Background Systemic thermal therapy (STT) has been associated with beneficial effects in patients with chronic heart failure (CHF). The fact, however, that it requires a dedicated as well as spacious facility and trained personnel makes it difficult to practice in the daily care of patients with CHF.
Objective The aim of this study was to determine whether the leg thermal therapy (LTT) has a positive impact similar to that of STT in patients with CHF.
Methods and Results Twenty patients with CHF (57±17 years old, left ventricular ejection fraction=30±10%) received LTT (45°C) for 20 minutes. Immediately after the treatment, the core temperature had increased (+0.3±0.3°C) (p<0.01). While the LTT had no significant effects on the heart rate, systolic arterial pressure, and diastolic blood pressure, it increased the cardiac output (mixed venous oxygen saturation; +2±3%) and decrease the pulmonary capillary wedge pressure (-2±2 mmHg). The LTT significantly improved the flow-mediated vasodilatation (FMD) from 4.8±2.6 to 7.1±3.6%, the antioxidative markers, thiol from 4.0±0.7 to 4.5±0.9 μmoL/g, and the marker of oxidative deoxyribonucleic acid (DNA) damage, urine 8-hydroxy-2'deoxyguanosine (8OHdG) from 100 to 82±3%, respectively (p<0.05). No patient had any adverse effects associated with LTT.
Conclusion LTT acutely improved FMD, and oxidative stress in patients with CHF. Although the long-term effect of LTT remains to be investigated, its practicality which is comparable to that of STT would make it an attractive therapeutic strategy for patients with CHF.

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Objective Red blood cell distribution width (RDW) is a numerical measure of erythrocyte size variation. It has been recently reported to be an independent prognostic marker of heart failure (HF). Previous studies on RDW were mostly designed for middle-aged and elderly patients (60-79 years old), therefore, there is no established limit for super-elderly patients (≥80 years old). The purpose of this study was to evaluate RDW as an effective tool to detect fatal HF in super-elderly patients.
Methods The medical records and death certificates of 160 consecutive patients admitted to the Department of Cardiology in Juntendo Tokyo Koto Geriatric Medical Center and who died from June 2002 to October 2010 were reviewed. The causes of death were reviewed, and the factors, including RDW, that might have been related to the fatal HF were evaluated using multivariate logistic regression analysis.
Results HF was the major cause of death [52 patients (32.5%), 29 females, age 84.0±7.5 years], followed by pneumonia (18.8%, 30/160), and acute myocardial infarction (16.3%, 26/160). The most common cause of HF was atrial fibrillation (36.6%, 19/52), followed by hypertensive heart disease (19.2%, 10/52) and valvular disorders (17.3%, 9/52). The multivariate logistic regression analysis found that a high RDW (≥16.5%) was an independent factor related to fatal HF (OR 2.36, 95% CI 1.10, 5.04, p=0.03).
Conclusion HF was the major cause of death, and RDW ≥16.5 was significantly associated with fatal HF in super-elderly patients.

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Objective We sought to determine the value of electrocardiographic right ventricular hypertrophy (ECG-RVH) in pulmonary hypertension (PH) patients with right ventricular systolic dysfunction defined by cardiac magnetic resonance (CMR-RVSD).
Patients A total of 31 consecutive patients with PH with a mean pulmonary arterial pressure of >25 mmHg underwent both ECG and CMR studies. Patients were divided into 2 groups according to the presence of RVSD, defined as a RV ejection fraction <35%. Logistic regression modeling was performed to define the association between ECG-RVH and CMR-RVSD.
Results About half of the patients had RVSD (n=15 ; 48%). The R to S wave ratio (p=0.01) or incidence of qR pattern (p=0.002) in lead V₁ was significantly greater in patients with PH complicated by RVSD than in those without RVSD. These 2 patterns were significant predictors of RVSD [odds ratio (OR), 19.3 for qR; OR, 14.0 for R/S>1] and when each of these ECG findings was assigned with a point proportional to OR (score of 2 for qR in lead V₁ and score of 1 for R/S>1 in lead V₁), the incidence of RVSD increased by the total ECG score.
Conclusion The combination of ECG-RVH findings, especially in lead V₁, predicts the presence of RVSD defined by CMR. ECG might be a useful tool for estimating the presence of RVSD in patients with PH.

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Objective β-adrenergic antagonists (β-blockers) are often used to attenuate the hyperadrenergic symptoms of Graves' disease (GD), including palpitation. Although β-blockers reduce the heart rate, cardiac output and oxygen consumption, no firm evidence exists regarding the effects of combined therapy with β-blockers and anti-thyroid drugs. The objective is to elucidate the effects of β-blockers on anti-thyroid drug therapy in GD.
Methods Patients newly diagnosed with mild GD were randomly assigned to receive methimazole with or without β-blockers in a prospective multi-center survey. The heart rate and thyroid function were measured and the quality of life was assessed using original and SF-36 questionnaires at 0 and 4 weeks.
Results A total of 28 patients were enrolled in the study. Fourteen patients (one man, 13 women) were randomly assigned to the group treated with β-blockers and 14 patients (one man, 13 women) were randomly assigned to the group not treated with β-blockers. Although no significant differences in the improvement of thyroid function were observed between the two groups, the heart rates improved more significantly in the group treated with β-blockers. Specific symptoms, such as easy fatigability and shortness of breath, also improved more significantly with the β-blocker treatment. In addition, 'physical functioning' assessed with the SF-36 questionnaires significantly improved only in the group treated with β-blockers.
Conclusion Although β-blockers may not reinforce the effects of anti-thyroid drugs on thyroid function, at least during the course of one month, they are effective in reducing heart rates and ameliorating specific symptoms in patients with mild GD.

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Objective Decreased lung function as assessed by forced vital capacity (FVC) and forced expiratory volume in one second (FEV₁) is shown to be associated with cardiovascular morbidity and mortality. Although the underlying mechanisms for this association remain unknown, metabolic syndrome and obstructive sleep apnea (OSA) may have a role. We analyzed the relationships between metabolic syndrome and OSA in a cross-sectional health survey of middle-aged male employees.
Methods In this secondary analysis, we re-analyzed the relationships of lung function determined by spirometry with metabolic syndrome and OSA based on the respiratory disturbance index (RDI) with a type 3 portable monitor.
Results We analyzed 273 subjects. Independent of age, body mass index (BMI) and smoking, quartiles for lower FVC and FEV₁ were associated with a higher risk of metabolic syndrome compared with quartiles for the highest FVC and FEV₁, respectively. A similar trend was observed regarding the risk associated with waist circumference, and in FVC cases, dyslipidemia. The risk of hyperglycemia was significantly higher in quartiles for the second lowest FVC and FEV₁ than in quartiles for the highest FVC and FEV₁, respectively. A significant trend for an increase in RDI was observed in accordance with quartiles for lower FVC, but not FEV₁.
Conclusion There was a significant relationship between lung function impairment and metabolic syndrome through mainly abdominal obesity, partially through hyperglycemia, and also through dyslipidemia, but only with respect to restrictive lung function. Restrictive lung function was also related to OSA. This epidemiologic evidence may indicate underlying mechanisms between decreased lung function and cardiovascular risk.

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Objective To compare the efficacy and safety of sedation with dexmedetomidine vs. midazolam for patients with acute cardiogenic pulmonary edema and hypoxemia during the treatment of non-invasive ventilation (NIV).
Methods The intensive care unit (ICU) patients treated in our hospital between March 2008 and August 2011 who had acute pulmonary edema and hyoxemia in NIV failure due to patient refusal to continue the NIV sessions (due to discomfort) were enrolled in this study. The patients were divided into two groups by the random numerical table method. They were treated with either midazolam (29 cases) or dexmedetomidine (33 cases). The patients were sedated (Ramsay scale 2-3) by a continuous perfusion of midazolam or dexmedetomidine during the NIV session. Cardiorespiratory and ventilatory parameters, the results of the blood gas analysis, and adverse events were prospectively recorded. The main outcome measure was the percentage of endotracheal intubation during NIV. Secondary endpoints included the duration of non-invasive mechanical ventilation, length of ICU stay, and adverse events.
Results In both groups of patients, the expected sedative scores were obtained. The cardiorespiratory symptoms and signs (oxygenation index, pH value, and respiratory rate) were significantly improved in both groups. In the dexmedetomidine-treated group, the patients had a further decreased percentage of failure of NIV requiring endotracheal intubation (ETI) and a more prolonged mean time to ETI (p=0.042, p=0.024). Furthermore, when compared with the group treated with midazolam, the overall duration of mechanical ventilation and the duration of ICU hospitalization in the group treated with dexmedetomidine were markedly decreased, and weaning from mechanical ventilation was easier (p=0.010, p=0.042). Despite the fact that more dexmedetomidine-treated patients developed bradycardia (18.2% vs. 0, p=0.016), no patients required an intervention or interruption of study drug infusion. Conversely, the incidence of respiratory infections and vomiting was lower in the dexmedetomidine-treated patients (p=0.026, p=0.010).
Conclusion Dexmedetomidine led to a more desired level of awaking sedation, shortened the duration of mechanical ventilation and the length of the ICU stay, and further reduced the prevalence of nosocomial infection for NIV sedation in patients with acute cardiogenic pulmonary edema. It appears to provide several advantages and safe control compared with the γ-amino butyric acid (GABA) agonist midazolam.

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Objective A significant number of patients with Fisher syndrome (FS) exhibit sensory symptoms in addition to the classical triad of opthalmoplegia, ataxia and areflexia. Previous studies have shown the amplitudes of sensory nerve action potentials (SNAPs) to decrease in patients with FS, thus implying the presence of an axonal pathology in the sensory nerves.
Methods We included ten consecutive patients with FS who were divided into the following two groups: those with hypesthesia (group H) and those without hypesthesia (group NS). The parameters obtained from nerve conduction studies (amplitudes of compound muscle action potentials, motor conduction velocities, amplitudes/duration of SNAPs and sensory conduction velocities) were retrospectively compared between the two groups. In addition, follow-up sensory nerve conduction studies were conducted in one representative patient from each group.
Results Of the 10 patients with FS, four (40%) showed hypesthesia and eight (80%) showed distal paresthesia. The amplitudes of the SNAPs of both the median and sural nerves were lower in group H than in group NS. Moreover, the duration of the sural SNAPs was longer in group H than in group NS. Desynchronization of SNAPs in the acute phase was observed during follow-up in both patients who underwent follow-up studies.
Conclusion The prolonged duration of SNAPs in group H and the desynchronization of SNAPs in the two patients who underwent follow-up studies suggest the presence of a concomitant demyelinating process in the sensory nerves.

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Objective Scrub typhus is an important febrile disease in Asia, and antibiotics have been used to treat this disease. The purpose of this study was to generate large-scale evidence of the efficacy of different antibiotic regimens for treating scrub typhus using a meta-analysis.
Methods PubMed, Elsevier ScienceDirect, Chinese Biomedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), and Wanfang (Chinese) were searched to identify relevant articles. The data from eligible citations were extracted by two reviewers. All analyses were performed using the Cochrane Collaboration Review Manager 4.2 and Stata 10.0 software programs.
Results We conducted a meta-analysis of 17 separate studies that evaluated the efficacy of treatment with the different antibiotic regimens for scrub typhus. The median time (h) to clearance of fever in the azithromycin-treated group was longer than that in the chloramphenicol-treated group (weighted mean difference [WMD] = 12.66, 95% confidence interval [CI]: 2.26,23.06). Adverse events were 2.95 (95%CI: 1.32, 6.61) times more likely to occur in the azithromycin-treated group than in the chloramphenicol-treated group. The clearance time (days) for the main symptoms (including fever, headache, rash and lymphadenectasis) in the doxycycline-treated group was shorter than that in the chloramphenicol-treated group (WMD = -0.4, 95%CI: -0.53, -0.26) in five trials. Adverse drug events occurred significantly less frequently in the azithromycin-treated group than in the doxycycline-treated group (relative risk [RR] = 0.47, 95%CI: 0.31,0.71).
Conclusion Doxycycline was found to act more quickly, but more adverse drug events occur when using this regimen compared to azithromycin and chloramphenicol.

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We herein present a case of IIc-like metastatic lung cancer that was detected early. A 65-year-old man presented with diarrhea and difficulty walking. Colonoscopy demonstrated a depressed lesion in the descending colon. However, the appearance of the lesion by endoscopy with a magnifying objective was not compatible with early colon cancer. Therefore, we performed diagnostic endoscopic mucosal resection to allow for an examination of the whole lesion. A histological examination demonstrated lung cancer metastasis to the colon. Only 1.6% of lung cancers metastasize to the large intestine, and metastatic colorectal cancer is not usually detected at an early stage. In the present case, however, endoscopy and a histological examination revealed alterations in the mucosal configuration of the lesion, which were unusual for early colon cancer.

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We report the case of a 41-year-old woman with hepatocellular carcinoma (HCC). She received living donor liver transplantation (LDLT) from her husband for HCC at 36 years of age. She had few risk factors for HCC recurrence, such as elevated alpha-fetoprotein (AFP), protein induced by vitamin K absence (PIVKA) II, vascular invasion, and number, size of tumors. However, recurrent tumors were found in the graft at 54 months after LDLT. Although we examined the sex chromosomes of the HCC by fluorescence in situ hybridization (FISH) methods, the origin of the HCC was unclear. This is a very rare case of recurrent HCC appearing more than 4 years after LDLT in the absence of risk factors for recurrence.

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We herein present a rare case of myocardial metastases from intrahepatic cholangiocarcinoma. The patient was a 68-year-old man diagnosed with stage IVb intrahepatic cholangiocarcinoma. Growing low-density nodules at the cardiac interventricular septum and the left lateral wall were found on contrast enhanced computed tomography (CECT). He died and an autopsy was performed. Intrahepatic cholangiocarcinoma was found to have metastasized to the myocardium, and these myocardial metastases conformed to the low-density nodules detected on CECT. A hilar nodule in the lung was revealed to be lung squamous cell carcinoma. This was a rare case of intrahepatic cholangiocarcinoma with myocardial metastases and lung squamous cell carcinoma.

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We present a case of pulmonary arterial hypertension (PAH), which is potentially related to treatment with dasatinib (Sprycel^®). A 61-year-old woman, who had been treated with dasatinib for 27 months for chronic myeloid leukemia (CML), visited our hospital complaining of dyspnea. In right heart catheterization, her mean pulmonary arterial pressure was 35 mmHg. After other possible etiologies to cause PAH were excluded, the patient was diagnosed as a dasatinib-related PAH. As notified by U.S. Food and Drug Administration (FDA) in October 2011, we recommend routine cardiopulmonary evaluation before and during treatment with dasatinib in CML patients in terms of the adverse effects of PAH.

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A 51-year-old woman was admitted to our hospital with heart failure due to aortic regurgitation. Examination showed severe aortic regurgitation mainly due to cleft-like deformity of the right coronary cusp and single coronary artery. Aortic valve replacement was performed, and the deformity was seen in all aortic cusps. Histological study showed elongation of the leaflets by myofibrotic and fibrotic hyperplasia without calcification and inflammation in the aortic valve. This deformity likely arose from an acquired modification of a congenitally malformed aortic valve.

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We herein report the case of an idiopathic aneurysm of the accessory hemiazygos vein diagnosed by contrast-enhanced computed tomography (CT) and angiography. The patient was asymptomatic, and a posterior mediastinal mass was incidentally found on CT. Because endoscopic ultrasonography showed no thrombus formation in the aneurysm, the patient was therefore managed conservatively.

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We report an adult case of internal mammary arteriovenous fistula which was found to have a continuous murmur. The fistula was clearly demonstrated by multi-detector row computed tomography and selective digital subtraction angiography, and was successfully occluded by transcatheter embolization.

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We herein report the histopathology of a rare case of an idiopathic internal mammary artery aneurysm in a 61-year-old asymptomatic woman. Chest radiography during an annual medical check-up incidentally revealed the aneurysm, which was initially mistaken for a mediastinal tumor. Given that a rupture of the aneurysm could have been life-threatening, it was removed surgically, and found to possess a paper-thin arterial wall with cystic medial degeneration.

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A 23-year-old pregnant woman had a stillbirth at 30 weeks gestation due to abrupt diabetic ketoacidosis. The patient had a normal HbA1c, severe hyperglycemia, negative islet cell autoantibodies, and very low insulin secretion capacity. The viral markers associated with fulminant type 1 diabetes were negative. The patient's human leukocyte antigen genotypes were DRB1*04:06 and DQB1*03:01/05:02, not common subtypes for fulminant type 1 diabetes. This is the first Vietnamese patient with fulminant type 1 diabetes mellitus.

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We herein report the case of a 41-year-old male patient with an incidentally identified large adrenal ganglioneuroma (GN). His endocrine examinations were normal except for one episode of elevated urinary dopamine and noradrenaline levels. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) showed a large solid tumor with calcifications and a slightly lobular edge in the right adrenal gland. We performed open tumor excision and diagnosed it as adrenal ganglioneuroma. Adrenal GN is a rare benign tumor, and its hormonal activity and imaging characteristics are occasionally very similar to those of other adrenal tumors. Therefore, it needs careful evaluation by endocrine examinations and multiple imaging procedures to rule out other types of tumors.

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Patients with acute adrenal insufficiency may have musculoskeletal symptoms including flexion contractures, myopathy and hyperkalaemic neuromyopathy. However, the association between rhabdomyolysis and acute adrenal insufficiency is extremely rare and has only been reported infrequently in the literature. Hyponatraemia is often present in association with acute adrenal insufficiency complicated by rhabdomyolysis. We herein report the case of a patient with acute primary adrenal insufficiency and severe hyponatraemia complicated by rhabdomyolysis and acute kidney injury.

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A 44-year-old woman with iron deficiency anemia was on a continuous course of intravenous saccharated ferric oxide (SFO). She came to our hospital because of right hip joint pain. She was found to have hypophosphatemia caused by impaired phosphorus resorption and her fibroblast growth factor 23 (FGF-23) levels were elevated. Therefore, she was diagnosed with FGF-23-related osteomalacia due to SFO administration. Discontinuation of the SFO treatment rapidly improved the impaired phosphorus resorption and also normalized the blood levels of phosphorus and FGF-23. During the treatment with SFO, it is important to regularly measure the blood levels of phosphorus in order to prevent the occurrence of osteomalacia.

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Thyroid storm is a rare disorder with a sudden onset, rapid progression and high mortality. We experienced a case of thyroid storm which had a devastating course, including multiple organ failure (MOF), severe hypoglycemia, disseminated intravascular coagulation (DIC), and stroke. It was difficult to make a diagnosis of thyroid storm in the present patient, because she did not have a history of thyroid disease and her serum FT3 level was normal. Clinicians should be aware that thyroid storm can occur even when there is an almost normal level of thyroid hormones, and that intensive anticoagulation is required for patients with atrial fibrillation to prevent stroke after thyroid storm.

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IgG4-related systemic disease (IgG4-RSD) is an autoimmune disease that includes a wide variety of lesions. IgG4-RSD is characterized by high levels of serum IgG4, abundant levels of IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. Tubulointerstitial nephritis (TIN) is a major finding when the kidneys are involved and is effectively treated with corticosteroid therapy. We herein describe two cases of IgG4-related TIN. Such cases have rarely been reported in China.

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A 68-year-old woman showed rapidly progressive glomerulonephritis based on the fact that she had 1+ proteinuria (1.1 g/day), a 3+ occult blood reaction, blood urea nitrogen of 32.4 mg/dL, serum creatinine of 2.96 mg/dL, and myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibodies (ANCA) at 52 ELISA Unit (normal range: below 10). A renal biopsy demonstrated a bubbling appearance associated with cellular crescent formation with segmental necrosis. Immunofluorescence studies showed granular IgG3λ deposition along the basement membrane and in the mesangial area. This is the first English-language case report describing MPO-ANCA positive crescentic glomerulonephritis in a patient demonstrating monoclonal immunoglobulin deposition disease with mainly membranous features.

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A 26-year-old man with unresectable inflammatory myofibroblastic tumor (IMT) presented with multiple metastases in the thoracic vertebra and lymph nodes as detected by positron emission tomography (PET) received chemotherapy with carboplatin plus paclitaxel. After three cycles of chemotherapy, fluorine-18-fluorodeoxyglucose (FDG)-PET/CT revealed tumor regression and significant reduction of FDG uptake in all lesions. The patient received six cycles of chemotherapy without any severe adverse event, and there was no sign of disease progression for seven months. This regimen is well tolerated and may be considered the treatment of choice for unresectable IMT.

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Chyloptysis is a very rare clinical finding. We describe a 44-year-old man who presented with cough and milky-white sputum. Fiberoptic bronchoscopy revealed white sputum, which originated from the right B₆ bronchus. The finding of elevated triglyceride levels in his sputum led to the diagnosis of chyloptysis. He had a surgical history of ligation of the thoracic duct for idiopathic chylopericarditis 7 years-previously. He also suffered from postoperative bilateral empyema. Since then, his pleural cavity has been adhered bilaterally. It is thought that his abnormal postoperative lymphatic flow caused the chyloptysis.

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Although rare, gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. The asynchronous occurrence of other malignancies in patients with GIST during the clinical course is relatively common. However, the synchronous coexistence of GIST and lung cancer has only rarely been reported. We experienced a case of coincidental primary lung adenocarcinoma and intestinal GIST. The present case is not only of interest due to the rare coincidence of GIST and lung cancer, but also because there was an epidermal growth factor receptor gene mutation in the lung cancer and a c-kit mutation in the GIST.

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A 74-year-old Japanese man with myelodysplastic syndrome (MDS) received chemotherapy with azacitidine. From the second day after starting the administration, he complained of fever, cough and shortness of breath. Chest roentgenography and computed tomography showed consolidations and ground-glass opacities. His symptoms grew from worse to life-threatening. We diagnosed him with azacitidine-induced pneumonitis and began administering corticosteroids. Thereafter, his symptoms and radiographic abnormalities improved. Azacitidine is a hypomethylating agent that improves the survival of MDS patients. Although this drug is commonly well tolerated and rarely causes severe lung injury, it is important to consider the potentially serious adverse effects of azacitidine-induced pneumonitis.

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We report a 67-year-old woman who was diagnosed with hepatic portal venous gas associated with severe graft-versus-host disease (GVHD) of the gastrointestinal tract. The patient received allogenic peripheral blood stem cell transplantation from a haploidentical son against Philadelphia chromosome-positive acute lymphocytic leukemia. The patient developed grade 3 intestinal GVHD on day 90 from the transplantation. On day 149, she presented septic shock and computed tomography (CT) scan revealed hepatic portal venous gas (HPVG); an ileocecal resection was performed immediately. The damage of gastrointestinal mucosa by GVHD resulted in the invasion of gas-producing bacteria. Although HPVG-associated gastrointestinal GVHD is extremely rare, we should pay special attention to this pathogenesis.

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A 63-year-old woman had anti-Hu-associated paraneoplastic encephalomyelitis (anti-Hu syndrome) caused by esophageal small cell carcinoma (SCC). The patient developed bilateral limbic encephalitis, followed by myelitis, brain stem encephalitis, and autonomic failure. Extensive examination demonstrated SCC of the abdominal lymph nodes that was retrospectively diagnosed as metastasis of esophageal SCC on autopsy. The neuropathological findings were characterized by widespread neuronal loss and gliosis in the central nervous system, as well as patchy loss of myelin and axons in the spinal nerve roots with perivascular lymphocytic infiltration. This is the first detailed clinical and neuropathological report of anti-Hu syndrome caused by esophageal SCC.

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A 60-year-old woman developed opsoclonus-myoclonus syndrome (OMS) a week after being diagnosed with influenza A infection by a rapid antigen test. She had no loss of consciousness. Opsoclonus, myoclonus, and truncal ataxia were noted. Two weeks after treatment with intravenous immunoglobulin and corticosteroids, her opsoclonus, myoclonus, and truncal ataxia disappeared. No malignant tumors were detected during the 3-year follow-up period. There has been no previous report of postinfectious OMS following confirmed influenza A infection. OMS without a loss of consciousness has been reported to be statistically less common in cases of non-paraneoplastic OMS. This finding was consistent with the present patient's clinical manifestations.

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We herein report a 26-year-old man with Guillain-Barré Syndrome (GBS) coexisting facial nerve palsy (FP) and deafness. He developed deafness, facial weakness, and limb weakness and numbness. Neurological examination showed facial diplegia, bilateral hypoacusia, areflexia and sensorimotor deficits in the distal limbs. The nerve conduction study findings supported the diagnosis of the demyelinating polyneuropathy. An audiogram revealed sensorineural hearing loss of 40-50 dB. Auditory brainstem responses disclosed no elicitation of waves I to IV on both sides. Magnetic resonance imaging depicted abnormal enhancement in bilateral facial and acoustic nerves. Physicians should pay more attention to auditory dysfunction in GBS patients with FP.

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A nummular headache (NH) is a type of primary headache that results from cranial neuralgia without a known cause. We herein report the case of a woman who suffered two episodes of focal headache in the left parietal area with identical characteristics that were compatible with NH. During the recovery phase of the second NH episode, the pain resurged with shingles coinciding with the painful area. The patient's NH subsided in parallel with resolution of the shingles. These findings support a diagnosis of peripheral neuralgia with NH. Latent virus infections, such as Varicella-zoster virus, that frequently cause distal nerve damage in patients with zoster sine herpete may be associated with epicranial neuralgia and NH.

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Neuromyelitis optica spectrum disorder (NMOSD) is characterized by optic neuritis or transverse myelitis with anti-aquaporin 4 (AQP4) antibodies (1). We herein present the case of a patient with NMOSD who also was affected with peripheral neuropathy. A 58-year-old woman developed gait disturbance and sensory impairment in the lower limbs. She exhibited longitudinally extensive transverse myelitis with anti-AQP4 antibodies. Nerve conduction studies showed demyelinating changes. Laboratory findings showed hepatitis-C virus (HCV) infection. Her peripheral neuropathy improved after immunotherapy. There have been no previous reports of NMO or NMOSD associated with neuropathy. The HCV infection or undetermined humoral factors other than the anti-AQP4 antibodies may have caused her peripheral neuropathy.

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A 70-year-old woman developed marked akinesia after an anoxic event related to bronchiectasia. Magnetic resonance imaging studies revealed lesions in the bilateral globus pallidus and, to a lesser extent, in the putamen. Positron emission tomography studies with ¹⁸F-6-fluoro-L-dopa and ¹¹C-N-methylspiperone showed a decreased pre- and post-synaptic uptake in the striatum. Consistent with previous reports, the present case demonstrated the basal ganglia, particularly the globus pallidus, to be selectively susceptible to anoxic insults. Furthermore, a PET study indicated a disrupted presynaptic integrity of the dopaminergic terminals and decreased dopamine D₂ receptor binding, which together appear to underlie the pathophysiology of post-anoxic akinesia, at least in the present case.

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In patients with obstructive sleep apnea (OSA) and epilepsy, the frequency of generalized spike and wave complexes (GSWCs) usually decreases after the initiation of nasal continuous positive airway pressure (nCPAP) therapy. However, we herein report a patient who had a transient increase in GSWCs following nCPAP treatment. A woman with epilepsy underwent polysomnography, who showed severe OSA and 30 GSWCs during the sleep study. Polysomnography at the introduction nCPAP showed that the GSWCs increased to 94 times during the monitoring period, despite improvement of her OSA. Polysomnography was again performed four months later, and the GSWCs had decreased to 23 times. Physicians should therefore be cautious regarding a possible increase in epileptiform discharges and seizures immediately after the introduction of nCPAP.

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A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented. A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCAs). Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands. Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease (MD). Oral prednisolone (30 mg) was administered and the swelling of the lachrymal glands resolved. Churg-Strauss syndrome may be accompanied by Mikulicz's disease (an IgG4-related disease), and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist.

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A 62-year-old man presented with general fatigue. He was diagnosed with septic shock and severe pneumonia. The sputum at admission yielded methicillin-sensitive Staphylococcus aureus (MSSA) strain and methicillin-resistant S. aureus (MRSA) strain. Despite antibiotic treatment, he did not improve. A chest computed tomography (CT) revealed multilobar cavity lesions. Only MRSA strain was confirmed at that time. We diagnosed him with necrotizing pneumonia. Despite treatment with vancomycin, his pneumonia worsened and he died. At autopsy, many gram-positive cocci were observed in the lungs. The clinical presentation of our patient was different from typical CA-MRSA-mediated necrotizing pneumonia.

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We describe a case of 68-year-old Japanese man with HIV-1 infection who developed acute kidney injury with prominent tubular dysfunction immediately after starting tenofovir-containing antiretroviral therapy. Antiretroviral therapy was discontinued in two weeks but renal function, as well as tubular function, did not shown full recovery even at a 3-year follow-up examination. Acute tubular necrosis, a rare but well-known side effect of tenofovir, was suspected, but kidney biopsy confirmed interstitial nephritis. It is important to distinguish drug-induced interstitial nephritis from acute tubular necrosis, because early steroid administration can improve renal dysfunction caused by acute interstitial nephritis.

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This report presents a case of fulminant community-acquired Pseudomonas aeruginosa necrotizing pneumonia in a previously healthy young man, including an analysis of the virulence of the P.aeruginosa isolated from the patient. The patient was successfully treated with intensive care and antibiotic treatment. This study analyzed the pathogenicity of the isolated strain both in vivo (using a mouse pneumonia model) and in vitro (using biofilm production), but could not explain how an otherwise healthy young man developed such severe community-acquired P.aeruginosa pneumonia. Although rare in community-acquired pneumonia, P.aeruginosa infection should be considered in patients with severe rapidly progressive pneumonia.

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A 46-year-old man complained of ulcerovegetative lesions in the anogenital region, which he had noted one month prior to presentation. The patient had a history of travel to African countries. Therefore, the ulcerovegetative lesions of the patient were suspected to be granuloma inguinale (GI). Calymmatobacterium granulomatis was not observed in the direct examination of scrapings collected from the base of the ulcerovegetative lesion. Instead, a histological examination revealed cutaneous metastasis of mucinous adenocarcinoma of the rectum. Therefore, a diagnosis of GI was eliminated. As the patient did not report his history of rectal cancer and had travelled to African countries, we had primarily focused on the diagnosis of GI.

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