Morphological analysis of autophagy in axonal degeneration in gracile axonal dystrophy mice.

Gracile axonal dystrophy (gad) mutant mice present with autosomal recessive inherited sensory ataxia in the early stages, followed by age-dependent motor ataxia. This phenotype is caused by a mutation in the ubiquitin carboxyl-terminal hydrolase isozyme L1 (UCH-L1) gene and leads to a lack of expression of UCH-L1 protein, which is related with the autophagy pathway and the ubiquitin-proteasome system (UPS). To elucidate the pathophysiology of abnormal protein accumulation in gad mice, we focused on macroautophagy.

Durvalumab plus carboplatin-etoposide treatment in a patient with small-cell lung cancer on hemodialysis: a case report and literature review.

Little is known about the efficacy and safety of durvalumab plus carboplatin-etoposide treatment in patients with extensive-disease (ED) small-cell lung cancer (SCLC) on hemodialysis. Here, we present a case of a 67-year-old man with pleuroperitoneal communication on continuous ambulatory peritoneal dialysis who was diagnosed with ED-SCLC based on a cytological analysis of the peritoneal fluid. He was switched from peritoneal dialysis to hemodialysis and received durvalumab (1500 mg/body on day 1) plus carboplatin (area under the concentration-time curve = 5, 125 mg on day 1) and etoposide (50 mg/m on days 1 and 3) as first-line therapy.

Additional Steroid Therapy for Delayed Facial Palsy in Miller Fisher Syndrome.

Miller Fisher syndrome (MFS) is a variant of Guillain-Barré syndrome. Delayed facial palsy (DFP) is a symptom that occurs after other neurological symptoms begin to recover within four weeks from the onset of MFS. As there have been few detailed reports about DFP in MFS cases treated with additional immunotherapy, we investigated three cases of DFP in MFS treated with additional steroid therapies.

Phenological shifts in lake stratification under climate change.

One of the most important physical characteristics driving lifecycle events in lakes is stratification. Already subtle variations in the timing of stratification onset and break-up (phenology) are known to have major ecological effects, mainly by determining the availability of light, nutrients, carbon and oxygen to organisms. Despite its ecological importance, historic and future global changes in stratification phenology are unknown.

[Eclizumab in the treatment of myasthenia gravis crisis complicating invasive thymoma: a case study of efficacy].

A 40-year-old male patient was diagnosed with invasive thymoma and myasthenia gravis in 2015. In 2016 and 2017, he experienced myasthenic crises, with an increase in size of invasive thymoma. In 2018, he received chemotherapy for the invasive thymoma.

TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons.

Mislocalization and abnormal deposition of TDP-43 into the cytoplasm (TDP-43 proteinopathy) is a hallmark in neurons of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). However, the pathogenic mechanism of the diseases linked to TDP-43 is largely unknown. We hypothesized that the failure of mRNA transport to neuronal axons by TDP-43 may contribute to neurodegeneration in ALS and FTLD, and sought to examine the function of TDP-43 by identifying its target mRNA for axonal transport.

Satellite-measured water properties in high altitude Lake Tahoe.

It has been difficult in satellite remote sensing to derive accurate water optical, biological, and biogeochemical products over high-altitude inland waters due to issues in satellite data processing (i.e., atmospheric correction).

Changes in clinical features of multiple system atrophy in Japan.

Introduction: Multiple system atrophy (MSA) is an adult-onset progressive neurodegenerative disease that causes parkinsonism, cerebellar ataxia, and/or autonomic failure. MSA is categorized as MSA with predominant cerebellar ataxia (MSA-C) or MSA with predominant parkinsonism (MSA-P) according to the cardinal symptom at diagnosis. MSA-C has been reported to be the predominant presentation in Japan to date.

[Utility of the Predictive Score for Cisplatin-Induced Nephrotoxicity in Patients with Chemotherapy for Lung Cancer].

Background: Cisplatin(CDDP)-induced nephrotoxicity(CIN)is a critical complication of chemotherapy. Among patients undergoing chemotherapy with CDDP, short-hydration, and magnesium supplementation for lung cancer, this study was conducted to evaluate the frequency of CIN and utility of the predictive score.

Methods: Patients who underwent chemotherapy with CDDP for lung cancer were retrospectively investigated.

Histological characterisation of visceral changes in a patient with type 2 Gaucher disease treated with enzyme replacement therapy.

Gaucher disease is a lysosomal storage disease caused by deficiency of glucocerebrosidase and accumulation of glucocerebroside. Three major sub-types have been described, type 2 is an acute neurological form that exhibits serious general symptoms and poor prognosis, compared with the other types. This case was a girl diagnosed with type 2 Gaucher disease at 12months of age who presented with poor weight gain from infancy, stridor, hypertonia, hepatosplenomegaly, trismus and an eye movement disorder.

Streptococcus panodentis sp. nov. from the oral cavities of chimpanzees.

Three strains TKU9, TKU49 and TKU50(T) , were isolated from the oral cavities of chimpanzees (Pan troglodytes). The isolates were all gram-positive, facultative anaerobic cocci that lacked catalase activity. Analysis of partial 16S rRNA gene sequences showed that the most closely related species was Streptococcus infantis (96.

[Recurrence of chronic active Epstein-Barr virus infection presenting with myelopathy after umbilical cord blood transplantation].

A 38-year-old man was admitted to our hospital with neck pain, dysesthesia of both hands, and weakness of the left upper limb. He had been diagnosed with a chronic active Epstein-Barr virus infection (CAEBV) at the age of 34 and had undergone umbilical cord blood transplantation at the age of 37. MRI of the spinal cord revealed an intramedullary hyperintense lesion on T₂-weighted images with gadolinium enhancement.

Identification of the neural correlates of cyclothymic temperament using a working memory task in fMRI.

Background: Recently, we reported a significantly negative association of cyclothymic temperament scores with activations of the left lingual gyrus during esthetic judgments of paintings, suggesting that cyclothymic temperament may be associated with the left lingual gyrus. In view of potential associations of cyclothymic temperament, bipolar disorder and dementia, this study examined the relationship of temperament to lingual gyrus activity using a working memory task as a new context.

Methods: N-back tasks (0-, 1-, 2- and 3-back tasks) were performed on 34 healthy subjects using functional MRI.

Upregulation of store-operated Ca(2+) entry in the naïve CD4(+) T cells with aberrant cytokine releasing in active rheumatoid arthritis.

The regulated control of Ca(2+) influx is essential for the activation and function of the adaptive immune response, as Ca(2+) is a key regulator of important transcription factors. To determine whether Ca(2+) release-activated Ca(2+) (CRAC) channels contribute to the abnormal behaviour of T cells in patients with rheumatoid arthritis (RA), we performed a cross-sectional study to characterize the expression and functional status of CRACM1 channels in RA patients. Peripheral blood was obtained from 50 RA patients, 50 osteoarthritis (OA) patients and healthy donors.

Intracranial arachnoid cysts in a chimpanzee (Pan troglodytes).

An intracranial arachnoid cyst was detected in a 32-year-old, 44.6-kg, female chimpanzee at the Primate Research Institute, Kyoto University. Magnetic resonance imaging (MRI) and computed tomography (CT) were performed and the cognitive studies in which she participated were reviewed.

Irreversible neurological defects in the lower extremities after haploidentical stem cell transplantation: possible association with nelarabine.

Severe peripheral neuropathy and myelopathy are rare complications after stem cell transplantation (SCT). In our institution, seven patients of precursor T lymphoblastic leukemia/lymphoma without the central nervous involvement who had been treated by nelarabine to control their diseases received SCT from HLA-haploidentical familial donor (HLA-haploidentical SCT) with the conditioning regimen including high-dose cytarabine (HDAC). Three of evaluable six patients developed irreversible paresthesia and muscle weakness in both lower extremities after neutrophil engraftment.

Behavioral recovery from tetraparesis in a captive chimpanzee.

An adult male chimpanzee living in a captive social group at the Primate Research Institute of Kyoto University developed acute tetraparesis. He was paralyzed and received intensive care and veterinary treatment as previously reported in Miyabe-Nishiwaki et al. (J Med Primatol 39:336-346, 2010).

Efficient synthesis of oxygenated terphenyls and other oligomers: sequential arylation reactions through phenol oxidation-rearomatization.

One by one: starting from simple phenols, a diverse series of oxygenated terphenyl compounds can be prepared in a concise and practical manner using sequential arylation reactions involving phenol oxidation/rearomatization and quinone monoacetal intermediates. Many of the terphenyl products can be used for preparing well-defined oligomers and, furthermore, contain valuable functional groups that can be transformed for further diversification.

Streptococcus troglodytae sp. nov., from the chimpanzee oral cavity.

Six strains, TKU 25, TKU 28, TKU 30, TKU 31(T), TKU 33 and TKU 34, were isolated from the oral cavity of a chimpanzee (Pan troglodytes). Colonies of strains grown on Mitis-Salivarius agar were similar in morphology to that of Streptococcus mutans. The novel strains were Gram-stain-positive, facultatively anaerobic cocci that lacked catalase activity.

Correlation of subthalamic nuclei T2 relaxation times with neuropsychological symptoms in patients with Parkinson's disease.

The subthalamic nucleus (STN) is a frequent target of deep brain stimulation (DBS), which is used to treat patients with advanced Parkinson's disease (PD). However, few studies have assessed the relationship between the STN and the clinical characteristics of PD patients. We identified the STN of 17 PD patients and 7 control subjects using coronal Short TI Inversion Recovery (STIR) magnetic resonance imaging (MRI) and estimated the T2 relaxation time (T2) of the STN on the subsequent coronal images that were acquired from T2-weighted MRI.

Idiopathic childhood occipital epilepsy of Gastaut: report of 12 patients.

This study sought to present clinical and outcome data of patients with idiopathic childhood occipital epilepsy of Gastaut, to validate previously reported characteristics of this epilepsy. The study group was comprised of 12 affected children (three boys and nine girls), with a median age of onset at 10.3 years.

An Asian case of fibroblastic rheumatism: clinical, radiological, and histological features.

We report a case of fibroblastic rheumatism (FR) in a 61-year-old woman. The patient showed sclerodactyly and polyarthritis that involved both her hands and feet joints. Levels of C-reactive protein and matrix metallopeptidase-3 were within normal range.

Activation of epidermal growth factor receptor gene is involved in transforming growth factor-beta-mediated fibronectin expression in a chondrocyte progenitor cell line.

Chondrocytes produce many types of ECM to maintain elasticity and plasticity in articular cartilage of revolute joints. Both transforming growth factor beta (TGF-beta) and bone morphogenetic proteins (BMPs) induce extracellular matrix proteins such as type IIalpha1 collagen and aggrecan during chondrogenic differentiation in vitro. However, differences in the matrix gene expression pattern by the stimulation of TGF-betas and BMPs remains unclear.