Anti-disaster measures along with disaster medicine aims at reducing loss of property and life and facilitating grief work of the suffered people. In contrast the care system for patients with intractable disease has the same aim. According to the experiences of two large earthquakes including Chuetsu (2004) and Chuetsu-oki earthquake (2007), earthquake-resistant buildings are necessary for maintaining hospital function as well as reviving community after occurrence of large earthquake. A list of patients living with ventilator and their individual care plan designed for disaster need to be prepared to transport each patient to the hospital at appropriate timing, when electricity and visiting nurse care system are damaged. Satellite telephone is very useful for communicating with such patients and medical teams because telephone connection is limited to only the specific calling number just after occurrence of earthquake.

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In japan, many patients equipped with TMV are under medical treatment at home after 1990. These patients can't put out sputa in trachea, so that these patient's family members must suck these patient's intratracheal sputa all days. Mr Yamamoto and Mr Tokunaga, main researchers of this study, began the study on the automatic SS of itratracheal sputa from 1999. In first stage, They developed the intermittent SS in detaining the suction tube within tracheal cannula, monitering the intratracheal pressure, but this system takes the ventilation away from the patient. Hypoventilation caused by this SS may cause the serious accident in patient. Therefore, we remodel the SS from intermittent SS to rollerpomp-type SS continuing to suck the itratracheal sputa with low volume from 2004, and thereafter we made up the SS of piston pomp type-SS finally at 2007.
We developed the tracheal cannula with double suction holes of inner and lower hole in the lower part of its cannula together with the suction machine.
We think that the practical use of this automatic SS will bring these patients with TMV and their family members great benefits. We desire that the practical use of this SS will be realized as soon as possible.

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Parkinson's disease (PD) is the most common neurodegenerative disease after Alzheimer's disease. Although the pathogenetic mechanisms underlying PD is largely unknowm, it is widely believed that multiple genetic as well as environmental factors play critical roles in the development of PD. Alpha synuclein (a-SYN) and the disturbance of a-SYN metabolism seems to play the most important role in PD pathogenesis. a-SYN, the gene causative in PARK1, is the major component of Lewy bodies. Gene duplication or triplication of a-SYN leads to autosomal dominant form of familial PD, suggesting that disturbance of a-SYN may represent the etiology of sporadic PD. GBA and LRRK, the genes responsible for Gaucher disease and PARK8, respetively, are also likely to affect the development of PD. Studies of PD brains suggest that mitochondrial impairment and oxidative stress may contribute to the pathogenesis of sporadic PD. Mitochondrial complex I inhibitors, such as MPTP and rotenone, induce selective dopaminergic neuronal death, suggesting that chemicals may constitute risk factors of sporadic PD. Not only continuous infusion, but oral intake of rotenone is effective to establish animal models of PD. Further studies will reveal genetic or environmental risk factors, that are the good targets for prevention and therapy of PD.

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It is proposed that α-synucleinopathy initially affects the medulla oblongata and then progresses to more rostral brain areas ("Braak hypothesis"). According to this hypothesis, substantia nigra is affected in the later stages of PD. Another region affected in the earlier stages was reported to be olfactory bulb, although the following processes were not described in detail. On the other hand, several lines of evidence suggest that non-motor symptoms including constipation, depression, REM-sleep behavior disorder (RBD) and hyposmia may be prodromal symptoms in PD. The pathological staging postulated by the Braak hypothesis is in good agreement with the fact that these non-motor symptoms precede motor symptoms in PD, because affected brain areas in the early stages, such as dorsal vagal nucleus, locus ceruleus and olfactory bulb, are related to these non-motor features. Recently, it was reported that although half of brains corresponded to the Braak hypothesis, there were a high proportion of cases which did not fit the Braak's staging system and majority of the latter demonstrated amygdale-predominant α-synucleinopathy. It was also demonstrated that the Lewy pathology in olfactory bulb was closely related to the presence of alpha-synuclein pathology in amygdala. The amygdala is one of the main systems in odor perception and in PD, cortical neurons in corticomedial complex of amygdale, which have major olfactory connections, are selectively affected even in the early stages of the disease. We recently obtained the data suggesting that metabolic changes in the amygdala were associated with low scores in odor identification test. These data suggest that not only the olfactory bulb, but also the amygdala is also responsible for hyposmia in PD and that there may be another pathological process, which starts from the olfactory bulb and involves the amygdala.

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Advances in the field of stem cell research have raised hopes of creating novel cell replacement therapies for Parkinson disease (PD), although double-blinded clinical trials have met with controversial success in patients implanted with fetal midbrain tissue and autopsy results have shown that some of the grafted fetal neurons displayed pathological changes typical of PD. Dopaminergic neurons have been efficiently derived from stem cells using various methods, and beneficial effects after transplantation have been demonstrated in animal models of PD. Some obstacles remain to be overcome before stem cell therapy can be routinely and safely used to treat PD in humans. A widely used prodrug/suicide gene therapy would be applied to stem cells to reduce risk of tumor formation. Since grafts were transplanted ectopically into the striatum instead of the substantia nigra in most current protocols, surviving dopaminergic neurons would not have to be the same subtype as the nigral cells. If the main mechanism underlying any functional recovery achieved by cell therapies is restoration of dopaminergic neurotransmission, then viral vector-mediated gene delivery of dopamine-synthesizing enzymes represents a more straightforward approach. Future targets for cell therapy should include some types of Parkinsonism with degeneration of striatal neurons.

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Understanding of the pathogenesis of neuromyelitis optica (NMO) is rapidly growing. In our immunohistochemical studies from 2006, the loss of AQP4 was evident in about 90% of NMO lesions, especially in perivascular areas of acute inflammatory lesions where immunoglobulins and complements were deposited. Glial fibrillary acidic protein (GFAP) was also weak or lost in those lesions. In contrast, myelin basic protein (MBP)-stained myelinated fibers were relatively preserved in those lesions where AQP4 was completely lost. In contrast to NMO lesions, AQP4 and GFAP were preserved or increased in demyelinating MS lesions. The loss of AQP4 in acute inflammatory lesions was evident in the largest areas compared with GFAP or MBP, which probably suggested the primary loss of AQP4 on astrocytes and the secondarily demyelination. In contrast, the immunostaining patterns in more chronic lesions of NMO mostly lacked AQP4 but were necrotic heterogeneously with demyelination and gliosis, or completely burn-out. Swelling and regressive changes of astrocytes were easily evident. In addition, the lesions lacking AQP4 was appeared by passive-transferred Lewis rats with human purified IgG from NMO patients. Accordingly, these evidences strongly suggest its humoral autoimmune astrocytopathy in the pathomechanism of NMO.

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Recently a specific auto-antibody response has been found in patients with neuromyelitis optica, which is directed against the astrocytic water channel aquaporin 4. In experimental models these antibodies do not induce disease or lesions in the central nervous system, when present in the circulation of normal rats. However, when T-cell mediated inflammation is induced in such animals, circulating antibodies against aquaporin 4 can reach the brain and induce lesions in the central nervous system, which are closely similar to those, seen in patients with neuromyelitis optica.

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The commonly used clinical diagnostic criteria for NMO still requires the presence of clinical episodes of both optic neuritis and myelitis. We believe this criteria has disadvantages for making early diagnosis in patients who fail to meet the criteria yet have the same disease process. Therefore we propose a simple new criteria which requires one of the following two: (1) centrally located, fully contiguous long spinal cord lesion (LCL) extending three or more spinal segments length or long segmental spinal cord atrophy and (2) presence of specific antibody to aquaporin-4 (AQP4). We also propose a new naming "autoimmune astrocytopathy" for such patients, since the autoimmune destructive process against astrocytes is the characteristic common underlying mechanism, the majority of patients show the clinical brain symptoms, and anti-AQP4 antibodies are frequently, but not exclusively, associated. The reanalysis of Japanese interferon β-1b clinical trial data failed to show efficacy in patients meeting the diagnostic criteria for multiple sclerosis and showed MRI evidence of LCL. Although there is no evidence-based guideline for NMO, expert opinions generally agree with the treatment strategy. My treatment algorithm is shown in the table. At acute exacerbation, high-dose steroid infusion is the choice. If two cycles of infusion treatment fails to show recovery, plasma-exchange treatment should be initiated quickly. Medium dose oral steroids are started immediately after the end of high dose steroid infusion therapy. In moderate to severe activity cases additional usage of one of the immunosuppressants, Mizoribine, Tacrolimus, or Azathiopurine is quite useful, and the immunosuppressive effects on NMO are higher in this order, but the safety is higher at the opposite order. Therefore, selection of one of immunosuppressants should be made on individual basis and slow achievement of the immunosuppressive effects should be kept in mind especially in case of Azathiopurine. If any of these immunosuppressants are not successful in inducing stable state, then Mitoxantrone or Tituximab is the choice. The monthly infusion of former drug usually stablizes the disease activity quite quickly, but treatment duration is limited due to it's cardiotoxicity. Rituximab is also quite powerful in stabilizing the activity, but there still exist some non-or poor responder to this medicine, and insurance coverage is not expected for MS or NMO in Japan. Cyclophosphamide infusion is also an alternative choice for very difficult cases. Physicians working on NMO treatment in Japan are very much hoping the initiation of well controlled clinical trials in order to treat our patients with more scientific strategy based on evidences.

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Spinocerebellar ataxia (SCA) is a group of degenerative ataxias with autosomal dominant inheritance. The most common form of mutation that causes SCA is the expansion of trinucleotide (CAG) repeat encoding polyglutamine. These "polyglutamine disorders" are, SCA1, SCA2, Machado-Joseph disease, SCA6, SCA7, SCA17 and DRPLA. Another dynamic mutation, yet a non-coding one, has been identified as the cause of SCA8, SCA10 and SCA12. This mutation includes, trinucleotide (CAG/CTG) expansion causing SCA8 and SCA12, and pentanuclotide (ATTCT) expansion leading SCA10. In addition to these dynamic mutations, static mutations, such as missense mutations and deletions, have been identified to cause SCA5, SCA11, SCA13, SCA14, SCA15 and SCA27.
Since 1992, authors have been involved in identifying the mutation (s) of autosomal dominant cerebellar ataxia with rather pure cerebellar syndrome (ADCAIII). About a half of our cohort with ADCAIII were SCA6, caused by a small CAG repeat expansion in the α1A-voltage-dependent calcium channel gene. Recent study in patients' brains suggested that a small polyglutamine expansion leads a portion of this channel protein to aggregate in the Purkinje cell. Another type of ADCAIII is the chromosome 16q22.1-linked ADCA. By a comprehensive positional cloning strategy, we have found a genetic change that segregate with the disease. Identifying the mutation of 16q-ADCA is imperative for understanding molecular basis of this disease.

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The intracellular accumulation of unfolded or misfolded proteins is believed to contribute to aging and age-related neurodegenerative diseases. However, the links between age-dependent proteotoxicity and cellular protein degradation systems remain poorly understood. Here, we show that 26S proteasome activity and abundance attenuate with age, which is associated with the impaired assembly of the 26S proteasome with the 19S regulatory particle (RP) and the 20S proteasome. In a genetic gain-of-function screen using Drosophila, we characterized Rpn11, which encodes a subunit of the 19S RP, as a suppressor of expanded polyglutamine-induced progressive neurodegeneration. Rpn11 overexpression suppressed the age-related reduction of the 26S proteasome activity, resulting in the extension of flies' life spans with suppression of the age-dependent accumulation of ubiquitinated proteins. On the other hand, the loss of function of Rpn11 caused an early onset of reduced 26S proteasome activity and a premature age-dependent accumulation of ubiquitinated proteins. It also caused a shorter life span and an enhanced neurodegenerative phenotype. Our results suggest that maintaining the 26S proteasome with age could extend the life span and suppress the age-related progression of polyglutamine diseases.

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Abnormal aggregation and deposition of misfolded proteins have been recognized as a common molecular pathogenesis of various neurodegenerative diseases including Alzheimer's, Parkinson's, and the polyglutamine (polyQ) diseases. The polyQ diseases, including Huntington's disease and various spinocerebellar ataxias, are caused by abnormal expansions of the polyQ stretch (>35-40) within disease-causative proteins, which are thought to trigger their misfolding and aggregation, leading to their deposition as inclusion bodies, and eventually resulting in neurodegeneration. We found that the expanded polyQ protein undergoes a conformational transition to a β-sheet dominant structure in the monomeric state, triggering cytotoxicity, and subsequently resulting in formation of insoluble amyloid-like fibrillar aggregates. Targeting misfolding and aggregation of the expanded polyQ protein, we demonstrated that QBP1 (PolyQ-Binding Peptide 1: SNWKWWPGIFD) prevents the toxic β-sheet transition and aggregation of the expanded polyQ protein in vitro and suppresses polyQ-induced neurodegeneration in Drosophila. From high-throughput screening of a chemical compound library (46,000), we have identified approximately 100 polyQ aggregate inhibitors as therapeutic candidates so far. We also found that 17-AAG, an HSF1-activating compound, suppresses polyQ-induced neurodegeneration in Drosophila through induction of endogenous molecular chaperones. We propose that our therapeutic strategy targeting protein misfolding and aggregation can also be applied to other neurodegenerative diseases.

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球脊髄性筋萎縮症（SBMA）は成人男性に発症する運動ニューロン疾患であり，アンドロゲン受容体遺伝子（AR）のCAGくりかえし配列の異常延長を原因とするポリグルタミン病である．病因蛋白質である変異ARが運動ニューロン内に蓄積し，転写障害や軸索輸送障害など様々な細胞機能低下をひきおこすという病態仮説が提唱されている．変異ARの蓄積はテストステロンに依存しており，男性ホルモン抑制剤であるリュープロレリン酢酸塩の有効性がSBMA患者を対象とした第II相臨床試験で示されている．今後SBMAの治療法開発を更に進めるためには，多彩な分子生物学的アプローチと，その効果を臨床試験で検証するための方法論を確立する必要がある．

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老人斑の脳内沈着はアルツハイマー病（AD）を特徴づける病理像であり，その生体画像化はAD早期診断における有用な指標となる．¹¹C-PIBを代表とする数多くのβシート結合薬剤がアミロイドイメージング用PETトレーサーとして開発され，軽度認知障害（MCI）の段階での早期診断精度が大幅に向上した．健常成人の中にも本検査で異常所見を示す症例が一定の割合で存在することから，発症前段階でのアミロイドの沈着が示唆される．ただし予後との関係は不明であり，発症前診断法としてのエビデンスを確立するには長期縦断研究による検証が求められる．βシート結合プローブをもちいた本検査は，線維化蛋白の蓄積するアルツハイマー病以外のミスフォールディング病にも応用可能である．

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ミクログリアは脳実質内グリア細胞の10%を占め，安静時ではramified型をして繊毛突起を出して絶えず移動して免疫監視作用にかかわっている．ところが，脳組織が傷害を受けると傷害された神経細胞やアストロサイトからの刺激によってameboid型と形を変え，ミクログリアが傷害側まで誘導される．血管障害や変性疾患などの神経疾患だけでなく，直接的な脳病理学的所見が不明確とされる精神疾患においても，神経細胞やアストロサイトの異常によってミクログリア活性が上昇していることが最近報告されている．活性化ミクログリアはグルタミン酸神経シナプスなどを剥離して異常興奮を抑制する神経保護に関与する一方，炎症性サイトカインを放出し細胞傷害を惹起する．すなわちミクログリアの活性化こそ脳内での炎症の存在を示す証拠となる．この神経炎症を死後脳でなく，生きた脳で捉えることは疾患の病態を評価し，治療方針の決定に重要となる．活性化したミクログリアには，末梢性ベンゾジアゼピン受容体が多数発現し，その受容体に結合するトレーサーとPETを使うことで可視化できる．様々なトレーサー開発がおこなわれているが，中でも[¹¹C]（R）-PK11195は感度は低いが世界で広く臨床利用されているPETトレーサーである．このトレーサーは傷害性ミクログリアと保護的ミクログリア（果たして末梢性ベンゾジアゼピン受容体の差で差別化できるか疑問であるが）の区別なく，活性化したミクログリアを検出することができる．本シンポジウムでは神経・精神疾患の患者脳におけるミクログリア活性について述べる．

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Nonclinical and clinical evidences have supported the view that accumulations of neurotoxic amyloid components initiate chain reactions of molecular and cellular pathologies, eventually leading to neuronal death and symptomatic onsets of neurodegenerative diseases. As this amyloid-triggered cascade is virtually composed of bidirectional causalities between upstream and downstream events, it is of critical significance to monitor all key processes, including amyloidosis, neuroinflammation, disrupted calcium homeostasis and impaired neurotransmissions, in living brains toward therapeutic regulations of the entire cascade. Positron emission tomography (PET) offers quantitative mapping of these alterations with the aid of multiple classes of radioprobes. Comparative PET assays of humans and animal models in conjunction with cognitive, biochemical and histopathological assessments have revealed toxic subspecies of amyloid β peptide, tau proteins and microglia detectable by specific molecular probes. Dysregulated neurotransmissions are also capturable by PET techniques, while it should be noted that the accessibility of binding components to exogenous radioligands does not simply reflect their amounts but also is affected by their translocation, posttranslational modifications and interaction with endogenous ligands and other molecules. Clarification of these changes in target elements brings mechanistic insights into the molecular etiology of neuropsychiatric disorders.

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For the research on human higher brain functions, both neuropsychological and neuroimaging studies are useful, but each of these two methodologies has merits and demerits. In neuropsychological studies, damaged regions are necessary for the normal execution of cognitive functions underlying the symptoms that patients show. However, it is usually difficult to determine to what extent the lesion has affected a specific psychological subprocess amongst several subprocesses related to the symptoms. On the other hand, in neuroimaging studies, activated regions may not be necessary for but simply participate in a targeted higher brain function. However, if we can set up an adequate experimental design, it provides an opportunity to identify brain regions related to a specific psychological subprocess necessary for the targeted higher brain function. In this symposium, I talked about several neuroimaging studies using positron emission tomography and functional magnetic resonance imaging. We can take advantage of neuroimaging techniques for the research on human brain functions, but it should be kept in mind that neuroimaging techniques are not the purpose of the research but simply one of the useful means.

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プリオン病の研究の歴史は，神経学会が始まってからの50年に集約される．ニューギニアの地方疾患であったkuruから，感染性の疾患であることを証明し，神経内科医のもっとも恐れるCJDがどのようにしてプリオン病と呼ばれるようになったかを解説し，これから新興しうるプリオン病まで言及した．

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The Creutzfeldt-Jakob Disease (CJD) Surveillance Committee has identified 1,241 patients with prion diseases during 1999-2009, including 953 with sporadic CJD (sCJD)(76.8%), 207 with genetic prion diseases (16.7%), 78 with environmentally acquired prion diseases (6.3%), and 3 with unclassified CJD. Among atypical cases of sCJD, most common was MM2 type including the cortical and thalamic forms. The genetic cases included 84 with a PrP V180I mutation (40.6%), 37 with a P102L mutation (17.9%), 34 with a E200K mutation (16.4%), 32 with a M232R mutation (15.5%), 4 with a P105L mutation (1.9%), and so on. The environmentally acquired cases included 77 with dura mater graft-associated CJD (dCJD) and one with variant CJD (vCJD). Combined with the results by the previous surveillance systems, a total number of dCJD in Japan was 135. The vCJD patient had a history of short stay in the UK and presented with periodic electroencephalogram in the late stage. Although there was no evidence of association of surgical procedures or blood transfusion with sCJD, 4.5% of the sCJD patients underwent operations after the onset of sCJD, including neurosurgical for 0.8% and ophthalmic for 1.9%, requiring more attention on prion diseases to reduce the iatrogenic risk.

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There is no established treatment for prion diseases; however, recently several drug candidates, including pentosan polysulfate and doxycycline, have been clinically used on a trial basis to prevent accumulation of abnormal prion protein in the brain. So far the outcome of the trials is still very far from the goal where a complete cure of the diseases is expected. In order to bridge the gap between the reality and the ideal, the followings are suggested. First, combination therapy needs to be developed against multi-targets: inhibition of prion replication; degradation and scavengery of prion; inhibition of prion-related neurodegeneration. Secondly, preclinical diagnostic means, by which healthy prion-carriers can be revealed before the onset of the diseases, should be explored for earlier therapeutic interventions. The last is to disclose intrinsic disease susceptibility factors and environmental factors, both of which could solely or jointly facilitate in suppressing prion replication and disease progress. Exploitation of these items should be tough but will be deserved for overcoming the fatal diseases.

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Hereditary neuropathies are classified into HMSN/Charcot-Marie-Tooth disease (CMT), familial amyloid polyneuropathy (FAP), hereditary motor neuropathies (HMN) and hereditary sensory (and autonomic) neuropathies (HSAN). The clinical features of HMSN are generally characterized as distal dominant motor and sensory involvements. However, we have reported a novel HMSN with proximal dominancy (HMSN-P) originated in Okinawa and Shiga prefectures, Japan. The gene locus is located in the centromere region of chromosome 3. In 2008, a new family with the HMSN-P was reported from Brazilians of Japanese ancestry. This Brazilian family was initially diagnosed as having "a familial ALS". The HMSN-P linked to ch.3 is not limited in Japan, but may be present in the worldwide. The overseas scientific research for the elucidation of the mechanism of HMSN-P supported by JSPS KAKENHI (21406026) is planning. Recently several other types of HMSN-P have been reported; HMSN-P with urinary disturbance and paroxysmal dry cough, a patient with both CMT 1A and mild spinal muscular atrophy and CMT1A with severe paresis of the proximal lower limb muscles. Therefore the clinical concept of HMSN is not limited as the disease with distal dominant motor sensory involvement. HMSN has the wider spectrum from distal to proximal and motor/sensory to autonomic neuropathies.

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Familial amyloid polyneuropathy (FAP) was long considered to be an incurable disease, but a new therapeutic approach was developed 15 years ago. As the liver produces most of the transthyretin (TTR) in serum, it was assumed that the replacement of a liver expressing an abnormal TTR gene should stop the production of the variant TTR, the serum amyloid precursor in FAP. Until now about 1,500 FAP patients underwent liver transplantation, and the 10-year-survival rate is about 77%. After operation the progression of FAP symptoms certainly stopped, and patients who were in an early stage of the disease and underwent successful operations showed considerable improvement in their quality of life. Electrophysiological study of peripheral nerve function has demonstrated that liver transplantation can halt the progression of peripheral neuropathy in FAP patients, and histopathological regression of amyloid deposits was seen on the patients with long post-transplatation courses. Pharmacological therapies have been considered for FAP patients and among them, diflunisal, one of non-steroidal anti-inflammatory drugs, is very promising. TTR tetramer dissociation is an initial step for the process of TTR-derived amyloid fibril formation associated with FAP and diflinisal can inhibit this process by stabilization of the TTR tetramer. Clinical trial of this drug for FAP patients is now going worldwide.

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Such neuropathies as Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and IgM paraproteinemic neuropathy are caused by autoimmune mechanisms. IgM paraproteinemic neuropathies are known to be intractable. Rituximab has recently been reported to be effective for IgM paraproteinemic neuropathy with anti-MAG IgM M-protein in a placebo-controlled trial. The effect should be confirmed with a larger trial. The use of this drug also may be tried for other type of IgM paraproteinemic neuropathy and for intractable CIDP in future. Antiganglioside IgG antibodies are frequently present in the acute-phase sera from GBS patients. Recently, presence of the antibodies that recognize a conformational epitope formed by carbohydrate portions of two different gangliosides (ganglioside complex) has been reported. Antibodies against a complex formed by GD1a and GD1b (anti-GD1a/GD1b antibodies) are associated with severe GBS. Anti-GM1/GalNAc-GD1a antibodies are found to be associated with pure motor GBS with frequent conduction block. Anti-ganglioside complex antibodies may be useful diagnostic and prognostic markers of GBS. Future study is necessary to clarify the pathogenetic mechanisms in which those antibodies are specifically involved.

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It is important to know the cellular properties of endoneurial microvascular endothelial cells (PnMECs) and microvascular pericytes which constitute blood-nerve barrier (BNB), since this barrier structure in the peripheral nervous system (PNS) may play pivotal pathophysiological roles in various disorders of the PNS including inflammatory neuropathies (i.e. Guillain-Barré syndrome), vasculitic neuropathies, hereditary neuropathies and diabetic neuropathy. However, in contrast to blood-brain barrier (BBB), very few studies have been directed to BNB and no adequate cell lines originating from BNB had been launched. In our laboratory, we successfully established human immortalized cell lines originating from BNB using temperature-sensitive SV40 large T antigen and the cellular properties of human cell lines are presented in this paper. Human PnMEC cell line showed high transendothelial electrical resistance and expressed tight junction components and various types of influx as well as efflux transporters that have been reported to function at BBB. Human pericyte cell line also possessed tight junction proteins except claudin-5 and secrete various cytokines and growth factors including bFGF, VEGF, GDNF, NGF, BDNF and angiopoietin-1. Co-culture with pericytes or pericyte-conditioned media strengthend barrier properties of PnMEC, suggesting that in the PNS, peripheral nerve pericytes support the BNB function and play the same role of astrocytes in the BBB. Future accumulation of the knowledge concerning the cellular properties of BNB-forming cells will open the door to novel therapeutic strategies for intractable peripheral neuropathies.

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The first medical society of Japanese neurologists and psychiatrists was founded in 1902, but psychiatrists gradually dominated in number. New "Japanese Society of Neurology" (JSN) was founded in 1960. The number of members was only 643 in 1960, while it rose up to 8,555 in 2009, including regular, junior, senior and associate members. JSN contributed much to solve the causes and treatment of the medicosocial and iatrogenic diseases such as Minamata disease and SMON (subacute myelopticoneuropathy) at its early period. In undergraduate education at medical school, neurology is one of the core subjects in the curriculum, and almost all the 80 medical schools have at least one faculty neurologist. The Board of neurology of JSN was started in 1975, as the third earliest of the Japanese Medical Associations. It takes at least 6 years' clinical training after graduating from the medical school to take the neurology Board examinations. By 2009, 4,000 members passed the Board examinations. In 2002 JSN published evidence-based "Treatment Guidelines 2002" of 6 diseases: Parkinson's disease, stroke, chronic headache, dementia and ALS. As to the international issues, JSN hosted the 12^th World Congress of Neurology in 1981, and international activities markedly increased after that. The first informal meeting with JSN and Korean Neurological Association (KNA) was held at the 48^th JSN Annual Meeting in Nagoya in May 2007. In May 2008 the KNA-JSN 1^st Joint symposium was held at the 49^th Annual Meeting of JSN in Yokohama on "International comparison of neurological disorders: focusing on spinocerebellar atrophies (SCA) and epilepsies". In May 2009, KNA-JNS 2nd Joint Symposium was held at the 50^th JSN Annual Meeting in Sendai, inviting a speaker from Taiwan Neurological Society, on the subject "History and Education of Neurology in Japan, Korea and Taiwan". In this symposium, a strategy to make up the Northeast Asian Neurological Association was discussed.

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