the patients and their follow-up was started by the incident committee. It is very important for all the nations to cooperate with each other in order to overcome this intractable disease.
Education Program 6
  • 松本 英之, 宇川 義一
    2010 年 50 巻 11 号 p. 803-807
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    磁気刺激法が開発されて20年以上経過し,臨床神経学の場面で様々に応用されている.単発刺激は臨床検査としてもちいられており,反復刺激はその長時間持続する効果から治療応用が期待されている.臨床検査でもっとも一般的なのは中枢運動伝導時間(CMCT)の測定であるが,ここでは脳幹磁気刺激法,小脳磁気刺激法の有用性についても述べる.将来の治療法として反復刺激の研究が進んでいるが,ここではサルで判明している反復刺激の効果と,本邦の大規模調査で示されたパーキンソン病患者に対する反復刺激の有効性について解説する.更に,今後の治療応用が期待される新しい反復刺激法(QPS)についても触れる.
Hot Topics 1
Hot Topics 2
Hot Topics 3
  • 田中 惠子
    2010 年 50 巻 11 号 p. 813-815
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    Varieties of autoantibodies are known to relate to autoimmune neurological disorders as the diagnostic and therapeutic markers. Some of them affected directly to the pathomechanisms of neurological diseases. Recently several autoantibodies with such roles have been reported showing the common characters as recognizing cell surface antigens. Among them, anti-aquaporin 4 antibody (AQP4-Ab) in neuromyelitis optica (NMO) and anti-NMDA receptor antibody (NMDAR-Ab) in non-herpetic limbic encephalitis are drawn considerable attention. The features of NMO with AQP4-Ab are as higher age at onset, extreme women preponderance, severe optic neuritis and myelitis with longitudinary extended spinal cord lesions. AQP4-Ab binds to the astrocytic endfeet extended toward cerebrospinal fluid space or vessel wall, related to the common lesions of NMO, and passive transfer of the antibody with complements to rodents showed NMO pathology.
    The NMDAR-Ab related encephalitis is seen in young women having ovary teratoma showing memory and consciousness disturbances, agitation, epilepsy, respiratory failure, autonomic disorders and involuntary movements. We showed this antibody really affects to NMDAR specific signal transduction using rodent hippocampal slices with suppression of long-term potentiation induction.
    The discovery of newly characterized autoantibodies with relation to certain neurological diseases will be expected to expand in the future.
Hot Topics 4
  • 平 孝臣
    2010 年 50 巻 11 号 p. 816-819
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    バクロフェン髄腔内投与治療(Intrathecal baclofen therapy,ITB)はGABA-B受容体のアゴニストであるバクロフェンを脊髄髄液腔内に持続的に注入し,その他の治療で十分にコントロールできない重症の痙縮を治療するものである.本治療はバクロフェンを脊髄の後角の抑制性神経細胞のみに作用させることが目的であり,経口投与の数百分の1程度の微量の薬剤で強力な効果がえられる.薬剤投与には体内に植え込んだ持続薬液投与ポンプから微量のバクロフェンを脊髄髄液腔内に慢性注入するものであり,精度が高く何年もの長期間にわたって安定して動作する植え込み型ポンプという機器に大きく依存している.このような機器にまつわるトラブルにより離脱症候群などの合併症もまれではなく,注意深いフォローアップ体制が不可欠である.
Hot Topics 5
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Hot Topics 7
  • 笠井 清登
    2010 年 50 巻 11 号 p. 822-824
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    The editorial of the new-year issue of Nature 2010 features "A decade for psychiatric disorders". The DALY estimation clearly shows that psychiatric disorders are the top source for burden of diseases to the individual life and society. Schizophrenia is a most devastating psychiatric disorder in which the onset is usually at youth and the cognitive dysfunction persists for life-long in some patients. Schizophrenia is associated with neurodevelopmental abnormalities. It has been unknown whether post-onset progressive pathology is also present in schizophrenia until the recent sophistication of in vivo neuroimaging techniques. Longitudinal neuroimaging studies on first-episode schizophrenia have shown a progressive deterioration of structure and function of neocortical regions in the early stage of the disorder. Insult to dendritic spines through glutamatergic dysfunction may underlie this process, which may in turn be a promising molecular target for intervention to improve the functional outcome of schizophrenia. More recently, the question of whether early intervention can be targeted at prodromal stage of schizophrenia has called special attention in psychiatry. In University of Tokyo, the integrative neuroimaging studies for schizophrenia targeting early intervention and prevention (IN-STEP) is ongoing. Through these efforts, we would like to contribute to the establishment of "youth mental health", where every youth in the community can know, prevent, and have easy access to needs- and value-based services, and pursue mental well-being and recovery.
East Asian Neurology Forum 1
East Asian Neurology Forum 2
East Asian Neurology Forum 3
East Asian Neurology Forum 4
  • Chih-Ping Chung, Han-Hwa Hu
    2010 年 50 巻 11 号 p. 828-829
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    The presentation discussed certain issues in the management and research of cerebrovascular diseases in Taiwan. In the first part of the presentation, the acute management of stroke in Taiwan, I have revealed the results of the Taiwan Thrombolytic Therapy for Acute Ischemic Stroke (TTT-AIS) study (rt-PA). TTT-AIS was a multicentre, observational study, which enrolled 244 eligible patients with acute ischemic stroke from 23 hospitals from December 2004 to July 2008. The standard-dose group (0.9mg/kg) had higher rates of symptomatic ICH and mortality within three months, twice that of the lower-dose group. This pattern was more prominent in older patients. Besides, significantly lower independence rate was also observed among patients ≥70 years old receiving standard-dose than lower-dose. This study suggests that the standard dose of alteplase 0.9mg/kg may not be optimal for treating aged Chinese. However, the optimal dose of rt-PA for ischemic stroke in Chinese should be based on more broad and convincing evidences. Randomized trials of lower versus higher dose of rt-PA are needed. The second part of the presentation discussed about the relationship between age-related cerebral white matter lesions (leukoaraiosis) and jugular venous reflux (JVR), one of the plenty researches about cerebral venous insufficiency in Taiwan. The presentation introduced current evidences and rationales favoring venous ischemia as a role in the pathophysiology of leukoaraiosis.
シンポジウム1:神経内科における大規模臨床治験
シンポジウム2:アカデミア発の創薬・治療研究
シンポジウム3:神経難病の克服―単一遺伝子病からのアプローチ―
  • 水澤 英洋, 辻 省次
    2010 年 50 巻 11 号 p. 848
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
  • 福武 敏夫
    2010 年 50 巻 11 号 p. 849-851
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    CARASIL (Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy) is the second known single-gene disorder directly affecting cerebral small vessels. The acronym CARASIL was proposed by Bowler and Hachinski (1994), based on its recessive inheritance and resemblance to CADASIL (R instead of D). The first CARASIL patients were most probably described in preliminary reports in 1965-66, and later in Japanese and English articles in 1969-1976. In 1985, the author and colleagues reported on another family of three brothers with strikingly similar clinical features, including not only neurological symptoms but also recurrent acute lumbago and premature alopecia, and cerebral white matter disease on CT scans, proposing that these characteristics can constitute a new systemic syndrome. According to our clinical and pathological/neuroradiological criteria, similar patients have been reported, almost exclusively from Japan, with a total reaching 50 until today. In five consanguineous families including ours, Hara et al. (2009) identified homozygous mutations in the HTRA1 gene on chromosome 10q25. Since no founder haplotype has been identified, the author and allied researchers suspect that this disorder will be found more widely. This review summarizes the historical background, epidemiology, characteristic clinical findings, neuroimaging, and clinical perspectives after the gene identification of this disorder.
  • 原 賢寿
    2010 年 50 巻 11 号 p. 852-854
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    Hypertension is a well known risk factor for cerebral small vessel disease (SVD) characterized by MRI white matter hyperintensities called "leukoaraiosis". However, the molecular basis of SVD remains to be elucidated. Both twin and family studies have shown that leukoaraiosis is the most heritable cerebrovascular phenotype with a heritability estimated to be between 55% and 71%, suggesting genetic factors for SVD. Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is hereditary SVD lacking hypertension. We have recently identified the causative gene, FHtrA1, for CARASIL by genome-wide linkage study and a candidate gene approach. HtrA1 is a serine protease that represses signaling by TGF-β family members. We found that mutated HtrA1 did not repress signaling by the TGF-β family members (BMP2, BMP4, and TGF-β1), resulting in vascular fibrosis with synthesis of extracellular matrix proteins. Our results indicate that disinhibition of TGF-β signaling underlies the molecular basis of CARASIL. Marfan's syndrome is an autosomal dominant connective tissue disorder caused by disinhibition of TGF-β signaling associated with FBN1 mutations. In a small cohort study, angiotensin II-receptor blockers (ARBs) therapy in patients with Marfan's syndrome significantly slowed the rate of progressive aortic-root dilatation. This study provides a potential for developing a therapy targeting TGF-β signaling for SVD.
  • 宮嶋 裕明, 河野 智
    2010 年 50 巻 11 号 p. 855-857
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    Wernicke's encephalopathy is a syndrome characterized by ataxia, ophthalmoplegia, and confusion with thiamine deficiency. We reported on two Japanese brothers with a newly discovered recessively inherited syndrome similar to Wernicke's encephalopathy that developed in the second decade of life; this syndrome was manifested clinically as thiamine-responsive diplopia, ataxia and confusion without serum thiamine deficiency. The patients had complex partial seizure. The administration of high-dose thiamine improved these symptoms. MRI of the brain showed high-intensity signals in the bilateral medial thalamus and periaqueductal region on fluid-attenuated inversion recovery images; these signals were characteristic of findings in Wernicke's encephalopathy. There was no history of chronic alcoholism. The clinical and images features resembling Wernicke's encephalopathy in these patients suggested that the syndrome was caused by a genetic disorder of thiamine metabolism. Genomic analysis of SLC10A3 encoding human thiamine transporter 2 revealed that the patients were compound heterozygotes for the K44E and E320Q mutations. Gene-expression analyses of mammalian culture cells showed that intracellular thiamine uptake activities were decreased significantly. High expression of SLC19A3 RNA in the thalamus may explain the selective thalamic lesions on MRI. The identification of this syndrome proves insight into the thiamine metabolism associated with Wernicke's encephalopathy in humans.
  • 渡瀬 啓, 石川 欽也, 水澤 英洋
    2010 年 50 巻 11 号 p. 858-860
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
    Spinocerebellar ataxia type 6 (SCA6) is one of the common dominantly inherited ataxias in Japan, featuring late-onset ataxia and selective Purkinje cell (PC) degeneration. Molecular pathogenesis of SCA6 has been attracting considerable attention since it is caused by small CAG repeat expansions within the Cav2.1 voltage-gated Ca++ channel gene (CACNA1A). During the past 9 years, efforts have been made to generate and analyze a precise SCA6 model in order to disclose its molecular pathogenesis in vivo. Evidence indicates that the SCA6 mutation does not directly change the basic properties of the channel but rather exerts neurotoxicity through a mechanism associated with age-dependent accumulation of the expanded polyglutamine protein. We envisage further analysis on a knockin model developing PC degeneration at their young age will lead to elucidation of the molecular pathways involved in SCA6 and thus be useful for developing therapeutic strategies against the disease.
  • 青木 正志
    2010 年 50 巻 11 号 p. 861
    発行日: 2010年
    公開日: 2011/03/28
    ジャーナル フリー
シンポジウム4:パーキンソン病の分子病態機序のブレークスルー
シンポジウム5:中枢神経の免疫疾患とグリア
シンポジウム6:脳血管障害治療の次のブレークスルーを目指して
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