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Research on Caucasian and Japanese subjects shows a correlation between large normal CAG allele frequencies and the CAG-repeat expansions coinciding with dominantly inherited spinocerebellar ataxias (SCAs). Dominant SCA types and dentatorubralpallidoluysian atrophy were found primarily in Caucasian and Japanese populations respectively, with prevalences matching comparatively large numbers of normal alleles.

Relatively little is known about the factors controlling ectomycorrhizal fungal communities. One possible factor is forest litter chemistry. In a series of experiments we demonstrated that the growth of ectomycorrhizal fungi able to colonize red pine (Pinus resinosa Ait.) are differentially affected by red pine needles and needle chemical components. For example, water extracts of pine needles stimulated the growth of Suillus intermedius (Smith & Thiers) Smith & Theirs and inhibited the growth of Amanita rubescens Pers. Catechin and epicatechin gallate, components of the water extract, acted similarly to the extract. The volatile compounds α- and β-pinene also had differential effects on the growth of the various species of ectomycorrhizal fungi. Our results suggest that forest litter chemistry has the potential differentially to affect the growth of ectomycorrhizal fungal species and so could affect the structure of ectomycorrhizal fungal communities. [ABSTRACT FROM AUTHOR]

Delusional misidentification, a rare syndrome in which a patient displays persistent delusional misidentification of individuals or objects, occurs in several types of dementia. However, the pathology of delusional misidentification is still unclear, and there was no data pertaining to striate-frontal projection. Here, we report a case of delusional misidentification following frontotemporal dementia in which complex striate-frontal and some specific frontal gyrus dysfunction were observed. In our presented case, delusional misidentification progressed following frontal atrophy. Believing that her actual daughter had been replaced by her niece, her symptoms of delusional misidentification and frontal atrophy progressed in the short term, and social arrangement was necessary three months after the onset. There were no abnormal neurological findings including parkinsonism and general cognitive function test scores were preserved. Validated by dopamine transporter single-photon emission computed tomography, right unilateral striatal uptake decreased significantly without parkinsonism or Parkinson's disease. In addition, of specific concern, functional magnetic resonance images showed left opercular inferior frontal gyrus and right superior frontal gyrus dysfunctions. Our case study highlights complex striate-frontal projection and specific frontal gyrus dysfunctions associated with the pathology of delusional misidentification syndrome., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Atrial switch procedures such as the Mustard operation were previously popular for the complete transposition of the great arteries (i.e. dextro-transposition of the great arteries [d-TGA]). Patients with d-TGA who underwent atrial switch procedures approximately three decades ago have now entered adulthood. A female patient in her 30s with d-TGA had a paradoxical embolic stroke following the initiation of a low-dose oestrogen plus progesterone oral pill for dysmenorrhoea. She underwent Mustard surgery when she was 2 years old. Following a series of procedures including implantation of a permanent pacemaker that was required because of sinus node dysfunction, she had reached adulthood, was living by herself and working independently. One month after taking the low-dose oestrogen plus progesterone oral pill, venous thrombosis occurred in the left soleus and left peroneal veins; and she experienced an acute ischaemic stroke in the right middle cerebral artery area. Transoesophageal echocardiography revealed that the shunt was present only during the Valsalva manoeuvre. Based on the examinations, the patient was diagnosed with juvenile ischaemic stroke as a result of a paradoxical embolism. These findings suggest that paradoxical cerebral embolism can occur as a late complication in patients with d-TGA who underwent the Mustard operation as children., Competing Interests: Declaration of conflicting interestThe authors declare that there are no conflicts of interest.

Objective: We investigated the effect of the pandemic on neurological hospitalizations and complications associated with severe acute respiratory syndrome coronavirus 2 infection or vaccinations., Methods: We retrospectively analyzed data of patients hospitalized in our neurology division from 1 April 2019 to 31 March 2022 as the opt-out study. We classified the neurological diseases into nine subgroups, evaluated changes of neurological disease characteristics, and analyzed patients hospitalized with the complications from severe acute respiratory syndrome coronavirus 2 infection or after the coronavirus disease 2019 vaccination over three eras based on the pandemic stages: (1) pre-pandemic, (2) during the pandemic but before vaccines, and (3) during the pandemic with vaccines., Results: Overall, 1756 patients were included in the analyses. The patient characteristics significantly changed throughout the pandemic ( p  < 0.01). Although the number of autoimmune cases did not change throughout the pandemic ( p  = 0.53), that of psychological cases and that of unknown cases were significantly changed ( p  < 0.05, p  < 0.01). There were four infectious cases and 11 cases following vaccination from 1 April 2020 to 31 March 2022. The 11 postvaccination cases involved 10 kinds of neurological diseases., Conclusions: The neurological characteristics significantly changed throughout the pandemic and there were diverse neurological complications following vaccinations., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Some neurological complications are associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). A 74-year-old man was diagnosed with infection by SARS-CoV-2. Eighteen days after SARS-CoV-2 infection, he developed disturbed consciousness and aseptic meningoencephalitis. An analysis of cerebrospinal flood revealed an elevated cell count (184/μL) and protein level (260 mg/dL). Cranial magnetic resonance imaging showed no abnormalities. By contrast, 123 I-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography showed a significant decrease in cerebral blood flow (CBF) in the left parietal and occipital lobes. He died suddenly 3 months after being transferred to a rehabilitation clinic without any clear cause of death. The SARS-CoV-2 infection can cause aseptic meningoencephalitis with a distinctive decrease in CBF pattern without magnetic resonance image abnormality or intracranial artery stenosis., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Background/aim: Our recent studies have indicated that trace copper co-existed with iron in hemosiderin particles of human genetic iron overload. To understand this phenomenon, we analyzed hemosiderin particles in iron-overloaded rat liver by using scanning transmission electron microscopy - energy-dispersive X-ray (STEM-EDX) spectroscopy., Materials and Methods: Samples for STEM-EDX spectroscopy were prepared from the liver of rats administered an intraperitoneal injection of dextran iron., Results: The micro-domain analysis with STEM-EDX spectroscopy showed that dense bodies contained high levels of iron and trace copper. Quantitative analysis of copper levels in the liver specimen using atomic spectrophotometry showed that copper concentration in the liver was not increased by iron overload. These findings suggest that the overload of iron induced distribution of trace copper to hemosiderin particles without changing cellular copper levels., Conclusion: Co-existence of copper with iron was observed in hemosiderin particles of the liver of an experimental model of iron overload, suggesting that iron overload induced distribution of trace copper into hemosiderin particles., (Copyright © 2024, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background: Orofacial pain conditions are complex disorders that involve biological, social, and psychological factors. Temporomandibular Disorders (TMDs) are one of the most common orofacial pain conditions, and our previous literature review indicated that exercise therapy has shown promise in reducing TMD-related pain. However, more evidence is needed to firmly establish its effectiveness., Objectives: This systematic review aims to investigate the effectiveness of exercise therapy on pain relief and jaw mobility in patients with pain-related TMDs., Methods: To include randomized controlled trials (RCTs) written in English, a literature search was performed using PubMed, Scopus, Web of Science, Cochrane Library, Ovid, EBM reviews, and Academic Search Complete initially from 4th November 2020 until March 2022. A PICOS for this review was as follows; P: Patients with TMD myalgia or arthralgia, I: Excursion exercise, Stretch exercises, Resistance exercise, or Coordination exercise, C: No treatment or education only. O: Pain intensity and Range of Motion (ROM), S: RCTs. After title screening, a full-text assessment was done to extract data. According to Risk of Bias (RoB) 2.0, risk of bias was assessed in each included paper by 2 reviewers independently., Results: A total of 3,388 titles were identified from the electronic database search. After the screening and full-text evaluation, only 5 studies (145 participants) were eligible to be included. Among the exercise modalities, coordination exercise showed a significant effect on pain relief and improvement of joint mobility., Discussion: Due to the heterogeneity and small sample size of the included studies, a meta-analysis was not feasible. However, this systematic review suggested that exercise therapy, especially coordinate exercise, can be effective in managing painful TMD conditions. Further research is needed to establish optimal parameters for this patient population, as well as standardization and consistency in terminology and treatment structure., 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. The handling editor NC declared a past co-authorship with the author AS., (© 2023 Shimada, Ogawa, Sammour, Narihara, Kinomura, Koide, Noma and Sasaki.)

Background: Valproic acid is associated with increased risks of tremor and parkinsonism., Case Report: A 67-year-old man with a diagnosis of epilepsy who had been treated with valproic acid (VPA) for 32 years noticed right-dominant upper-limb resting tremor accompanied by mild rigidity and bradykinesia. He was initially diagnosed with tremor-dominant Parkinson's disease (TDPD), but dopamine transporter single-photon emission computed tomography demonstrated no nigrostriatal degeneration. At 3 months after discontinuing VPA, his symptoms dramatically improved., Discussion: VPA-induced tremor usually consists of postural or kinetic tremor without asymmetry. Our case indicated that careful evaluation is needed, even in cases of asymmetrical resting tremor and mild parkinsonism resembling TDPD after long term exposure to VPA., Highlights: We report an atypical case of valproic acid-induced tremor and parkinsonism that mimics tremor-dominant Parkinson's disease. Physicians should not exclude the possible relation to valproic acid in patients presenting unilateral resting tremor and parkinsonism even in the absence of long-term side effects., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2023 The Author(s).)

We herein report a 70-year-old man diagnosed with IgG4-related hypertrophic pachymeningitis with skull base involvement, who presented with isolated glossopharyngeal and vagus nerve palsy. Contrast-enhanced magnetic resonance imaging (MRI) showed enhanced dural thickening of the posterior clivus and skull base involvement. When a patient with hypertrophic pachymeningitis presents with isolated cranial neuropathy without systemic manifestations or definite MRI abnormalities, it is difficult to make a diagnosis, and the patient may be misdiagnosed. This case suggests that a detailed radiological evaluation including contrast enhancement of the skull base is very important in patients with isolated glossopharyngeal and vagus nerve palsy.

Conformational fluctuation, namely, protein interconversion between different conformations, is crucial to protein function. Outer surface protein A (OspA), comprising N- and C-terminal globular domains linked by a central β-sheet, is expressed on the surface of Borrelia burgdorferi , the causative agent of Lyme disease, and recognizes the TROSPA receptor in the tick gut. Solution nuclear magnetic resonance studies have shown that the central β-sheet and C-terminal domain containing TROSPA recognition sites are less stable than the N-terminal domain, revealing an intermediate conformation between the basic folded and completely unfolded proteins. We previously suggested that exposure of receptor-binding sites following denaturation of the C-terminal domain is advantageous for OspA binding to the receptor. Here, we observed amplification of a specific protein fluctuation by pressure perturbation and site-specific mutagenesis. The salt-bridge-destabilized mutant E160D and the cavity-enlarged mutant I243A favored the intermediate. The proportion of the intermediate accounted for almost 100% in E160D at 250 MPa. Strategies using a suitably chosen point mutation with high pressure are generally applicable for amplification of specific conformational fluctuation and potentially improve our understanding of the intermediate conformations of proteins. Knowledge of various conformations, including OspA intermediates, may be useful for designing a vaccine for Lyme disease.

Despite emerging importance of tumor cells-derived exosomes in cancer metastasis, the heterogeneity of exosome populations has largely hampered systemic characterization of their molecular composition, biogenesis, and functions. This study communicates a novel method for predicting and targeting pre-metastatic sites based on an exosome model "fluorescent cancer glyconanosomes" displaying N-glycans of cultured tumor cells. Glycoblotting by antiadhesive quantum dots provides a nice tool to shed light on the pivotal functions of the glycocalyx reconstructed from four cancer cell types without bias due to other compositions of exosomes. In vivo imaging revealed that circulation, clearance, and organotropic biodistribution of cancer glyconanosomes in mice depend strongly on cancer cell-type-specific N-glycosylation patterns, the compositions of key glycotypes, particularly dominant abundances of high mannose-type N-glycans and the position-specific sialylation. Notably, organ biodistribution of cancer glyconanosomes is reproducible artificially by mimicking cancer cell-type-specific N-glycosylation patterns, demonstrating that nanosomal glycoblotting method serves as promising tools for predicting and targeting pre-metastatic sites determined by the glycocalyx of extracellular vesicles disseminated from the primary cancer site., (Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.)