Progressive supranuclear palsy (PSP) is a distinct neurodegenerative disorder with symptoms of supranuclear ophthalmoplegia affecting chiefly vertical gaze, pseudobulbar palsy, and postural instability with falls. It is sometimes difficult to distinguish clinically PSP from other parkinsonian syndromes, including corticobasal degeneration (CBD), Parkinson's disease, and multiple system atrophy. To identify the biomarker(s) associated with PSP and CBD for better understanding of a pathophysiology and clinical course, a multicenter registration consortium, the Japanese Longitudinal Biomarker Study in PSP and CBD (JALPAC), was established. Clinical information with biological samples (serum, plasma, and cerebrospinal fluid), gene (genomic DNA and lymphoblastoid cell), and magnetic resonance imaging (MRI) data were collected. The initial registration of 147 cases (78 males ; mean age, 72.6 years), including PSP in 87 patients, CBD in 36, others in 24 and the follow up registration of 32 cases one year later were carried out, and a total of 179 registrations was performed from November 2014 to October 2016. In the initial registration 147 particients, the clinical information, biological samples (serum 142 (100%), plasma 134 (94.4%), cerebrospinal fluid 107 (75.4%)), genomic DNA 133 (96.4%), lymphoblastoid cell 132 (95.7%) and MRI data 96 (67.6%) were stored. JALPAC has established an efficient registration and follow–up system with biological samples, genomic DNA, and MRI data from PSP/CBD patients in Japan. JALPAC will contribute to investigations of the natural history of PSP/CBD and identification of PSP/CBD–associated biomarkers and genes, and will promote clinical research.

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.

Dominantly Inherited Alzheimer Network (DIAN), Dominantly Inherited Alzheimer Network Trial Unit (DIAN–TU), and DIAN–Japan were reviewed. In Japan, participants enrolment is initiated from 2016. These global studies would contribute to clarify the signature of Alzheimer's pathology and validity of biomarkers. These studies now move to clinical trials for prevention of Alzheimer's disease. Thus, strong evidences from DIAN and DIAN–TU are now getting hot attentions and their desirable contribution are waiting.

Initial–phase Intensive Support Team (IPIST) is composed of multi–disciplinary experts in dementia, such as nurses, public health nurses, occupational therapists, psychiatric social workers, care workers, et cetera. When the team gets a request from person concerned, that there's a possible need to support for an individual who has dementia or who is suspected to have dementia, the team visits his or her home to assess the condition and environment. After the initial home visit, a team member meeting is held to make a plan for support program under the assistance of experienced physician in dementia treatment and support. Following the plan, the team provides an integrated and intensive support to enable the person with dementia to keep control of his or her own life as long as possible. The support continues until a certain degree of effect is confirmed and the community services including home doctors and other social resources take over the support. Furthermore, the team conducts ex–post monitoring. According to the New Orange Plan, every municipality in Japan is required to implement IPIST by the end of JFY 2017.

In Osaka City, an initial dementia support team was established as a model project at one region comprehensive support center in 2014. In 2015 three wards were examined with different doctor requirements and in 2016 it expanded to 24 teams in all 24 wards. Most of the team members doctors are non-specialists, the dementia disease medical center laid the backup system. Dementia support physicians who were in charge of the team members told us that participation in this project made it easier to recommend medical consultation to specialized medical institutions, through cooperation with local doctors and district medical associations. The cooperation with medical institutions became smooth, and regional collaboration was promoted, and the opinion that the role of dementia support doctor in the region became clear has been increasing.

There are a lot of elderly living alone or those who are refused support among the recipients. In addition, as the need for juvenile dementia has also arisen, the Community Support Promotion Committee, together with the team, will be placed at the same regional comprehensive support center, support to difficult cases will be required, and cases where continuous support beyond 6 months are required. It was here to be responsible for assisting. For the future development of this project, strengthen regional networks and promote relationships. It is thought that support based on a long-term perspective such as human resource development is necessary. It is suggested that this project is effective as an activity in the city.

Japan has reach to a super aging society. Especially, Dementia measures are an important subject. For that reason, Initial–phase Intensive Support Team for dementia started. As one of them, health professional staffs visit the user's home. They advise to the family, and support users in a short period. They can deal flexibly by going around various places. As a result, we can get the cooperation of medical, nursing care and the region. With that Dementia people were able to the social inclusion. Role of Occupational therapists is to understand users from the cognitive empathy perspective. Further, it is important to prepare the living environment with family and other specialists.

City of Setagaya established 27 regional community comprehensive care centers since 2006. All are managed by private consignment. In addition, we have been working on comprehensive dementia measures.

In 2013, we established the “dementia home life support center concept” to establish an early response system for dementia and to enhance future dementia measures.

At the same time, we started a model project for Initial–Phase Intensive Support Team of Dementia, and we worked to create a mechanism to ensure accurate grasp of people with dementia who needed assistance by the community comprehensive care center, and to early support.

As a basic municipality, in order to realize effective early response and early support of dementia, not only the Initial–Phase Intensive Support Team of Dementia work but also various business and regional services are utilized according to the needs of the target. It is important to demonstrate governance that aims to construct a regional comprehensive care system for dementia under cooperation and collaboration with citizens, nursing care providers, regional organizations, etc.

In the district, since the study of the dementia home life support center concept was started, in consideration of establishing the early dementia support team in 2013, consideration of the business scheme and the management system Securing, making decisions within the administration, explaining to the Congress, obtaining budget, etc.

82歳女性．呼吸困難のため当院入院．両側性声帯麻痺，末梢神経伝導検査で多発ニューロパチーを認め免疫グロブリン療法1クール，副腎皮質ステロイドパルス療法を2クール施行し改善した．8か月後に再度両側性声帯麻痺を認め，副腎皮質ステロイドパルス療法を2クール施行し改善，prednisolone（PSL）加療を継続した．11か月後にも再び両側性声帯麻痺を生じたが，副腎皮質ステロイドパルス療法1クールを施行し改善した．以後はPSLにAzathioprine（AZT）を併用し安定した．monoclonal gammopathy of undetermined significance（MGUS）とされるIgMλ型M蛋白血症を認め，これによるニューロパチーを考慮した．免疫治療により改善するM蛋白血症併発両側性声帯麻痺の存在が示され，本邦初論文として報告する．

症例は74歳男性．発疹と共に四肢，体幹の筋力低下が進行し，その半年後に嚥下障害が出現した．検査の結果，皮膚筋炎と診断し，高容量のステロイド内服（prednisolone 60mg/day）を開始したが，ステロイドミオパチーにより，状態はさらに悪化した．6週間後に免疫グロブリン大量静注療法（intravenous immunoglobulin：IVIg）（0.4g/kg/day×5days）を併用し，四肢の筋力低下や筋原性酵素の上昇は比較的早期に回復に向かったが，1回のIVIgで，嚥下障害への効果は十分ではなかった．IVIgは，月1回ペースで計3回施行した．反復唾液嚥下テストは0回から3回に回復したが，依然として嚥下障害は重度であり，経皮内視鏡的胃瘻造設術を施行した．嚥下造影検査では食道入口部の開大不全が顕著であった．その後，輪状咽頭筋部の機能不全に対して，バルーン拡張法をほぼ毎日2ヶ月間繰り返した所，嚥下障害はさらに回復に向かい，入院から9ヶ月後にはゼリー食の摂取が可能となった．本症例のごとく皮膚筋炎の難治性嚥下障害にステロイド治療やIVIgのみで十分な効果が得られない場合には，バルーン拡張法による輪状咽頭筋部の機能不全の改善が有用なことがあり，試みるべきである．