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A letter to the editor is presented entitled "A Case of Progressive Posterior Cortical Atrophy (PCA) With Vivid Hallucination: Are Some Ghost Tales Vivid Hallucinations in Normal People?" which is published within the issue.

Background: The composite physiologic index (CPI) was developed to estimate the extent of interstitial lung disease (ILD) in idiopathic pulmonary fibrosis (IPF) patients based on pulmonary function tests (PFTs). The CALIPER-revised version of the CPI (CALIPER-CPI) was also developed to estimate the volume fraction of ILD measured by CALIPER, an automated quantitative CT postprocessing software. Recently, artificial intelligence-based quantitative CT image analysis software (AIQCT), which can be used to quantify the bronchial volume separately from the ILD volume, was developed and validated in IPF. The aim of this study was to develop AIQCT-derived CPI formulas to quantify CT abnormalities in IPF and to investigate the associations of these CPI formulas with survival., Methods: The first cohort included 116 patients with IPF. In this cohort, ILD, bronchial, and hyperlucent volumes on CT were quantified using AIQCT. New CPI formulas were developed based on PFTs to estimate the volume fraction of ILD (ILD-CPI), the sum of the ILD and bronchial volume fractions (ILDB-CPI), and the sum of the ILD, bronchial and hyperlucent volume fractions (ILDBH-CPI). The associations of the original CPI, the CALIPER-CPI and the AIQCT-derived CPIs with survival were analyzed in the first cohort and in a second cohort of patients with IPF (n = 72)., Results: In the first cohort, over a median observation time of 92.8 months, 79 patients (68.1%) died, and one patient (0.9%) underwent living-donor lung transplantation. The original CPI, the CALIPER-CPI, and all AIQCT-derived CPIs were associated with overall survival (hazard ratios: 1.07-1.22). The C-index of the ILDB-CPI (0.759) was the highest among all AIQCT-derived CPIs and was comparable to that of the original CPI (0.765) and the CALIPER-CPI (0.749). The C-index of the ILDBH-CPI (0.729) was lower than that of the other CPI variables. The second cohort yielded similar C-indices as the first cohort for the original CPI (0.738), CALIPER-CPI (0.757) and ILDB-CPI (0.749)., Conclusions: The ILDB-CPI can predict the outcomes of IPF patients with a similar performance to that of the original CPI and the CALIPER-CPI. Adding the hyperlucent volume to the CPI formula did not improve its predictive accuracy for mortality., Trial Registration: None (no health care interventions were performed)., Competing Interests: Declarations. Ethics approval and consent to participate: The Institutional Review Board of Kyoto University and the Ethics Committee of Tenri Hospital approved this study (approval numbers R1353, E2119 and No. 635, respectively). The requirement for written informed consent was waived due to the retrospective design of this study. This study was conducted in accordance with the amended Declaration of Helsinki. Consent for publication: Not applicable. Competing interests: Michihiro Uyama has no conflicts of interest; Tomohiro Handa received a research grant from FUJIFILM Corporation and Daiichi Sankyo Company, Limited and belongs to an endowed department sponsored by Teijin Pharma Limited; Ryuji Uozumi received personal fees from Daiichi Sankyo, Eisai, Sawai Pharmaceutical, SBI Pharmaceuticals, Statcom and EPS Corporation and lecture fees from Janssen Pharmaceutical and SAS Institute Japan outside the submitted work; Seishu Hashimoto has no conflicts of interest; Yoshio Taguchi has no conflicts of interest; Kohei Ikezoe received a research grant from FUJIFILM Corporation and Daiichi Sankyo Company, Limited; Kiminobu Tanizawa received a research grant from FUJIFILM Corporation and Daiichi Sankyo Company, Limited; Naoya Tanabe received a research grant from FUJIFILM Corporation and Daiichi Sankyo Company, Limited; Tsuyoshi Oguma received a research grant from FUJIFILM Corporation and Daiichi Sankyo Company, Limited; Atsushi Matsunashi has no conflicts of interest; Takafumi Niwamoto has no conflicts of interest; Hiroshi Shima has no conflicts of interest; Ryobu Mori has no conflicts of interest; Tomoki Maetani has no conflicts of interest; Yusuke Shiraishi has no conflicts of interest; Tomomi W. Nobashi has no conflicts of interest; Ryo Sakamoto has no conflicts of interest; Takeshi Kubo has no conflicts of interest; Akihiko Yoshizawa has no conflicts of interest; Kazuhiro Terada has no conflicts of interest; Yuji Nakamoto has no conflicts of interest; and Toyohiro Hirai received a research grant from FUJIFILM Corporation and Daiichi Sankyo Company, Limited., (© 2024. The Author(s).)

Erdheim-Chester disease (ECD) is a rare type of non-Langerhans cell histiocytosis, characterized by the infiltration of disease-specific foamy histiocytes, polymorphic granulomas, and fibrosis. Although cardiovascular involvement is observed radiologically in approximately half of ECD patients, only a few reports have described its pathological features. We herein report the autopsy of an ECD patient with pulmonary, cardiovascular, and retroperitoneal involvement that may have caused his death. Autopsy revealed the pathological association of coronary and renal arterial stenosis with the BRAF V600E gene mutation. BRAF mutations should be considered in patients with ECD, especially in those with arterial lesions.

Objective: To explore the characteristics and prognostic impact of chronic lung allograft dysfunction (CLAD) after deceased-donor lung transplantation and living-donor lobar lung transplantation, wherein the lower lobes from 2 donors are usually transplanted into one recipient., Methods: The clinical data of 123 deceased-donor and 67 living-donor lung transplantations performed in adult patients at our institution between June 2008 and September 2019 were retrospectively reviewed. The cumulative incidence of CLAD was evaluated on a per-recipient and per-donor graft basis using the Kaplan-Meier method., Results: A smaller number of human leukocyte antigen mismatches, shorter ischemic time, and lower incidence of grade 3 primary graft dysfunction were observed in living-donor transplantation than in deceased-donor transplantation (P < .001). Restrictive allograft syndrome-type CLAD occurred in 9 (20.9%) of 43 patients with CLAD after deceased-donor transplantation and 9 (45.0%) of 20 patients with CLAD after living-donor transplantation. CLAD occurred unilaterally in 15 patients (75.0%) after bilateral living-donor transplantation. Despite the greater incidence of restrictive allograft syndrome-type CLAD after living-donor transplantation, the overall survival rates after the transplantation and survival rates after the onset of CLAD were comparable between the patients receiving deceased-donor transplants and living-donor transplants. The cumulative incidence of CLAD per recipient was similar between recipients of deceased-donor and the living-donor transplants (P = .32). In the per-donor graft analysis, the cumulative incidence of CLAD was significantly lower in the living-donor grafts than in the deceased-donor grafts (P = .003)., Conclusions: The manifestation of CLAD after living-donor lobar lung transplantation is unique and differs from that after deceased-donor lung transplantation., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

A 52-year-old man had developed hearing loss since childhood, as well as recurrent foot ulcers and osteomyelitis since his forties. He presented with gait disturbance and dysarthria that had worsened over four years and a month, respectively. Neurological exams revealed cognitive impairment, proximal weakness of the lower extremities, generalized hyperrflexia, ataxia, sensory disturbances predominant in deep sensation, urinary retention, and gait instability. On nerve conduction study, no sensory nerve action potentials were evoked in the upper and lower limbs. Since his grandmother suffered from similar symptoms, we investigated genetic analysis, which revealed a missense mutation (c.1483T>C, p.Y495H) in DNA methyltransferase 1 gene. He was subsequently diagnosed with hereditary sensory and autonomic neuropathy 1E (HSAN1E). It is important to recognize that increased deep tendon reflex can be observed in HSAN1E.

Background: Interstitial lung abnormalities (ILAs) on CT may affect the clinical outcomes in patients with chronic obstructive pulmonary disease (COPD), but their quantification remains unestablished. This study examined whether artificial intelligence (AI)-based segmentation could be applied to identify ILAs using two COPD cohorts., Methods: ILAs were diagnosed visually based on the Fleischner Society definition. Using an AI-based method, ground-glass opacities, reticulations, and honeycombing were segmented, and their volumes were summed to obtain the percentage ratio of interstitial lung disease-associated volume to total lung volume (ILDvol%). The optimal ILDvol% threshold for ILA detection was determined in cross-sectional data of the discovery and validation cohorts. The 5-year longitudinal changes in ILDvol% were calculated in discovery cohort patients who underwent baseline and follow-up CT scans., Results: ILAs were found in 32 (14%) and 15 (10%) patients with COPD in the discovery (n = 234) and validation (n = 153) cohorts, respectively. ILDvol% was higher in patients with ILAs than in those without ILA in both cohorts. The optimal ILDvol% threshold in the discovery cohort was 1.203%, and good sensitivity and specificity (93.3% and 76.3%) were confirmed in the validation cohort. 124 patients took follow-up CT scan during 5 ± 1 years. 8 out of 124 patients (7%) developed ILAs. In a multivariable model, an increase in ILDvol% was associated with ILA development after adjusting for age, sex, BMI, and smoking exposure., Conclusion: AI-based CT quantification of ILDvol% may be a reproducible method for identifying and monitoring ILAs in patients with COPD., (© 2024. The Author(s).)

Background: The definition of progressive pulmonary fibrosis is based on a 1-year lung function decline., Objectives: To evaluate the epidemiology and clinical relevance of 1-year lung function decline in sarcoidosis., Methods: A retrospective observational study at a general sarcoidosis clinic., Results: Of the 198 patients, 42 (18.4 %) had a 1-year lung function decline (absolute 12-month decline in percentage predicted forced vital capacity [%FVC] of ≥5 % or percentage predicted diffusion capacity for carbon monoxide [%DLCO] of ≥10 %). A 1-year lung function decline was associated with a 2-year lung function decline (a relative 24-month decline in %FVC of ≥10 % or %DLCO of ≥15 %), which occurred in 13 (7.4 %) of the 175 patients with 24-month follow-up results. A 1-year lung function decline was not associated with survival; a 2-year lung function decline predicted mortality., Conclusions: Compared with a 24-month decline, a 12-month decline in lung function did not predict worse survival in sarcoidosis., Competing Interests: Conflict of interest Kiminobu Tanizawa received a research grant from the FUJIFILM Corporation; Tomohiro Handa received a research grant from the FUJIFILM Corporation and belongs to an endowed department sponsored by Teijin Pharma Ltd; Sonoko Nagai has no conflicts of interest; Kohei Ikezoe received a research grant from the FUJIFILM Corporation; Takeshi Kubo has no conflicts of interest; Takafumi Yokomatsu has no conflicts of interest; Seigen Ueda has no conflicts of interest; Yasutaka Fushimi has no conflicts of interest; Shumpei Ogino has no conflicts of interest; Kizuku Watanabe has no conflicts of interest; Yoshinari Nakatsuka belonged to an endowed department sponsored by Philips Respironics, Teijin Pharma Ltd., Fukuda Denshi Inc., and Fukuda Lifetec Keiji; Toyohiro Hirai received a research grant from the FUJIFILM Corporation; and Takateru Izumi has no conflicts of interest., (© 2023 Published by Elsevier B.V. on behalf of The Japanese Respiratory Society.)

Introduction: Lung transplantation (LT) recipients are at risk of bone mineral density (BMD) loss. Pre- and post-LT BMD loss has been reported in some cross-sectional studies; however, there are limited studies regarding the serial BMD change in LT recipients. The aim of this study was to investigate the serial BMD changes and the clinical characteristics associated with BMD decline., Methods: This was a single-center, retrospective observational study. BMD was serially measured in thoracic vertebral bodies (Th4, 7, 10) using computed tomography (CT) before and 3 and 12 months after LT. The frequency of osteoporosis and factors associated with pre-LT osteoporosis and post-LT BMD loss were evaluated. The frequency of post-LT compression fracture and its associated factors were also analyzed., Results: This study included 128 adult LT recipients. LT recipients had decreased BMD (151.8 ± 42.2 mg/mL) before LT compared with age-, sex-, and smoking index-matched controls (176.2 ± 35.7 mg/mL). The diagnosis of COPD was associated with pre-LT osteoporosis. LT recipients experience further BMD decline after transplantation, and the percentage of recipients classified as exhibiting osteoporosis increased from 20% at baseline to 43% at 12 months. Recipients who had been taking no or small doses of glucocorticoids before LT had rapid BMD loss after LT. Early bisphosphonate use (within 3 months) after LT attenuated BMD loss and decreased new-onset compression fracture., Conclusion: LT recipients are at high risk for BMD loss and compression fracture after LT. Early bisphosphonate use may decrease BMD loss and compression fracture., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Pulmonary and extrapulmonary complications after coronavirus disease 2019 (COVID-19) have been major public health concerns during the COVID-19 pandemic. Although post-COVID-19 pulmonary manifestations cover a wide spectrum, eosinophilic pneumonia (EP) has rarely been reported. To date, only four cases of EP potentially triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection have been reported, all of which required systemic corticosteroid therapy. We herein report the first case of post-COVID-19 EP resolution without systemic corticosteroid therapy. We also review the literature regarding EP associated with SARS-CoV-2 infection and vaccination.

Cerebellar climbing fibers convey sensorimotor information and their errors, which are used for motor control and learning. Furthermore, they represent reward-related information. Despite such functional diversity of climbing fiber signals, it is still unclear whether each climbing fiber conveys the information of single or multiple modalities and how the climbing fibers conveying different information are distributed over the cerebellar cortex. Here we perform two-photon calcium imaging from cerebellar Purkinje cells in mice engaged in a voluntary forelimb lever-pull task and demonstrate that climbing fiber responses in 68% of Purkinje cells can be explained by the combination of multiple behavioral variables such as lever movement, licking, and reward delivery. Neighboring Purkinje cells exhibit similar climbing fiber response properties, form functional clusters, and share noise fluctuations of responses. Taken together, individual climbing fibers convey behavioral information on multiplex variables and are spatially organized into the functional modules of the cerebellar cortex., (© 2023. Springer Nature Limited.)

Advanced systemic sclerosis-associated interstitial lung disease (SSc-ILD) can be treated with lung transplantation. There is limited data on lung transplantation outcomes in patients with SSc-ILD, in non-Western populations.We assessed survival data of patients with SSc-ILD, on the lung transplant (LT) waiting list, and evaluated post-transplant outcomes in patients from an Asian LT center. In this single-center retrospective study, 29 patients with SSc-ILD, registered for deceased LT at Kyoto University Hospital, between 2010 and 2022, were identified. We investigated post-transplant outcomes in recipients who underwent LT for SSc-ILD, between February 2002 and April 2022. Ten patients received deceased-donor LT (34%), two received living-donor LT (7%), seven died waiting for LT (24%), and ten survived on the waiting list (34%). Median duration from registration to deceased-donor LT was 28.9 months and that from registration to living-donor LT or death was 6.5 months. Analysis of 15 recipients showed improved forced vital capacity with a median of 55.1% at baseline, 65.8% at 6 months, and 80.3% at 12 months post-transplant. The 5-year survival rate for post-transplant patients with SSc-ILD was 86.2%. The higher post-transplant survival rate at our institute than previously reported suggests that lung transplantation is acceptable in Asian patients with SSc-ILD., (© 2023. The Author(s).)

Background: Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant muscular disorder characterized by asymmetric muscle wasting and weakness. FSHD can be subdivided into two types: FSHD1, caused by contraction of the D4Z4 repeat on chromosome 4q35, and FSHD2, caused by mild contraction of the D4Z4 repeat plus aberrant hypomethylation mediated by genetic variants in SMCHD1, DNMT3B, or LRIF1. Genetic diagnosis of FSHD is challenging because of the complex procedures required., Methods: We applied Nanopore CRISPR/Cas9-targeted resequencing for the diagnosis of FSHD by simultaneous detection of D4Z4 repeat length and methylation status at nucleotide level in genetically-confirmed and suspected patients., Results: We found significant hypomethylation of contracted 4q-D4Z4 repeats in FSHD1, and both 4q- and 10q-D4Z4 repeats in FSHD2. We also found that the hypomethylation in the contracted D4Z4 in FSHD1 is moderately correlated with patient phenotypes., Conclusions: Our method contributes to the development for the diagnosis of FSHD using Nanopore long-read sequencing. This finding might give insight into the mechanisms by which repeat contraction causes disease pathogenesis., (© 2022. The Author(s).)