Objective The aim of this study was to determine the safety and clinical efficacy of docetaxel+cisplatin+5-fluorouracil (DCF) as neoadjuvant chemotherapy (NAC).

Methods In this single-center study, patient background and treatment outcomes (NAC efficacy assessment, NAC adverse events, short-term postoperative outcomes, and one-year postoperative outcomes) in patients treated with preoperative DCF and preoperative cisplatin+5-FU (CF) were compared retrospectively.

Patients Seventeen patients diagnosed with esophageal squamous cell carcinoma (ESCC) and treated with preoperative DCF therapy and 50 patients treated with preoperative CF therapy between January 2013 and July 2019 were included in this study.

Results There were significant differences in clinical T factor and clinical stage between the CF and DCF groups (p<0.05). All patients in the DCF therapy group were above clinical T3 and clinical stage III. The clinical response after NAC was partial response (PR) for 23 patients (46.0%) in the CF group and 13 patients (76.5%) in the DCF group (p=0.030). Regarding adverse events in NAC, neutropenia, febrile neutropenia (FN), diarrhea, and stomatitis were observed more frequently in the DCF group than in the CF group (p<0.05). The postoperative results [overall survival (OS), recurrence-free survival (RFS), one-year OS, one-year RFS] of the DCF group were comparable to those of the CF group.

Conclusion DCF therapy has been recognized as an effective treatment option for advanced ESCC. However, the indication for DCF therapy should be chosen carefully because of the high incidence of adverse events.

Objectives Neoadjuvant therapy followed by radical resection improves the borderline-resectable pancreatic cancer (BRPC) prognosis; however, the optimal therapeutic regimen remains unclear. Gemcitabine plus nab-paclitaxel (GnP) showed a high anti-tumor effect in primary lesions in a prospective study for metastatic disease. However, evidence concerning its feasibility is still lacking in patients with BRPC. We therefore evaluated the tolerability of neoadjuvant GnP (NAC-GnP) for BRPC.

Methods This single-center prospective study evaluated 10 patients with BRPC who were treated with two cycles of NAC-GnP. The primary endpoint was feasibility for NAC-GnP. Treatment feasibility was defined as a successful outcome in at least eight patients.

Results Ten patients who had BRPC in contact with the celiac artery (n=5), superior mesenteric artery (n=3), or hepatic artery (n=2) were enrolled. The median age was 75 (range, 40-82) years old. Grade 3 anorexia and grade 2 pneumonia occurred in one patient each, so treatment was feasible in eight patients. The median primary tumor reduction and response rates were 33% (range, 0-68%) and 60%, respectively. Six of eight patients who had abnormal CA19-9 levels at the time of enrolment showed a decrease in CA19-9 levels, with a median decrease of 72%. Five patients underwent radical resection, including R0 resection in four. Postoperative grade IIIa Clavien-Dindo complications occurred in one patient (upper gastrointestinal bleeding and pancreatic fistula).

Conclusion Two-cycle NAC-GnP is a feasible treatment for patients with BRPC. Further studies on NAC-GnP in patients with BRPC are warranted.

Objective Pancreatic cystic lesions (PCLs) are known risk factors for pancreatic cancer. Therefore, this study explored the predictors identifying PCLs in a general population and developed a scoring system to help more efficiently diagnose these entities during medical checkups.

Methods We reviewed 9,369 examinees of abdominal ultrasound (AUS) during medical checkups between January 2013 and November 2019. Predictors of PCLs were identified using a multivariate logistic regression analysis, and we constructed a scoring system based on these predictors.

Results PCLs were detected in 118 (1.3%). Age 50-59 years old [odds ratio (OR) 2.52, 95% confidence interval (CI) 1.18-5.35], 60-69 years old (OR 3.91, 95% CI 1.86-8.26), and ≥70 years old (OR 10.5, 95% CI 5.03-21.7) as well as abdominal pain (OR 1.85, 95% CI 1.14-3.00), alcohol consumption (OR 1.72, 95% CI 1.03-2.89), a family history of pancreatic cancer (OR 2.41, 95% CI 1.09-5.34), and pre-diabetes or diabetes (OR 1.78, 95% CI 1.05-3.00) were predictors of PCLs. The following scores were assigned according to regression coefficients: age (50-59 years old, 1 point; 60-69 years old, 1.5 points; ≥70 years old, 2.5 points); abdominal pain, 1 point, alcohol consumption, 1 point; a family history of pancreatic cancer, 1 point; and pre-diabetes, 1 point. The PCL detection rate increased with the total score: 0.2% for total score 0 point, 5.4% for ≥4.0 points. The area under the curve of the scoring system was 0.75 (95% CI 0.70-0.79).

Conclusion Our scoring system allows the risk of PCLs to be determined and may help more efficiently diagnose these entities.

Objective In the Japanese Community-Based Integrated Care System (CBICS), access to formal care services is based on voluntary consultations mainly from the families of people with dementia (PWD). The problem is that some people need services but do not consult their municipalities for various reasons. The purpose of this study is to examine the possibility of using health insurance claims data to identify and characterize these PWD.

Methods Using health insurance claims data, we selected PWD prescribed with anti-dementia drugs. Of them, excluding those with a usage history of long-term care insurance services or other formal services, facility residents and deaths, we identified PWD not accessing the CBICS. We conducted a visit survey on their status, home care environment and reasons for not accessing services, a proposal for using services and a one-year follow-up.

Result Based on the data of 1,809 late-stage elderly who resided in the Tamaki-cho, a town in Mie Prefecture, Japan, for a 2-month period, 16 PWD not accessing the CBICS were identified, and 15 PWD and their families participated in this study. Ten were men and 13 were physically and cognitively relatively independent. All lived with a family caregiver and refused services. Ten families needed but had not accessed the services due to refusal by PWD and other reasons. As a result, seven of these PWD started using long-term care insurance services or dementia prevention services.

Conclusion PWD not accessing the CBICS can be identified using health insurance claims data. The results proved that the municipality has a cost-effective way of providing their services to PWD and their families, even if they have never consulted voluntarily.

Objective We investigated the clinical characteristics of patients with reversible cerebral vasoconstrictor syndrome who had a history of migraine before the onset and considered the relationship between these two pathologies.

Methods We investigated 98 patients who underwent magnetic resonance angiography within 14 days of the onset of reversible cerebral vasoconstriction syndrome at our hospital. Of these, 11 cases involved recurrences, so data from 87 patients were analyzed.

Materials All consecutive patients diagnosed with reversible cerebral vasoconstrictor syndrome at our institution between October 2010 and July 2021.

Results Fifty of the 87 patients (57%) had a history of migraine. A multivariate analysis revealed that the following clinical factors were significantly more frequent in patients with a history of migraine than in those without such a history: female sex; emotional situations as a trigger of the onset; presence of deep and subcortical white matter hyperintensity, absence of vasoconstriction in the M1 portion of the middle cerebral artery, and absence of other cerebral lesions on initial magnetic resonance imaging; absence of vasoconstriction of the basilar artery on follow-up magnetic resonance imaging; and progression of deep and subcortical white matter hyperintensity in the chronic stage.

Conclusion Reversible cerebral vasoconstrictor syndrome patients with a history of migraine showed clinical features of migraine, including one aspect of cerebral small-vessel disease due to endothelial dysfunction, as a common causative condition.

Objective During the coronavirus disease 2019 (COVID-19) pandemic, many social activities have moved online using applications for digital devices (e.g. computers, smartphones). We investigated the needs of telemedicine and trends in medical status and social care situations of Japanese patients with neurological disorders in order to estimate their affinity for an online telemedicine application.

Methods We designed an original questionnaire for the present study that asked participants what problems they had with hospital visits, how the COVID-19 pandemic had affected their lives, and whether or not they would like to receive telemedicine.

Patients The present study included volunteer caregivers, participants with Parkinson's disease (PD), epilepsy, stroke, dementia, immune-mediated neurological disease (IMMD), spinocerebellar degeneration (SCD), amyotrophic lateral sclerosis (ALS), headache, myopathy, and other neurological diseases from Okayama University Hospital.

Results A total of 29.6% of patients wanted to use telemedicine. Patients with headaches (60.0%) and epilepsy (38.1%) were more likely to want to use telemedicine than patients with PD (17.8%) or stroke (19.0%). Almost 90% of patients had access to a digital device, and there was no association between favoring telemedicine, ownership of a digital device, hospital visiting time, or waiting time at the hospital, although age was associated with motivation to telemedicine use (52.6 vs. 62.2 years old, p<0.001).

Conclusion We can contribute to the management of the COVID-19 pandemic and the medical economy by promoting telemedicine, especially for young patients with headaches or epilepsy.

Objective Sarcopenia is characterized by a loss of muscle mass and strength, which leads to frailty and mortality. Rheumatoid arthritis (RA) is considered to be a cause of sarcopenia. The present study assessed the effectiveness of biological disease-modifying antirheumatic drugs (bDMARDs) on sarcopenia.

Methods This was a prospective cohort study including 48 patients [11 men, 37 women; 67.5 (57.0-74.8) years old] with RA who started bDMARDs in Niigata Rheumatic Center. We monitored the physical ability, nutritional status and body composition at the baseline, 6 months and 12 months. The physical activity was measured by the Health Assessment Questionnaire (HAQ) and 10-m walking test (10MWT). The nutritional status was assessed by the controlling nutrition status (CONUT) score.

Results Among the 48 patients who started bDMARDs, 21 were classified as having sarcopenia. The physical activity and nutritional status were significantly ameliorated after 12 months of bDMARDs. The body composition analysis showed a significant increase in the body weight but no significant increase in the skeletal muscle mass index. The proportion of patients diagnosed with sarcopenia decreased significantly after 12 months of bDMARDs (43.8% vs. 27.1%, p=0.039). Among the 21 patients who were diagnosed with sarcopenia when starting bDMARDs, the skeletal muscle index was significantly increased after 12 months of bDMARDs. [5.22 (4.76-5.43) kg/m² vs. 5.44 (4.84-5.77), p=0.039].

Conclusion Biologics may be useful in the treatment of sarcopenia through mechanisms such as improving the disease activity, physical activity and nutritional status.

Two adult cases of acute gastric mucosal lesions (AGML) caused by Helicobacter pylori infection were confirmed by spontaneous eradication during the follow-up period. The clinical course of the initial infection by H. pylori in adults with AGML remains unclear, whether it is transient or progresses to a persistent infection. In these two reported cases, gastric biopsies at the time of the onset revealed the presence of H. pylori; however, serum H. pylori antibodies performed at the same time were negative. Retesting for H. pylori serum antibody, after six months in one and after two months in the other, was negative, confirming spontaneous eradication.

A 44-year-old woman presented with severe anemia. We strongly suspected gastrointestinal bleeding; however, esophagogastroduodenoscopy, colonoscopy, and computed tomography showed no bleeding sources. Video capsule endoscopy revealed an actively bleeding submucosal lesion within the jejunum. Double-balloon enteroscopy revealed a 20-mm continuously bleeding submucosal lesion in the distal jejunum. We suspected small intestinal vascular malformation and performed surgical resection. The resected specimen pathologically comprised dilated, thin-walled lymphatic channels and blood vessels involving the small intestinal submucosa. Therefore, the patient was diagnosed with small intestinal lymphatic-venous malformation. Postoperatively, the patient recovered well, and recurrence was not observed.

Extra-gastrointestinal stromal tumors (EGISTs) are rare mesenchymal tumors that arise from the abdominal, pelvic or retroperitoneal region, unrelated to the gastrointestinal tract. However, cases with a plasmoid morphology are extremely rare. we hererin report a 49-year-old man with abdominal pain who underwent magnetic resonance imaging that revealed an irregular tumor (103×71 mm) in size, in the space between stomach and pancreas, diagnosed as an EGISIT, we also reviewed the clinicopathological characteristics and immunohistochemical characteristics, molecular genetic features and differential diagnoses previously reported in the literature.

Glycemic disorders involving large glucose fluctuations and recurrent hypoglycemia may lead to adverse cardiovascular events, including acute coronary syndrome (ACS). Flash glucose monitoring (FGM) has reportedly been useful for detecting latent glycemic disorders. However, only a few studies have so far reported latent glycemic disorders in coronary artery disease. Thus, we herein present a unique case of ACS due to intraplaque hemorrhage in a post-gastrectomy patient who had no apparent coronary risk, except for a latent severe glycemic disorder detected via FGM. This masked etiology should be considered in ACS patients who have no apparent cardiovascular risks in order to improve their cardiovascular outcomes.

Electrocardiogram (ECG) findings showing ST-segment depression in a wide range of leads and ST-segment elevation in aVR are found in patients with acute coronary syndrome with multivessel coronary lesions and left main trunk lesions. A 64-year-old man with a history of eosinophilic granulomatosis presented with chest pain and dyspnea. Although an ECG showed the above findings, he was diagnosed with acute severe aortic regurgitation (AR) complicating aortic root dissection and successfully underwent urgent Bentall operation. These ECG findings indicated that acute severe AR caused subendocardial ischemia.

A 19-year-old Japanese man was hospitalized for cardiogenic shock 28 days after receiving a second dose of the coronavirus disease 2019 (COVID-19) mRNA-1273 vaccine. He had had a high fever for three days with vomiting and abdominal pain before arriving at our hospital. The patient visited a local hospital and was diagnosed with heart failure and acute appendicitis. An endomyocardial biopsy specimen showed myocarditis. Thereafter, Impella CP left ventricular assist device implantation and venoarterial peripheral extracorporeal membranous oxygenation were initiated immediately along with inotropic support and steroid pulse therapy. Given these findings, he was finally diagnosed with multiple inflammatory syndrome and fulminant myocarditis.

We evaluated kidney histology in a 43-year-old woman with autosomal dominant tubulointerstitial kidney disease subtype hepatocyte nuclear factor 1β. Magnetic resonance imaging showed multiple cysts in the renal medullary area, and computed tomography showed hypoplasia of the pancreatic body and tail. A kidney biopsy showed thinning of the cortex, size reduction of glomerular tuft area, proximal tubule clustering, fibrosis around the tubules, loss of peritubular capillaries, and multilayered epithelial cells of cortical collecting ducts; this last finding was consistent with so-called medullary dysplasia specific to congenital disease, in which the renal pelvic epithelial cells enter the collecting duct.

A 71-year-old man was admitted for left-sided chest pain. He had a history of diabetes, treatment with epidermal growth factor receptor-tyrosine kinase inhibitor for advanced non-small-cell lung cancer, and corticosteroid treatment for underlying lung diseases. Chest computed tomography showed consolidations in the bilateral lower lobes, and Aspergillus fumigatus was detected by bronchoscopy. Invasive pulmonary aspergillosis was suspected, and antifungal therapy with voriconazole was initiated; however, the patient passed away suddenly. Autopsy revealed disseminated Aspergillus infection and intra-abdominal hemorrhage due to the rupture of a splenic vein aneurysm caused by Aspergillus necrotizing vasculitis, which was considered the cause of death.

Patients after lung transplantation are at risk for Nocardia infections. We herein report a case of lung and cerebral nocardiosis caused by Nocardia elegans, a rare species of Nocardia, in a lung transplant recipient. Antibiotic therapy, including sulfamethoxazole-trimethoprim (ST), and brain abscess drainage improved symptoms and imaging findings. A literature review of N. elegans infections showed that 12 of 14 cases (85.7%) were reported from East Asia, particularly Japan (9 cases, 64.2%). The lungs were the predominant site (12/14 cases, 85.7%), and most of the cases were susceptible to ST (9/10 cases, 90%).

An 82-year-old woman complained of recurring cough and shortness of breath and was diagnosed with progressive multiple myeloma (MM). Chest computed tomography (CT) revealed bilateral ground-glass opacity and interlobular septal thickening predominantly in the lower lung zones. Histopathologic findings obtained by a transbronchial lung cryobiopsy (TBLC) revealed alveolitis and granulomas consistent with granulomatous-lymphocytic interstitial lung disease (GLILD). Aggressive chemotherapy for MM contributed to the improvement in respiratory symptoms and abnormal chest CT findings. In cases of MM with lung abnormalities, the possibility of GLILD must be ruled out, and a TBLC should be considered to attain an accurate diagnosis.

Approximately 50% of idiopathic inflammatory myopathies (IIMs) are associated with interstitial lung disease (ILD). Typically, IIM-ILD manifests as nonspecific interstitial pneumonia. We herein report a rare case of a 78-year-old man with autoimmune pulmonary alveolar proteinosis (PAP) that developed during IIM treatment. The diagnosis of autoimmune PAP was based on detecting anti-granulocyte-macrophage colony-stimulating factor antibodies. We postulated that PAP may have been induced by IIM treatment with prednisolone. Our case suggests that the possibility of autoimmune PAP should be considered in patients with lung lesions during the clinical course of IIM.

Tocilizumab has been used to treat idiopathic multicentric Castleman disease (iMCD). As tocilizumab prevents interleukin-6 from exerting pro-inflammatory effects, there is some concern about a delayed diagnosis of severe infections during tocilizumab treatment. Although serious infections during tocilizumab therapy have been previously described in patients with rheumatoid arthritis, they have not been reported in iMCD. We herein report a case of disseminated Staphylococcus aureus infection after a superficial skin wound followed by psoas and mediastinal abscesses with pyogenic spondylodiscitis in an iMCD patient with diabetes. Physicians should be alert for the occurrence of disseminated S. aureus infection after even minor skin injury during tocilizumab therapy.

We herein report a rare case of spinal cord compression due to epidural involvement of acute myeloid leukemia (AML). A 14-year-old boy presented with a 7-day history of back pain, paraplegia and hypoesthesia. Contrast-enhanced computed tomography revealed an epidural mass. Emergency laminectomy and resection of the mass were performed. Histopathologically, the resected mass was comparable to an extramedullary mass of AML. Chemotherapy was initiated, and complete remission was achieved. Neurological sequelae remained after the treatment. Based on the present and previous reports, spinal cord compression from epidural AML involvement may progress rapidly.

Many patients with primary vitreoretinal lymphoma (PVRL) exhibit central nervous system (CNS) involvement either at the diagnosis or during follow-up. The prognosis in the patients of PVRL with relapsed or refractory CNS remains extremely poor. We herein report a patient with refractory PVRL who had recurrence in the spinal cord despite receiving high-dose methotrexate-based chemotherapy and whole-brain radiotherapy. The patient surprisingly responded to tirabrutinib temporarily. We believe that this case suggests the utility of this new target therapy.

Recent studies have reported that autoantibodies against glial fibrillary acidic protein (GFAP), a major cytoskeletal protein expressed in astrocytes, can lead to GFAP astrocytopathy, an autoimmune central nervous system inflammatory disease. We herein report the unique case of a 59-year-old Japanese woman with GFAP astrocytopathy who presented with characteristic symptoms, including signs of meningeal irritation, cerebellar ataxia, and bladder/rectal dysfunction, in the absence of specific findings on initial brain magnetic resonance imaging (MRI). The patient exhibited new abnormal changes mainly in the brainstem on follow-up MRI, illustrating the need to recognize that MRI abnormalities may appear later in GFAP astrocytopathy.

The coexistence of multiple autoantibodies associated with autoimmune encephalitis (AE) is rare. A 63-year-old woman developed psychosis and consciousness disorder. Her cerebrospinal fluid was positive for anti-N-methyl-D-aspartate receptor antibodies, and her serum was positive for anti-Hu antibodies. Enhanced computed tomography revealed a mass in the right pulmonary hilum. AE complicated with small-cell lung cancer was diagnosed. Immunotherapy (steroid therapy and intravenous immunoglobulin) and four courses of carboplatin-etoposide chemotherapy were required to improve her neurological symptoms. When the coexistence of multiple antibodies is detected, despite its rarity, aggressive detection and treatment of any underlying malignancy may be recommended.

A 36-year-old man developed polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome after conversion from solitary plasmacytoma of bone to multiple myeloma. Twenty-four days following the neurological onset, he lost his independent walking ability. The level of serum vascular endothelial growth factor (VEGF) at diagnosis was 5,250 pg/mL. Three months after initiating treatment, he regained his independent walking ability in line with a reduction in the elevated serum VEGF level. Due to their genomic instability gained during conversion, myeloma cells may overproduce humoral factors and cytokines, possibly contributing to the development of neuropathy as well as the production of VEGF.

We herein report a 49-year-old Japanese man with relapsing polychondritis (RP) and aseptic meningoencephalitis. Four years ago, the patient was diagnosed with RP. Prednisolone (PSL) was started at 30 mg/day, and the symptoms promptly disappeared. However, cognitive impairment gradually appeared from six months before hospitalization. Methylprednisolone pulse therapy was immediately initiated, followed by administration of PSL at 1 mg/kg/day. Intravenous cyclophosphamide was combined with PSL. After treatment, the patient's cognitive impairment clearly improved. In conclusion, RP rarely causes aseptic meningoencephalitis, highlighting the need for prompt and aggressive immunosuppressive therapy.