Clinical Practice Guideline
for Dementia 2017

Publication of the English Version of“Clinical Practice Guideline for Dementia 2017”
The first “Treatment Guideline for Dementia” was released in 2002. This was followed by publication of the “Treatment
Guideline for Dementia 2010” using the Clinical Question (CQ) format in 2010. In 2014, revision of the “Treatment
Guideline for Dementia 2010” was decided. Around this time, preparation of the English version of the “Clinical Practice
Guideline for Chronic Headache 2013” was in progress, aiming to be published in 2015. Furthermore, the XXIII World
Congress of Neurology was scheduled to be held in Kyoto in September 2017. These events prompted a plan to publish theEnglish version of the revised treatment guideline for dementia.
In August 2017, the “Clinical Practice Guideline for Dementia 2017” in Japanese language was published. However, theEnglish version was not ready in time for the XXIII World Congress of Neurology. Nevertheless, discussions were continued
toward publication of the English version as originally planned.
After the publication of the “Clinical Practice Guideline for Dementia 2017” (Japanese version), some questions andopinions were received. The Committee addressed these issues and made some revisions, and at the same time simplified thecontents to some extent to prepare the text for the English version of the Guideline.
The policies of developing clinical practice guidelines are gradually evolving, and the current guideline is expected to berevised in the future. For the development of future guideline, some issues in the “Clinical Practice Guideline for Dementia
2017” have to be addressed. These will be discussed in the next revision. With anticipation to develop further improved andmore refined clinical practice guideline for dementia in the future, we embarked on preparing the English version of “Clinical
Practice Guideline for Dementia 2017”.
Kenji Nakajima
Chairman
Dementia Clinical Practice Guideline Development Committee
Japanese Society of Neurology

ii

Clinical Practice Guideline for Dementia 2017

Preface:
Clinical Practice Guideline for Dementia 2017, Japanese version
July 2017

The first “Treatment Guideline for Dementia” was published in 2002. Then in 2010, six academic societies; namely, theJapanese Society of Neurology, Japanese Society of Neurological Therapeutics, Japanese Society of Psychiatry and Neurology,
Japan Society for Dementia Research, Japan Geriatrics Society and Japanese Psychogeriatric Society, jointly developed the“Treatment Guideline for Dementia 2010” using the Clinical Question (CQ) format. Thereafter, with the addition of some
new knowledge, the “Treatment Guideline for Dementia 2010, Compact Edition” was published in 2012. Subsequent to thispublication, revision of the guideline was discussed at the Japanese Society of Neurology Guideline Executive Committee,
and revision of the guideline was decided in 2014.

[Target Readers of this Guideline]
Along with the 2002 and 2010 guidelines, the current guideline was prepared assuming that the readers are general
doctors in principle. However, the preparation work was done with the intention that this guideline will also be read bypeople other than doctors.

[Flow of Guideline Revision]
In 2014, after revision of the guideline was decided, chairman of the committee was elected and the office of guideline
preparation was placed in the chairman’s affiliated facility. In the same manner as the previous guideline, the revision task
was to be undertaken jointly by the six dementia-related societies mentioned above. Another five societies were invited toparticipate or collaborate. Committee members, research collaborators, and evaluation/coordination committee members
were elected from the six societies. The method for the revision work was discussed and decided at the committee, and it was
decided that each committee member would provide feedback to his/her academic society as the work progressed. Moreover,although the previous guidelines were called “treatment guidelines”, the contents also included diagnosis. Like the previous
guidelines, the revised guideline is not limited to treatment but covers the entire clinical practice from diagnosis to treatment
of dementia. Therefore, the revised guideline was developed as a “clinical practice guideline”
The Japanese Society of Neurology Guideline Executive Committee considered that although the current revision was inprinciple to be implemented according to the Minds Handbook for Clinical Practice Guideline Development 2014, thesystem was still not fully developed in Japan. As a policy to respond appropriately to the actual situation, it was decided thatthe specific methods for guideline development should be decided through discussions at the Dementia Clinical Practice
Guideline Development Committee.
Based on the above guideline development policy, the guideline: (1) used the CQ format; (2) confirmed the sources offunding for guideline preparation and managed the conflict of interest (COI) of committee members; (3) conducted literaturesearch using a uniform method; (4) discussed and decided evidence level and strength of recommendation based on the
GRADE system recommended by Minds 2014; (5) listened to opinions from patient groups; (6) for CQs in which“recommendation” statement was difficult to compose because the strength of recommendation could not be decided, an“Answer” statement was prepared; (7) the draft guideline was reviewed by the evaluation/coordination committee andexternal committees, and (8) the draft guideline was opened to the public, and comments were invited. Thereafter, the“Clinical Practice Guideline for Dementia” was finalized.

[Funding Sources and Conflict of Interest (COI)]
Funds necessary for preparation of this guideline were borne by the Japanese Society of Neurology. The funds provided
payment of expenses such as meeting room charges for committee meetings and transportation expenses for attending thecommittee, but did not provide remuneration for committee members and research collaborators for drafting manuscripts
and participation in meetings.
This guideline was prepared based on appropriate COI management according to the “Regulations related to preparationof Japanese Society of Neurology clinical practice guideline”, “Guide to preparation of Japanese Society of Neurology
clinical practice guideline” and “Rules for establishment and operation of Japanese Society of Neurology conflict of interest
committee”.

Prefaceiii

Every year, the chairman, vice chairman, committee members, research collaborators, and evaluation/coordinationcommittee members declared COI to the Chairman of Board of Directors of the Japanese Society of Neurology. Declaration
was based on the following criteria: board member renumeration, etc. (1 million yen or more); stocks, etc. (1 million yen or
more, or 5% or more of the total stocks); patent royalty (1 million yen or more); lecture fees, etc. (500,000 yen or more);manuscript fee, etc. (500,000 yen or more); contract research fee, joint research fees, etc. (2 million yen or more, 1 million
yen or more for the 2015 declaration); scholarship (incentive) donation, etc. (2 million yen or more, 1 million yen or more
for the 2015 declaration); endowed chair; provision of travel and gifts (50,000 yen or more).
Enterprises that declared COI are as follows:
Ajinomoto Co., Inc.; Astellas Pharma Inc.; AstraZeneca Co., Ltd.; Igaku-Shoin, Ltd.; Iset Co.; Eisai Co., Ltd.; MSD
K.K.; Otsuka Pharmaceutical Co., Ltd.; Ono Pharmaceutical Co., Ltd.; Kyowa Hakko Kirin Co., Ltd.; GlaxoSmithKline
K.K.; Kowa Pharmaceutical Co., Ltd.; Social and Medical Corporation Kowakai Sapporo Shirakabadai Hospital; Medical
Corporation Seijinkai; Daiichi Sankyo Co., Ltd.; Sumitomo Dainippon Pharma Co., Ltd.; Takeda Pharmaceutical Co.,
Ltd.; Chugai Pharmaceutical Co., Ltd.; Nippon Boehringer Ingelheim Co., Ltd.; Nippon Medi-Physics Co., Ltd.; Novartis
Pharma K.K.; Bayer Yakuhin Ltd.; Pfizer Japan, Inc.; Mochida Pharmaceutical Co., Ltd.; Morimoto Pharma Co., Ltd.;
Janssen Pharmaceutical K.K.

[Process of Guideline Revision, Determination of Level of Evidence and Recommended Grade]
The first committee meeting was held in September 2014, and guideline development work began. First, we invited an
instructor from Minds to confirm the Minds policy for guideline development. We also invited external committee members
to participate, and decided the committee composition, guideline development policy, schedule, and items, and also decided
work sharing. The scope was discussed. The scope of this guideline was decided to cover from diagnosis to treatment andlong-term care for dementia. CQs would be prepared taking into consideration that the guideline is a reference material for
doctors to decide the best methods of clinical care. Comments would be written as general items according to the scope. CQs
were to be formulated with reference to PICO (P: patients, problem, population; I: interventions; C: comparisons, controls,comparators; O: outcomes).
Thereafter, the Guideline Executive Committee decided that this guideline is expected to be written concisely with
contents that support routine clinical care. To address this point, it was necessary to consider the user-friendliness ofguidelines; i.e., users can easily access the content they want to read from the table of contents. Initially, the plan was to limit
CQs to important clinical issues, while general clinical and epidemiological features are described under the preface of eachsection. However, the general features also would have to be presented in a way such that readers can easily find what theywant to read. Eventually, the plan was changed: all the contents are presented based on CQs. This format is similar to thatused in the previous version. For this reason, items concerning specific clinical issues are mixed with items describing generalclinical and epidemiological features, all under CQs (without the initially planned preface). Despite the adoption of thisformat in the present guideline, which is also partially due to consideration of continuity with the previous version, continuous
efforts have to be made to find the optimal format for use in the next revision.
We determined key words (KW) for the CQs and requested Professor Shinichi Abe at the Academic Information Center,
the Jikei University School of Medicine to perform search of literature. Since literature search for the previous guideline wasconducted until 2008, the search period for this guideline was in principle from 2009. Therefore, literature search from 2009
until April 2015 was conducted during May to July 2015. From the reference list obtained from the search, the referenceswere evaluated using evaluation sheets, not only by subcommittee members and research collaborators, but also bycollaborators recommended by each committee member. The level of evidence for each outcome and the body of evidence
were evaluated. For CQs that did not have sufficient references from this search process, the key words were changed asnecessary, and literature search was repeated. Furthermore, additional manual search was conducted when it was deemednecessary by the committee.
Regarding systematic reviews, the system for conducting quantitative systematic reviews including meta-analysis is not yet
adequately established. Therefore, following the policy of the Japanese Society of Neurology Guideline Executive Committee,
quantitative systematic review was a non-binding target, and would be conducted if feasible based on the judgment of eachcommittee member, while the main work was to conduct systematic literature search followed by qualitative systematic
review, as was done in previous guideline development. All the collaborators of this work will be acknowledged with gratitude
at the end of this message.
The level of evidence was evaluated not for individual reference, but by each outcome for studies grouped by study design,
such as randomized controlled trial and observational study. The body of evidence was evaluated for risk of bias, indirectness,iv

Clinical Practice Guideline for Dementia 2017

inconsistency, imprecision, and publication bias. In the next revision, the methods of conducting systematic review including
quantitative systematic review will be thoroughly examined before the work begins. Next, the responsible area for eachcommittee member and research coordinator was decided. Texts for CQs, recommendation statements, and comments andevidence were drafted, and the draft was discussed and decided by all members of the committee.
Table 1. Recommendation grade and level of confidence.
Recommendation grade:
1 (Strong): Recommend to “perform” or “not to perform”
2 (Weak): Propose to “perform” or “not to perform”
Strength of body of evidence:
A: Strong
B: Moderate
C: Weak
D: Very weak
The manuscript thus prepared was reviewed by the evaluation/coordination committee. In addition, external committee
members and persons related to patient organizations were invited to review the manuscript, and this guideline development
committee also participated to listen to their opinions.
Public comments were invited during August 1–21, 2016. The comments received were reviewed and corrections were
made. There were opinions pointing out the need for including more recent references, especially reports from Japan, after
April 2015, which exceeded the period of literature search set for this guideline. This was discussed at the guideline
development committee and additional manual search as needed was adopted. Since the public comments also included arequest to conduct another round of public comment invitation, public comments were again invited during November
16–30, 2016.

[Contents and Items in this Guideline]
This guideline also places importance on continuity, and the contents almost conform to the previous “Treatment
Guideline for Dementia 2010”. The guideline covers general items including definition, epidemiology, symptoms, assessment
scales, diagnosis, examinations, non-pharmacological treatments and pharmacotherapy, interventions for delirium andcoexisting diseases, risk factors, prevention, mild cognitive impairment, severity and interventions by severity, long-termcare, social resources, community liaison, ethics, and legal issues. In addition, for diseases of dementia by etiology, which aredealt with in the section of “Specific Diseases”, Alzheimer’s disease dementia, Lewy body disease, frontotemporal lobar
degeneration (FTLD), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), argyrophilic grain dementia,
senile dementia of the neurofibrillary tangle type, vascular dementia, prion disease, and medical diseases are included.
For PSP, CBD, and Huntington’s disease, although management of motor symptoms is important, including these
discussions will further expand the volume of the guideline. Therefore, it was decided to limit discussions to cognitive
impairment only, which is in line with the previous guideline. Incidentally, a guideline for these diseases has been planned,
which includes motor symptoms and all other disorders except cognitive impairment. The guideline was in the process ofdevelopment as of November 2016.
Although molecular pathological classification shows that FTLD includes PSP and CBD, clinical differentiation of these
diseases is possible based on clinical symptoms and other tests. Furthermore, since FTLD, PSP, and CBD are specified
separately as designated intractable disease in Japan, these three diseases are handled as related diseases and described inseparate chapters in this clinical practice guideline. For differential use of terms between FTLD and frontotemporal dementia(FTD), and between CBD and corticobasal syndrome (CBS), please refer to the descriptions in the corresponding chapters.
On the other hand, since a separate guideline for prion disease has been developed (Clinical Practice Guideline for Prion
Disease 2014), prion disease is described briefly in this guideline while citing the Clinical Practice Guideline for Prion
Disease 2014.
In an evaluation questionnaire after publication of the previous guideline, there was opinion that medical diseases causedby vitamin deficiency should also be included. In this guideline, therefore, vitamin deficiency, hypothyroidism, neurosyphilis,
hepatic encephalopathy, and idiopathic normal pressure hydrocephalus (iNPH) are described. In the next revision, while
reviewing the target diseases to be included in the guideline, it seems necessary to discuss whether the above diseases/items
should be included.

Prefacev

[Names of therapeutic drugs and terms]
In principle, the terms used in the previous “Treatment Guideline for Dementia 2010” are adopted in this guideline.
<Names of therapeutic drugs>
Drug that are approved for dementia treatment in Japan and those that are used clinically even though they are notcovered by health insurance for dementia treatment in Japan are written in katakana. Drugs that are used only overseas but
not used in Japan are written in English.
<“Cognitive impairment” and “behavioral and psychological symptoms of dementia (BPSD)”>“Core symptom” of dementia is described as “cognitive impairment”. The term “peripheral symptoms” is not used, and is
replaced by “behavioral and psychological symptoms of dementia (BPSD)”. A combination of cognitive impairment an
BPSD is described as “dementia symptoms”.
<Description of severity>
Regarding the severity of dementia, the terms “severe” and “high level” are used almost synonymously for the advanced
stage. “Severe” was used in the Treatment Guideline for Dementia 2002 and 2010. Following these previous guidelines,“severe” is used in this guideline.
<Classification related to time of onset: “juvenile dementia”>
Depending on the time of onset, terms such as “juvenile dementia”, “pre-senile dementia”, “senile dementia”, “early-onset
dementia”, and “late-onset dementia” are used. There is opinion that use of the term “juvenile dementia” may not be desirable,
because the same term may denote different age groups [Japan Society for Dementia Research (Ed.): Textbook of Dementia,
2008]. However, the term “early-onset dementia” is used in administrative documents such as “Measures for early-onset
dementia” (2009) (Ministry of Health, Labor and Welfare), as well as the Orange Plan and the New Orange Plan. In
response to these developments, a patient aged under 65 years with dementia is referred to as “early-onset dementia” in thisguideline (see “Chapter 5: Various Systems and Social Resources for Supporting Persons with Dementia and Their Families;
C. Early-onset Dementia”).
<“Alzheimer’s disease” and “Alzheimer’s disease dementia”
The term “Alzheimer’s disease” may refer to the pathological state of the disease or may be used to describe the clinicalsyndrome when dementia symptoms due to “Alzheimer’s disease” have become evident. According to the National Instituteon Aging and Alzheimer’s Association (2011), “Alzheimer’s disease” is defined as a term encompassing the underlying
pathophysiological processes, and “Alzheimer’s disease” is distinguished from “Alzheimer’s disease dementia” which describes
the state of dementia caused by “Alzheimer’s disease”. In Japan, “Alzheimer’s disease dementia” has been used from the past
as a term to describe the state of dementia considered to be caused by “Alzheimer’s disease”. Therefore, in this guideline, theterm “Alzheimer disease dementia” is used for dementia that is considered to have occurred based on the pathologicalbackground of “Alzheimer’s disease”. However, this guideline does not differentiate between “Alzheimer disease dementia”(diagnosed from clinical features) and “Alzheimer disease dementia caused by Alzheimer’s disease” (evidence that dementia
is caused by Alzheimer’s disease has been confirmed), because this differentiation has little utility in clinical practice.
<Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5)>
In DSM-5 published by the American Psychiatric Association in 2013, the term “neurocognitive disorder” was introduced.
This term includes “delirium” and “major and mild neurocognitive disorder”. The Japanese translations for “major and mild
neurocognitive disorder” are “dementia (DSM-5)” and “mild cognitive impairment (DSM-5)”, respectively.
<“Mild cognitive impairment” and “MCI”>
In this guideline, “mild cognitive impairment” and “MCI” are described as ”mild cognitive impairment (MCI)”.
<Others>
Terms including “senile dementia of the neurofibrillary tangle type (SD-NFT)”, “tangle-predominant senile dementia/
NFT-predominant form of senile dementia”, “tangle only dementia”, and “primary age-related tauopathy (PART)” are useduniformly in the guideline.

vi

Clinical Practice Guideline for Dementia 2017

[Using this clinical practice guideline in clinical care for dementia]
This guideline provides reference materials to support the clinical practice and care for dementia with the purpose toimprove clinical practice for dementia, and does not intend to constrain clinical practice for dementia in the clinical setting.
With future developments and changes in medical care and research, as well as diversification of persons with dementia andthe environment surrounding these persons, guidelines are expected to change over time. This guideline does not restrict thedecision of clinicians over treatment, neither does it stipulate medical treatment in response to changes in the clinical setting.
Sometimes the recommendations in this guideline may not apply. There are cases where the physician in charge may perform
treatments that deviate from the descriptions in this guideline, and cases where the deviation may be appropriate. In actual
clinical practice, it is important to formulate an individualized treatment plan that responds to the dementia of each patient,
without being restricted by the contents of this guideline. In addition, the contents described in this guideline do not serveas grounds for medical lawsuits.

[Promotion of utilization of the guideline, future plans including the next revision, and evaluation]
In order to promote the utilization of this guideline, the guideline is scheduled to be introduced and publicized by postingon the websites of the academic societies that participated in guideline development, presented at scientific conferences andother meetings of the academic societies, and published in scientific journals.
For the convenience of readers, a compact edition has been published both for the Treatment Guideline for Dementia
2002 and 2010 editions. For this guideline also, publication of a compact version will be considered. Furthermore, theJapanese Society of Neurology has published the English version of other clinical practice guideline, and we hope that thepossibility of publishing the English version of the Clinical Practice Guideline for Dementia will be discussed at the guideline
development committee.
In the case that addition or correction is necessary due to new knowledge obtained after publication of this guideline,
additional or revised CQ will be prepared as a supplement and posted on the society website. In addition, the JapaneseSociety of Neurology has a policy of revising clinical practice guidelines every five years. This guideline development
committee will also consider the next revision and composition of the next guideline development committee, and the results
will be submitted to the Japanese Society of Neurology Guideline Executive Committee for deliberation.
The previous two guidelines for the treatment of dementia were evaluated by an evaluation committee set up at theJapanese Society of Neurology. It is anticipated that the current Clinical Practice Guideline for Dementia 2017 will also be
evaluated by an evaluation committee.

[Acknowledgment to committee members and research collaborators]
Revision and development of the present guideline were undertaken with the collaboration of six dementia-related
associations. I would like to express my gratitude to all members of the committee and the research collaborators for their
participation in the committee and for their great efforts despite their busy schedules. This guideline was developed with thesupport of many colleagues such as evaluation/coordinating committee members, external committee members, andcollaborators. I would like to thank them all for their collaboration. I would also like to thank Ms. Noriko Ono and Ms.
Yoriko Matsushita from the Tokyo Branch of the Alzheimer’s Association Japan for their opinions in preparing the guideline.
Furthermore, I would like to express my sincere appreciation to those who have provided many valuable comments in theinvitation of public comments. I would also like to thank staff members of Minds for their advice.
Kenji Nakajima
Chairman
Dementia Clinical Practice Guideline Development Committee
Japanese Society of Neurology

Prefacevii

LIST OF AUTHORS
Editing Supervisors:
Japanese Society of Neurology
Japanese Society of Neurological Therapeutics, Japanese Society of Psychiatry and Neurology, Japan Society for Dementia
Research, Japan Geriatrics Society, Japanese Psychogeriatric Society

Editors:
Dementia Clinical Practice Guideline Development Committee
Participating societies: Japanese Society of Neurology, Japanese Society of Neurological Therapeutics, Japanese Society ofPsychiatry and Neurology, Japan Society for Dementia Research, Japan Geriatrics Society, Japanese Psychogeriatric Society

Chairman
Kenji Nakajima: Matsue Medical Center

Vice-chairman
Hidekazu Tomimoto: Department of Neurology, Mie University

Committee members
Ikuko Aeba: Department of Neurology, Higashinagoya National Hospital
Masahiro Akishita: Geriatric Medicine, University of Tokyo Hospital
Shuichi Awata: Tokyo Metropolitan Institute of Gerontology
Setsu Iijima: National Rehabilitation Center for Persons with Disabilities
Takeshi Ikeuchi: Department of Molecular Genetics, Brain Research Institute, Niigata University
Manabu Ikeda: Department of Psychiatry, Osaka University Graduate School of Medicine
Masafumi Ihara: Division of Cerebrovascular Medicine and Neurology, National Cerebral and Cardiovascular Center
Hiroaki Kazui: Department of Psychiatry, Osaka University Graduate School of Medicine
Nobuo Sanjo: Department of Neurology and Neurological Science, Tokyo Medical and Dental University
Mikio Shoji: Department of Neurology, Hirosaki University Graduate school of Medicine
Kyoko Suzuki: Department of Clinical Neuroscience, Yamagata University Graduate School of Medical Science
Makio Takahashi: Department of Neurology, Osaka Red Cross Hospital
Akira Tamaoka: Graduate School of Comprehensive Human Sciences, University of Tsukuba
Seishi Terada: Department of Neuropsychiatry, Okayama University
Aki Nakanishi: Department of Neurology, Osaka City Kosaiin Hospital
Haruo Hanyu: Geriatric Medicine, Tokyo Medical University
Hideo Hara: Division of Neurology, Saga University
Katsuyoshi Mizukami: Faculty of Health and Sport Sciences, University of Tsukuba
Yukihiko Washimi: Department of Brain Function Examination, National Center for Geriatrics and Gerontology
Hirohisa Watanabe: Brain and Mind Research Center, Nagoya University
Kenji Wada: Division of Neurology, Tottori University

Research Collaborators
Haruhiko Akiyama: Dementia Research Project, Tokyo Metropolitan Institute of Medical Science
Tadashi Adachi: Division of Neurology, Tottori University
Atsushi Iwata: Department of Neurology, University of Tokyo
Tomomi Ogihara: Department of Psychiatry, Shinshu University
Naoto Kamimura: Department of Neuropsychiatry, Kochi University
Takayoshi Shimohata: Department of Neurology, Brain Research Institute, Niigata University
Kazuko Hasegawa: Department of Neurology, Sagamihara National Hospital
Osamo Yokoto: Kinoko Espoir Hospitalviii

Clinical Practice Guideline for Dementia 2017

Evaluation/Coordination Committee Members
Takashi Asada: Center for Brain Integration Research, Tokyo Medical and Dental University
Naoji Amano: Okaya City Hospital
Hiroyuki Arai: Institute of Development, Aging and Cancer, Tohoko University
Fukashi Udaka: Sumitomo Hospital
Kochi Okamoto: Geriatrics Research InstituteShun Shimohama: Department of Neurology, Sapporo Medical University
Masatoshi Takeda: Aino University
Akira Honma: Otafuku Monowasure Clinic
Masahito Yamada: Department of Neurology and Neurobiology of Aging, Kanazawa University(Late)Dr. Hideo Mori: Department of Neurology, Juntendo University Koshigaya Hospital

External Committee Member
Masayo Kojima: Department of Medical Education, Nagoya City University

Collaborators
Yusuke Yakushiji: Division of Neurology, Saga University
Makoto Eriguchi: Division of Neurology, Saga University
Masafumi Kosugi: Division of Neurology, Saga University
Jun Tanaka: Division of Neurology, Saga University
Kohie Suzuyama: Department of Neurology, Saga University
Yasuo Harigaya: Japanese Red Cross Maebashi Hospital
Etsuro Matsubara: Department of Neurology, Oita University
Noriyuki Kimura: Department of Neurology, Oita University
Takeshi Kawarabayashi: Department of Neurology, Hirosaki University
Masaki Ikeda: Department of Neurology, Guma University
Kokoro Ozaki: Department of Neurology and Neurological Science, Tokyo Medical and Dental University
Maya Higuma: Department of Neurology and Neurological Science, Tokyo Medical and Dental University
Yoko Ito: Department of Neurology and Neurological Science, Tokyo Medical and Dental University
Fumiko Furukawa: Department of Neurology and Neurological Science, Tokyo Medical and Dental University
Takefumi Sato: Department of Neurology and Neurological Science, Tokyo Medical and Dental University
Ryo Tagawa: Tagawa Kusunoki Clinic
Yumi Kameyama: Geriatric Medicine, University of Tokyo HospitalMichihito Masuda: Department of Neurology, Nagoya University
Kazunori Imai: Department of Neurology, Nagoya University

Prefaceix

Table of Contents
Publication of the English Version of “Clinical Practice Guideline for Dementia 2017”
Preface: Clinical Practice Guideline for Dementia 2017, Japanese version

Chapter 1

Overview of Dementia: Epidemiology, Definitions, Terms

CQ 1-1
CQ 1-2
CQ 1-3
CQ 1-4
CQ 1-5
CQ 1-6
CQ 1-7
CQ 1-8What are the diagnostic criteria for dementia?...........................................................................................  2
What other terms are related to dementia?.................................................................................................  3
What are the causes of dementia and how are they classified?....................................................................  5
What pathological conditions should be differentiated from dementia?.....................................................  6
What is the prevalence of dementia in Japan?............................................................................................  7
Are there changes in prevalence depending on subtype of dementia?.........................................................  8
Is the disease duration of dementia getting longer?....................................................................................  9
What is the pathological background of dementia?.................................................................................... 10

Chapter 2

Syndrome, Rating Scales, Tests, Diagnosis

CQ 2-1
CQ 2-2
CQ 2-3
CQ 2-4

What kinds of cognitive function impairment are found in dementia? ..................................................... 15
What are the behavioral and psychological symptoms of dementia (BPSD)? ............................................ 17
What are the useful scales for assessing cognitive impairment in dementia? ............................................. 18
What are the useful rating scales for behavioral and psychological symptoms of dementia (BPSD),
activities of daily living (ADL), and global severity?.................................................................................. 19
How is quality of life (QOL) evaluated in persons with dementia? ........................................................... 21
How is primary progressive aphasia (PPA) classified and evaluated?.......................................................... 22
How is dementia diagnosed and differentiated?......................................................................................... 24
How are imaging examinations carried out in dementia?.......................................................................... 25
What are the useful blood and cerebrospinal fluid tests for the diagnosis of dementia?............................. 27
Which physical and neurological findings require attention when diagnosing dementia?.......................... 29
What are the drugs that affect the diagnosis of dementia?......................................................................... 30
Are there useful genetic tests for the diagnosis of dementia?...................................................................... 32

CQ 2-5
CQ 2-6
CQ 2-7
CQ 2-8
CQ 2-9
CQ 2-10
CQ 2-11
CQ 2-12Chapter 3 Treatment
A | Overview of Treatment
CQ 3A-1
CQ 3A-2
CQ 3A-3
CQ 3A-4
CQ 3A-5
CQ 3A-6
CQ 3A-7-1
CQ 3A-7-2

How should interventions and support be given following a diagnosis of dementia?................................. 34
How should pharmacotherapy, non-pharmacologic interventions and care be provided during treatment
of dementia? .............................................................................................................................................. 35
What are the principles and precautions of pharmacotherapy in older people with dementia?................... 37
What are the adverse events of psychotropic drugs [such as falls, declined activity of daily living (ADL),
cognitive impairment, and aspiration pneumonia]?.................................................................................... 38
What are the treatment procedures when using pharmacotherapy for dementia?...................................... 39
What are the adverse events due to cholinesterase inhibitors and NMDA receptor antagonists, and whatshould be done in case of these events?..................................................................................................... 40
What are the non-pharmacological therapies for dementia?...................................................................... 42
What are the symptoms that respond to non-pharmacological therapies for dementia?............................. 43

B | Treatment for behavioral and psychological symptoms of dementia (BPSD)
CQ 3B-1
CQ 3B-2
CQ 3B-3
CQ 3B-4
CQ 3B-5x

What are the effective non-pharmacological therapies and pharmacotherapy for anxiety?........................ 44
What are the effective non-pharmacological therapies and pharmacotherapy for agitation?..................... 46
What are the effective non-pharmacological therapies and pharmacotherapy for hallucinations anddelusions?................................................................................................................................................... 48
What are the effective non-pharmacological therapies and pharmacotherapy for depression?.................... 50
What are the effective non-pharmacological therapies and pharmacotherapy for wandering?.................... 52

Clinical Practice Guideline for Dementia 2017

CQ 3B-6
CQ 3B-7

What are the effective non-pharmacological therapies and pharmacotherapy for sleep disturbance(excluding REM sleep behavior disorder)? ................................................................................................ 54
What are the effective non-pharmacological therapies and pharmacotherapy for apathy?.......................... 56

C | Interventions for Coexisting Diseases
CQ 3C-1
CQ 3C-2
CQ 3C-3
CQ 3C-4
CQ 3C-5
CQ 3C-6
CQ 3C-7
CQ 3C-8
CQ 3C-9
CQ 3C-10
CQ 3C-11
CQ 3C-12
CQ 3C-13

How is delirium treated?............................................................................................................................ 58
How epilepsy including seizures should be treated in patients with dementia?.......................................... 59
What are the interventions for dysphagia (including prevention of aspiration pneumonia)?...................... 61
What are the interventions for eating disorder and undernutrition?........................................................... 63
What are the interventions for sarcopenia and frailty?.............................................................................. 64
What are the interventions and preventive measures for falls and fractures?.............................................. 65
What are the interventions for pressure ulcers?.......................................................................................... 67
What are the approaches to treat acute physical illness?............................................................................. 68
How should decisions be made regarding invasive examinations and treatments including dialysis anddental treatment?....................................................................................................................................... 69
What are the interventions for edema?....................................................................................................... 71
What are the interventions for urinary disorder?....................................................................................... 72
What are the interventions for constipation?.............................................................................................. 74
How should diabetes, hypertension and other lifestyle-related diseases be managed?................................ 75

Chapter 4 Clinical Course and Treatment
A | Risk Factors and Protective Factors of Dementia
CQ 4A-1
CQ 4A-2
CQ 4A-3
CQ 4A-4
CQ 4A-5
CQ 4A-6
CQ 4A-7
CQ 4A-8
CQ 4A-9
CQ 4A-10
CQ 4A-11
CQ 4A-12

What are the risk factors and protective factors of dementia?..................................................................... 78
Are hypertension control and antihypertensive drugs effective in dementia prevention? .......................... 80
Is diabetes control effective in dementia prevention? ................................................................................. 82
Is treatment for dyslipidemia effective for dementia prevention? ............................................................... 83
Does metabolic syndrome worsen dementia? ............................................................................................ 85
Does smoking worsen dementia? .............................................................................................................. 86
Is exercise effective in preventing dementia? .............................................................................................. 87
Are leisure activities, social participation, mental activities, cognitive training, and artistic activities suchas music effective in preventing dementia and cognitive decline in older people?....................................... 88
Are there dietary factors associated with dementia? ................................................................................ 90
Is moderate drinking effective in preventing cognitive decline and dementia? .......................................... 91
Does sleep apnea syndrome worsen cognitive function? ............................................................................ 92
Are depression and bipolar disorder risk factors for dementia? .................................................................. 93

B | Mild Cognitive Impairment
CQ 4B-1
CQ 4B-2
CQ 4B-3
CQ 4B-4
CQ 4B-5
CQ 4B-6
CQ 4B-7

What are the prevalence and incidence of mild cognitive impairment (MCI)? ......................................... 94
What is the conversion rate from mild cognitive impairment (MCI) to dementia, and the reversion ratefrom MCI to cognitive normal? ................................................................................................................ 95
What are the useful biomarkers for predicting conversion of mild cognitive impairment (MCI) to
dementia?................................................................................................................................................... 96
What rating scales are recommended when mild cognitive impairment (MCI) is suspected? ................... 98
How is mild cognitive impairment (MCI) diagnosed? .............................................................................. 99
Are there methods to prevent progression from mild cognitive impairment (MCI) to dementia?..............101
What kinds of guidance and support are available for people with mild cognitive impairment (MCI)?.....103

C | Disease Severity and Interventions According to Severity
CQ 4C-1
CQ 4C-2
CQ 4C-3What kind of guidance and support can be given to persons with mild to moderate dementia?................104
What kind of guidance and support can be given to caregivers of persons with mild to moderatedementia? ..................................................................................................................................................106
What kind of guidance and support can be given to persons with severe dementia?..................................107xi

CQ 4C-4
CQ 4C-5

What kind of guidance and support can be given to caregivers of persons with severe dementia?..............108
How should end-of-life care be given to persons with dementia?...............................................................109

Chapter 5 Various Systems and Social Resources for Supporting Persons with Dementia
and Their Families
A | Various Systems and Social Resources for Supporting Medical and Long-term Care for Persons
with Dementia
CQ 5A-1
CQ 5A-2
CQ 5A-3
CQ 5A-4
CQ 5A-5

What are the functions and roles of the medical center for dementia?.......................................................112
What are the roles of certified dementia support doctors? ......................................................................... 114
What are the roles of long-term care insurance system for persons with dementia and theircaregivers?.................................................................................................................................................. 115
What are the functions and roles of Community General Support Center?............................................... 117
What are the functions and roles of the initial-phase intensive support team for dementia?...................... 118

B | Advocacy for People with Dementia
CQ 5B-1
CQ 5B-2
CQ 5B-3

Is it possible to evaluate the abilities of judgment and decision-making in persons with dementia?........... 119
How can the adult guardianship system be used to protect the rights of people with dementia?................121
What role does the Prevention of Elder Abuse Act play in preventing abuse to persons
with dementia? ..........................................................................................................................................123

C | Early-onset Dementia
CQ 5C-1
CQ 5C-2
CQ 5C-3
CQ 5C-4

What is early-onset dementia?....................................................................................................................125
What are the support systems to help persons with early-onset dementia with their economic
challenges?.................................................................................................................................................127
What are the systems that can be used to support the living of persons with early-onset dementia? .........129
What kind of consultation support is available for persons with early-onset dementia?.............................131

D | Road Traffic Act
CQ 5D-1

What should be done if one finds that a person diagnosed with dementia holds a driver license and is
still driving?...............................................................................................................................................132

Chapter 6

Alzheimer’s Disease Dementia

CQ 6-1
CQ 6-2
CQ 6-3
CQ 6-4
CQ 6-5
CQ 6-6
CQ 6-7
CQ 6-8
CQ 6-9
CQ 6-10What are the features and key points of diagnosis for neuropsychiatric symptoms in Alzheimer’s
disease dementia?.......................................................................................................................................137
What are the diagnostic criteria for Alzheimer’s disease dementia?............................................................139
What are the characteristic image findings of Alzheimer’s disease dementia?............................................140
Is APOE genetic testing useful for the diagnosis of Alzheimer’s disease dementia?....................................142
What are the useful biomarkers for the diagnosis of Alzheimer’s disease dementia? ..................................143
Is amyloid PET examination useful for the diagnosis of Alzheimer’s disease dementia? ............................ 145
What are the pharmacotherapy and treatment algorithms for Alzheimer’s disease dementia? ...................147
What are the effects of non-pharmacological therapies for Alzheimer’s disease dementia?......................... 151
What are the key points in care for Alzheimer’s disease dementia?............................................................ 153
What kind of social support is available for Alzheimer’s disease dementia?................................................154

Chapter 7

Dementia with Lewy bodies

CQ 7-1

What are the diagnostic criteria and key points for early diagnosis of dementia with Lewy bodies(DLB)?.......................................................................................................................................................156
What are the clinical and pathological differences between dementia with Lewy bodies (DLB) andParkinson’s disease with dementia (PDD)?................................................................................................. 157
What are the characteristic laboratory and imaging biomarker of dementia with Lewy bodies(DLB)? ...................................................................................................................................................... 159
What are the clinical course and prognosis of dementia with Lewy bodies (DLB)?................................... 161

CQ 7-2
CQ 7-3
CQ 7-4xii

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CQ 7-5
CQ 7-6

CQ 7-9
CQ 7-10What is the treatment strategy planned for dementia with Lewy bodies (DLB)?.......................................162
Are there any drugs for the treatment of cognitive impairment in dementia with Lewy bodies(DLB)?.......................................................................................................................................................163
Are there any treatments for behavioral and psychological symptoms of dementia (BPSD) and REM
sleep behavior disorder (RBD) in dementia with Lewy bodies (DLB)?......................................................165
Are there any treatments for autonomic symptoms (such as orthostatic hypotension, constipation,
sweating, urination disorder) in dementia with Lewy bodies (DLB)?.........................................................167
What are the suitable treatments for parkinsonism in dementia with Lewy bodies (DLB)?.......................168
What are the non-pharmacological interventions for dementia with Lewy bodies (DLB)?........................169

Chapter 8

Frontotemporal Lobar Degeneration

CQ 8-1

What are the diagnostic key points and diagnostic criteria for frontotemporal lobar degeneration(FTLD)?.................................................................................................................................................... 174
What are the characteristics of image findings of frontotemporal lobar degeneration (FTLD)?.................176
Are there effective drugs for frontotemporal lobar degeneration (FTLD)?.................................................178
Are there effective non-pharmacological therapies for frontotemporal lobar degeneration (FTLD)?..........180
What kind of instructions should be given to families and caregivers of frontotemporal lobar
degeneration (FTLD) patients?..................................................................................................................183

CQ 7-7
CQ 7-8

CQ 8-2
CQ 8-3
CQ 8-4
CQ 8-5Chapter 9

Progressive Supranuclear Palsy (PSP)CQ 9-1
CQ 9-2

What are the features of dementia symptoms in progressive supranuclear palsy (PSP)?.............................187
Are there useful therapies for cognitive impairment in progressive supranuclear palsy (PSP)? ...................188

Chapter 10

Corticobasal Degeneration

CQ 10-1

What are the features of cognitive impairment in corticobasal degeneration (CBD), and what are
the test methods? ...................................................................................................................................... 191
Are there effective pharmacological and non-pharmacological therapies for cognitive impairment
in corticobasal degeneration (CBD)? .........................................................................................................192

CQ 10-2

Chapter 11

Argyrophilic Grain Dementia

CQ 11-1
CQ 11-2
CQ 11-3What is the frequency of argyrophilic grain disease (AGD)?......................................................................194
How is a clinical diagnosis made for argyrophilic grain dementia?............................................................195
What kinds of treatments are available for argyrophilic grain dementia? ..................................................196

Chapter 12 Senile Dementia of the Neurofibrillary Tangle TypeCQ 12-1

What kind of disease is senile dementia of the neurofibrillary tangle type (SD-NFT)?.............................198

Chapter 13

Huntington Disease

CQ 13-1

What are the features and diagnosis of cognitive symptoms in Huntington disease?.................................202

Chapter 14

Vascular Dementia

CQ 14-1
CQ 14-2
CQ 14-3
CQ 14-4
CQ 14-5
CQ 14-6
CQ 14-7
CQ 14-8
CQ 14-9
CQ 14-10

What are the diagnostic criteria for vascular dementia (VaD)?.................................................................. 204
How are the types of vascular dementia (VaD) classified?..........................................................................205
What are the imaging features of vascular dementia (VaD)? .................................................................... 206
What is the condition when vascular dementia (VaD) coexists with Alzheimer’s disease dementia?......... 208
What are the clinical course and prognosis of vascular dementia (VaD)?.................................................. 209
What are the systemic complications and associated symptoms of vascular dementia (VaD)? ..................210
What are the risk factors for vascular dementia (VaD) and how are they controlled?................................. 211
What is antithrombotic therapy for vascular dementia (VaD)?..................................................................213
Are there effective drugs for cognitive impairment in vascular dementia (VaD)? ...................................... 215
What is the clinical position of cerebral amyloid angiopathy (CAA)?........................................................ 217

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Chapter 15 Prion Disease
CQ 15-1
CQ 15-2
CQ 15-3
CQ 15-4
CQ 15-5

What are the clinical features of sporadic Creutzfeldt-Jakob disease (CJD)?............................................. 220
What are the electroencephalographic, cerebrospinal fluid, and MRI findings of sporadic
Creutzfeldt-Jakob disease (CJD)? ..............................................................................................................221
What are the types and features of genetic prion disease in Japan?........................................................... 222
What are the types and features of acquired (infectious) prion disease in Japan?.......................................223
What are the infection control measures and effective sterilization methods for prion disease? ................224

Chapter 16

Medical Diseases and Others

CQ 16-1
CQ 16-2
CQ 16-3
CQ 16-4
CQ 16-5

What are the features of cognitive impairment due to vitamin deficiency?............................................... 226
What are the features of cognitive impairment due to hypothyroidism?................................................... 228
What are the features of cognitive decline due to neurosyphilis?...............................................................229
What are the features of cognitive impairment caused by hepatic encephalopathy (HE)?..........................230
What are the features, diagnosis, and treatment strategies for dementia symptoms of idiopathic normalpressure hydrocephalus (iNPH)? ...............................................................................................................231

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