Objective Metabolic syndrome and dental erosion have been demonstrated to correlate with gastroesophageal acid reflux disease (GERD), while periodontitis has been reported to have a positive relationship with metabolic syndrome. However, no correlation between periodontitis and GERD has yet been reported. We therefore investigated the relationship between periodontitis and GERD.

Methods The subjects consisted of 280 individuals who visited the Health Center for a detailed medical checkup examination. Each underwent upper endoscopy and periodontitis examinations, with the latter performed by measuring the concentrations of lactate dehydrogenase and hemoglobin in saliva. The subjects were divided into those with positive and negative periodontitis findings, and the prevalence rates of endoscopically proven reflux esophagitis, dyslipidemia, hypertension, and hyperglycemia were compared.

Results The number of subjects positive for periodontitis was 93, while 187 had negative findings. The prevalence of reflux esophagitis was not different between the positive and negative groups (8.6% vs. 8.0%). In addition, a multiple logistic regression analysis did not identify a positive relationship between the presence of periodontitis and reflux esophagitis. On the other hand, dyslipidemia and hypertension were more frequently observed in the subjects that were positive for periodontitis.

Conclusion We did not find an association between periodontitis and reflux esophagitis in the present study. On the other hand, the presence of periodontitis was found to correlate with hypertension and dyslipidemia.

Objective To retrospectively evaluate the risk factors for post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis (PEP) and stent dysfunction after performing preoperative biliary drainage (BD) in patients with malignant biliary stricture.

Methods Between January 2003 and February 2013, 105 consecutive patients who had undergone transpapillary BD before surgery were enrolled in this study. Procedure-related complications, stent dysfunction rates, and their respective risk factors were investigated. PEP was defined according to the consensus guidelines.

Results Fifty-five patients had bile duct cancer, 31 had pancreatic cancer, 16 had ampullary cancer, and 3 had gallbladder cancer. Endoscopic biliary stenting (EBS) and nasobiliary drainage (NBD) were performed in 84 patients and 21 patients, respectively. PEP occurred in 10% of the patients, with a significantly higher frequency in those with hilar/upper bile duct stricture (p=0.026) and a normal bilirubin level at admission (p=0.016). Of the 84 patients who underwent initial EBS, stent dysfunction occurred in 13%. The mean number of days from EBS to stent dysfunction was 14±12 days. A multivariate analysis revealed a male gender (p=0.048), a stent diameter ≤8 Fr (p=0.036), and an ERCP procedure time ≥45 minutes (p=0.021) to be risk factors for stent dysfunction. No NBD tube dysfunction was observed.

Conclusion Patients with upper/hilar bile duct stricture or a normal bilirubin level are at high risk of developing PEP after preoperative BD. NBD or EBS with a large-bore stent is therefore recommended as preoperative BD.

Objective Little is known about the outcome of repeat catheter ablation of long-standing persistent atrial fibrillation (AF) in patients with a total AF duration of more than 2 years. The main objective of this study was to explore the results and factors affecting the clinical success rate of these repeat procedures.

Methods We enrolled 99 patients with a total AF duration of more than 2 years and recurrent atrial arrhythmias after the initial catheter ablation of long-standing persistent AF. The enrolled patients were divided into two groups named the AF-recurrence group (50 patients) and the atrial tachycardia (AT)-recurrence group (49 patients) and all underwent a strict follow-up. The quality of life (QOL) and AF-related symptom classification were assessed at baseline and at 24 months post re-ablation.

Results After a mean follow-up of 31 months, 30 (30.3%) patients were free from arrhythmia recurrence, and the success rate in the AT-recurrence group was higher than that in the AF-recurrence group (32.7% vs. 28.0%, p=0.614). A Cox regression analysis revealed a CHA₂DS₂-VASc score ≥3 to be a predictor of recurrence. AF recurrent patients with an abnormal renal function were more prone to undergo a failed procedure. However, an abnormal renal function had no effect on the outcome of the repeat procedure for patients with AT recurrence. At the 24-month follow-up, patients maintaining sinus rhythm (SR) had a significantly improved QOL and AF-related symptoms.

Conclusion The success rate of repeat procedures for long-standing persistent AF and a total AF duration of more than 2 years is poor for patients with a CHA₂DS₂-VASc score ≥3. An impaired renal function has an unfavorable effect on the outcome for patients with AF recurrence. For patients maintaining SR, both the QOL and AF symptomatology improve significantly.

Objective The close relationship between fatty liver and metabolic syndrome suggests that individuals with fatty liver may have multiple coronary risk factors. In the present study, we investigated the relationships among fatty liver, abdominal fat distribution, and coronary risk markers.

Methods and Results Eighty-seven pairs of men and 42 pairs of women who were matched for age and body mass index were enrolled in the present study. The obesity-related markers, abdominal fat distribution (examined by CT), and coronary risk markers were compared in subjects with and without fatty liver. The visceral fat area was significantly larger in the men with fatty liver than in the men without fatty liver. The plasma levels of triglyceride and low-density lipoprotein cholesterol (LDL-C), as well as the homeostasis model assessment-insulin resistance level, were higher in both males and females with fatty liver than in those without fatty liver, while the plasma levels of high-density lipoprotein cholesterol (HDL-C) and adiponectin were lower in the males and females with fatty liver. The plasma levels of apolipoprotein B, remnant-like particle cholesterol (RLP-C), and oxidized LDL were higher in men with fatty liver, but not in women with fatty liver.

Conclusion Both males and females with fatty liver had lower insulin sensitivity, lower plasma levels of HDL-C and adiponectin, and higher triglyceride and LDL-C levels. However, the plasma levels of apolipoprotein B, RLP-C, and oxidized LDL were only higher and closely associated with fatty liver in men. Men with fatty liver had a higher risk of coronary disease than women with fatty liver.

Objective The purpose of this study was to measure serum 25-hydroxyvitamin D [25(OH)D] levels in Japanese patients with diabetic nephropathy and determine the relationship between 25(OH)D concentrations and various factors.

Methods The study subjects included 442 patients with type 2 diabetes. Their serum levels of creatinine, HbA1c, intact-parathyroid hormone, urinary albumin, 25(OH)D, and 1,25-dihydroxyvitamin D [1,25(OH)₂D] were measured and their estimated glomerular filtration rate (eGFR) was determined. The patients were divided into four groups based on the risk for progression to chronic kidney disease (CKD): low, moderate, high, very high, based on their eGFR and their level of albuminuria.

Results The median 25(OH)D level was 14.6 ng/mL; 11% of the patients had 25(OH)D deficiency (<10 ng/mL), and 2% of patients had active vitamin D deficiency, as defined by a 1,25(OH)₂D level of <22 pg/mL. The serum 25(OH)D level was correlated with the serum 1,25(OH)₂D level in patients with a very high risk for CKD, but not in those with a moderate or high risk for CKD.

Conclusion Although the vitamin D levels of the Japanese patients with diabetic nephropathy and CKD were low, the prevalence of vitamin D deficiency, as defined by the 1,25(OH)₂D level, was low. Albuminuria, younger age, and female gender were associated with a low 25(OH)D level. The serum level of 25(OH)D should be monitored to assess the vitamin D status of patients with nephropathy and CKD.

Objective We conducted the present study to evaluate the prevalence of chronic kidney disease (CKD) and CKD complications among evacuees and non-evacuees after the Great East Japan Earthquake and Fukushima Daiichi nuclear disaster.

Methods Twenty-seven thousand and eighty-eight subjects who were living near the Fukushima Daiichi Nuclear Power Plant in Fukushima Prefecture in Japan, aged ≥40 years by the Heath Care Insures, were included in the analyses. Metabolic factors were compared between the evacuees and non-evacuees stratified by the eGFR and proteinuria grades.

Results The prevalence of CKD with a low eGFR (<60 mL/min/1.73 m²) and proteinuria were 21.59% and 1.85%, respectively. The risk of CKD complications was classified into four grades according to the eGFR and proteinuria grades. The prevalence of diabetes, hypertension, and dyslipidemia were significantly higher in the very high risk group than in the low risk group. The prevalence of diabetes and dyslipidemia were significantly higher in evacuees than in non-evacuees in only the low risk group. However, a multivariate logistic regression analysis showed that evacuation was not significantly associated with the risk of a low eGFR or proteinuria.

Conclusion This study did not reach the definitive conclusion that evacuation elevated the risk of CKD complication, although evacuation might lead to increased CKD complications in the future. We believe that this information is important for follow-up and lifestyle change recommendations for evacuees.

Objective An association has been reported between inflammatory myopathies (IMs), which include polymyositis (PM) and dermatomyositis (DM), and malignancy, and the concept of cancer-associated myositis (CAM) was recently proposed. We herein attempted to determine the features and etiologies of these myopathies.

Methods We analyzed the gene expression levels via microarray and real-time quantitative reverse transcription polymerase chain reaction analyses to identify genes that were specifically upregulated or downregulated with suspected inflammatory involvement and verified the microarray data via an immunohistochemical (IHC) analysis in additional cases.

Patients We selected 14 patients with the following conditions: PM without malignancy (n=3), DM without malignancy (n=3), CAM (n=3), and Controls (no pathological changes or malignancy; n=5).

Results PM was distinct from DM and CAM in a clustering analysis and exhibited the highest numbers of overexpressed genes and specific pathologies in a gene ontology analysis. The IHC analysis confirmed the gene expression results.

Conclusion PM is associated with severe inflammatory pathological findings, primarily in the cell-mediated immune system. DM and CAM exhibit similarities in the gene expression and IHC results, which suggest that humoral immunity is the main etiology for both myopathies, indicating the importance of cancer screening in patients with IMs, particularly DM.

Objective We examined whether infliximab (IFX) therapy was more effective than methotrexate (MTX) monotherapy to achieve an improvement in depressive states in Rheumatoid Arthritis (RA) patients.

Methods We examined 152 RA patients (72 IFX patients and 80 MTX patients). We conducted an open-label cohort study to evaluate the disease activity of RA (Simplified Disease Activity Index; SDAI), depressive states (Hamilton Rating Scale for Depression; HAM-D), Activity of Daily Living (ADL) (modified Health Assessment Questionnaire; mHAQ) and Quality of Life (QOL) [Short Form (SF)-36] in patients before and 6 months after receiving therapy. The HAM-D, SDAI, mHAQ and SF-36 scores after 6 months of therapy were measured as the outcomes.

Results We analyzed 60 IFX patients and 53 MTX patients. The HAM-D scores significantly improved in both groups (p<0.001), but there was no significant difference in the effectiveness between the IFX and MTX therapies (p=0.792). The SDAI scores significantly improved in both groups after therapy (p<0.001), and IFX therapy was more effective than MTX therapy (p=0.004). The mHAQ and HAM-D scores also improved significantly in both groups after therapy (p<0.001), but no significant difference in the effectiveness between the IFX and MTX therapies was observed (p=0.272, 0.792). The scores of all 8 items of the SF-36 improved in both groups after therapy, but IFX therapy was more effective than MTX therapy in only 4 of the 8 items (p<0.05).

Conclusion Both IFX and MTX therapy improved the clinical efficacy, ADL, QOL and depressive states. However, no significant differences regarding an improvement in the depressive states and ADL were observed between IFX therapy and MTX monotherapy.

Objective Antibody testing for endemic viruses in healthcare workers is used as an index of immunoprotection in Japan. However, it remains unclear how these antibody titers chronologically change and how they should be interpreted.

Methods We retrospectively collected two sets of antibody titers to measles and rubella, measured in 2013 and within the preceding 5 years, in adult hospital workers by an enzyme-linked immunoassay and calculated in international units. Subjects infected with, or vaccinated against, these viruses over this period were eliminated. Seropositivities and geometric mean titers were analyzed. Decay rates and half-lives of antibodies were calculated using a mixed-effect model according to the subjects' ages and antibody titers.

Results We analyzed 469 subjects for measles and 439 for rubella. Comparison with previous data revealed a mean measurement interval of 1,026 days between the previous and present tests, with seropositivity rates of 98.0% (previous) vs. 99.3% (present) for measles; 974 days and 90.7% vs. 94.9%, respectively, for rubella. For measles and rubella, 97.4% and 86.1%, respectively, of previously seropositive subjects remained positive in the present test. The geometric mean titers in the present and previous tests were 924.3 IU/mL and 853.2 IU/mL (measles) and 46.23 IU/mL and 40.78 IU/mL (rubella), respectively. In the mixed-effect model, measles and rubella antibody titers showed an increasing trend with age.

Conclusion Seropositivities against measles and rubella can remain high for more than 5 years. Among adult hospital workers in Japan, the antibody titers against measles and rubella have a sufficient lifetime persistence.

Endometriosis is a quite common pathology, however, intestinal endometriosis is a rare condition, which typically occurs with chronic symptoms. Its acute presentation is very infrequent. We herein report four cases of intestinal endometriosis, in which the clinical debut occurred acutely: two as an acute small bowel obstruction and two as a small bowel perforation. None of the cases had a preoperative diagnosis of endometriosis. The interest of these cases lies in this exceptional form of presentation, such as a surgical acute abdomen. Therefore, intestinal endometriosis should be taken into account in the differential diagnosis of an acute obstructive or perforative process of the small or large bowel.

A 72-year-old man with the history of small bowel carcinoma was diagnosed to have a sessile polyp in the jejunum during a follow-up examination. We performed double-balloon endoscopy and removed the polyp by means of cold snare polypectomy. The follow-up endoscopy showed no residual lesion. Cold snare polypectomy has been established as a safe and effective method for the endoscopic treatment of colonic polyps with a low risk of causing electrocautery burns and perforation. However, this technique has not yet been applied for small bowel lesions. This case report, for the first time, shows the feasibility of performing cold snare polypectomy for small bowel polyps.

Paraneoplastic neurological syndromes (PNSs) are rare nervous system dysfunctions in cancer patients, which are primarily observed with small-cell lung cancer, gynecological cancer, and thymoma. We herein present an uncommon case of PNS in an anti-Hu antibody-positive patient with human epidermal growth factor receptor (HER)-2-positive gastric cancer (GC), who developed limbic encephalitis and a worsening cognitive function. Trastuzumab-combined chemotherapy was initiated and appeared to be partially effective for controlling the neurological symptoms and tumor volume. Chemotherapy failure eventually led to uncontrollable neurological symptoms. This is the first case demonstrating that trastuzumab-combined chemotherapy may be effective for controlling neurological symptoms of PNS in HER2-positive GC patients.

To investigate the mechanism of phytobezoar dissolution by Coca-Cola^®, persimmon phytobezoar pieces removed from a 60-year-old Japanese woman were analyzed by energy dispersive X-ray spectroscopy. The amount of calcium significantly decreased after dissolution treatment using Coca-Cola^®, suggesting a potential contribution of calcium to dissolution mechanisms. Moreover, immersion in Coca-Cola^® for 120 hours on the exterior surface revealed that Coca-Cola^® did not permeate persimmon phytobezoars. This is the first study to investigate the mechanisms of persimmon phytobezoar permeability and dissolution induced by Coca-Cola^®.

An 87-year-old woman on oral prednisolone was diagnosed with a cholecystoduodenal fistula (CDF) caused by a cytomegalovirus-associated duodenal ulcer (DU) and was managed conservatively. A CDF caused by a DU is extremely rare. Although surgical repair is recommended for the treatment of a CDF caused by cholecystolithiasis, appropriate treatment for CDF caused by a DU remains controversial. This case report of a CDF caused by a DU suggests that conservative treatment is feasible in the absence of DU-associated complications, such as an untreatable hemorrhage or obstruction; this finding is compatible with previously reported cases that were conservatively treated.

A 63-year-old woman presented to our hospital with elevated levels of serum IgG4, marked wall thickening of the gallbladder, hepatomegaly, and abdominal lymphadenopathy. She experienced a recurrent fever and leg edema. Her laboratory data demonstrated anemia, hypoalbuminemia, and elevated serum levels of interleukin-6 and C-reactive protein. The patient was eventually diagnosed with IgG4-related disease according to the comprehensive diagnostic criteria, although the patient exhibited common clinical manifestations of multicentric Castleman disease such as a fever, anemia, lymphadenopathy, and elevated levels of serum interleukin-6 and C-reactive protein. This case report highlights the difficulties in differentiating between these two diseases.

A 58-year-old man, who had presented with a large cyst between the pancreatic tail and splenic hilum 6 years previously, was referred to our hospital with exacerbation of abdominal distention. Computed tomography revealed a well-demarcated, unilocular cyst, with a beak sign for the pancreas, without wall thickening or nodules suggestive of a non-neoplastic cyst. Compared with 6 years previously, the cyst had increased in size from 14.7 cm to 19.5 cm, and the serum carcinogenic antigen 19-9 level had increased from 635 U/mL to 1,918 U/mL. To prevent spontaneous rupture, laparotomy was performed, and the cyst was pathologically diagnosed as a splenic epithelial cyst.

Coronary artery fistulas are rare and the feeding artery is ectatic and tortuous. It is not well-known whether coronary artery ectasia (CAE) is a risk factor of acute coronary syndrome (ACS) in the puerperal periods. A 40-year-old woman with a coronary artery fistula and an ectatic right coronary artery (RCA) had delivered twins. A month later, she had chest pain and coronary angiography revealed thrombogenesis in the RCA. She had no risk factors of cardiovascular disease or thrombogenesis. We should recognize that CAE is a risk factor for ACS in women in the perinatal and puerperal periods.

A 44-year-old woman, who had been previously diagnosed with coronary spastic angina and treated with standard medical therapy including calcium channel blockers, was admitted to our hospital due to chest pain at rest. Her chest pain attacks were concentrated just before and during menstruation. Despite the administration of an intravenous infusion of nitroglycerin and nicorandil, strong heart attacks with ST elevation occurred frequently after this admission. However, following continuous combined estrogen-progestin hormonal contraception use (estradiol plus dienogest), her attacks disappeared completely. Reduced estrogen levels before and during menstruation were speculated to be associated with her angina attacks.

We herein report an adult case of unicommissural unicuspid aortic valve (UAV). A 59-year-old man, who was noted to have a cardiac murmur at 31 years of age, was admitted to our hospital due to acute heart failure. Severe calcification in the aortic valve with severe low-flow/low-gradient aortic stenosis and moderate aortic regurgitation was observed and thought to be the cause of heart failure, however, the etiology of aortic valve dysfunction was not clear. Aortic valve replacement was subsequently performed, and unicommissural UAV was diagnosed according to the intraoperative findings. UAV is very rare congenital aortic valve disease which is rarely diagnosed preoperatively.

A 73-year-old Japanese woman with untreated Graves' hyperthyroidism developed glucocorticoid-induced adrenal insufficiency (AI) after a supraphysiological dose of prednisolone therapy for bronchial asthma. Days later, she had high plasma adrenocorticotropic hormone (ACTH) levels and was expected to recover from glucocorticoid-induced AI. Her plasma ACTH levels remained high over 3 months during a physiological dose of hydrocortisone replacement. However, she suffered a further decrease in her serum cortisol level and was diagnosed with isolated adrenocorticotropin deficiency (IAD), in which bioinactive ACTH likely caused the high ACTH value. IAD should be considered as an unusual disorder associated with Graves' disease, especially in older patients.

Carnitine palmitoyltransferase II (CPT II) deficiency is a rare inherited disorder related to recurrent episodes of rhabdomyolysis. The adult myopathic form of CPT II deficiency is relatively benign and difficult to diagnose. The point mutation S113L in CPT2 is very common in Caucasian patients, whereas F383Y is the most common mutation among Japanese patients. We herein present a case of CPT II deficiency in a Japanese patient homozygous for the missense mutation S113L. The patient showed a decreased frequency of rhabdomyolysis recurrence after the administration of a diet containing medium-chain triglyceride oil and supplementation with carnitine and bezafibrate.

A 30-year-old woman with proteinuria first noted at 26 weeks of gestation was admitted to undergo further evaluation. A renal biopsy revealed membranous nephropathy (MN). There was no evidence of any secondary MN. Prednisolone was initiated 6 months after delivery. Four months later, her urine protein became negative. Enhanced granular staining for thrombospondin type-1 domain-containing 7A (THSD7A) in the glomeruli was retrospectively detected in a biopsy specimen. A literature review revealed that 60% of cases of THSD7A-related MN occurred in women of childbearing age. Therefore, THSD7A-related MN should be considered in female patients presenting with idiopathic MN in childbearing age.

A 46-year-old woman suddenly developed peripheral edema. Her massive proteinuria, hypoproteinemia, and renal biopsy findings yielded the diagnosis of minimal change disease (MCD). In addition, lung Mycobacterium avium infection was diagnosed according to a positive culture of her bronchoalveolar lavage fluid. The lung lesion was improved by anti-nontuberculous mycobacteria therapy. Surprisingly, her proteinuria also gradually decreased and she attained complete remission of MCD without any immunosuppressive therapy. She has subsequently remained in complete remission. We herein report an interesting case of MCD with lung Mycobacterium avium infection, suggesting a causal relationship among infection, immune system abnormality, and MCD/nephrotic syndrome.

We herein report a case of primary marginal zone lymphoma (MZL) of the posterior mediastinum in an 84-year-old woman. Computed tomography of the chest showed a posterior mediastinal mass in the right thoracic paravertebral region with right pleural effusion. Pathological findings of a surgical biopsy from the posterior mediastinum, along with immunohistochemical and flow cytometric results, indicated MZL. The patient was treated with chemotherapy and radiation therapy for the mediastinal lesion and achieved complete remission. A relapse occurred 3 months after the initial treatment regimen. However, a second relapse has not occurred more than 2 years after second-line chemotherapy. This is the first case of MZL originating in the posterior mediastinum.

Pulmonary tumor embolism (PTE) and intravascular lymphoma cause rapidly progressive deterioration and an antemortem diagnosis is difficult. The usefulness of pulmonary microvascular cytology (PMC) in the diagnosis of these disorders has been reported in sporadic case reports. We retrospectively evaluated the records of 7 patients with tumor cells in the pulmonary microvasculature (4 with PTE and 3 with malignant lymphoma) who underwent pulmonary microvascular cytology. Two of the 4 patients with PTE and 2 of the 3 patients with malignant lymphoma (all 3 had intravascular metastasis) had positive PMC results. These findings suggested that PMC may be useful in the diagnosis of these disorders.

A 50-year-old man presented to our hospital in 1995. Invasive thymoma was diagnosed and extended thymectomy and left upper lobe partial resection were performed. In 2013, he complained of dyspnea. Chest computed tomography showed postoperative recurrence of invasive thymoma. Several chemotherapies were administered. Severe anemia and an increase in the total bilirubin level were observed with chemotherapies. In additional, an examination showed that the direct Coombs test was positive. Cold agglutinin was also high. We herein experienced a rare case of postoperative recurrence of invasive thymoma with cold agglutinin disease and autoimmune hemolytic anemia.

We herein report a case of atypical drug-induced hypersensitivity syndrome (DIHS) involving serological reactivation of cytomegalovirus induced by carbamazepine with pulmonary and skin manifestations. These lesions were not present on admission, but developed on virus reactivation as indicated by the presence of inclusion bodies and multinucleated giant cells in alveolar cells with CD8⁺ T lymphocyte infiltration on a transbronchial lung biopsy. Although the precise mechanism of DIHS remains unknown, this case suggests the crucial role of viral reactivation in pulmonary lesions in DIHS.

Hereditary hemochromatosis (HH) is an inherited disorder usually seen in Northern Europeans, which results in iron overload syndrome. A few cases have also been reported in Japan. We herein report a Japanese man presenting with fever, arthritis, liver dysfunction, and hyperferritinemia who was diagnosed with type 4 HH. He was heterozygous for the 1520A>G (His507Arg) mutation in the ferroportin-1 gene (SLC40A1). He had a familial cataract as an infant, but hereditary hyperferritinemia cataract syndrome was excluded. This is the first report of type 4 HH with juvenile cataracts and suggests that there is an association between hyperferritinemia and early cataract formation.

Dasatinib has been associated with an increased risk of bleeding, with the most prominent risk noted in patients with advanced-stage chronic myeloid leukemia and thrombocytopenia. We herein report two cases of Philadelphia chromosome-positive acute lymphoblastic leukemia in which a subdural hematoma developed in association with low-dose (40-50 mg/day) dasatinib treatment and lumbar puncture for intrathecal methotrexate injection. Both patients were in complete remission, with normal platelet counts and coagulation status. We suggest that dasatinib, even at a low dose, may impair platelet aggregation and that lumbar puncture may increase the risk of a subdural hematoma (occasionally bilateral) in patients receiving dasatinib.

A 70-year-old woman with Charcot-Marie-Tooth disease (CMT) suffered from nephrotic syndrome and a renal biopsy revealed non-AA amyloid depositions that contained immunoglobulin light chain λ. Her serum λ free LC was elevated to 80.8 mg/L and she was diagnosed with primary amyloid light-chain (AL) amyloidosis. She was subsequently treated with lenalidomide, cyclophosphamide, and dexamethasone (RCD). After 14 cycles of RCD, she achieved complete remission. Her serum albumin levels gradually normalized to 3.1 g/dL. No exacerbation of neurologic symptoms related to CMT was observed. Thus, RCD may be a well-tolerated and effective regimen for treating AL amyloidosis in patients with CMT disease.

We herein report an elderly patient with a history of restless legs syndrome (RLS) who developed unpleasant sensations in the lower abdomen and perineum. The patient' s symptoms occurred during the evening and at rest and forced the patient to void, resulting in some symptom relief. Urological examinations and spinal magnetic resonance imaging findings were unremarkable. The adjunctive use of rotigotine resolved her symptoms. The symptoms were considered to be "restless bladder". Further studies are required to elucidate whether restless bladder is an identical condition to the clinical entities of other RLS variants or whether it represents an unusual feature of RLS.

A patient with xerostomia and xerophthalmia due to Sjögren's syndrome presented with acute motor-dominant polyneuropathy and multiple mononeuropathy with antiganglioside antibodies. Nerve conduction studies and a sural nerve biopsy revealed the neuropathy as a mixture of segmental demyelination and axonal degeneration. Positive results were obtained for several antiganglioside antibodies. Corticosteroid treatment proved effective. The neuropathy was considered to represent a mixture of polyneuropathy as Guillain-Barré syndrome and multiple mononeuropathy via Sjögren's syndrome. We speculate that Guillain-Barré syndrome occurred in the patient and Guillain-Barré syndrome itself activated multiple mononeuropathy via Sjögren's syndrome.

A 72-year-old, seemingly healthy, Japanese man suddenly lost consciousness. At the emergency room, the patient's Glasgow coma scale score was 10 and a thoracic breathing pattern was observed. An arterial blood gas analysis indicated acute hypercarbic respiratory failure. He was placed on non-invasive positive pressure ventilation. The next day he was alert. Manual muscle testing revealed that his face, neck and limb muscle strength were normal. He could walk, and Gowers' sign was not observed. Computed tomography showed atrophy of the paravertebral, abdominal wall and diaphragm crura muscles, without apparent limb muscle involvement. Pompe's disease was diagnosed based on the results of biochemical and genetic tests for acid alpha-glucosidase.

A 27-year-old HIV-infected pregnant Japanese woman was admitted to our hospital at gestational week 14. The patient's HIV viral load was 71,000 copies/mL, and her CD4 cell count was 147 cells/mm³. Zidovudine, lamivudine, and lopinavir/ritonavir were administered at gestational week 18. Because the viral load increased to 222,000 copies/mL at the initiation of antiretroviral therapy, we added raltegravir. The decrease in the viral load was satisfactory, and a caesarean delivery was performed. Although the plasma concentration of raltegravir in the neonate was significantly high (2,482 ng/mL), no adverse event was confirmed. There was no evidence of the mother-to-child transmission of HIV.

Gram-negative fusiform rods were detected in a blood culture obtained from a 63-year-old man who had been hospitalized for a long duration for severe heart failure. Although the organism could not be identified using a conventional method, it was finally identified as a bacterium of the Capnocytophaga ochracea group based on the results of biochemical testing, 16S rRNA sequencing and a matrix-assisted laser desorption ionization time-of-flight mass spectrometry analysis. Although neutropenic patients with poor oral hygiene are exclusively vulnerable to Capnocytophaga bacteremia, this case was unique because such predisposing conditions were not noted. A multi-centered investigation is warranted for a better understanding of this clinically rare, but potentially pathogenic organism.