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We analyzed 20 patients diagnosed with autoimmune neurological diseases with seizure predominance. In these patients, we examined the usefulness of Antibody Prevalence in Epilepsy and Encephalopathy (APE 2 ) score and Antibodies Contributing to Focal Epilepsy Signs and Symptoms (ACES) score in autoimmune encephalitis (AE) for facilitating early treatment. APE 2 score was positive in 19 of 20 patients. ACES score was positive in 15 of 20 patients, and 4 of 5 of the patients with negative ACES score did not have AE. Comprehensive assessment including the use of the above scores is desirable in the early stage of AE.

Foreign body granuloma(FBG)is a granuloma that occurs due to chronic inflammation caused by various residual foreign objects. In the field of gastrointestinal surgery, intraperitoneal foreign body granulomas(IPFBGs)are often caused by sutures materials or residual gauzes, but those caused by food residue are extremely rare. We present an IPFBG case of food residue caused by anastomotic leakage, which was difficult to be distinguished from peritoneal dissemination. The patient is a 74- year-old male. Anastomotic leakage occurred following low anterior resection for rectal cancer, peritoneal drainage and ileostomy were performed. 1.5 years after rectal resection, liver metastasis was diagnosed by CT and peritoneal dissemination was diagnosed by PET-CT. Both lesions were resected at the same time. The pathological findings were liver metastasis and FBG. It was presumed to be an FBG formed by food residue left behind after anastomotic leakage. It has reported that FBG caused by residual gauzes were shown a ring-shaped uptake by PET-CT, but that was not observed in our case. In addition, since a nodule suspected of liver metastasis was observed simultaneously, we considered no differential diagnosis other than peritoneal dissemination. IPFBG resembling peritoneal dissemination, occurred after anastomotic leakage. A food residue can cause IPFBG, it is necessary to consider IPFBG in decision making treatment strategy for peritoneal nodule.

In past reports, the incidence of gastric perforation accounts for 0.08 to 3.6% of all gastric cancers, and the proportion of perforated gastric cancer(PGC)in gastric perforations is 26 to 32%. In the treatment of PGC, critical care for peritonitis, diagnosis of gastric cancer and curability for gastric cancer are required simultaneously, so it is not easy to decide the treatment strategies. Therefore, for the purpose to consider treatment strategies for PGC, we conducted a clinicopathological study on PGC in our hospital for the past 12 years. There were 22 cases of PGC, and we analyzed clinicopathologically 19 cases excluding perforation during endoscopic resection and perforation during chemotherapy. The R0 surgery group tended to have a good prognosis even in PGC cases, and there was surgery-related death in the one-stage gastrectomy group. So it was considered desirable to perform radical surgery after the general condition was stable by the treatment of peritonitis was given priority in the PGC.

Hashimoto encephalopathy (HE) has been recognized as a new clinical disease based on an autoimmune mechanism associated with Hashimoto's thyroiditis. HE is successfully treated with steroids. In 2005, we discovered serum autoantibodies against the NH 2 -terminal of α-enolase (NAE) as a highly specific diagnostic biomarker for HE. We analyzed the serum anti-NAE autoantibodies and clinical features in many cases of HE from institutions in Japan and other countries. Approximately half of the patients with HE had anti-NAE antibodies. In our study, HE was widely distributed in patients aged 50-60 years. Most patients with HE were in euthyroid states, and all patients had anti-thyroid antibodies. The common neuropsychiatric features of these patients were consciousness disturbance and psychosis, followed by cognitive dysfunction, involuntary movements, seizures, and ataxia. Abnormalities in EEG and decreased cerebral blood flow on the brain SPECT were common findings. In contrast, abnormalities on the brain MRI were rare except for diffuse subcortical lesions and limbic lesions. Patients with HE had varied clinical phenotypes, including acute encephalopathy, chronic psychiatric form, and other particular clinical presentations, such as limbic encephalitis, progressive cerebellar ataxia, and Creutzfeldt-Jakob disease (CJD)-mimic form. The cerebellar ataxic form of HE clinically mimics spinocerebellar degeneration (SCD) and is characterized by the absence of nystagmus, absent or mild cerebellar atrophy, and lazy background activities on electroencephalography (EEG). Taken together, these features should indicate the possibility of encephalopathy associated with thyroid disorders.

Oxidative stress and mitochondrial dysfunction are assumed to be the pathogenic molecular mechanisms underlying various neurodegenerative diseases. We applied positron emission tomography (PET) with [ 62 Cu] diacetyl-bis (N 4 -methylthiosemicarbazone) ( 62 Cu-ATSM) to image cerebral oxidative stress based on mitochondrial dysfunction in living patients. In our previous study, we observed an increased retention of Cu-ATSM in in vitro cell lines with mitochondrial respiratory failure, suggesting that 62 Cu-ATSM uptake can be a promising biomarker for evaluating oxidative stress in patients with mitochondrial or neurodegenerative diseases. PET imaging with 62 Cu-ATSM successfully demonstrated the increased uptake in brain lesions of a patient with mitochondrial disease (MELAS), in the striatum of patients with Parkinson's disease, and in the motor cortex and motor-related cortices of patients with amyotrophic lateral sclerosis. The uptake for these disease-related brain regions strongly correlated with disease severity, indicating that oxidative stress based on mitochondrial dysfunction is associated with the neurodegenerative process in these diseases. 62 Cu-ATSM PET imaging for oxidative stress has improved our insights into the pathological mechanisms of neurodegenerative diseases and may be a promising tool for monitoring further antioxidant and mitochondrial therapies.

Encephalopathy occasionally occurs in association with thyroid disorders, most of which are treatable. These encephalopathies include a neuropsychiatric disorder associated with hypothyroidism named myxedema encephalopathy. Moreover, Hashimoto's encephalopathy (HE) has been recognized as a new clinical disease based on an autoimmune mechanism associated with Hashimoto's thyroiditis, and can be successfully treated using steroids. Recently, we discovered that the serum autoantibodies against the NH 2 -terminal of α-enolase (NAE) were a highly specific diagnostic biomarker for HE. We analyzed the serum anti-NAE autoantibodies and the clinical features in many cases of HE from institutions across Japan and other countries. About half the patients with HE had anti-NAE antibodies. Patient age was widely distributed with two peaks (around 20-30 years old and 60-80 years old). Most patients with HE were in euthyroid states and all patients had anti-thyroid antibodies. The common neuropsychiatric features include disturbance of consciousness, psychosis, cognitive dysfunction, involuntary movements, seizures, and ataxia. Electroencephalograph (EEG) abnormalities and decreased cerebral blood flow on brain single positron emission computed tomography are common findings, whereas abnormalities on brain magnetic resonance imaging are rare. Patients with HE present with various clinical phenotypes such as an acute encephalopathy form and chronic psychiatric form. Other clinical forms include limbic encephalitis, progressive cerebellar ataxia, and Creutzfeldt-Jakob disease (CJD)-mimic forms. The cerebellar ataxia form of HE clinically mimics spinocerebellar degeneration (SCD) and is characterized by the absence of nystagmus, absent or mild cerebellar atrophy, and lazy background activity on EEG. Taken together, clinicians should pay attention to the possibility of encephalopathy associated with thyroid disorders.

Nonalcoholic fatty liver disease (NAFLD), especially nonalcoholic steatohepatitis (NASH), is a disease that may lead to liver cirrhosis or hepatocellular carcinoma. The number of patients is increasing steadily and the pathology is diverse. Effective treatment is still mainly focused on diet and exercise therapy. With the advent of the next-generation sequencer, the intestinal flora in NAFLD/NASH is being elucidated. We believe that elevation of endotoxin in the blood caused by intestinal bacterial dysbiosis caused by intestinal permeability enhancement is important for NASH pathology and control of intestinal permeability enhancement has a new possibility for treatment of NAFLD/NASH.

Aim: Despite the significant advances in chemotherapy, the prognosis of unresectable or recurrent gastric cancer is still very poor. Given that older adults are likely to have a number of concomitant diseases and an impaired major organ function, cancer chemotherapy in elderly patients requires particular caution. We examined what factors are associated with the overall survival of gastric cancer patients undergoing chemotherapy., Methods: A retrospective chart review of gastric cancer patients receiving oral fluoropyrimidines (N=130) was performed at Nagoya Memorial Hospital over 9 years. The overall survival was calculated from the beginning of chemotherapy until death or the most recent date of follow-up. The Kaplan-Meier method was used to plot survival curves, which were compared using the log-rank test. A multivariate analysis was performed using stepwise Cox proportional hazards models. A comprehensive geriatric assessment was conducted for the elderly patients. The chart review was approved by the ethics committee of Nagoya Memorial Hospital., Results: The objective response rate and overall survival did not differ markedly between the patients < 75 years (N=64) and those ≥ 75 years of age (N=28). The addition of lentinan significantly prolonged the survival of the stage 4 gastric cancer patients. In a multivariate analysis of those ≥ 75 years of age, the only independent prognostic factor for the survival was the functional capacity, as measured by the TMIG Index of Competence., Conclusions: This comprehensive geriatric assessment was useful for predicting the longevity of patients with stage 4 gastric cancer ≥ 75 years of age.

Nipah and Hendra virus were first identified in mid 1990s in Australia and Malaysia, caus- ing epidemics with high mortality rate in affected animals and humans. Since their first emer- gence, they continued to re-emerge in Australia and South East Asia almost every year. Nipah and Hendra virus were classified in the new genus Henipavirus because of their un- common features amongst Paramyxoviridae. Henipaviruses are zoonotic paramyxoviruses with a broad tropism, and cause severe acute respiratory disease and encephalitis. Their high virulence and wide host range make them to be given Biosecurity Level 4 status. This review summarizes details of Henipavirus emergence, reservoir hosts and pathology, and introduce recent progress in vaccines and antivirals.

Endocrine diseases affecting various organs, such as the pituitary gland, the thyroid, the parathyroid, the adrenal glands and the pancreas, occasionally cause dementia. While Alzheimer's disease (AD) is the main cause of dementia in the elderly and is untreatable, dementia caused by endocrine diseases is treatable in most cases. However, patients with dementia associated with endocrine diseases show memory impairments similar to those found in AD, often leading to misdiagnoses. Patients with endocrine diseases often present with other characteristic systemic and neuropsychiatric symptoms caused by altered hormone levels. Such neuropsychiatric symptoms include involuntary movements, depression, seizures, and muscle weakness. In these cases, abnormalities in imaging and blood or urine tests are helpful in making a differential diagnosis. As delays in the diagnosis and treatment of these patients may cause irreversible brain damage, it is imperative for clinicians to carefully exclude the possibility of latent endocrine diseases when treating patients with dementia.

A 15-year-old boy was admitted to our hospital with a recent increase in the size of a preexisting pancreatic pseudocyst. At 11 years of age, he was diagnosed with acute lymphoid leukemia (ALL) and received chemotherapy with L-asparaginase (L-Asp); he developed the pancreatic pseudocyst following L-Asp-induced acute pancreatitis. The pancreatic pseudocyst had increased to 120mm in diameter. He developed epigastralgia and portal hypertension. Endoscopic ultrasound (EUS)-guided cystogastrostomy with the placement of a 7-cm 7-Fr plastic stent and a 5-Fr NB pigtail catheter led to the near-complete resolution of the pseudocyst. There were no signs of recurrence within the first year after intervention. EUS-guided drainage, increasingly used for pseudocysts, should be considered as an effective treatment approach for pediatric pancreatic pseudocysts.

A 56-year-old man was diagnosed with immunoglobulin (Ig) A-κ multiple myeloma in July 2007. After three courses of vincristine, adriamycin, and dexamethasone (VAD) chemotherapy, autologous peripheral blood stem cell transplantation was performed and achieved a very good partial response. In February 2010, an increase in the M-protein concentration and plasmacytoma in the L3/4 lumbar vertebrae were observed, and radiation treatment was performed. This was followed by administrations of bortezomib, lenalidomide, and thalidomide, none of which achieved a good response. In November 2011, the patient presented with obstructive jaundice, and imaging tests revealed tumorous lesions in the lower bile duct region and bilateral kidneys. Plasmacytoma was diagnosed from biopsy of the right renal mass. Radiotherapy to the common bile duct tumor resulted in jaundice amelioration, but the patient died despite subsequent treatment efforts. Autopsy revealed multiple extramedullary lesions in the abdominal cavity and in the region around the common bile duct. CD138 shedding was observed in the myeloma cells. We include a discussion of the literature on CD138 shedding and 38 reports of obstructive jaundice associated with extramedullary disease.

Dementia with Lewy bodies (DLB) is clinically characterized by progressive dementia that is frequently accompanied by neurological and psychiatric manifestations. Hashimoto's encephalopathy (HE) is a rare autoimmune disease with neurological and psychiatric manifestations that is not well understood. However, this disease has attracted growing attention as a treatable dementia. Although autoimmune mechanisms are thought to play a pathogenic role in HE, the etiology of the disease remains unclear. Recently, it was reported that the serum in patients with HE is frequency positive for autoantibodies against the anti-NH 2 -terminal of α-enolase (anti-NAE), indicating a useful serological diagnostic marker for HE. We report the case of an 81-year-old Japanese woman with probable DLB and hypothyroidism. In her serum, elevated anti-thyroid antibodies and positive autoantibodies against anti-NAE were observed. Elevated levels of anti-glutamate receptor ε2 subunit (GluRε2) antibodies were also detected in her cerebrospinal fluid. Because her clinical condition became stable after treatment with cholinesterase inhibitor, levodopa, and levothyroxine, immunotherapy was not performed. Although the relationship between autoimmunity and cognitive decline in this patient was unclear, the present observations suggest the coexistence of neurodegeneration and autoimmunity as the underlying pathogenic mechanism.

A 30-year-old woman was admitted to the first institution with subacutely progressive aphasia and depression. Despite of lacking conclusive evidence on magnetic resonance imaging, cerebrospinal fluid examination, or electroencephalogram, we tentatively diagnosed her disease as limbic encephalopathy due to its acute progression. High-dose methylprednisolone was started on admission. However, symptoms did not improve. To make matters worse, psychiatric symptoms, such as hallucinations and emotional incontinence, appeared on the same day. Additional treatment with plasma exchange and intravenous immunoglobulin administration was also ineffective. Therefore, we could not manage the patient in a general ward due to severe psychiatric symptoms. The patient was transferred to a psychiatric ward in the second institution. She received both psychiatric treatment and steroid therapy, including a second course of intravenous high-dose methylprednisolone, followed by long-term oral prednisolone. Her symptoms gradually improved. A final diagnosis of Hashimoto's encephalopathy was made based on the patient's clinical course and positive results for both serum anti-thyroid antibody and anti-NAE antibody. In our case, long-term oral steroid therapy under psychiatric treatment was effective for good outcome.

We report a case of fulminant hepatitis that was caused by XELOX therapy administered for metastatic rectal cancer. A 69- year-old man with metastatic rectal cancer received 4 courses XELOX therapy. He was subsequently admitted to our hospital with general fatigue. Shenzhen flapping and altered consciousness were noticed on the fifth day of hospitalization. A liver biopsy was subsequently performed. The patient was diagnosed with liver failure due to sinusoidal obstruction syndrome caused by oxaliplatin. This case provides valuable information as there are only a few reports of fulminant hepatitis caused by oxaliplatin.

We describe a patient, 61-year-old left-handed Japanese woman, who presented with left hemi-facial metamorphopsia after infarct that extended from the splenium of the corpus callosum to the major forceps on the right side. Past medical history revealed a right putaminal hemorrhage with amnesic aphasia. She complained that the right side of people's faces, that is, the left side when visualized by her, seemed distorted. When she looked at other people's faces, the right half of the faces looked smaller than the left half, and the eyes, noses, and mouths appeared to be hanging toward the center of their faces. This phenomenon was observed for whomever she visualized. She stated that objects other than the face looked normal. Her visual acuity and visual field were normal. Callosal disconnection syndrome was not presented. Magnetic resonance imaging of the brain on diffusion weighted image revealed a high intensity area that extended from the splenium of the corpus callosum to the major forceps on the right side. Electroencephalography did not show any epileptic discharge. Her visual symptoms improved gradually. The mechanism of hemi-facial metamorphopsia remains obscure. We hypothesized that this patient developed left hemi-facial metamorphopsia because of the disrupted transfer of visual information of the left side of face at the splenium of the corpus callosum and the major forceps, which may be the responsible lesion of hemi-facial metamorphopsia.

Nipah virus (NiV), a paramyxovirus, was first discovered in Malaysia in 1998 in an outbreak of infection in pigs and humans, and incurred a high fatality rate in humans. We established a system that enabled the rescue of replicating NiVs from a cloned DNA. Using the system, we analyzed the functions of accessory proteins in infected cells and the implications in in vivo pathogenicity. Further, we have developed a recombinant measles virus (rMV) vaccine expressing NiV envelope glycoproteins, which appeared to be an appropriate to NiV vaccine candidate for use in humans.

A 67-year-old man living alone was admitted for acute disturbance of consciousness during winter. He presented with semicoma, a decorticate posture, and exaggerated tendon reflexes of the limbs, but brainstem reflexes were intact. The carboxyhemoglobin (COHb) level was normal in arterial blood gas on admission, and protein in cerebrospinal fluid was increased without pleocytosis. Brain MRI showed diffuse T2 high intensities in the deep white matter bilaterally without a contrast effect and abnormal T1 intensity in the pallidum. (1)H-MR spectroscopy (MRS) of the white matter lesion demonstrated findings suggesting demyelination as an increased choline peak, enhanced anaerobic metabolism as increased lactate and lipids peaks, and reduced neurons as a decreased N-acetylaspartate peak, which corresponded to delayed encephalopathy due to the interval form of carbon monoxide (CO) poisoning. The possibility of CO exposure due to coal briquette use 2 weeks before the symptomatic onset was indicated by his family, so he was diagnosed with CO poisoning. His consciousness slightly improved with corticosteroid therapy and repetitive hyperbaric oxygen therapy, but brain MRI and MRS findings did not improve. Characteristic MRS findings of leukoencephalopathy are helpful for diagnosing the interval form of CO poisoning in the case of a normal COHb level.

Endoscopic ultrasound-guided fine needle aspiration cytology/biopsy is now widely used to diagnose pancreatic tumors. The procedure was introduced to Mie University Hospital in 2006. A pathologist and cytotechnologist collaborate in the endoscopy room, and immediate on-site diagnosis is routinely performed in our hospital. If the pathologist is not able to come to the bed-side, telediagnosis is done using a digital camera attached to a smart phone. The authors emphasize that communication between physicians, cytotechnologists, and pathologists is very important. Therefore, the presence of pathologists in the endoscopy room promotes physicians' skills as well as the accuracy of this procedure.

The patients with nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepa-titis(NASH) often have dyslipidemia along with other features of metabolic syndrome such as obesity, diabetes mellitus, and hypertension. The dyslipidemia in NAFLD is characterized by increased serum triglycerides, increased small dense low-density lipoprotein, and low high-density lipoprotein cholesterol. The pathogenesis of dyslipidemia in NAFLD is not well understood, but it is likely related to hepatic overproduction of the very low-density lipoprotein 1 and decreasing clearance of lipoproteins from the circulation. Recently it is showed that free fatty acids induce hepatocyte lipoapoptosis in vitro. In this manuscript we discuss about "hypertriglyceridemia and NASH/NAFLD".

We report a 53-year-old male with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome who relapsed after autologous peripheral stem cell transplantation (ASCT), but responded extremely well to lenalidomide (LEN) plus low dose dexamethasone (Ld) therapy. The patient had been diagnosed with POEMS syndrome in November 2006, and underwent ASCT in February 2007. In July 2011, he developed respiratory distress, generalized edema, and massive bilateral pleural effusion. Plasma vascular endothelial growth factor (VEGF) and M protein were increased, strongly indicating a relapse. Ld therapy was remarkably effective for these symptoms, resulting in complete remission with M-protein becoming undetectable by immunofixation. Since completing 11 courses of therapy with an every 4 weeks regimen, he has remained in clinical remission. The patient's activities of daily living have also markedly improved from total physical incapacity to being able to stand with slight assistance. LEN is associated with a lower incidence of peripheral neuropathy than other new drugs. Although LEN has occasionally been given to patients with POEMS syndrome in recent years, there are still few reports on its use for patients with recurrent disease after ASCT. Our successful management of this patient suggests that Ld therapy is not only relatively safe but also a promising option for POEMS syndrome relapsing after ASCT.

Hashimoto's encephalopathy (HE) is a treatable disease based on autoimmune mechanisms associated with Hashimoto's thyroiditis. We recently discovered the serum autoantibodies (Abs) against the NH2-terminal of alpha-enolase (NAE) as a diagnostic biomarker for HE. The serum anti-NAE Abs are not detected in normal individuals and other disorders such as infections, collagen diseases, multiple sclerosis and other autoimmune conditions. The specificity of the serum anti-NAE Abs is 91% for HE whereas the sensitivity is 50%. In our clinical study of 80 cases of HE with anti-NAE Abs, the acute encephalopathic form was the most common clinical feature, and followed by chronic psychiatric form and progressive ataxia form. The common neuropsychiatric features were consciousness disturbance, psychosis (especially delirium and hallucination), seizures and dementia. Abnormalities on EEG and decreased blood flow on SPECT were common while abnormalities on brain MRI were rare. The early diagnosis and treatment for HE could lead to good recovery from the disease. Taken together, the serum anti-NAE Abs are a useful diagnostic biomarker for HE.

Encephalopathy occasionally occurs in association with thyroid disorders, but most of these are treatable. These encephalopathies include a neuropsychiatric disorder associated with hypothyroidism, called myxedema encephalopathy. Moreover, Hashimoto's encephalopathy (HE) has been recognized as a new clinical disease based on an autoimmune mechanism associated with Hashimoto's thyroiditis. Steroid treatment was successfully administered to these patients. Recently, we discovered that the serum autoantibodies against the NH2-terminal of α-enolase (NAE) are highly specific diagnostic biomarkers for HE. Further, we analyzed serum anti-NAE autoantibodies and the clinical features in many cases of HE from institutions throughout Japan and other countries. Approximately half of assessed HE patients carry anti-NAE antibodies. The age was widely distributed with 2 peaks (20-30 years and 50-70 years). Most HE patients were in euthyroid states, and all patients had anti-thyroid (TG) antibodies and anti-thyroid peroxidase (TPO) antibodies. Anti-TSH receptor (TSH-R) antibodies were observed in some cases. The common neuropsychiatry features are consciousness disturbance and psychosis, followed by cognitive dysfunction, involuntary movements, seizures, and ataxia. Abnormalities on electroencephalography (EEG) and decreased cerebral blood flow on brain SPECT were common findings, whereas abnormal findings on brain magnetic resonance imaging (MRI) were rare. HE patients have various clinical phenotypes such as the acute encephalopathy form, the chronic psychiatric form, and other particular clinical forms, including limbic encephalitis, progressive cerebellar ataxia, and Creutzfeldt-Jakob disease (CJD)-like form. The cerebellar ataxic form of HE clinically mimics spinocerebellar degeneration (SCD) and is characterized by the absence of nystagmus, absent or mild cerebellar atrophy, and lazy background activities on EEG. Taken together, these data suggest that the possibility of encephalopathy associated with thyroid disorders must be considered.

Objectives: The lead content of excavated bone samples from archaeological sites in Hokkaido was measured to obtain insight into the source of human lead contamination known in the historic Japanese Edo era., Methods: Fifty-seven rib samples excavated from 11 sites of five different eras in Hokkaido were analyzed for lead (Pb), calcium (Ca), and iron (Fe) contents by ICP mass spectrometry and ICP emission spectrometry., Results: The Pb/Ca ratio (mg Pb/kg Ca) was low (approximately 2.0) and constant from the Jomon (approximately 5000 BP) to the Satsumon (approximately 750 BP) eras; however, its median increased to 11 in the Modern era. This elevation of Pb/Ca ratio in the bone samples from sites of the Modern era was not considered to be due to a greater bone contamination with soil particles because of similar Fe concentrations in the bone samples from this era to those in other eras. This historic trend of Pb/Ca ratio was similar to that observed in other parts of Japan. The elevated Pb/Ca ratio in the bone samples excavated from sites in the Modern era in other parts of Japan, that is, Edo era, has been ascribed to the usage of face powder containing Pb; however, people inhabiting Hokkaido in those days, the Ainu, were not considered to have the custom of using face powder., Conclusions: Contamination source(s) other than face powder was postulated in the Modern era of both Hokkaido and other parts of Japan.

Here, we report a case of Hashimoto's encephalopathy (HE) mimicking Creutzfeldt-Jakob disease (CJD). A 57-year-old man was admitted to our hospital for status epilepticus. He had gradually presented personality change over the last two years. On admission, he was in state of akinetic mutism. He exhibited seizures on the left side of his body, including the face, and intermittent myoclonic movement. Routine laboratory tests showed no abnormalities, including thyroid functions. An EEG study showed typical periodic synchronous discharge (PSD). Brain MRI showed high-intensity areas in the bilateral frontal cortex, thalamus, and right insula on diffusion-weighted imaging (DWI). So, initially, sporadic CJD was suspected. However, there were no abnormalities in the caudate or putamen on MRI. Anti-TG and anti-TPO antibodies, as well as anti-NAE antibody were all positive. He was administered methylpredonisolone pulse therapy. Subsequently, his consciousness levels and EEG and MRI findings markedly improved. So, he was finally diagnosed with HE. HE should be considered in patients with PSD on EEG, even if the patients have typical MRI abnormalities of CJD. Anti-thyroid antibodies should be examined in such patients.

A 42-year-old woman presented with rapid myalgia of the thigh and muscle weakness in the proximal limbs with markedly elevated serum CK. Despite positive for antibodies to anti-nuclear, anti-ds-DNA, anti-ss-DNA, anti-Sm, anti-SS-A/Ro, C-ANCA, anti-U1-RNP and anti-ribosome and slight lymphocytopenia and hypocomplementemia, there was no symptom associated with systemic lupus erythematosus (SLE). Proteinuria and hematuria were initially considered to be associated with renal damage due to myoglobinuria. Muscle MRI demonstrated high signal intensities in the rectus femoris. Muscle biopsy of the rectus femoris demonstrated a mild variation in fiber size, a few necrotic and several regenerating fibers and minimal lymphocytic infiltration in the endomysium, which suggested myopathic changes with mild necrotic and regenerating processes. Thus she was diagnosed as idiopathic myositis at first, and was treated by corticosteroid therapy. Her myalgia and CK level improved, but the proteinuria and hematuria were persistent. A renal biopsy demonstrated lupus nephritis, and SLE with myositis was confirmed. She was treated with additional tacrolimus administration, and her proteinuria and hematuria also improved. The present case suggests that patients who predominantly present with myositis accompanied by nephritis and autoantibodies should be considered as SLE with myositis.

Over the last few years, various autoantibodies against cell surface or intracellular antigens were identified in association with several forms of encephalitis, i.e. autoimmune encephalitis. Immunoprecipitaion and sequence analysis of the target protein (proteomics) provided the identification of the antigens corresponding to autoantibodies in autoimmune encephalitis. Appropriate preparation of antigens (synthesized peptides, or recombinant proteins prepared in E.coli or cultured mammalian cells) and assay systems (immunoblot, ELISA, immunoprecipitation or cell-based assay) should be selected for detection of each autoantibodies.Limbic encephalitis characterized by psychosis, dementia, seizures and abnormal movements is a common form of autoimmune encephalitis. Autoantibodies against the NMDA receptor, the AMPA receptor or the VGKC complex (LGI1 and Caspr2) were identified in limbic encephalitis with or without tumor association. Especially, limbic encephalitis associated with anti-NMDA receptor and ovarian teratoma became a distinct neurologic disorder to date.The intracellular antigens are also involved in several forms of encephalitis. Cerebellar ataxia is a common form of autoimmune encephalitis (cerebellits). The autoimmune cerebellar ataxia consists of paraneoplastic ataxia (anti-Yo etc.), anti-GAD-autoantibodies associated ataxia, gluten ataxia (anti-gliadin) and ataxic form of Hashimoto's encephalitis (anti-NAE).The early and accurate diagnosis of autoimmune encephalitis is important because most patients show responses to immunotherapy.

We have created an IT network with a chat feature and have provided at-home medical care to one ALS patient through hospital-home cooperation. The IT network was operated by staff involved in hospital and at-home medical care, who recorded the details of the medical care they provided in the chat server installed at the University of Fukui Hospital via cellular phones or personal computers. During the 51-day operating period of the network, information was entered 118 times; all staff could browse this information. Hospital staff supported home medical care staff by sending replies to the questions of home staff. This experience suggested that the use of the IT network could increase the level of contribution by neurology specialists in home medical care.

This study is based on clinical information on 894 subjects diagnosed with influenza (H1N1) 2009 in Nara Prefecture from June 15, 2009, to March 4, 2010, and from July 9, 2010, to March 6, 2011. Clinical data for 2009-2010 and 2010-2011 was compared. Results showed that 43% of 2009-2010 subjects were 0-9 years old and 38% were 10-19 years old. They also showed that 25% of 2010-2011 0-9 years old, 20% 10-19 years old, 20% 20-29 years old and 16% 30-39 years old. Both seasons showed a high percentage of subjects 0-9 years old. Numbers of subjects aged 20-39 years old increased in 2010-2011. Results thus suggest that an age shift occurred in subjects, in Nara Prefecture. The most frequent symptom was fever, e.g., 38 degrees C, in 88%. Upper airway inflammation was seen in 68%, lower airway inflammation in 20% and gastroenteritis in 6%. Lower airway inflammation decreased from 20% in 2009-2010 to 7% in 2010-2011. Neuraminidase inhibitor was prescribed for 408 (46%) subjects, oseltamivir for 262 (63%), zanamivir for 120 (29%), peramivir for 10 (2.4%), and laninamivir for 12 (2.9%). Two neuraminidase inhibitors were prescribed for 11 subjects. Oseltamivir prescription rates were lower among subjects 10-19 years old, following guidelines for the use of antiinfluenza drugs.

With the increase of lifestyle-related diseases, metabolic syndrome has clearly increased in recent years. Fatty liver, which is the manifestation of metabolic syndrome in the liver, has received little attention because it is not the actual cause of death. However, with the developing increase of metabolic syndrome, non-alcoholic fatty liver disease(NAFLD), especially nonalcoholic steatohepatits(NASH), has increased, and has received much attention. In chronic liver disease, liver cirrhosis develops, finally leading to liver failure. The most serious chronic liver disease is progression to liver cancer. Once hepatic fibrosis develops, the hepatic carcinogenic rate has been increased. Hepatic carcinogenic rate has been reported to reach 8%per year in hepatitis type C-related liver cirrhosis. Although little has been reported about NASH-related liver cancer, NASH-related hepatic carcinogenic rate reached to about 2. 6%per year. In this review, we will describe the epidemiology, gender differences, risk factors and pathogenesis of NASH-related liver cancer.

A 59-year-old man with a long history of under-treated diabetes mellitus presented with severe inflammation that had spread from the sinus to the left orbital cavity. The bilateral internal carotid arteries were severely stenotic, causing multiple infarctions in the brain parenchyma. There was no β-D-glucan detected in the cerebrospinal fluid. Based on the presence of central nervous system (CNS) inflammation and vascular involvements that spread from the sinusitis, we tentatively diagnosed this patient as having invasive fungal CNS infection, i.e. zygomycosis or aspergillosis. Although the patient was treated with anti-fungal drugs such as liposomal amphotericin B and voriconazole, he died of respiratory failure. Pathological examination of the autopsied tissues demonstrated zygomycosis in the brain and heart. The prevalence of zygomycosis is generally very low (-5% of CNS infections) compared with that of other fungal infections. The lack of an appropriate diagnostic marker may lead to the under- or mis-diagnosis of zygomycosis. Moreover, it is hard to differentiate zygomycosis from aspergillosis because the two diseases share common clinical features such as the association of sinusitis and vascular involvement. The clinically diagnostic points that discriminate zygomycosis from aspergillosis are as followed; i) β-D-glucan is negative in zygomycosis but positive in aspergillosis; ii) diabetes is more frequent in patients with zygomycosis to those with aspergillosis; iii) the infectious lesion in aspergillosis shows an iso-low-intensity on T(2) weighted MRI image but shows a high intensity lesion in zygomycosis. The mortality rate of CNS zygomycosis is so high that an early diagnosis of it is warranted and the start appropriate anti-fungal treatments or surgical drainage in the early stage of the disease.

Hashimoto's encephalopathy (HE) presents with a variety of neurologic and neuropsychiatric features. Sixteen percentages of HE patients show cerebellar ataxia as a main neurological feature. The clinical features, treatments, laboratory features, brain images, and serum anti-NH(2)-terminal of α-enolase autoantibodies (anti-NAE Abs), which is a useful diagnostic marker of HE, were investigated in 8 patients. They presented with pure cerebellar ataxia and fulfilled the diagnostic criteria for HE based on the presence of anti-thyroid antibodies and responsiveness to immunotherapy, and were compared with clinical features in other autoimmune cerebellar ataxia associated with anti-GAD, anti-gliadin or anti-Yo. All autoimmune cerebellar ataxic patients presented with truncal ataxia, while nystagmus was absent in HE patients. Most of ataxic form of HE patients had insidious onset mimicking spinocerebellar degeneration, but brain magnetic resonance imaging showed no or mild atrophy of the cerebellum in all patients. Ataxic form of HE patients demonstrated an absence of nystagmus and tended to show a better response to immunotherapy. When a pure cerebellar ataxic patient who presents with truncal ataxia without nystagmus and cerebellar atrophy on brain MRI, HE should be considered as a differential diagnosis and anti-NAE Abs should be examined for the screening of this disease.

Hashimoto's encephalopathy (HE), which is occasionally associated with chronic thyroiditis (Hashimoto's disease), presents with a variety of neurologic and neuropsychiatric features. We investigated the clinical features and treatments in 80 cases of HE with serum anti-NH(2)-terminal of α-enolase [anti-NAE] autoantibodies, a useful diagnostic marker of this disease. The acute encephalopathy form was the most common clinical phenotype (58%), followed by chronic psychiatric form (17%), cerebellar ataxic form (16%), and other forms such as Creutzfeltd-Jakob disease (CJD)-like form and limbic encephalitis. The common neuropsychiatric features were consciousness disturbance (66%), psychosis (53%) and dementia (38%). Involuntary movements (31%, tremor or myoclonus), seizures (31%) or ataxia (28%) occasionally occurred. Abnormalities on EEG (80%) were common while abnormalities on brain MRI were relatively rare (36%). On the treatments for HE, steroid therapy was most usually applied and successful. Intravenous immunoglobulin therapy or plasma exchange was successfully applied for the treatment of HE in some cases of HE. Taken together, we should be aware of the possibility of HE for early diagnosis and treatment of this disease.

Patients with T4 esophageal cancer generally have poor prognosis. Of these patients, prognosis of non-responder to chemoradiothrapy (CRT) is extremely poor. We report a case of residual lymph node metastasis following definitive CRT, which showed a good response to outpatient clinic-based chemotherapy consisting of docetaxel (DOC) followed by S-1 in a patient with T4 esophageal cancer. The patient was a 65-year-old man with the diagnosis of squamous cell carcinoma of the middle thoracic esophagus with the 5 cm size lymph node metastasis with tracheal invasion along right recurrent laryngeal nerve [T4 (106recR-rt subclavian A) N2M0, Stage IVa]. He underwent induction chemotherapy with two courses of FP followed by one course of DCF. As a result, primary tumor was judged as complete response on endoscopy, and the lymph node lesion was judged as partial response, but unresectable on CT. Then, he underwent definitive CRT (FP+60 Gy). Following CRT, although the lymph node lesion was judged as non-CR on CT, a significant decrease of FDG uptake (PET-CR) was observed on PET-CT. Five months later, a recurrence of the lymph node lesion was observed on PET-CT. Then, he underwent outpatient clinic-based chemotherapy with DOC( 60 mg/m², triweekly) followed by S-1( 80 mg/ body/day, 6 weeks/course, administration for 4 weeks with 2 weeks cessation). DOC was administered for 8 months, and was converted to S-1 because of the regrowth of the lesion on PET-CT. After 3 months following initiation of S-1, a remarkable decrease of the lesion was observed on PET-CT. During outpatient clinic observation, the residual lymph node lesion after definitive CRT was well controlled over 1 year, and no new metastatic lesions were observed at other sites. Sequential chemotherapy with DOC followed by S-1 may be effective in controlling progression of resistant tumor against prior CRT.

Despite recent advances in uncovering the neural signature of memory processing in humans, spatiotemporal characteristics of cerebral activity during mental transformation of the visual (color) memory to the visuo-spatial memory has not been studied. The aim of this study was to investigate the electrophysiological correlates of internal memory transformation based on the event-related potential analysis using a root mean square (RMS) and a standardized low resolution electromagnetic tomography (sLORETA). Eleven healthy human subjects completed a modified delayed matching-to-sample test in which the visual(color) short-term memory had to be transformed into the visuo-spatial memory according to the matching probe. In comparison with a non-memory control condition, identifiable RMS peak was observed 352ms after the matching probe presentation in the condition of memory transformation. In this time, sLORETA demonstrated the higher current density in the prefrontal region. Our results suggested that the prefrontal region is associated with the internal memory transformation process depicting a spatiotemporal profile similar to P3b.

Serum CRP has been shown to be associated with the progression of esophageal cancer. The purpose of this study was to examine the relationship between treatment response and serum CRP levels in time course during definitive chemoradiotherapy (CRT) in terms of early prediction of CRT response by serum CRP. The subjects of this study were 36 patients with cT3/cT4 esophageal squamous cell carcinoma who underwent definitive CRT in our hospital. Serum CRP levels during definitive CRT (pretreatment, 1W, 2W and 3W after CRT initiation) were compared between CR and non-CR group. In addition, partition model was constructed to discriminate CR with non-CR and the prediction accuracy was evaluated. The patients were consisted of 28 males and 8 females. At pretreatment diagnosis, tumors were categorized as T3 (n=21) and T4 (n=15). Thirty four patients received FP-based chemotherapy and 2 patients received docetaxel-based chemotherapy. Treatment responses were categorized as CR (n=8), PR (n=14), NC (n=2) and PD (n=12). Serum CRP levels at the time of 2W after CRT initiation (CRT2W) in CR group were low compared to those in non-CR group (p=0.071). The partition model was constructed based on CRP levels at CRT2W. The prediction accuracies to discriminate CR from non-CR by CRP≤0.1 were 50%, 82%, and 75% in sensitivity, specificity and accuracy, respectively. Serum CRP is a useful biomarker for an early prediction of CRT response.

We reported a 61-year-old man who had developed acute cerebellar ataxia in the trunk and the lower limbs. His chemical blood analysis showed very mild hypothyroidism and the presence of serum anti-thyroid peroxidase (TPO) antibody and anti-NH2 terminal of alpha-enolase (NAE) antibody. While cerebellar atrophy was not evident on magnetic resonance imaging (MRI) of the brain, 99mTc-ECD SPECT using the easy Z-score imaging system (eZIS) showed decreased regional cerebral blood flow (rCBF) in the vermis of cerebellum. His cerebellar ataxia improved spontaneously within three weeks. The present case is very rare and suggests that anti-NAE autoantibody may be associated with actue cerebellar ataxia.

The patient was a 57-year-old male, who developed bloody stool around May 2006. He was examined by another physician in the department of gastroenterology in our hospital. Gastrointestinal (GI) endoscopy showed a duodenal ulcer, and the biopsy specimen revealed angiitis of the duodenum. He was admitted to our hospital on June 2006. Serum level of creatinine (Cr) was rapidly increased with hematuria and proteinuria. The titer of MPO-ANCA was elevated, and he was diagnosed with microscopic polyangiitis. Steroid pulse therapy was initiated, followed by the administration of prednisolone (PSL : 1 mg/kg/day) and intravenous cyclophosphamide (IVCY). Serum Cr was gradually decreased, but bloody stool was observed from the 10th hospital day. GI endoscopy showed bleeding from the duodenal ulcer. Steroid pulse therapy was performed, and the dose of PSL was increased to 1.5 mg/kg. Endoscopic hemostatic therapy was repeatedly performed without clinical improvement. Pancreatoduodenectomy was performed on the 15th hospital day. However, bleeding from the small intestine was observed repeatedly and the computed tomography of the chest showed cavity-forming nodules, which were diagnosed with angiitis by the biopsy specimen. The additional treatments of steroid pulse therapy, intravenous immunoglobulin therapy, IVCY and Rituximab did not result in favorable response. We report a refractory case of ANCA-related angiitis presented with gastrointestinal ulcer, rapidly progressive nephritis and multiple lung nodules.

We report 2 patients showing invasion of aspergillosis into the central nerve system (CNS). Patient 1, an 81-year-old woman, underwent surgery for sphenoidal sinusitis. She developed cerebral infarction with unconsciousness on 12th postoperative day. CSF examination demonstrated pleocytosis with increased protein and aspergillus antigen. She was diagnosed as having invasion of aspergillosis into the CNS, and was treated with voriconazole. Her clinical manifestations and CSF findings markedly improved. However, the effects of voriconazole gradually attenuated and she demonstrated recurrence of the cerebral infarction. After 2 months, she died of systemic aspergillosis and sepsis. Autopsy studies. Severe atherosclerotic changes with calcification were demonstrated in the bilateral carotid and basilar arteries, and many aspergillus were clustered in the vessel walls. Granulomatous inflammatory lesions with aspergillus were also demonstrated in the area surrounding the chiasm. There were no massive infarcts or bleeding in the brain, but multiple small infarcts were present. Patinet 2, a 64-year-old man, showing bilateral visual loss, was receiving treatment with corticosteroids under a diagnosis of optic neuritis. Two weeks later, he developed cerebral infarction. CSF examination showed pleocytosis with increased protein and aspergillus antigen. He was diagnosed as having invasive aspergillosis from the sphenoidal sinusitis into the CNS. He was treated with voriconazole, and unconsciousness and CSF findings improved transiently. However, he developed a recurrence of the brain infarction and pneumonia and finally died 6 months later. Treatment by voriconazole was definitely effective in both patients, but both patients died of recurrent cerebral infarction, possibly due to resistance for voriconazole, or developing multicellular filamentous biofilms. Voriconazole is recommended as the first choice of antifungal agents for aspergillosis. Aspergillus infection is strongly invasive into arterial vessels. It is important to consider the possible occurrence of cerebrovascular disease when treating invasion of aspergillosis into the CNS.

The patient was a 53-year-old woman with an 18-year history of recurrent oral aphtae, genital ulcers and folliculitis-like erupsions without mucocutaneus symptoms. She was admitted to our hospital for headache, and presented with meningeal irritation, dysarthria and right pyramidal signs. Brain MRI showed abnormal intensities extending from the right midbrain to the bilateral corona radiata, accompanied by contrast enhancement. She was diagnosed as having an incomplete form of neuro-Behçet disease (NBD) based on the diagnostic criteria for NBD. However, the HLA-type was defined as B54 and Cw1, which is common and specific in neuro-Sweet disease (NSD). Oral administration of prednisolone was markedly effective for the neurological symptoms and improved radiological findings, suggesting NSD rather than NBD as the clinical diagnosis for this patient Since she presented with clinical features that appeared in both diseases, the definitive diagnosis was clinically difficult. While tapering the dosage of prednisolone, we carefully observed the appearance of skin lesions and erythema nodosum appearing on her right lower leg. Skin biopsy demonstrated the features of erythema nodosum: lobular panniculitis with the accumulation of neutrophils and lymphocytes with necrotic fatty cells in the subcutaneous area, which was compatible with skin lesions in NBD. In our case, pathological findings of the skin lesion were required to differentiate between NBD and NSD, indicating the need for careful follow-up of dermatologic signs appearing in such a case.