An autopsied case report of spastic paraplegia with thin corpus callosum carrying a novel mutation in the SPG11 gene: widespread degeneration with eosinophilic inclusions.

BACKGROUND: The detailed neuropathological features of patients with autosomal recessive hereditary spastic paraplegia with a thin corpus callosum (TCC) and SPG11 mutations are poorly understood, as only a few autopsies have been reported. Herein, we describe the clinicopathological findings of a patient with this disease who received long-term care at our medical facility. CASE PRESENTATION: A Japanese man exhibited a mild developmental delay in early childhood and intellectual disability, followed by the appearance of a spastic gait by age 13. At the age of 25 years, he became bedridden and needed a ventilator. Genetic analysis revealed a homozygous splice site variant in the SPG11 gene (c. 4162-2A > G) after the provision of genetic counselling and acquisition of informed consent from his parents. He died of pneumonia at the age of 44. His brain weighed 967 g and was characterized by a TCC, and his spinal cord was flattened. Microscopically, degeneration was observed in the posterior spinocerebellar tract, the gracile fasciculus, and the posterior column in addition to the corticospinal tract. Marked neuronal loss and gliosis were observed in the anterior horn, Clarke's column, and hypoglossal and facial nuclei. Various types of neurons, in addition to motor neurons, showed coarse eosinophilic granules that were immunoreactive for p62. The loss of pigmented neurons with gliosis was apparent in both the substantia nigra and locus coeruleus. Lateral geniculate body degeneration was a characteristic feature of this patient. Furthermore, peripheral Lewy body-related α-synucleinopathy and scattered α-synuclein-immunoreactive neurites in the locus coeruleus and reticular formation of the brainstem were observed. CONCLUSIONS: In patients with hereditary spastic paraplegia with SPG11 mutations, a variety of clinical phenotypes develop due to widespread lesions containing p62-immunoreactive neuronal cytoplasmic inclusions. We herein report the lateral geniculate body as another degenerative site related to SPG11-related pathologies that should be studied in future investigations.

Tufted astrocyte-like glia in two autopsy cases of multiple system atrophy: Is it a concomitant neurodegenerative disorder with multiple system atrophy and progressive supranuclear palsy?

Tufted astrocytes are one of the core histopathological features of progressive supranuclear palsy (PSP). To our knowledge, only three cases of multiple system atrophy (MSA) with PSP pathology have been reported. Here, we report two autopsy cases of MSA associated with the appearance of tufted astrocyte-like glia (TuALG). Clinically, the patients' symptoms were atypical of MSA; one showed vertical gaze palsy, and the other was a long-term survivor who progressed to a bedridden state shortly after the onset of the disease. These neuropathological observations were characterized by the copresence of MSA-specific changes and TuALG in some of the cerebral cortices but few or none of the other PSP tau pathologies. These cases might emphasize the significance of TuALG in non-PSP neurodegenerative disorders.

Huxleyan utopia or Huxleyan dystopia? "Scientific humanism", Faure's legacy and the ascendancy of neuroliberalism in education.

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 as neuroliberalism remain under-theorised. Neuroliberalism combines neoliberal ideas concerning the role of markets in addressing social problems with beliefs about human nature ostensibly grounded in the behavioural, psychological and neurological sciences. This article critically examines a recent initiative of one of UNESCO's Category 1 Institutes - the Mahatma Gandhi Institute of Education for Peace and Sustainable Development (MGIEP) - that seeks to mainstream neuroscience and digital technology within global educational policy. Comparing the visions of the 1972 Faure, the 1996 Delors and the 2021 Futures of Education reports with MGIEP's International Science and Evidence Based Education Assessment (ISEEA), the authors analyse continuity and change in UNESCO's attempts to articulate a vision of "scientific humanism" which advocates the use of science for the betterment of humanity. They argue that ISEEA's overall recommendations - as represented in its Summary for Decision Makers (SDM) - reinforce a reductive, depoliticised vision of education which threatens to exacerbate educational inequality while enhancing the profits and power of Big Tech. These recommendations exemplify a neuroliberal turn in global education policy discourse, marking a stark departure from the central focus on ethics and democratic politics characteristic of UNESCO's landmark education reports. Reanimating, in cruder form, visions of a scientifically-organised utopia of the kind that attracted UNESCO's inaugural Director-General, Julian Huxley, ISEEA's recommendations actually point towards the sort of dystopian "brave new world" of which his brother, Aldous Huxley, warned.

Utopie huxleyenne ou dystopie huxleyenne? « L'humanisme scientifique ¼, 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 neurolibéralisme. Le neurolibéralisme associe des idées néolibérales sur le rôle des marchés pour résoudre des problèmes sociaux avec la conviction que la nature humaine est prétendument ancrée dans les sciences comportementales, psychologiques et neurologiques. Cet article porte un regard critique sur une initiative récente d'un des instituts de catégorie 1 de l'UNESCO, l'Institut Mahatma Gandhi d'éducation pour la paix et le développement durable (MGIEP), qui cherche à intégrer les neurosciences et la technologie du numérique dans la politique mondiale de l'éducation. Les auteurs comparent les visions des rapports Faure en 1972 et Delors en 1996 et du rapport de 2021 sur les futurs de l'éducation avec l'évaluation internationale de l'éducation basée sur la science et des éléments concrets (ISEEA) réalisée par le MGIEP, pour analyser la continuité et les changements dans les tentatives de l'UNESCO d'articuler une vision de « l'humanisme scientifique ¼ prônant d'utiliser la science pour améliorer l'humanité. Ils avancent que les recommandations principales de l'ISEEA, telles que l'évaluation les présente dans son récapitulatif à l'intention des décideurs, renforce une vision réductrice et dépolitisée de l'éducation, qui menace d'exacerber les inégalités en matière d'éducation tout en accroissant les profits et la puissance des big tech. Ces recommandations illustrent un tournant neurolibéral dans le discours mondial sur la politique de l'éducation, qui se démarque absolument de l'intérêt central pour l'éthique et la politique démocratique, caractéristiques des rapports historiques de l'UNESCO sur l'éducation. Ravivant, sous une forme plus rudimentaire, des visions d'une utopie structurée scientifiquement du type de celles qui attiraient le premier directeur général de l'UNESCO, Julian Huxley, les recommandations de l'ISEEA laissent en réalité entrevoir la sorte de « meilleur des mondes ¼ dystopique contre laquelle son frère, Aldous Huxley, mettait en garde.

Non-motor manifestations in ALS patients with tracheostomy and invasive ventilation.

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 <5 years, and more in patients with severe communication impairment than in those with preserved communication ability. DISCUSSION: 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.

Frequent globular neuronal cytoplasmic inclusions in the medial temporal region as a possible characteristic feature in multiple system atrophy with dementia.

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.

A Japanese familial ALS patient with autonomic failure and a p.Cys146Arg mutation in the gene for SOD1 (SOD1).

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.

Marked preservation of the visual and olfactory pathways in ALS patients in a totally locked-in state.

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.

Medial temporal regional argyrophilic grain as a possible important factor affecting dementia in Parkinson's disease.

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.

Frontotemporal lobar degeneration with writing disturbance mainly consisting of omission of kana letters.

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.

A Japanese patient with familial ALS and a p.K510M mutation in the gene for FUS (FUS) resulting in the totally locked-in state.

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.

[Issues of transition support to adulthood and the practices of pediatric-adult healthcare transition in neurological diseases].

A workshop of the Special Committee on Measures for Transition from Pediatric to Adult Health Care, the Japanese Society of Neurology was held to discuss various issues and practices involved in healthcare transition. The following points were addressed: (1) the history of, and issues involved in, promoting support for patients requiring medical care, (2) cooperation between pediatric medical centers and university hospitals, (3) collaboration between pediatrics and neurology in medical and rehabilitation facilities, and (4) a questionnaire survey of members of the Japanese Society of Neurology. The reasons for extreme difficulties in pediatric-adult healthcare transition for patients with neurological diseases, especially those who require continuous intensive medical care over a long period of time, include the difference in the operating systems of pediatric and adult departments, in addition to the difference in the diseases treated during childhood and adulthood. For holistic transition support, it is necessary to strengthen cooperation not only among medical professionals, but also among multiple professions, as well as between local communities and government.

Medical Needs of Adults with Down Syndrome Presenting at a Regional Medical and Rehabilitation Center in Japan.

BACKGROUND: Down syndrome (DS) is the most frequent chromosomal aberration; however, knowledge of associated health issues in adulthood is inadequate. We analyzed health data from Japanese adults with DS. METHODS: We conducted a retrospective chart review of 151 patients with DS who visited the Internal Medicine Outpatient Department of the Tokyo Metropolitan Kita Medical and Rehabilitation Center for the Disabled. RESULTS: Endocrine disorders such as obesity, hyperlipidemia, and hyperuricemia were most common in adulthood (≤40 years) and senescence (>40 years); neurological diseases were more prevalent in senescence. Multimorbidity was noted even patients with DS who were younger than 30 years, and the prevalence increased with age. Only 21 patients (13.9%) with DS visited our hospital with referral letters from pediatricians; 94 patients (62.3%) visited without such referrals from other medical institutions. Patients without a referral letter had a mean of 3.1 comorbidities per patient. Moreover, medical care for some people with DS was interrupted during childhood. CONCLUSIONS: Prevention and detection of comorbidities in patients with DS requires continuous medical care from childhood through adulthood. Recently, DS has been diagnosed by chromosome testing and genetic counseling. Clinical geneticists and genetic counselors can help patients with DS, and their caregivers, to obtain appropriate health care and achieve well-being on their own by seamlessly engaging them throughout childhood and adulthood.

Benefits and Challenges of Pediatric-to-Adult Health Care Transition in Childhood-Onset Neurologic Conditions.

Background and Objectives: Although 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. Methods: This 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. Results: Responses 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. Discussion: Systems 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.

Differential immunophenotypes of neuronal cytoplasmic inclusions in the dentate gyrus of multiple system atrophy and their association with clinicopathological features.

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.

[Current practices of transition from pediatric to adult health care for patients with neurological disease: promote the cooperation between child and adult neurologists].

The Special Committee for Measures Against Transition from Pediatric to Adult Health Care of the Japanese Society of Neurology, which consists of child and adult neurologists, started to tackle the issues of pediatric to adult health care transition for patients with neurological disease in July 2020. The Committee held a workshop with a theme of "cooperation between child and adult neurologists," which is a critical issue in the pediatric to adult health care transition. To solve the many problems in the pediatric to adult health care transition, it is crucial that child and adult neurologists and primary care physicians cooperate on the following issues: preparing child neurologists for the transition, encouraging adult neurologists to study child neurology, promoting the formation of multidisciplinary teams, improving the medical system and medical fees, appealing to governmental agencies for issues of community health care and welfare services.

Somatosensory pathway dysfunction in patients with amyotrophic lateral sclerosis in a completely locked-in state.

OBJECTIVE: To investigate somatosensory pathway function in patients with amyotrophic lateral sclerosis (ALS) dependent on invasive ventilation and in a completely locked-in state (CLIS). METHODS: We examined median nerve somatosensory evoked potentials (SEPs) in 17 ALS patients in a CLIS, including 11 patients with sporadic ALS, one with familial ALS with genes not examined, four with a Cu/Zn superoxide-dismutase-1 (SOD1) gene variant (Val118Leu, Gly93Ser, Cys146Arg), and one with a fused-in-sarcoma gene variant (P525L). We evaluated N9, N13, N20 and P25, and central conduction time (CCT); the data were compared with those of 73 healthy controls. RESULTS: N20 and N13 were abolished in 12 and 10 patients, and their latencies was prolonged in four and three patients, respectively. The CCT was prolonged in five patients with measurable N13 and N20. Two patients with SOD1 gene mutations had absent or slightly visible N9. Compared to the CCT and latencies and amplitudes of N13 and N20 in the controls, those in the patient cohort were significantly abnormal. CONCLUSIONS: The central somatosensory pathway is severely involved in patients with ALS in a CLIS. SIGNIFICANCE: Our findings suggest that median nerve SEP cannot be utilized for communication in patients with ALS in a CLIS.

Novel G37V mutation of SOD1 gene in autopsied patient with familial amyotrophic lateral sclerosis.

We report a novel missense mutation (G37V) in exon 2 of the superoxide dismutase-1 gene in a 63-years-old Japanese male with purely lower motor neuron disease. His disease duration was 14 months, and he died of respiratory failure. The disease in this patient with the G37V mutation showed a rapid progression, although patients with G37R mutation are known to have a long survival.

Supranuclear ophthalmoparesis and vacuolar degeneration of the cerebral white matter in amyotrophic lateral sclerosis: a clinicopathological study.

Possible clinicopathological relationship between vacuolar degeneration of cerebral white matter and clinical manifestation, especially of supranuclear ophthalmoparesis, both infrequent in amyotrophic lateral sclerosis (ALS) patients, was tested. Of 104 ALS sequential series, cases with vacuolar degeneration of the cerebral white matter were selected to yield 14 cases pathologically surveyed in this study. Clinical features were retrospectively assessed in their clinical records. Microscopic examination clarified vacuolar changes with fibrous gliosis, infiltration of macrophages, axonal degeneration with segmental dilatation and partial loss of myelin on electron microscopy. This histological change was extended into the cerebral white matter just under the cortices but sometimes accentuated as restricted areas along the pyramidal tract and precentral regions. In a patient with the most extensive focal lesion, these white matter vacuolar changes were detected with magnetic resonance imaging. The clinical manifestations linked to this focal vacuolar degeneration were disturbance of vertical ocular movements and shorter duration of the illness, compared with patients without vacuolar degeneration. In conclusion, histological demonstration of characteristic vacuolar degeneration in the white matter of ALS and its focal accentuation along precentral-pyramidal tracts are mutually related and possibly linked to clinical manifestations such as supranuclear ophthalmoparesis, an exceptional but possible manifestation of ALS.

Phosphorylated α-synuclein immunoreactivity in the posterior pituitary lobe.

Parkinson's disease is now recognized as a major form of α-synucleinopathy involving both the central and peripheral nervous systems. However, no research has focused on the posterior pituitary lobe (PPL), despite the fact that this organ also plays an important role in systemic homeostasis. In the present study, we aimed to distinguish phosphorylated α-synuclein (pαSyn)-positive deposits in the PPL, as is observed in Lewy body- and non-Lewy body-related disorders. PαSyn deposits were immunohistochemically analyzed using formalin-fixed, paraffin-embedded PPL specimens obtained from 60 autopsy cases. Among the cases with Lewy body-related disorders, PPL pαSyn deposits were observed in almost all cases of Parkinson's disease (22/23), and in one case of dementia with Lewy bodies (1/1). On the other hand, only 3/36 cases of non-Lewy body-related disorders had pαSyn immunoreactivity in the PPL. The present study confirms the presence of pαSyn-positive deposits, as demonstrated by high specificity (97.1%) and sensitivity (88.5%), in both Parkinson's disease and dementia with Lewy bodies, suggesting that this finding can be a useful hallmark of Lewy body-related disorders.

[Current Situation and Issues in the Transition from Pediatric to Adult Health Care: A Neurologist's Perspective].

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.