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Although hippocampal pathologies of multiple system atrophy (MSA) and their association with dementia have been reported, no studies have reported clinicopathological differences among MSA patients with and without neuronal cytoplasmic inclusions (NCIs) in the dentate gyrus (dntNCIs). We investigated hippocampal NCI pathology in 18 MSA patient autopsies, focusing on phosphorylated α-synuclein (pAS)- and phosphorylated tau (pT)-positive dntNCIs. There were 8 MSA patients without and 10 with dntNCIs. The latter group was subclassified by immunophenotype: those with pAS-positive dntNCIs (pAS-dntNCI subtype), those with pT-positive dntNCIs (pT-dntNCI subtype), and those with both types of dntNCIs. MSA patients with dntNCIs survived longer with prolonged tracheostomy and had dementia more frequently than those without dntNCIs. The brain weights of patients with dntNCIs were lower than those without dntNCIs. The presence of dementia was similar among the dntNCI subtypes. The pAS-dntNCI subtype was associated with longer survival and smaller brain weights; the pT-dntNCI subtype exhibited more frequent tau pathologies than the pAS-dntNCI subtype. Thus, MSA with dntNCIs is a possible pathological subtype of longer survivors that correlates with longer disease duration, prolonged tracheostomy, and high frequency of dementia. Understanding clinicopathological differences in MSA patients with and without dntNCIs may lead to improved personalized management strategies.

In addition to the longstanding threat posed by narrow economism, faith in the possibility of peace and progress through democratic politics - central to the humanistic vision of the 1972 Faure report - today faces additional challenges. These challenges include the ascendancy of neurocentrism in the global policyscape. Whereas the effects of neoliberalism on education have been extensively critiqued, the implications of a newer, related ideological framework known asUtopie huxleyenne ou dystopie huxleyenne? « L’humanisme scientifique », l’héritage de Faure et la montée du néolibéralisme dans l’éducation – Outre faire face à la menace que pose depuis longtemps l’économisme étroit, la foi dans la possibilité que la politique démocratique peut produire paix et progrès – un pilier de la vision humaniste du rapport Faure paru en 1972 – se heurte aujourd’hui à d’autres défis, entre autres à la montée du neurocentrisme dans le champ politique mondial. Tandis que les effets du néolibéralisme sur l’éducation ont été abondamment critiqués, peu d’hypothèses ont été formulées au sujet de ce qu’implique une notion plus récente, mais liée à lui sur le plan idéologique et connue sous le nom de

Our hospital provides medical care and rehabilitation for individuals with disabilities. In our hospital, both neurologists and pediatricians have been working on the transition from pediatric to adult healthcare. We used a transition readiness checklist and pediatrician-neurologist transition consultation. We aimed to promote appropriate medical care, community-based healthcare coordination, and welfare services through interprofessional care with other health professionals. We assisted patients and their families in improving self-management and discussed patient issues with their best interests in mind through a shared decision-making process. Recently, the need for neurologists in transitioning patients from pediatric to adult healthcare has been increasing.

Background and ObjectivesAlthough the importance of pediatric-to-adult health care transition (HCT) has been recognized, individuals with childhood-onset neurologic conditions often encounter challenges during pediatric-to-adult HCT, and HCT benefits for this population remain elusive. We assessed the current HCT situation in individuals with childhood-onset neurologic conditions to develop an improved transition system that incorporates patient perspectives.MethodsThis cross-sectional study was conducted at the Tokyo Metropolitan Kita Medical and Rehabilitation Center for the Disabled from November 2020 to December 2020. We targeted adults with childhood-onset neurologic conditions who visited the Department of Internal Medicine and their families. Questionnaires provided to 127 patients asked them about their experiences with pediatric-to-adult HCT (i.e., educational opportunities regarding HCT during pediatric visits, difficulties in transition, and the merits/demerits of adult practice) and their families' perspectives regarding pediatric-to-adult HCT. We also reviewed the patients' medical records to examine the severity of their disabilities.ResultsResponses were collected from 111 patients (response rate: 87%). Most patients had both severe physical and intellectual disabilities, and approximately half had a physical disability level of Gross Motor Function Classification System V and a profound intellectual disability. Half of the respondents were not transitioned through pediatric-to-adult HCT by their pediatricians, and they visited adult departments by themselves without a formal referral process. They experienced difficulties during HCT, such as a lack of knowledge regarding adult health care providers and consultants. However, those who underwent HCT benefited from it in terms of their health, experience, and service use, such as age- and condition-appropriate care, seeing adult specialists, and the introduction of adult services. They also addressed challenges in managing appointments and having adult doctors understand their medical history. Nonetheless, they were not informed about diseases and medical and welfare resources for adulthood during pediatric visits and desired to discuss future plans with pediatricians.DiscussionSystems that provide sufficient pediatric-to-adult HCT for individuals with childhood-onset neurologic conditions are required. Lifelong education for patients and families, training for pediatricians on HCT and neurologists on childhood-onset conditions and disabilities, and clinical practice and human resources that support patients and families are warranted.

An improvement in efficacy treatment and development of the social support system has led to many patients with neurological disease being able to reach adulthood. Therefore health care for life from pediatrics to adulthood has become necessary. The Special Committee for Measures Against Transition from Pediatric to Adult Health Care of the Japanese Society of Neurology officially started to examine the current situation and issues of transition from pediatric to adult health care in July 2020. Pediatric neurologists and adult neurologists have an awareness of this issue of constructing a better transition from pediatric to adult health care. However, there are some tasks that need to be resolved in the medical system. We intend to improve the understanding of transition and assessment of medical service fees for transition in cooperation with the Japanese Society of Neurology and the Japanese Society of Child Neurology.

Hundreds of sustainability programs have emerged at universities and colleges around the world over the past 2 decades. A prime question for employers, students, educators, and program administrators is what competencies these programs develop in students. This study explores convergence on competencies for sustainability programs. We conducted a Delphi study with 14 international experts in sustainability education on the framework of key competencies in sustainability by Wiek et al. (Sustain Sci 6: 203–218, 2011), the most frequently cited framework to date. While experts generally agreed with the framework, they propose two additional competencies, suggest a hierarchy of competencies, and specify learning objectives for students interested in a career as sustainability researcher. The refined framework can inform program development, implementation, and evaluation to enhance employability of graduates and facilitate comparison of sustainability programs worldwide.

Three patients with neurodegenerative diseases who had developed repeated aspiration pneumonia underwent laryngeal closure, a surgical procedure at the larynx to prevent aspiration. None of these patients have developed aspiration pneumonia since the procedure. One patient needed endoscopic suction and cough assist machine to clear thick sputum, because tracheostomy bypassed the upper airway and so prevented moisturization of inhaled air. While two patients achieved freedom from tracheal cannulation, one needed continued cannulation because of narrowing of the stoma due to improvements in the nutritional condition. One patient was able to resume oral intake. Although the right timing to perform the procedure and optimal care along with long-term observation are important, laryngeal closure is an effective option for patients with neurodegenerative diseases to prevent recurrent aspiration pneumonia.

Education is indispensable for the flourishing of people from all backgrounds and stages of life. However, given the accelerating demographic, environmental, economical, socio-political, and technological changes—and their associated risks and opportunities—there is increasing consensus that our current educational systems are falling short and that we need to repurpose education and rethink the organization of learning to meet the challenges of the 21st century. The United Nations Educational Scientific and Cultural Organization (UNESCO) “Futures of Education” initiative was formally launched at the United Nations General Assembly in 2019 to provide such a vision of education for the future. The International Scientific and Evidence-based Education (ISEE) Assessment synthesizes knowledge streams generated by different communities and stakeholders at all levels and scales and will thereby essentially contribute to re-envisioning this future of education. The overall aim of the ISEE Assessment is to pool the expertise from a broad range of knowledge holders and stakeholders to undertake a scientifically robust and evidence-based assessment in an open and inclusive manner of our current educational systems and its necessary reforms. In this commentary, we discuss the aims and goals of the ISEE Assessment. We describe how the ISEE Assessment will address key questions on the purpose of education and what, how, where and when we learn, and evaluate the alignment of today’s education and theory of learning with the current and forthcoming needs and challenges and to inform policymaking for future education.

We investigated the patients followed in our hospital's adult neurology department to evaluate issues during the transition from pediatric to adult health care for patients with special health-care needs for neurological diseases. There has been an increase in the number of transition patients, and they were often recommended for the transition by pediatricians. Many patients had complications such as epilepsy, and there were also patients with an intractable disease. Therefore, patients undergoing this transition need neurologists. The transition requires a long time, and there is a difference in the medical administrative fees between pediatric and adult health care. The Japanese Society of Neurology and related societies need to take measures to improve these health-care transitions.

Introduction: This study aimed to investigate non-motor manifestations in amyotrophic lateral sclerosis (ALS) patients with tracheostomy and invasive ventilation (TIV) and their relevance to disease progression. Methods: Sixty-seven ALS patients with TIV were enrolled, and followed-up prospectively. The patients were classified at the final evaluation into two subgroups according to the duration of TIV use or disease stage measured by communication impairment. We identified non-motor manifestations and investigated their frequencies and differences across the stages. Results: The non-motor manifestations were macroglossia (22.4%), unstable blood pressure (38.8%), hypothermia (26.9%), dysuria (50.7%), and hyperglycemia (12.1%). These manifestations occurred significantly more frequently in patients with TIV ≥5 years than in patients with TIV

Progressive supranuclear palsy – Richardson syndrome (PSP-RS) was first described in 1964 by Steele et al. Tau pathology has not been reported in the hypoglossal nuclei of PSP-RS patients, whereas Steele et al. described gliosis with no remarkable neuronal losses in the hypoglossal nucleus. This study aimed to investigate the distribution and degree of tau pathology-associated neurodegeneration, with an emphasis on the hypoglossal nucleus, in patients with PSP-RS. Six clinicopathologically proven PSP-RS cases were included in this study. All patients were clinicopathologically and immunohistochemically re-evaluated. This study confirmed the following neuropathological characteristics of PSP-RS: (1) neurodegeneration usually affects the striatonigral system and cerebellar dentate nucleus; (2) the cerebellar afferent system in PSP-RS is affected by absent-to-mild neurodegeneration; and (3) the extent of tau distribution throughout the central nervous system is greater than the extent of neurodegeneration. Furthermore, we found that subthalamic neurodegeneration was more prominent in the ventromedial region than in the dorsolateral region. Nevertheless, the tau pathology showed no remarkable differences between these two sites. Interestingly, the tau pathology was frequently observed in the hypoglossal nuclei of PSP-RS patients. Gradient neurodegeneration of the subthalamus and tau pathology in the hypoglossal nucleus could be regarded as essential pathological features of PSP-RS.

We describe a Japanese man with familial amyotrophic lateral sclerosis (ALS) associated with a p.Cys146Arg mutation in the copper/zinc superoxide dismutase gene (SOD1). The patient developed bulbar signs followed by rapidly progressive limb muscle weakness. The prominent clinical feature was orthostatic hypotension due to autonomic failure, which occurred after he underwent tracheostomy 1 year and 3 months after the onset. Thereafter, he required mechanical ventilation and progressed to communication stage V (totally locked-in state) 7 years after the onset. Neuropathology showed ALS with posterior column degeneration and multiple system degeneration. Severe neuronal loss in the intermediolateral nucleus was also observed. Two previously reported cases of ALS patients with autonomic failure showed severe neuronal loss in the intermediolateral nucleus in addition to degeneration of the motor neurons. Thus, autonomic failure due to neuronal loss in the intermediolateral nucleus could present in patients with ALS associated with certain mutations in SOD1.

Multiple system atrophy (MSA) is an adult-onset neurodegenerative disease, which is characterized clinically by parkinsonism, cerebellar ataxia and/or autonomic dysfunction, and pathologically by alpha-synuclein-related multisystem neurodegeneration, so-called alpha-synucleinopathy, which particularly involves the striatonigral and olivopontocerebellar systems, with glial cytoplasmic inclusions and neuronal cytoplasmic/nuclear inclusions (NCIs/NNIs). In the recent consensus criteria for the diagnosis of MSA, dementia is described as one of the features not supporting a diagnosis of MSA. However, MSA with dementia has been reported, although the location of the lesion responsible for the dementia remains unclear. In the present study, we aimed to investigate where this lesion may be found, by analyzing 12 autopsy-proven MSA cases, with a particular focus on the medial temporal region. Three of 12 cases with MSA had dementia (MSA-D). Compared with MSA cases without dementia, MSA-D cases had frequent globular NCIs (G-NCIs) in the medial temporal region, especially in their subiculum. In addition, MSA-D cases could be divided into two types; MSA-D with distinct fronto-temporal lobar degeneration (FTLD type) and without distinct fronto-temporal lobar degeneration (non-FTLD type). There was no association between dementia and Alzheimer pathologies, such as neurofibrillary tangles and senile plaques. We suggest that frequent G-NCIs in the medial temporal region, and particularly the subiculum, is one of the important pathological findings of MSA-D, even when a case with MSA-D reveals no significant cerebral atrophy.

Patients with amyotrophic lateral sclerosis (ALS) often suffer from salivation problems such as drooling and dry mouth. We examined resting salivation rate cross-sectionally in 66 advanced ALS patients with tracheostomy invasive ventilation using a cotton roll method, and investigated clinical factors associated with salivation rate. Resting salivation rate in the patients was well preserved (median value 0.6 g/min), and was significantly more increased in patients with impairment of jaw movement (P = 0.007) or mouth opening (P = 0.003) than in patients with less impairment, and in patients with the mouth being constantly open ≥ 10 mm in rostrocaudal length than in patients with < 10 mm. These data indicate that salivation rate was increased with progression of dysfunction of voluntary jaw movement. Appropriate oral care is required in advanced ALS patients to maintain their oral hygiene and to avoid penetration of saliva into the airway.

Introduction It is unclear whether tau-positive granular glial pathology is a characteristic feature of MSA. We aimed to analyse the prevalence and significance of tau-positive granular glial pathology in MSA. Methods Fourteen MSA cases were clinicopathologically investigated, focusing on tau-positive granular glial pathology in the frontal and temporal white matter and putamen. Results In five MSA cases, the temporal white matter showed AT8-positive granular glial pathology; this pathology was detected in the frontal white matter in three cases. AT8-positive granular glia in the white matter were associated with long disease duration with long-term tube feeding and/or long-term tracheotomy. Alpha-synuclein-positive glial cytoplasmic inclusion intensity was not associated with AT8-positive granular glial pathology. The tau isoform of AT8-positive granular glia in the cerebral white matter exhibited three-repeat, not four-repeat, tau. Ten MSA patients showed tau-positive granular glial pathology in the putamen; the tau isoform was predominantly three-repeat tau and four-repeat tau in cases with disease duration ≥13 years and Conclusions Tau-positive granular glia in the putamen is a characteristic pathological feature of MSA. Tau-positive granular glia appear in the cerebral white matter in MSA patients and are associated with long disease duration with long-term tube feeding and/or long-term tracheotomy.

Materials and methods The present paper examines the brains and spinal cords in 7 patients with amyotrophic lateral sclerosis (ALS) receiving artificial respirator support in a totally locked-in state (TLS) neuropathologically in order to clarify whether any anatomical structures in the central nervous system are preserved. Results and conclusion We found that the visual and olfactory pathways, hypothalamus, nucleus basalis of Meynert, and commissura anterior were remarkably well preserved, whereas the somatosensory, auditory, and gustatory pathways in the brain stem and/or spinal cord showed severe deterioration.

Predictors of communication impairment in patients with amyotrophic lateral sclerosis (ALS) using tracheostomy-invasive ventilation (TIV) were investigated. Seventy-six ALS patients using TIV were enrolled and classified into three subgroups of communication ability: patients who could communicate with communication devices (Stage I), patients who had difficulty with communication (Stage II, III, or IV), and patients who could not communicate by any means (Stage V). Predictors of communication impairment were analysed by the Cox proportional hazard model. Results demonstrated that there were no significant differences in disease duration between subgroups. Within 24 months after disease onset, patients who needed TIV and tube feeding, developed oculomotor impairment or became totally quadriplegic and progressed from Stage I to II and V significantly earlier. Multivariate analyses revealed that within 24 months from onset, the need for TIV and progression to total quadriplegia were significant events in patients who progressed to Stage II, whereas the development of oculomotor limitation was significant in patients who progressed to Stage V. In conclusion, TIV, impaired oculomotor movement and total quadriplegia are predictors of severe communication impairment. Rapid disease progression might indicate future communication impairment after the use of TIV. We highly recommend early detection of impaired communication and identification of the best methods of communication.

Argyrophilic grain (ArG) is the main pathological feature of argyrophilic grain disease (AGD) and is clinically characterized by cognitive impairment, behavioral abnormalities, personality changes, and emotional imbalances. However, ArG can not only be found in AGD but also in various other neurological disorders, including Parkinson's disease (PD). The association of ArG with psychosis and/or dementia in various neurological disorders remains unknown; in this study, we have investigated this in PD. The distribution and degree of ArG deposition, spongiform change in the transentorhinal cortex (TER SpC), and phosphorylated alpha-synuclein-positive neurites in CA2/3 were assessed, and we used formalin-fixed, paraffin-embedded specimens obtained from the anterior/posterior medial temporal region of 20 autopsy cases diagnosed as PD. These cases were clinically divided into two groups: PD without dementia (PDND) and PD with dementia (PDD). Most PDD cases revealed scattered to numerous ArG or moderate to severe TER SpC, both of which were rarely observed in the PDND group. Furthermore, by the degree of ArG density and TER SpC, the PDD group was further divided into three subtypes: PDD with ArG, with TER SpC and without ArG/TER SpC. Scattered-to-numerous ArG and/or moderate-to-severe TER SpC were observed only in PDD, which suggested that both ArG and TER SpC could be important factors affecting dementia in PD and that their distribution and degree are equally important.

This article maps and explains Brazil’s policies, strategies, plans and initiatives related to Climate Change Education (CCE), in the overall context of Environmental Education (EE) and Education for Sustainable Development (ESD). The case of Brazil offers useful insights on how to enhance climate response through education because of its unique strength in climate policy making and its established EE tradition. The article traces the development of EE in Brazil and provides an overview of the relations between EE and climate change legislations and public policies. Although Brazil established a strong legal framework for EE and the Ministries of the Environment and Education have adopted a number of initiatives to implement the EE policies, climate change legislation addresses education rather superficially. Many other challenges remain, including fully integrating EE in the mainstream work of the Ministry of Education, enhancing the impact of EE policies and programmes, and creating synergies between EE and a number of climate-related education initiatives developed in other areas such as science and technology.

Although the role of education in addressing the challenges of climate change is increasingly recognized, the education sector remains underutilized as a strategic resource to mitigate and adapt to climate change. Education stakeholders in many countries have yet to develop a coherent framework for climate change education (CCE). This article underscores the critical role that education can and should play in addressing and responding to climate change in all of its complexity. It provides rationales as to why CCE should be addressed in the context of Education for Sustainable Development (ESD). Advancing CCE in the context of ESD, or Climate Change Education for Sustainable Development (CCESD), requires enhancement of learners’ understanding of the causes and consequences of climate change and their readiness to take actions to address it. The article presents key organizing principles of CCESD and outlines key knowledge, skills, attitudes, dispositions and competences to be fostered through it.

This study aimed to investigate non-motor manifestations in amyotrophic lateral sclerosis (ALS) patients with tracheostomy and invasive ventilation (TIV) and their relevance to disease progression.Sixty-seven ALS patients with TIV were enrolled, and followed-up prospectively. The patients were classified at the final evaluation into two subgroups according to the duration of TIV use or disease stage measured by communication impairment. We identified non-motor manifestations and investigated their frequencies and differences across the stages.The non-motor manifestations were macroglossia (22.4%), unstable blood pressure (38.8%), hypothermia (26.9%), dysuria (50.7%), and hyperglycemia (12.1%). These manifestations occurred significantly more frequently in patients with TIV ≥5 years than in patients with TIV5 years, and more in patients with severe communication impairment than in those with preserved communication ability.Non-motor manifestations are observed at a high rate in ALS patients with TIV, and are possibly related to disease progression. Muscle Nerve 57: 735-741, 2018.

We describe a Japanese patient with familial amyotrophic lateral sclerosis (ALS) and a p.K510M mutation in the fused in sarcoma gene (FUS). The patient's condition was characterized clinically by an early onset and rapid progression. The patient eventually required mechanical ventilation and progressed to the totally locked-in state. Neuropathologically, multiple system degeneration with many FUS-immunoreactive structures was observed. The involvement of the globus pallidus, subthalamic nucleus, substantia nigra, cerebellar efferent system, and both upper and lower motor neurons in the present patient was comparable to that described for ALS patients with different mutations in FUS, all of whom progressed to the totally locked-in state. However, the patient also exhibited degeneration of the cerebellar afferent system and posterior column. Furthermore, the appearance of non-compact FUS-immunoreactive neuronal cytoplasmic inclusions and many FUS-immunoreactive glial cytoplasmic inclusions were unique to the present patient. These features suggest that the morphological characteristics of the FUS-immunoreactive structures and distribution of the lesions vary with the diversity of mutations in FUS.

A right-handed woman developed pseudobulbar palsy and a particular writing disturbance mainly composed of omission of kana letters (OKL) at the age of 79, followed by gradual progression of generalized motor disturbance and mutism. She died at the age of 88. Postmortem examination revealed frontotemporal lobar degeneration. The precentral cortex and premotor area were the most severely degenerated among the affected frontal, parietal, and temporal lobes. The omission of kana letters has been recently reported as a characteristic feature of writing disturbance in Japanese amyotrophic lateral sclerosis (ALS). Our case indicates that OKL is not specific to ALS, and that the prefrontal and precentral cortices, common lesions between our case and ALS, are responsible for OKL. This case also shows that OKL can be caused by a pathomechanism independent from other types of writing error. The neurolinguistic analysis of our case suggests the disturbance of the moraic frame of words in the transcription process of morae into kana letters or kana-letter cards.

ALS patients supported by a ventilator often suffered from difficulty in communicating with others. We herein proposed a new classification of clinical stages of advanced ALS focusing on the degree of communication disturbance. We analyzed the relationship between clinical findings and the prognosis for communication disturbance. Twenty-nine ALS patients without dementia were enrolled in the study. The proposed classification consisted of five stages. Stage I: communicate in sentences, stage II: communicate with one word answers only, stage III: communicate with nonverbal yes/no response only, stage IV: cannot communicate occasionally due to uncertain yes/no responses, stage V: cannot communicate by any means. Clinical analysis showed that patients who reached stage V had begun to use the ventilator significantly earlier than patients with the final stages of IV or less. In addition, patients in stage V frequently had a family history of ALS. Rapid disease progression before ventilator use in patients with a family history might predict a poor long-term prognosis for communication disorder after using the ventilator.

We evaluated the clinicopathological features of familial amyotrophic lateral sclerosis (ALS) with the fused in sarcoma (FUS) P525L mutation. Two sisters and their mother had a similar clinical course, which was characterized by the development of limb weakness at a young age with rapid disease progression. An elder sister, patient 1, progressed into a totally locked-in state requiring mechanical ventilation and died 26 years after the onset of the disease. In contrast, the younger sister, patient 2, died in the early stages of the disease. The patients had neuropathological findings that indicated a very active degeneration of motor neurons and multiple system degeneration, which led to marked brain and spinal cord atrophy in the long term clinical outcome. The multiple system degeneration included the frontal lobe, the basal ganglia and substantia nigra, cerebellum and related area. Compared with previously reported ALS cases, the severe degeneration of the frontal lobe and the striatum were the characteristic features in the patient 1 in this case study. The degeneration spread over multiple systems might be caused not only by the appearance of the FUS immunoreactive neuronal cytoplasmic inclusions but also by the degeneration of neuronal connections from the primary motor cortex and related areas.

Introduction Top-down and bottom-up ESD: divergence and convergence of Japanese ESD discourses and practices Yoko Mochizuki Part I: School-based approaches Chapter 1. Formal ESD in Japan: dissolving walls between classroom and community Jane Singer and Yoshiyuki Nagata Chapter 2. Implications of 3.11 for disaster education and education for sustainable development in Japan Aiko Sakurai and Rajib Shaw Chapter 3. Assessing sustainability learning and practice at Moriyama High School, Shiga, Japan Yi Zhou and Jane Singer Chapter 4. Globalising school education in Japan: an investigation using the academic ability model Toshiya Kodama Chapter 5. Perspectives on education for sustainable development through local cultural heritage Shizuo Nakazawa and Tadashi Izumitani Chapter 6. An investigation into fairness and bias in educational materials produced by the Japanese government to teach school children about nuclear power and radiation Shinobu Goto Chapter 7. Collaborating for change: teaching and assessing a university community sustainability course in Japan and Vietnam Tracey Gannon, Jane Singer and Benjamin McLellan Part II: Community-based approaches Chapter 8. Community-based, non-formal and informal ESD in Japan: where top-down and bottom-up approaches meet Fumiko Noguchi and Toyoshi Sasaki Chapter 9. Can civil society revitalise dying rural villages? The case of Kamiseya in Kyoto prefecture Binxian Ji and Katsue Fukamachi Chapter 10. Multi-stakeholder community education through environmental learning programmes in Nishinomiya Miki Yoshizumi Chapter 11. From challenge to opportunity: Japanese non-profit organisations harness post-3.11 civic engagement Sarajean Rossitto Chapter 12. A radical approach from the periphery: informal ESD through rights recovery for indigenous AinuFumiko Noguchi Chapter 13. The Tohoku Green Renaissance Project - networking green rebuilding activities after a mega-disaster Tsubasa Iwabuchi and Noriko Takemoto Chapter 14. The importance of genfukei (spirit of place) to citizen participation in community building in Zushi city Koichi Nagashima Chapter 15. Exploring the values of rural communities through place-based education in Niigata prefecture Takako Takano Postscript. Reflections on visions of rebuilding Tohoku: the future of ESD as a response to risk in JapanYoko Mochizuki with Makoto Hatakeyama

In the present study, we performed a comprehensive analysis to clarify the clinicopathological characteristics of patients with amyotrophic lateral sclerosis (ALS) that had progressed to result in a totally locked-in state (communication Stage V), in which all voluntary movements are lost and communication is impossible. In 11 patients, six had phosphorylated TAR DNA-binding protein 43 (pTDP-43)-immunoreactive (ir) neuronal cytoplasmic inclusions (NCI), two had fused in sarcoma (FUS)-ir NCI, and three had copper/zinc superoxide dismutase (SOD1)-ir NCI. The time from ALS onset to the need for tracheostomy invasive ventilation was less than 24 months in ten patients. Regardless of accumulated protein, all the patients showed common lesions in the pallido-nigro-luysian system, brainstem reticular formation, and cerebellar efferent system, in addition to motor neurons. In patients with pTDP-43-ir NCI, patients with NCI in the hippocampal dentate granule neurons (DG) showed a neuronal loss in the cerebral cortex, and patients without NCI in DG showed a preserved cerebral cortex. By contrast, in patients with FUS-ir NCI, patients with NCI in DG showed a preserved cerebral cortex and patients without NCI in DG showed marked cerebral degeneration. The cerebral cortex of patients with SOD1-ir NCI was preserved. Together, these findings suggest that lesions of the cerebrum are probably not necessary for progression to Stage V. In conclusion, patients with ALS that had progressed to result in communication Stage V showed rapidly-progressed symptoms, and their common lesions could cause the manifestations of communication Stage V., Article, ACTA NEUROPATHOLOGICA COMMUNICATIONS.4:107(2016)

PurposeThe purpose of this paper is to draw attention of the education for sustainable development (ESD) community to recent discussions on competence approaches and to examine the adequacy of a competence‐based model as the means of achieving educational and societal transformation towards sustainability. The paper analyses and highlights some important aspects of case studies of the contributing authors to the special issue.Design/methodology/approachThe paper is based on the review of relevant literature and reflections on the articles that constitute this special issue. It also reflects the authors' observations through their extensive interactions with theoreticians, practitioners and policy makers on ESD in the context of the United Nations decade of education for sustainable development (DESD) and higher education for sustainable development (HESD).FindingsThe paper recognises a highly complex nature of the conceptualizations of competences for SD and their articulation in educational programmes. It also highlights a growing interest in competence‐based approaches from institutions of higher education and their stakeholders in different parts of the world.Practical implicationsThe paper provides a broad picture of influential international processes and diverse players driving competence‐based approaches in ESD and indicates a need for more coherent critical multi‐level analysis of such processes.Originality/valueThe paper contributes to a broader debate on strategies of implementation of ESD and education for sustainability (EfS) by mapping arguments on competences for SD and sustainability with a particular focus on higher education institutions.

This paper examines how education for sustainable development (ESD) can be concretely advanced using the theoretical approaches of sustainable consumption and production (SCP) and sustainable livelihoods (SL). Five case examples illustrate a diverse set of strategic educational interventions focusing on: (1) education of specific organizational actors about these theoretical frameworks illustrated with case examples (such as SCP training by the United Nations University Institute of Advanced Studies [UNU-IAS] and CSR-Asia of government and business representatives), (2) regional education strategies focused on production and consumption in specific sectors (such as the food sector in Skane, Sweden), (3) social learning directed at innovation for sustainable development (such as competitions of solar boats developed by universities in the region of Friesland, the Netherlands), (4) education of consumers and firms made possible by the adoption of certification systems affirming SCP and SL (such as Cradle-to-Cradle certification of a paper company in the Netherlands or the establishment of Fair Trade cities in Sweden), or (5) reorienting communities to address underutilized productive physical capital within communities (such as the sharing productive capital project in rural areas of Saskatchewan, Canada). The cases are drawn from the projects that the UNU-IAS, four of its regional centers of expertise (RCE) on ESD and other affiliates have conducted. In addition to documenting the educational processes emerging from specific regions, the paper highlights findings related to the success of these projects and opportunities for further research, including regional and inter-regional approaches.

This article serves as a position paper of a consortium of universities in the Asia–Pacific region working to address challenges of sustainable development and rapidly changing social, economic and natural environments. Member universities of ProSPER.Net (Promotion of Sustainability in Postgraduate Education and Research Network) have embarked on a project to develop an alternative university appraisal system that would potentially become a viable alternative to the existing higher education ranking and assessment systems perceived as constraining, yet, powerful. The article discusses the changing landscape for knowledge creation and the need for universities to assume new roles in a new kind of modernity—variously termed as “liquid modernity” (Z. Bauman), “reflexive modernization” (U. Beck) or other neologisms. It recognises that the mainstream ranking and assessment systems are powerful guiding systems for higher education institutions (HEIs) and, if modified, could be a significant force for transformation towards a more sustainable future. Recognising the need for HEIs to address societal challenges and needs, the Alternative University Appraisal (AUA) project of ProSPER.Net starts by reviewing existing models of recognition and appraisal of various aspects of HEIs’ work and aims at creating space for individual and collective reflection on HEI practices and outcomes. In addition to extensive consultations among ProSPER.Net members, as well as with other higher education actors and international organisations addressing higher education for sustainability, cross-sectoral consultations, assessments of the uncertainties and pertinent trends, and engagement with policy-making processes would be required for the AUA system to become a guiding force that shapes higher education of today and tomorrow.

Glial cytoplasmic inclusions (GCIs) are a pathologic hallmark of multiple system atrophy (MSA), but their pathogenetic roles need to be clarified. To determine possible roles of GCIs in individual cells, serial optical sections obtained by confocal microscopy were reconstructed to yield 3-dimensional (3D) images of the nuclei to quantify nuclear volume. Oligodendroglial nuclear volumes were determined in the pons of 6 MSA and 7 control patients. The nuclear volumes were significantly smaller in the MSA group as a whole (135.81 +/- 60.83 microm, mean +/- SD; n = 404) than in the control group (188.05 +/- 55.71 microm; n = 308; p0.001). This difference was due to a significantly smaller nuclear volume of oligodendrocytes without GCIs (GCI group, 91.26 +/- 23.77 microm; n = 210) compared with the control group (p0.001) and compared with the oligodendrocytes with GCIs (GCI group, 184.03 +/- 51.18 microm; n = 194; p0.001); the difference between the latter GCI and control groups was not significant (p0.05). This selective decrease in nuclear volume restricted to the GCI group cannot be explained if nuclear shrinkage was accelerated in the presence of GCIs. Conversely, GCI formation might be linked, either directly or indirectly, to a mechanism that counteracts rather than accelerates nuclear shrinkage. This novel 3-dimensional strategy provides pivotal data that link GCI formation and degeneration in MSA.

PurposeThe purpose of this paper is to offer an overview of the United Nations University's Regional Centres of Expertise on Education for Sustainable Development (RCE) initiative–the global process created to support the implementation of the United Nations Decade of Education for Sustainable Development (DESD, 2005‐2014)–and discuss the roles of institutions of higher education (IHEs) in RCE efforts.Design/methodology/approachThe paper provides a historical overview of the RCE initiative, clarifies the philosophy behind it, and describes the guiding principles for RCE establishment and operations.FindingsThe paper reveals the UNU's views about effective strategies to promote Education for Sustainable Development (ESD) and discusses the roles of IHEs as partners within and among RCEs. Highlights the potential roles of IHEs in overcoming the compartmentalization of knowledge and linking policy and practice.Research limitations/implicationsThe paper recognises that RCE is an evolving concept and calls attention to the RCE process as a promising example of “social learning” and “communities of practice”, and at the same time, of “knowledge management system”.Practical implicationsThe paper clarifies the expected functions of RCEs as multi‐sectoral and interdisciplinary partnerships and “local‐regional knowledge base” and exhorts local ESD stakeholders, including higher education institutions to use RCEs as value‐adding learning networks at the local and global levels.Originality/valueThe paper refines the RCE concept and offers practical advice to RCEs and RCE candidates. Opportunities are indicated for higher education institutions in contributing towards ESD and sustainable development through RCEs.

We describe a 52-year-old man with body weight loss and bulbar palsy, who exhibited muscle atrophy and weakness with fasciculation especially in the respiratory muscles 4 years prior to death, necessitating respiratory support for 4 years, but who was able to walk until the end-stage. He had no significant family history. Neuropathological examination revealed severe loss of motor neurons in the spinal cord and brainstem, and ubiquitin-positive skein-like inclusions and Bunina bodies in the remaining neurons. In addition, prominent degeneration of the anterolateral funiculus and severe loss of neurons in the intermediate zone of the spinal cord were evident, without marked alteration of the corticospinal tracts. Degeneration of the subthalamic nucleus, increased iron deposition in the substantia nigra, and axonal swelling, residual nodules and acidophilic granules in the spinal ganglia were found. The patient's condition was considered to have been a forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) with pallido-nigro-luysian atrophy (PNLA). The neuropathological features of the present case appear to be important for understanding the nature of widespread-type ALS and MND with PNLA.

Concerns have been expressed about the United Nations University's (UNU) Regional Centres of Expertise on education for sustainable development (RCE) initiative. While many have discussed RCE's contribution to the United Nations Decade of Education for Sustainable Development (DESD), there has been no attempt to contextualise the RCE initiative in relation to similar, high-profile UN initiatives such as the Global Compact (GC) and UNESCO's World Heritage List, and to delineate the potential and limitations of RCE as a scheme to encourage recognition of ‘good practices’ and an institutional mechanism to address sustainable development. With a view to refining and advancing the RCE initiative conceptually and operationally, this article addresses some of the key issues that have been raised regarding the RCE programme. By comparing it to the World Heritage List and GC, the article clarifies the nature of designation of local networks as RCEs and offers suggestions on how to ensure the quality and validity of the RCE initiative in the long run.

Degeneration of the perforant pathway, not extensively surveyed so far in amyotrophic lateral sclerosis (ALS) with dementia, was found in eight out of twelve autopsied patients with clinically detectable dementia. Because the severity of degeneration of the entorhinal cortex and that of spongiosis of the outer half of the molecular layer of the dentate gyrus were correlated in these eight patients, it is suspected that the degeneration of the perforant pathway may explain these concomitant lesions. This was further corroborated by occasional involvement of the parahippocampal white matter and subiculum, other components of this pathway. Moreover, six of them manifested clinically detectable memory deficits and three of them exhibited amnesia or episodic memory impairments similar to Alzheimer's disease (AD). Abnormal intensity restricted the dentate gyrus on brain magnetic resonance imaging in a severe case looks like the degeneration of the molecular layer. This involvement of the perforant pathway in ALS patients and its correlation to memory deficits should be taken in account for evaluation of dementia.

The concept of Regional Centres of Expertise (RCEs) on Education for Sustainable Development (ESD) has been enthusiastically embraced by a diverse array of actors since it was first introduced by the United Nations University (UNU) in 2004. An RCE is conceived of as a ‘network’ of existing organisations of formal, nonformal and informal education, mobilised to promote ESD at the local-regional level. Derived from experiences of mobilising RCEs around the globe, this article offers a range of strategic concepts to explain and facilitate the transformation of RCEs into value-adding localised networks constituting a global network—an emerging ‘Global Learning Space for Sustainable Development’.