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We performed a plateau-linear reaction norm model (RNM) analysis of number born alive (NBA) in purebred Landrace pigs, where breeding value changes according to maximum temperature at mating day, using public meteorological observation data in Japan. We analysed 52,668 NBA records obtained from 10,320 Landrace sows. Pedigree data contained 99,201 animals. Off-farm daily temperature data at the nearest weather station from each of the farms were downloaded from the Japan Meteorological Agency website. A plateau-linear RNM analysis based on daily maximum temperature on mating day (threshold temperature of 16.6°C) was performed. The percentage of the records with daily maximum temperatures at mating days of ≤16.6, ≥25.0, ≥30.0 and ≥35.0°C were 34.3%, 33.6%, 14.0% and 0.8%, respectively. The value of Akaike's information criterion for the plateau-linear RNM was lower than that for a simple repeatability model (RM). With the plateau-linear RNM, estimated value of heritability ranged from 0.14 to 0.15, while that from the RM analysis was 0.15. Additive genetic correlation between intercept and slope terms was estimated to be -0.52 from the plateau-linear RNM analysis. Estimated additive genetic correlations were >0.9 between NBA at different temperatures ranging from 16.6 to 37.6°C. For the 10,320 sows, average values of prediction reliability of the intercept and slope terms for breeding values in the plateau-linear RNM were 0.47 and 0.16, respectively. Increasing weight for slope term in linear selection index could bring positive genetic gain in the slope part, but prediction accuracy would decrease. Our results imply that genetically improving heat tolerance in sows reared in Japan focusing on NBA using RNM is possible, while RNM is more complex to implement and interpret. Therefore, further study should be encouraged to make genetic improvement for heat tolerance in sows more efficient., (© 2024 Wiley‐VCH GmbH. Published by John Wiley & Sons Ltd.)

Introduction Short-term levodopa-carbidopa intestinal gel (LCIG) treatment using nasojejunal (NJ) tubes (NJ-LCIG test) is recommended for patients with advanced Parkinson's disease to ensure compatibility with this treatment system prior to permanent percutaneous endoscopic gastrojejunostomy. However, there have been no studies on NJ tube insertion by neurologists or on possible differences in treatment efficacy based on the NJ tube insertion method or tube tip position. We therefore investigated the effects of LCIG with NJ tube placement performed by a neurologist. Methods This retrospective observational study included 13 patients with advanced Parkinson's disease and NJ tube placement between March 1, 2020, and October 31, 2023. A neurologist performed all NJ tube placements, and the daily off-time and dyskinesia time before and after NJ tube placement were compared. We also investigated the effects of differences in the NJ tube tip site. Results NJ tubes were placed using either a combination of X-ray fluoroscopy-guided insertion and gastric motility methods (23.1%) or X-ray fluoroscopy-guided insertion alone (76.9%). All tubes were successfully placed in the descending duodenum (15.4%), ascending duodenum (23.1%), or jejunum (61.5%). The off time decreased significantly after the NJ-LCIG test (pre-NJ-LCIG test, 6.6 h [5.1-8.1] vs. post-NJ-LCIG test, 2.0 h [0.8-3.5], p<0.01). There was no difference in effectiveness based on the site of NJ tube tip placement. Conclusion Our results suggest that neurologists can place NJ tubes and that the NJ-LCIG test can also improve off-time, regardless of the placement site.

Objective: This study aimed to evaluate the feasibility of the recently commercialized reverse encoding distortion correction (RDC) method for echo-planar imaging (EPI) diffusion-weighted imaging (DWI) by applying clinical head MRI., Methods: This study included 50 consecutive patients who underwent head MRI, including single-shot (SS) EPI DWI and RDC-EPI DWI. For evaluation of normal structures, qualitative scores for image distortion, Dice similarity coefficient (DSC) values, distortion ratios, and mean apparent diffusion coefficient (ADC) values were assessed in the pons, temporal lobe at the skull base, and frontal lobe at the level of the lateral ventricles in 30 patients. To evaluate pathologies, qualitative scores for image distortion were assessed for 25 intracranial and 21 extracranial pathologies identified in 32 patients., Results: Qualitative scores for image distortion, DSC values, distortion ratios, and mean ADC values of the pons and temporal lobe were significantly different between SS-EPI DWI and RDC-EPI DWI, whereas those of the frontal lobe at the level of the lateral ventricles were not significantly different between the 2 DWIs. The qualitative scores for image distortion and mean ADC values of extracranial pathologies were significantly different between the DWIs, whereas those of intracranial pathologies were not significantly different., Conclusions: RDC-EPI DWI significantly reduced image distortion and showed higher mean ADC values of the brain parenchyma in the skull base and extracranial pathologies., Competing Interests: Y.I. is an employee of Canon Medical Systems Corporation. The other authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

A 49-year-old man presented for a screening esophagogastroduodenoscopy (EGD) as part of his registration for a donated kidney transplant. He had been on hemodialysis for 4 years for end-stage renal disease (ESRD) due to diabetic nephropathy. He was taking lanthanum carbonate (LaC) for hyperphosphatemia. A plain abdominal radiograph and CT showed the radiopaque appearance of LaC. EGD disclosed whitish spots resembling xanthomas in the stomach. Biopsies showed deposition of granular crystalline-like material and phagocytosis of these by multinucleated giant cells on HE staining. These histiocytes were positive for CD68. La deposition was suspected because of the long-term oral administration of LaC. LaC is commonly used to treat hyperphosphatemia in patients with ESRD. La deposition on the gastric mucosa presents with various endoscopic findings, including whitish spots, annular whitish mucosa, and diffuse whitish mucosa. The gastric endoscopic findings of La deposition reflect the infiltrated histiocytes and not the LC deposition itself. Our case highlights the importance of recognizing the rare but characteristic imaging findings of gastrointestinal La deposition in hemodialysis patients who are taking LaC.

Objectives: Previous studies have shown possible choroid plexus (CP) dysfunction in Alzheimer's disease (AD) and highlighted CP enlargement on magnetic resonance imaging (MRI) as a predictive factor of AD. However, few studies have assessed the relationship between CP volume (CPV) and mild cognitive impairment (MCI). In this large elderly population study, we investigated the changes in CPV in patients with MCI using MRI above 65 years., Methods: This cross-sectional study included 2144 participants (median age, 69 years; 60.9% females) who underwent 3T MRI; they were grouped as 218 MCI participants and 1904 cognitively healthy controls. The total intracranial volume (ICV), total brain volume (TBV), CPV, hippocampal volume (HV), and lateral ventricle volume (LVV) were calculated., Results: CPV/ICV was a significant independent predictor of MCI (p < 0.01) after adjusting for potential confounders (age, sex, hypertension, hyperlipidemia, diabetes, and education level). The CPV/ICV ratio was also a significant independent predictor of MCI after adjusting for the TBV/ICV ratio (p = 0.022) or HV/ICV ratio (p = 0.017), in addition to potential confounders. The CPV was significantly correlated with the LVV (r = 0.97, p < 0.01)., Conclusion: We identified a relationship between CPV and MCI, which could not be explained by the degree of brain atrophy. Our results support CP dysfunction in MCI., Clinical Relevance Statement: Choroid plexus volume measurement may serve as a valuable imaging biomarker for diagnosing and monitoring mild cognitive impairment. The enlargement of the choroid plexus, independent of brain atrophy, suggests its potential role in mild cognitive impairment pathology., Key Points: • The study examines choroid plexus volume in relation to cognitive decline in elderly. • Enlarged choroid plexus volume independently indicates mild cognitive impairment presence. • Choroid plexus volume could be a specific biomarker for early mild cognitive impairment diagnosis., (© 2024. The Author(s), under exclusive licence to European Society of Radiology.)

Background: Biallelic variants in PARK7 , which encodes protein-nucleic acid deglycase DJ-1, can cause early-onset Parkinson's disease (PD). Although many patients with PARK7 variants have been identified from European and Middle Eastern ethnic groups, there have been no reports in the Japanese population., Objectives: To determine the prevalence and clinical features of patients with PD harboring PARK7 variants in Japan., Methods: We performed a molecular genetic analysis of PD patients with PARK7 variants identified using comprehensive panel sequencing, to explore the details of variants. Moreover, clinical neurological features were investigated, including neuroimaging analyses. This study followed STROBE guidelines., Results: Four patients with biallelic rare variants of PARK7 were identified in the cohort. All four patients presented with levodopa-responsive parkinsonism, with an age at onset in the early 30s. Furthermore, two of the four patients had psychiatric complications. Dopamine transporter imaging revealed nigrostriatal pathway dysfunction., Conclusions: To our knowledge, this is the first report of Japanese patients with PARK7 variants. We identified a relatively low frequency of PARK7 variants in patients in Japan. As opposed to typical patients with sporadic PD, the identified patients developed the disease in their 30s and presented with a variety of non-motor symptoms and complications. Further studies are needed to identify the clinical features related to PARK7 variants in Japanese patients with PD, and to analyze the pathophysiology of how the variants identified in the present study might affect DJ-1 function., Competing Interests: The authors declare the following financial interests/personal relationships which may be considered as potential competing interests:Manabu Funayama reports was provided by Promotion and Mutual Aid Corporation for Private Schools of Japan. Nobutaka Hattori reports financial support was provided by 10.13039/501100012359Promotion and Mutual Aid Corporation for Private Schools of Japan. Manabu Funayama reports a relationship with The Michael J Fox Foundation that includes: funding grants. Taku Hatano reports a relationship with Sumitomo Pharma Co Ltd that includes: speaking and lecture fees. Taku Hatano reports a relationship with 10.13039/100008373Takeda Pharmaceutical Company Limited that includes: speaking and lecture fees. Taku Hatano reports a relationship with 10.13039/501100004095Kyowa Kirin Co Ltd that includes: speaking and lecture fees. Taku Hatano reports a relationship with 10.13039/100008792Novartis Pharma Kabushiki Kaisha that includes: speaking and lecture fees. Taku Hatano reports a relationship with 10.13039/501100003769Eisai Co Ltd that includes: speaking and lecture fees. Taku Hatano reports a relationship with 10.13039/100015377Nihon Medi-Physics Co Ltd that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with Sumitomo Pharma Co Ltd that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with 10.13039/100008373Takeda Pharmaceutical Company Limited that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with 10.13039/501100004095Kyowa Kirin Co Ltd that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with 10.13039/501100007132Otsuka Pharmaceutical Co Ltd that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with 10.13039/501100003769Eisai Co Ltd that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with DAIICHI SANKYO COMPANY, LIMITED that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with 10.13039/501100012351Mitsubishi Tanabe Pharma Corporation that includes: speaking and lecture fees. Hiroshi Nishida reports a relationship with Mochida Pharmaceutical Co Ltd that includes: speaking and lecture fees. Kazuyuki Noda reports a relationship with Sumitomo Pharma Co Ltd that includes: speaking and lecture fees. Kazuyuki Noda reports a relationship with 10.13039/100008373Takeda Pharmaceutical Company Limited that includes: speaking and lecture fees. Kazuyuki Noda reports a relationship with 10.13039/501100007132Otsuka Pharmaceutical Co Ltd that includes: speaking and lecture fees. Kazuyuki Noda reports a relationship with 10.13039/501100004095Kyowa Kirin Co Ltd that includes: speaking and lecture fees. Kazuyuki Noda reports a relationship with 10.13039/100015993Kowa Company Ltd that includes: speaking and lecture fees. Masahiko Tomiyama reports a relationship with Sumitomo Pharma Co Ltd that includes: speaking and lecture fees. Masahiko Tomiyama reports a relationship with 10.13039/100008373Takeda Pharmaceutical Company Limited that includes: speaking and lecture fees. Masahiko Tomiyama reports a relationship with 10.13039/501100004095Kyowa Kirin Co Ltd that includes: speaking and lecture fees. Masahiko Tomiyama reports a relationship with 10.13039/501100013170Ono Pharmaceutical Co Ltd that includes: speaking and lecture fees. Masahiko Tomiyama reports a relationship with 10.13039/501100003769Eisai Co Ltd that includes: speaking and lecture fees. Yuanzhe Li reports a relationship with Eisai Co Ltd that includes: employment. Yuanzhe Li reports a relationship with Nihon Medi-Physics Co Ltd that includes: employment. Nobutaka Hattori reports a relationship with Sumitomo Pharma Co Ltd that includes: board membership, consulting or advisory, paid expert testimony, and speaking and lecture fees. Nobutaka Hattori reports a relationship with 10.13039/100008373Takeda Pharmaceutical Company Limited that includes: board membership, consulting or advisory, paid expert testimony, and speaking and lecture fees. Nobutaka Hattori reports a relationship with 10.13039/501100004095Kyowa Kirin Co Ltd that includes: board membership, consulting or advisory, paid expert testimony, and speaking and lecture fees. Nobutaka Hattori reports a relationship with 10.13039/501100010486Teijin Pharma Limited that includes: board membership, consulting or advisory, paid expert testimony, and speaking and lecture fees. Nobutaka Hattori reports a relationship with PARKINSON Laboratories Co. Ltd that includes: consulting or advisory and equity or stocks. Nobutaka Hattori reports a relationship with 10.13039/501100013170Ono Pharmaceutical Co Ltd that includes: board membership, paid expert testimony, and speaking and lecture fees. Nobutaka Hattori reports a relationship with Riken Center for Brain Science that includes: employment. Nobutaka Hattori reports a relationship with Abbvie 10.13039/100013093GK that includes: speaking and lecture fees. Nobutaka Hattori reports a relationship with 10.13039/501100007132Otsuka Pharmaceutical Co Ltd that includes: speaking and lecture fees. Nobutaka Hattori reports a relationship with 10.13039/100008792Novartis Pharma Kabushiki Kaisha that includes: speaking and lecture fees. Nobutaka Hattori reports a relationship with 10.13039/501100003769Eisai Co Ltd that includes: speaking and lecture fees. Nobutaka Hattori reports a relationship with DAIICHI SANKYO COMPANY, LIMITED that includes: speaking and lecture fees. Nobutaka Hattori reports a relationship with 10.13039/501100013126FP Pharmaceutical Corporation that includes: speaking and lecture fees. Nobutaka Hattori reports a relationship with The Michael J Fox Foundation that includes: funding grants. Nobutaka Hattori reports a relationship with 10.13039/100008440International Parkinson and Movement Disorder Society that includes: funding grants. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Author(s).)

Atypical antipsychotics are considered to be better tolerated than typical antipsychotics; however, the risk of drug-induced movement disorders needs to be considered. Aripiprazole, a dopamine partial agonist, is one of the most frequently used atypical antipsychotics in children. In this report, we describe withdrawal dyskinesia after aripiprazole discontinuation in a child with autism spectrum disorder. The patient presented with oral dyskinesia after discontinuation of aripiprazole when he was 13 years old. Dyskinetic movements disappeared after reinitiation of aripiprazole. He developed oral dyskinesia again after a reduction of the aripiprazole dose when he was 14 years old. Dyskinesia gradually disappeared within a few months. Withdrawal dyskinesia associated with aripiprazole has been rarely reported in children. Moreover, there is no large study on the prevalence of dyskinesia associated with aripiprazole discontinuation either in adults or in children. However, relevant cases might be unreported, pretermitted, or regarded as akathisia or symptoms of attention-deficit hyperactivity disorder. The prevalence of withdrawal dyskinesia associated with aripiprazole, especially in children, may be more frequent than thought. Withdrawal dyskinesia is self-limited; however, such dyskinetic movements in children potentially result in irreversible effects that damage the quality of life. As such, physicians should be mindful when changing, reducing, or discontinuing antipsychotics in children., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nishijima et al.)

Rationale and Objectives: Although hyperintensity in the anterior portion of the callosal splenium on FLAIR (aCS-hyperintensity) is a common finding in elderly adults, no previous studies have examined the clinical significance. In this large elderly population study, we aimed to investigate the associations of aCS-hyperintensity with vascular risk factors, cognitive decline, and other MRI measurements., Materials and Methods: This cross-sectional study included 2110 participants (median age, 69 years; 61.1% females) who underwent 3 T MRI. The participants were grouped as 215 with mild cognitive impairment (MCI) and 1895 cognitively normal older adults (NOAs). Two neuroradiologists evaluated aCS-hyperintensity by using a four-point scale (none, mild, moderate, and severe). Periventricular hyperintensities (PVHs) were also rated on a four-point scale according to the Fazekas scale. The total intracranial volume (ICV), total brain volume, choroid plexus volume (CPV), and lateral ventricle volume (LVV) were calculated., Results: Logistic regression analysis showed diabetes was the main predictor of aCS-hyperintensity after adjusting for potential confounders (age, sex, hypertension, and hyperlipidemia) (p < 0.01), whereas PVH was associated with hypertension (p < 0.01). aCS-hyperintensity rated as "severe" was associated with a presence of MCI (p < 0.01). For the imaging factors, LVV was an independent predictor of aCS-hyperintensity when brain volume and PVH grade were added to the analysis (p < 0.01)., Conclusion: Cerebral small vessel disease due to diabetes is a major contributor to the development of aCS-hyperintensity. Cerebrospinal fluid clearance failure may also relate to aCS-hyperintensity, which may offer new insights into the pathologic processes underlying MCI., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Purpose: To propose a five-point scale for radiology report importance called Report Importance Category (RIC) and to compare the performance of natural language processing (NLP) algorithms in assessing RIC using head computed tomography (CT) reports written in Japanese., Materials and Methods: 3728 Japanese head CT reports performed at Osaka University Hospital in 2020 were included. RIC (category 0: no findings, category 1: minor findings, category 2: routine follow-up, category 3: careful follow-up, and category 4: examination or therapy) was established based not only on patient severity but also on the novelty of the information. The manual assessment of RIC for the reports was performed under the consensus of two out of four neuroradiologists. The performance of four NLP models for classifying RIC was compared using fivefold cross-validation: logistic regression, bidirectional long-short-term memory (BiLSTM), general bidirectional encoder representations of transformers (general BERT), and domain-specific BERT (BERT for medical domain)., Results: The proportion of each RIC in the whole data set was 15.0%, 26.7%, 44.2%, 7.7%, and 6.4%, respectively. Domain-specific BERT showed the highest accuracy (0.8434 ± 0.0063) in assessing RIC and significantly higher AUC in categories 1 (0.9813 ± 0.0011), 2 (0.9492 ± 0.0045), 3 (0.9637 ± 0.0050), and 4 (0.9548 ± 0.0074) than the other models (p < .05). Analysis using layer-integrated gradients showed that the domain-specific BERT model could detect important words, such as disease names in reports., Conclusions: Domain-specific BERT has superiority over the other models in assessing our newly proposed criteria called RIC of head CT radiology reports. The accumulation of similar and further studies of has a potential to contribute to medical safety by preventing missed important findings by clinicians., (© 2024. The Author(s).)

Purpose: To evaluate the diagnostic value of T1-weighted 3D fast spin-echo sequence (CUBE) with deep learning-based reconstruction (DLR) for depiction of pituitary adenoma and parasellar regions on contrast-enhanced MRI., Methods: We evaluated 24 patients with pituitary adenoma or residual tumor using CUBE with and without DLR, 1-mm slice thickness 2D T1WI (1-mm 2D T1WI) with DLR, and 3D spoiled gradient echo sequence (SPGR) as contrast-enhanced MRI. Depiction scores of pituitary adenoma and parasellar regions were assigned by two neuroradiologists, and contrast-to-noise ratio (CNR) was calculated., Results: CUBE with DLR showed significantly higher scores for depicting pituitary adenoma or residual tumor compared to CUBE without DLR, 1-mm 2D T1WI with DLR, and SPGR (p < 0.01). The depiction score for delineation of the boundary between adenoma and the cavernous sinus was higher for CUBE with DLR than for 1-mm 2D T1WI with DLR (p = 0.01), but the difference was not significant when compared to SPGR (p = 0.20). CUBE with DLR had better interobserver agreement for evaluating adenomas than 1-mm 2D T1WI with DLR (Kappa values, 0.75 vs. 0.41). The CNR of the adenoma to the brain parenchyma increased to a ratio of 3.6 (obtained by dividing 13.7, CNR of CUBE with DLR, by 3.8, that without DLR, p < 0.01). CUBE with DLR had a significantly higher CNR than SPGR, but not 1-mm 2D T1WI with DLR., Conclusion: On the contrast-enhanced MRI, compared to CUBE without DLR, 1-mm 2D T1WI with DLR and SPGR, CUBE with DLR improves the depiction of pituitary adenoma and parasellar regions., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Competing Interests: CONFLICT OF INTEREST STATEMENT: None declared.

Purpose: Brain MRI with high spatial resolution allows for a more detailed delineation of multiple sclerosis (MS) lesions. The recently developed deep learning-based reconstruction (DLR) technique enables image denoising with sharp edges and reduced artifacts, which improves the image quality of thin-slice 2D MRI. We, therefore, assessed the diagnostic value of 1 mm-slice-thickness 2D T2-weighted imaging (T2WI) with DLR (1 mm T2WI with DLR) compared with conventional MRI for identifying MS lesions., Methods: Conventional MRI (5 mm T2WI, 2D and 3D fluid-attenuated inversion recovery) and 1 mm T2WI with DLR (imaging time: 7 minutes) were performed in 42 MS patients. For lesion detection, two neuroradiologists counted the MS lesions in two reading sessions (conventional MRI interpretation with 5 mm T2WI and MRI interpretations with 1 mm T2WI with DLR). The numbers of lesions per region category (cerebral hemisphere, basal ganglia, brain stem, cerebellar hemisphere) were then compared between the two reading sessions., Results: For the detection of MS lesions by 2 neuroradiologists, the total number of detected MS lesions was significantly higher for MRI interpretation with 1 mm T2WI with DLR than for conventional MRI interpretation with 5 mm T2WI (765 lesions vs. 870 lesions at radiologist A, < 0.05). In particular, of the 33 lesions in the brain stem, radiologist A detected 21 (63.6%) additional lesions by 1 mm T2WI with DLR., Conclusion: Using the DLR technique, whole-brain 1 mm T2WI can be performed in about 7 minutes, which is feasible for routine clinical practice. MRI with 1 mm T2WI with DLR enabled increased MS lesion detection, particularly in the brain stem.

TDP-43 aggregates (skeins and round inclusions [RIs]) are frequent histopathological features of amyotrophic lateral sclerosis (ALS). We have shown that diffuse punctate cytoplasmic staining (DPCS) is the earliest pathologic manifestation of TDP-43 in ALS, corresponding to nonfibrillar TDP-43 located in the rough endoplasmic reticulum. Previous in vitro studies have suggested that TDP-43 inclusions may be derived from stress granules (SGs). Therefore, we investigated the involvement of SGs in the formation of TDP-43 inclusions. Formalin-fixed spinal cords of six ALS patients with a disease duration of less than 1 year (short duration), eight patients with a disease duration of 2-5 years (standard duration), and five normal controls were subjected to histopathological examination using antibodies against an SG marker, HuR. In normal controls, the cytoplasm of anterior horn cells was diffusely HuR-positive. In short-duration and standard-duration ALS, the number of HuR-positive anterior horn cells was significantly decreased relative to the controls. DPCS and RIs were more frequent in short-duration ALS than in standard-duration ALS. The majority of DPCS areas and a small proportion of RIs, but not skeins, were positive for HuR. Immunoelectron microscopy showed that ribosome-like granular structures in DPCS areas and RIs were labeled with anti-HuR, whereas skeins were not. These findings suggest that colocalization of TDP-43 and SGs occurs at the early stage of TDP-43 aggregation., (© 2023 The Authors. Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology.)

Introduction: Device-aided therapy (DAT) is an established treatment for improving the quality of life (QOL) in individuals with advanced Parkinson's disease (APD). Criteria for starting DAT, including motor and non-motor symptoms, have been proposed. However, it remains unclear whether QOL differences among patients with APD influence DAT introduction. Therefore, we aimed to investigate QOL differences between patients with and without DAT introduction., Methods: This retrospective observational cross-sectional study included 245 patients with PD who were followed up between January 1, 2020, and June 30, 2022. We defined cases that underwent DAT introduction after evaluation as "planned-DAT" and those that did not as "not-planned-DAT." We performed between-group comparisons of the PD questionnaire-39 (PDQ-39) summary index (SI) in patients with APD who met the 5-2-1 criteria (≥5 times the oral levodopa dose/day, ≥2 h of "off" symptoms/day, and ≥ 1 h of troublesome dyskinesia/day)., Results: Seventy-nine patients met the inclusion criteria for APD (median age: 68 [61.0-73.0] years; 62.8% [N = 52] women). The PDQ-39 SI scores were higher in the planned-DAT group (N = 12) than in the not-planned-DAT group (N = 67) (29.2 [22.1-33.6] vs. 19.0 [10.3-49.6] points, P < 0.05). After propensity-score matching according to age and sex, the PDQ-39 SI scores remained higher in the planned-DAT (N = 9) than in the not-planned-DAT group (N = 18) (40.0 [25.4-60.0] vs. 18.5 [7.9-46.8] points, P < 0.05)., Conclusions: Our results suggest that QOL assessment using PDQ-39 can be used to identify patients eligible for DAT., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier B.V.)

The quantification of stomatal pore size has long been a fundamental approach to understand the physiological response of plants in the context of environmental adaptation. Automation of such methodologies not only alleviates human labor and bias but also realizes new experimental research methods through massive analysis. Here, we present an image analysis pipeline that automatically quantifies stomatal aperture of Arabidopsis thaliana leaves from bright-field microscopy images containing mesophyll tissue as noisy backgrounds. By combining a You Only Look Once X-based stomatal detection submodule and a U-Net-based pore segmentation submodule, we achieved a mean average precision with an intersection of union (IoU) threshold of 50% value of 0.875 (stomata detection performance) and an IoU of 0.745 (pore segmentation performance) against images of leaf discs taken with a bright-field microscope. Moreover, we designed a portable imaging device that allows easy acquisition of stomatal images from detached/undetached intact leaves on-site. We demonstrated that this device in combination with fine-tuned models of the pipeline we generated here provides robust measurements that can substitute for manual measurement of stomatal responses against pathogen inoculation. Utilization of our hardware and pipeline for automated stomatal aperture measurements is expected to accelerate research on stomatal biology of model dicots., (© The Author(s) 2023. Published by Oxford University Press on behalf of Japanese Society of Plant Physiologists. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Case 1 presented with severe anemia and received an intrauterine blood cell transfusion at 33 weeks of gestation. The anemia spontaneously improved in early infancy. Case 2, the father of Case 1, had an uneventful birth with no evidence of anemia, though microcytic anemia was observed during childhood. The genetic analysis of the β-globin gene cluster identified a novel heterozygous deletion of DNA extending from the Gγ-globin gene downstream to the β-globin gene, confirming a diagnosis of ( G γ A γδβ) 0 -thalassemia. In cases where thalassemia is suspected based on blood tests, a genetic diagnosis should be performed for the sake of the offspring., (© 2023 Wiley Periodicals LLC.)

Although altered networks inside the hippocampus (hippocampal intra-networks) have been observed in dementia, the evaluation of hippocampal intra-networks using magnetic resonance imaging (MRI) is challenging. We employed conventional structural imaging and incident component analysis (ICA) to investigate the structural covariance of the hippocampal intra-networks. We aimed to assess altered hippocampal intra-networks in patients with mild cognitive impairment (MCI). A cross-sectional study of 2122 participants with 3T MRI (median age 69 years, 60.9% female) were divided into 218 patients with MCI and 1904 cognitively normal older adults (CNOA). By employing 3D T1-weighted imaging, voxels within the hippocampus were entered into the ICA analysis to extract the structural covariance intra-networks within the hippocampus. The ICA extracted 16 intra-networks from the hippocampal structural images, which were divided into two bilateral networks and 14 ipsilateral networks. Of the 16 intra-networks, two (one bilateral network and one ipsilateral networks) were significant predictors of MCI from the CNOA after adjusting for age, sex, education, disease history, and hippocampal volume/total intracranial volume ratio. In conclusion, we found that the relationship between hippocampal intra-networks and MCI was independent from the hippocampal volume. Our results suggest that altered hippocampal intra-networks may reflect a different pathology in MCI from that of brain atrophy., (© 2023. Springer Nature Limited.)

Background: A rat model of levodopa-induced dyskinesia (LID) showed enlarged axon terminals of striatal direct pathway neurons in the internal segment of the globus pallidus (GPi) with excessive gamma-aminobutyric acid (GABA) storage in them. Massive GABA release to GPi upon levodopa administration determines the emergence of LID., Objectives: We examined whether LID and axon terminal hypertrophy gradually develop with repeated levodopa treatment in Parkinsonian rats to examine if the hypertrophy reflects dyskinesia priming., Methods: 6-hydroxydopamine-lesioned hemiparkinsonian rats were randomly allocated to receive saline injections (placebo group, 14 days; n = 4), injections of 6 mg/kg levodopa methyl ester combined with 12.5 mg/kg benserazide (levodopa-treated groups, 3-day-treatment; n = 4, 7-day-treatment; n = 4, 14-day-treatment; n = 4), or injections of 6 mg/kg levodopa methyl ester with 12.5 mg/kg benserazide and 1 mg/kg 8-hydroxy-2-(di-n-propylamino)tetralin for 14 days (8-OH-DPAT-treated group; n = 4). We evaluated abnormal involuntary movement (AIM) scores and axon terminals in the GPi., Results: The AIM score increased with levodopa treatment, as did the hypertrophy of axon terminals in the GPi, showing an increased number of synaptic vesicles in hypertrophied terminals., Conclusion: Increased GABA storage in axon terminals of the direct pathway neurons represents the priming process of LID., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Nakamura, Nishijima, Mori, Kinoshita, Kon, Suzuki, Wakabayashi and Tomiyama.)

Background: l-3,4-dihydroxyphenylalanine (l-dopa) is the most effective drug for Parkinson's disease (PD); however, most PD patients develop motor fluctuations including wearing-off and l-dopa-induced dyskinesia (LID). Amantadine is beneficial for improving the motor symptoms, reducing "off" time, and ameliorating LID, although its long-term efficacy remains unknown., Objectives: To investigate the effects of amantadine on PD and LID using a rat model with repetitive drug treatment., Method: We utilized 6-hydroxydopamine injections to develop a hemiparkinsonian rat model. The rats were assigned to four groups: five rats received l-dopa and benserazide for 31 days, six rats received l-dopa and benserazide plus amantadine for 31 days, five rats received l-dopa and benserazide for 15 days followed by l-dopa and benserazide plus amantadine for 16 days, and five rats received l-dopa and benserazide plus amantadine for 15 days followed by l-dopa and benserazide treatment for 16 days. We evaluated the l-dopa-induced abnormal involuntary movements on treatment days 1, 7, 14, 16, 22, and 29. Subsequently, immunohistochemistry for drebrin was performed., Results: l-dopa-induced abnormal movements were reduced on the first day of amantadine treatment, and these effects disappeared with repetitive treatment. In contrast, the extension of l-dopa "on" time was observed after repetitive amantadine treatment. All groups showed enlarged drebrin immunoreactive dots in the dopamine-denervated striatum, indicating that amantadine did not prevent priming effects of repetitive l-dopa treatment., Conclusion: Anti-LID effect of amantadine diminished after repetitive treatment, and the effect of amantadine on wearing-off emerged after repetitive treatment in a hemiparkinsonian rat model. Fluctuations in amantadine effects should be considered when using it in clinical settings., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Aims: Synaptic dysfunction in Parkinson's disease is caused by propagation of pathogenic α-synuclein between neurons. Previously, in multiple system atrophy (MSA), pathologically characterised by ectopic deposition of abnormal α-synuclein predominantly in oligodendrocytes, we demonstrated that the occurrence of memory impairment was associated with the number of α-synuclein-positive neuronal cytoplasmic inclusions (NCIs) in the hippocampus. In the present study, we aimed to investigate how abnormal α-synuclein in the hippocampus can lead to memory impairment., Methods: We performed pathological and biochemical analyses using a mouse model of adult-onset MSA and human cases (MSA, N = 25; Parkinson's disease, N = 3; Alzheimer's disease, N = 2; normal controls, N = 11). In addition, the MSA model mice were examined behaviourally and physiologically., Results: In the MSA model, inducible human α-synuclein was first expressed in oligodendrocytes and subsequently accumulated in the cytoplasm of excitatory hippocampal neurons (NCI-like structures) and their presynaptic nerve terminals with the development of memory impairment. α-Synuclein oligomers increased simultaneously in the hippocampus of the MSA model. Hippocampal dendritic spines also decreased in number, followed by suppression of long-term potentiation. Consistent with these findings obtained in the MSA model, post-mortem analysis of human MSA brain tissues showed that cases of MSA with memory impairment developed more NCIs in excitatory hippocampal neurons along with α-synuclein oligomers than those without., Conclusions: Our results provide new insights into the role of α-synuclein oligomers as a possible pathological cause of memory impairment in MSA., (© 2022 British Neuropathological Society.)

Objectives: Forced vital capacity (FVC) is recommended as a respiratory function test in patients with amyotrophic lateral sclerosis (ALS). However, in ALS associated with orofacial palsy, FVC may be an unreliable test. Slow vital capacity (SVC) is an easier and more reliable test even in cases with bulbar symptoms. However, it remains unclear whether respiratory function tests using SVC and FVC are associated with prognosis after percutaneous endoscopic gastrostomy (PEG) placement. This study aimed to confirm whether both SVC and FVC are related to prognosis after PEG placement in patients with ALS., Materials and Methods: We conducted this retrospective observational cohort study of 69 consecutive patients diagnosed with sporadic ALS who underwent PEG placement between July 2007 and February 2020. We analyzed the association with mortality 6 months after PEG placement and evaluated long-term prognosis., Results: Forty-four patients met the inclusion criteria. In cases with decreased SVC (p < .01) and FVC (p < .01), a significant difference was observed in mortality 6 months after PEG placement, with an optimal cut-off of SVC ≤57.4% (sensitivity, 0.828; specificity, 0.867) and FVC ≤57.3% (sensitivity, 0.828; specificity, 0.867). Multivariate analysis showed that onset age ≥ 65 years (p < .05), SVC ≤57.4% (p < .01), and FVC ≤57.3% (p < .01) were associated with survival after PEG placement., Conclusions: SVC, like FVC, is an important prognostic factor after PEG placement in patients with ALS, and there is a possibility that evaluation using SVC can complement respiratory function testing even in cases where the evaluation of FVC is limited., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

α-Synuclein (α-Syn) binds to vesicle-associated membrane protein-binding protein B (VAPB) in the endoplasmic reticulum membrane. Recent studies have shown that α-Syn-immunoreactive Lewy pathology is characterized by membrane crowding, including vesicular structures. To elucidate the role of VAPB and vesicular structures in Parkinson's disease (PD) and in dementia with Lewy bodies (DLB), the relationships among VAPB, vesicular structures, and Lewy pathology were investigated by immunohistochemistry and immunoelectron microscopy in 8 PD and 4 DLB autopsy cases. The proportions of VAPB-negative neurons in the substantia nigra in PD and in the temporal cortex in DLB were significantly higher than those in 5 controls. In PD, the incidence of α-Syn inclusions in VAPB-negative neurons was significantly higher (77.4%) than in VAPB-positive neurons (1.6%) in the substantia nigra. In DLB, the incidence of α-Syn inclusions in VAPB-negative neurons was also significantly higher (65.3%) than in VAPB-positive neurons (2.8%) in the temporal cortex. Immunoelectron microscopy revealed that α-Syn and VAPB were localized to filamentous structures of Lewy bodies (LBs). However, only a few vesicular structures labeled with anti-α-Syn were observed within LBs. These findings suggest that reduction of VAPB is involved in the disease processes of PD and DLB, although vesicular structures may not directly contribute to the formation of LBs., (© The Author(s) 2022. Published by Oxford University Press on behalf of American Association of Neuropathologists, Inc. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Intermittent administration of L-dopa in Parkinson's disease is associated with L-dopa-induced dyskinesia (LID). Long-acting dopamine agonists may reduce the risk of LID by continuous dopaminergic stimulation. We examined the LID-like behavior, preprodynorphin messenger ribonucleic acid (mRNA) expression in the striatum (a neurochemical LID hallmark), and the volume of the entopeduncular nucleus (a pathological LID hallmark) in Parkinson's disease rat models that were treated with L-dopa and cabergoline. Cabergoline co-treatment with L-dopa reduced LID, striatal preprodynorphin mRNA expression, and hypertrophy of the entopeduncular nucleus, indicating that cabergoline has an anti-LID effect independent of the L-dopa-sparing effect., (Copyright © 2022 Elsevier Ltd and Japan Neuroscience Society. All rights reserved.)

In recent years, the term "functional" is starting to replace "psychogenic" when referring to a specific group of movement disorders, with an increasing emphasis being placed on the role of neurologists in the management of these disorders. These conditions are common and disabling, and sometimes difficult to diagnose. History taking and physical examination may highlight positive signs suggestive of diagnosis, which should not be based on exclusion. During examination, the distraction sign may be observed. When functional myoclonus is present, the patient's movements may vary over time. The role of a neurologist is not only to diagnose functional movement disorders, but also explain to the patient that the patient has a distinct neurological disorder and provide clinical evidence that supports the diagnosis. It is useful to share the positive signs with patients when explaining the diagnosis. In this article, I describe a patient with functional abdominal jerks to demonstrate how a diagnosis is made and how one can explain the diagnosis to the patient. Once a patient accepts and understands the diagnosis, treatment can be initiated. Collaboration with psychiatrists and physiotherapists is essential. It has been shown that cognitive behavioral therapy and physiotherapy are beneficial in ameliorating functional movements.

Transactivation response DNA-binding protein 43 (TDP-43)-immunoreactive neuronal cytoplasmic inclusions (NCIs) are the histopathological hallmarks of amyotrophic lateral sclerosis (ALS). They are classified as skein-like inclusions, round inclusions, dot-like inclusions, linear wisps, and diffuse punctate cytoplasmic staining (DPCS). We hypothesized that TDP-43-immunoreactive DPCS may form the early-stage pathology of ALS. Hence, we investigated phosphorylated TDP-43 pathology in the upper and lower motor neurons of patients with ALS and control participants. We designated patients whose disease duration was ≤1 year as short-duration ALS (n = 7) and those whose duration equaled 3-5 years as standard-duration ALS (n = 6). DPCS and skein-like inclusions were the most common NCIs in short-duration and standard-duration ALS, respectively. The density of DPCS was significantly higher in short-duration ALS than that in standard-duration ALS and was inversely correlated with disease duration. DPCS was not ubiquitinated and disappeared after proteinase K treatment, suggesting that it was not aggregated. Immunoelectron microscopy revealed that DPCS corresponded to nonfibrillar TDP-43 localized to the ribosomes of the rough endoplasmic reticulum (ER). These findings suggest that nonfibrillar TDP-43 accumulation in the rough ER is the earliest TDP-43 pathology in ALS, which may be helpful in developing future TDP-43 breakdown strategies for ALS., (© 2022 American Association of Neuropathologists, Inc. All rights reserved.)

We estimated heritabilities of semen production traits and their genetic correlations with litter traits and pork production traits in purebred Duroc pigs. Semen production traits were semen volume, sperm concentration, proportion of morphologically normal sperms, total number of sperm, and total number of morphologically normal sperm. Litter traits at farrowing were total number born, number born alive, number stillborn, total litter weight at birth, mean litter weight at birth, and piglet survival rate at birth. Litter traits at weaning were litter size at weaning, total litter weight at weaning, mean litter weight at weaning, and piglet survival rate from birth to weaning. Pork production traits were average daily gain, backfat thickness, and loin muscle area. We analyzed 45,913 semen collection records of 896 boars, 6,950 farrowing performance records of 1,400 sows, 2,237 weaning performance records of 586 sows, and individual growth performance records of 9,550 animals measured at approximately 5 mo of age. Heritabilities were estimated using a single-trait animal model. Genetic correlations were estimated using a 2-trait animal model. Estimated heritabilities of semen production traits ranged from 0.20 for sperm concentration to 0.29 for semen volume and were equal to or higher than those of litter traits, ranging from 0.06 for number stillborn and piglet survival rate at birth to 0.25 for mean litter weight at birth, but lower than those of pork production traits, ranging from 0.50 for average daily gain to 0.63 for backfat thickness. In many cases, the absolute values of estimated genetic correlations between semen production traits and other traits were smaller than 0.3. These estimated genetic parameters provide useful information for establishing a comprehensive pig breeding scheme., (© The Author(s) 2022. Published by Oxford University Press on behalf of the American Society of Animal Science.)

Background: Guillain-Barré syndrome (GBS) and spinal epidural abscess (SEA) are known as mimics of each other because they present with flaccid paralysis following an infection; however, they differ in the main causative bacteria. Nevertheless, the two diseases can occur simultaneously if there is a preceding Campylobacter infection. Here, we report the first case of SEA with GBS following Campylobacter coli infection., Case Presentation: A 71-year-old Japanese man presented with progressive back pain and paralysis of the lower limbs following enteritis. Magnetic resonance imaging showed a lumbar epidural abscess that required surgical decompression; therefore, surgical drainage was performed. Blood cultures revealed the presence of C. coli. Despite surgery, the paralysis progressed to the extremities. Nerve conduction studies led to the diagnosis of GBS. Anti-ganglioside antibodies in the patient suggested that GBS was preceded by Campylobacter infection. Intravascular immunoglobulin therapy attenuated the progression of the paralysis., Conclusions: We report a case of SEA and GBS following Campylobacter infection. A combination of the two diseases is rare; however, it could occur if the preceding infection is caused by Campylobacter spp. If a cause is known but the patient does not respond to the corresponding treatment, it is important to reconsider the diagnosis based on the medical history., (© 2021. The Author(s).)

The pathological hallmark of multiple system atrophy (MSA) is fibrillary aggregates of α-synuclein (α-Syn) in the cytoplasm and nucleus of both oligodendrocytes and neurons. In neurons, α-Syn localizes to the cytosolic and membrane compartments, including the synaptic vesicles, mitochondria, and endoplasmic reticulum (ER). α-Syn binds to vesicle-associated membrane protein-binding protein B (VAPB) in the ER membrane. Overexpression of wild-type and familial Parkinson's disease mutant α-Syn perturbs the association between the ER and mitochondria, leading to ER stress and ultimately neurodegeneration. We examined brains from MSA patients (n = 7) and control subjects (n = 5) using immunohistochemistry and immunoelectron microscopy with antibodies against VAPB and phosphorylated α-Syn. In controls, the cytoplasm of neurons and glial cells was positive for VAPB, whereas in MSA lesions VAPB immunoreactivity was decreased. The proportion of VAPB-negative neurons in the pontine nucleus was significantly higher in MSA (13.6%) than in controls (0.6%). The incidence of cytoplasmic inclusions in VAPB-negative neurons was significantly higher (42.2%) than that in VAPB-positive neurons (3.6%); 67.2% of inclusion-bearing oligodendrocytes and 51.1% of inclusion-containing neurons were negative for VAPB. Immunoelectron microscopy revealed that α-Syn and VAPB were localized to granulofilamentous structures in the cytoplasm of oligodendrocytes and neurons. Many vesicular structures labeled with anti-α-Syn were also observed within the granulofilamentous structures in the cytoplasm and nucleus of both oligodendrocytes and neurons. These findings suggest that, in MSA, reduction of VAPB is involved in the disease process and that vesicular structures are associated with inclusion formation., (© 2021 The Authors. Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology.)