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Immune checkpoint inhibitors (ICIs) have proven clinical benefits in various advanced cancers. However, despite their significant therapeutic efficacy, ICIs induce immune-related adverse events. Among these events, autoimmune meningoencephalitis often has severe effects on patients' outcomes, but its specific clinical features are still unclear. Here, we report two cases of ICI-associated meningoencephalitis with elevated interleukin-6 (IL-6) levels in the cerebrospinal fluid (CSF). A 47-year-old woman (Case 1) with renal cell carcinoma developed severe headache after a seventh nivolumab administration. A neurological examination revealed jolt accentuation signs and hyperreflexia in all extremities. CSF analysis revealed a high IL-6 value (6,620 pg/mL) with marked pleocytosis. A 70-year-old woman (Case 2) who received an initial administration of nivolumab plus ipilimumab for renal cell carcinoma developed alterations of consciousness. She presented with impaired consciousness, neck stiffness, and hyperreflexia in all extremities. CSF analysis demonstrated a high IL-6 value (49.3 pg/mL) with mild pleocytosis. Both patients were treated with steroid pulse therapy (methylprednisolone 1,000 mg/day, 3 days), followed by the administration of oral predonisolone. The symptoms and laboratory findings improved in both cases. CSF IL-6 values were proportional to the severity of meningoencephalitis and other clinical parameters. These findings may help elucidate the mechanisms of central nervous system complications that are caused by ICIs.

Myelin oligodendrocyte glycoprotein (MOG) antibodies are associated with relapsing inflammatory demyelinating disease. Pregnancy complicates the disease course, potentially leading to either symptom improvement or worsening. A 28-year-old woman with MOG antibody-associated encephalomyelitis had 2 pregnancies; her disease worsened during both postpartum periods despite continuing prednisolone and levetiracetam. The umbilical cord blood was positive for MOG antibodies following her second pregnancy, but neither baby had MOG antibody-associated disease. This is the first case report of MOG antibody-associated demyelinating disease that worsened postpartum despite continuous medication. Furthermore, we observed the placental transfer of MOG antibodies for the first time.

Laminopathy, caused by mutations in the LMNA gene, include a variety of diseases, such as Emery-Dreifuss muscular dystrophy. A Japanese woman developed progressive muscle weakness, muscle atrophy and joint contractures of upper and lower limbs after the age of two years old. She had restrictive respiratory dysfunction, and developed both supraventricular and ventricular arrhythmias after the fourth decade of life. At 55 years old, she had tracheostomy, required mechanical ventilation and was implanted with the implantable cardioverter defibrillator. The serum level of creatine kinase was within normal range. Electromyography showed polyphasic or large motor unit potentials and reduced interference pattern, while relatively normal recruitment. The exome analysis of disease-related genes revealed a heterozygous pathogenic variant c.1072G>A (p.E358K) in the LMNA gene, which contributed to the diagnosis of laminopathy.

Laminopathy, caused by mutations in the LMNA gene, include a variety of diseases, such as Emery-Dreifuss muscular dystrophy. A Japanese woman developed progressive muscle weakness, muscle atrophy and joint contractures of upper and lower limbs after the age of two years old. She had restrictive respiratory dysfunction, and developed both supraventricular and ventricular arrhythmias after the fourth decade of life. At 55 years old, she had tracheostomy, required mechanical ventilation and was implanted with the implantable cardioverter defibrillator. The serum level of creatine kinase was within normal range. Electromyography showed polyphasic or large motor unit potentials and reduced interference pattern, while relatively normal recruitment. The exome analysis of disease-related genes revealed a heterozygous pathogenic variant c.1072GA (p.E358K) in the LMNA gene, which contributed to the diagnosis of laminopathy.

Highlights • Double cancers of the bile duct are often associated with pancreaticobiliary maljunction. • Double cancers of the bile duct without pancreaticobiliary maljunction are very rare. • Double cancers of the bile duct may have a terribly poor prognosis., Background Double cancers of the biliary tract system are rare. Most of these cancers are synchronous double cancers of the gall bladder and bile duct, associated with pancreaticobiliary maljunction (PBM). Synchronous double cancers of the extrahepatic bile duct without PBM are especially rare, and only 4 cases have been reported. Case presentation A 78-year-old woman was admitted to our hospital for examination of hyperbilirubinemia and liver dysfunction. Contrast-enhanced abdominal computed tomography, Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography revealed 2 stenotic regions in the common bile duct: at its junction with the cystic duct and in the distal bile duct. No findings suggested PBM, such as a markedly long common channel. The diagnosis based on endoscopic brush cytology from both stricture portions was adenocarcinoma. The patient had a pylorus-preserving pancreaticoduodenectomy with regional lymph node resection. Macroscopically, there were 2 stenotic regions at the cystic duct junction and in the distal bile duct. Microscopically, the tumor at the junction of the cystic duct was a well-to-moderately differentiated adenocarcinoma. On the other hand, the tumor of the distal bile duct was a poorly differentiated adenocarcinoma. There was no evidence of communication between these 2 cancers. Conclusion Double cancers of the extrahepatic bile duct without PBM are very rare. Therefore, an accurate diagnosis prior to surgery is necessary. Furthermore, this rare condition seems to be associated with a poor prognosis.

Aim The aim of the present study was to evaluate the relationship between baseline white matter lesions (WML) and changes in regional cerebral blood flow during longitudinal follow up of patients with Alzheimer's disease (AD). Methods A total of 38 patients with AD were included in the study (16 men, 22 women; mean age 77.8 years). All patients were evaluated using the Mini-Mental State Examination and brain perfusion single-photon emission computed tomography at baseline with an approximately 2-year follow up. The patients were divided into two subgroups according to the presence of WML on magnetic resonance imaging. Single-photon emission computed tomography data were analyzed using a voxel-by-voxel group analysis with Statistical Parametric Mapping 8 and region of interest analysis using FineSRT. Changes in Mini-Mental State Examination scores and regional cerebral blood flow were analyzed using the Wilcoxon signed-rank test. Results Mean Mini-Mental State Examination scores in AD patients with WML significantly decreased from 19.4 ± 4.8 to 15.5 ± 6.5 (P = 0.003). Statistical Parametric Mapping 8 and FineSRT analysis showed more severe and widespread regional cerebral blood flow reduction, mainly in the frontal and mesial temporal regions in AD patients with WML compared with those without WML. Conclusion Baseline WML could predict a rapid progression of cognitive and brain functional impairment during longitudinal follow up in AD. Geriatr Gerontol Int 2016; 16: 836–842.

BACKGROUND We attempt to evaluate objectively the regional cerebral blood flow (rCBF) changes during long-term donepezil therapy and the relationship between the clinical response and rCBF change in patients with Alzheimer's disease (AD). PATIENTS AND METHODS Thirty-one patients with mild-to-moderate AD (11 men, 20 female; mean age, 76.2 ± 6.7 years) were treated with donepezil and underwent brain perfusion single-photon emission computed tomography (SPECT) twice with an interval of 24.5 ± 4.2 months. The rCBF was calculated using 3-dimensional stereotaxic region of interest template, a fully automated each region of interest technique. We compared the differences in rCBF between baseline and follow-up SPECT studies. Moreover, all patients were divided into stabilized (n= 14) and nonstabilized subgroups (n= 17) based on Mini-Mental State Examination (MMSE) score changes and the changes in rCBF were compared between two subgroups. RESULTS The mean MMSE score significantly decreased from 20.7 ± 4.6 at baseline to 16.5 ± 6.5 after 2 years. The mean rCBF significantly decreased in the widespread brain regions between the baseline and follow-up SPECT studies. The nonstabilized subgroup showed a significant decrease in rCBF of the parietal and temporal segments compared to the stabilized subgroup. CONCLUSION The progression of cognitive deterioration may be related to rCBF affected by the neuropathologic changes of AD.

Subclinical thyroid disease and even variations in thyroid function within the normal range is associated with cognitive function and a risk of Alzheimer disease (AD). Several studies reported the effect of thyroid hormones on cerebral blood flow. The aim of this study was to objectively evaluate regional cerebral blood flow (rCBF) in association with thyroid hormone levels within the normal range in patients with AD. Serum thyroid-stimulating hormone (TSH), free T3, and free T4 levels were measured in 62 patients with AD (23 men and 39 women; age 56 to 91 y; mean age 77.3 y) and 27 control subjects (9 men and 18 women; age 61 to 93 y; mean age 75.8 y). The 99mTc ethylcysteinate dimer single photon emission computed tomography was performed in all subjects. The rCBF in the region of interest was measured by the noninvasive Patlak plot method and calculated using FineSRT, which is a fully automated region of interest technique. No significant correlation was found between thyroid hormone levels and Mini-Mental State Examination scores or global CBF values. Serum levels of TSH, but not free T3 or free T4, were significantly inversely correlated with rCBF in the middle and inferior temporal regions of right cerebral hemisphere in patients with AD. Control subjects showed no significant correlation between thyroid hormone levels and rCBF. Although these findings of a regional relationship must be considers preliminary, this study proposed the hypothesis that altered TSH levels within the normal range may be related to brain perfusion in right temporal region.

Background and Aim: The purpose of the present study was to investigate the clinical significance of the highly sensitive fucosylated fraction of α-fetoprotein (hs-AFP-L3) in patients with chronic liver disease (CLD) and low serum α-fetoprotein (AFP) concentration. Methods: A total of 241 patients being treated at our institute with CLD and low serum AFP concentration (3–10 ng/mL) were investigated retrospectively. We measured total AFP and the percentage of AFP-L3 using a µTAS Wako i30 device. The possible presence of hepatocellular carcinoma (HCC) was thoroughly investigated by various examinations carried out from 1 month before to 1 month after measurements. In addition, hs-AFP-L3 elevated and non-elevated groups, divided by the cut-off value based on a receiver–operator characteristic (ROC) curve, were followed for possible future development of HCC. Results: hs-AFP-L3 was above the detectable range in 60 patients (24.9%). Among those AFP-L3 positive cases, 20 (33.3%) were found to be HCC prevalent, whereas HCC was found in just 16 patients (8.8%) with undetectable hs-AFP-L3 levels. We determined the cut-off value of hs-AFP-L3%, which shows the proportion of AFP L3 in total AFP, to be 5.75%. During the follow-up period, HCC was newly detected in six patients (22.2%) in the hs-AFP-L3% elevated group and in 10 (5.6%) in the non-elevated group. Analysis using the Kaplan–Meier method showed the HCC-free rate of the hs-AFP-L3% elevated group was significantly lower than that of the non-elevated group (P = 0.0038). Independent predicting variants were female sex (P = 0.0024) and hs-AFP-L3% elevation (P = 0.0036). Conclusion: Our results suggest hs-AFP-L3 level is a useful tumor marker for HCC in patients with CLD and low serum AFP concentration.

超音波診断用造影剤であるSonazoid®は，肝腫瘍性病変の造影剤として2007年以来広く使用されている．超音波の最大の長所であるリアルタイム画像を取得でき，今までにない詳細な血管イメージ及び灌流イメージと後血管イメージを得ることにより各種肝腫瘍性病変の質的診断と存在診断が可能となった．症例は50歳代，男性．腹部膨満感，腹痛を主訴に来院．腹部造影CTで膵尾部に5 cm大の低濃度腫瘍を認め，間接所見及び病理組織所見から最終的に膵尾部癌と診断された．術前の造影CTでは肝転移は認められなかったが，超音波検査では限局性低脂肪化域（focal spared area: FSA）を疑う複数の低エコー域を認めた．そのうち胆嚢近傍S4領域の病変は境界が比較的明瞭であったため，腫瘍性病変との鑑別が必要と考えSonazoid®造影超音波検査を行ったところ，動脈優位相で全体に淡く染影され，門脈優位相ではwashout，後血管相で欠損像を呈した．一方，他の低エコー域は欠損とはならなかった．よって，S4の病変は転移性肝腫瘍，その他はFSAと診断した．Sonazoid®造影超音波検査は，脂肪肝を背景に存在とする腫瘍性病変とFSAとの鑑別に有用と考える．

We report the case of a woman with primary progressive aphasia (PPA) presenting with conduction aphasia. Neurological findings showed bilateral finger tremor and signe de poignet fige in her right hand. Memory, orientation, and activities of daily living were well preserved. Linguistic examination showed severe impairment in repetition, fluent spontaneous speech with phonemic paraphasia, and relatively well preserved comprehension. Limb-kinetic apraxia and parkinsonism were not observed during the course of her illness. T1-weighted magnetic resonance imaging revealed severe atrophy of the left temporal lobe and dilatation of the left Sylvian fissure. Neuropathological findings demonstrated the most severe atrophy in the left superior temporal gyrus and Gallyas–Braak-positive or phosphorylated tau-immunoreactive cytoskeletal structures, which were consistent with corticobasal degeneration (CBD). We speculate that the progressive conduction aphasia of our patient might have been caused by left temporal lobe impairment. We suggest that progressive conduction aphasia may be a feature of CBD presenting with PPA.

The aim of the present study was to evaluate the relationship between baseline white matter lesions (WML) and changes in regional cerebral blood flow during longitudinal follow up of patients with Alzheimer's disease (AD).A total of 38 patients with AD were included in the study (16 men, 22 women; mean age 77.8 years). All patients were evaluated using the Mini-Mental State Examination and brain perfusion single-photon emission computed tomography at baseline with an approximately 2-year follow up. The patients were divided into two subgroups according to the presence of WML on magnetic resonance imaging. Single-photon emission computed tomography data were analyzed using a voxel-by-voxel group analysis with Statistical Parametric Mapping 8 and region of interest analysis using FineSRT. Changes in Mini-Mental State Examination scores and regional cerebral blood flow were analyzed using the Wilcoxon signed-rank test.Mean Mini-Mental State Examination scores in AD patients with WML significantly decreased from 19.4 ± 4.8 to 15.5 ± 6.5 (P = 0.003). Statistical Parametric Mapping 8 and FineSRT analysis showed more severe and widespread regional cerebral blood flow reduction, mainly in the frontal and mesial temporal regions in AD patients with WML compared with those without WML.Baseline WML could predict a rapid progression of cognitive and brain functional impairment during longitudinal follow up in AD. Geriatr Gerontol Int 2016; 16: 836-842.

Here we report a genetically confirmed case of Creutzfeldt-Jakob disease with a prion protein gene codon 180 mutation presenting atypical magnetic resonance imaging findings. The present case exhibited an acute onset and lateralized neurologic signs, and progressive cognitive impairment. No myoclonus or periodic synchronous discharges on electroencephalography were observed. Diffusion-weighted images revealed areas of high signal intensity in the right frontal and temporal cortices at onset that extended to the whole cortex and basal ganglia of the right cerebral hemisphere at 3 months. Although the cerebrospinal fluid (CSF) was initially negative for neuron specific enolase, tau protein, 14–3–3 protein, and abnormal prion protein, the CSF was positive for these brain-derived proteins at 3 months after onset.

Background: This study examined regional cerebral blood flow (rCBF) in Alzheimer's disease (AD) patients with and without subclinical hypothyroidism (SCH). Methods: Eleven AD patients with SCH and 141 AD patients without SCH underwent brain perfusion single photon emission computed tomography (SPECT). The SPECT data were analyzed by statistical parametric mapping (SPM8) and FineSRT. Results: AD patients with SCH showed a significantly decreased rCBF mainly in the temporal lobe and thalamus, whereas those without SCH showed a significantly decreased rCBF in the parietotemporal lobe and cingulate gyrus as well as the frontal lobe. Conclusion: Our findings suggest that SCH may affect cerebral perfusion in regions associated with the memory function.

We aimed to objectively examine the brain perfusion differences between PD, Parkinson variant of multiple system atrophy, and progressive supranuclear palsy. (99m) Tc ethylcysteinate dimer single-photon emission CT (SPECT) was performed in 28 patients with PD, 12 with Parkinson variant of multiple system atrophy, 19 with progressive supranuclear palsy, and 17 age- and sex-matched control subjects. A voxel-by-voxel group analysis, using statistical parametric mapping 8, was performed to detect the differences of regional cerebral blood flow among three diseases and control groups. Regional cerebral blood flow was measured using the noninvasive Patlak plot method and calculated using a fully automated region of interest technique. Progressive supranuclear palsy showed decreased regional cerebral blood flow in the cingulate gyrus and thalamus, whereas Parkinson variant of multiple system atrophy showed decreased regional cerebral blood flow in the cerebellum, compared with other patients and controls. Regional cerebral blood flow in the thalamus could be used to discriminate progressive supranuclear palsy from other diseases and control subjects with high sensitivity. These findings suggest that parkinsonian disorders, such as PD, Parkinson variant of multiple system atrophy, and progressive supranuclear palsy show a distinct SPECT pattern in the frontal cortex, thalamus, and cerebellum. Moreover, the measurements of regional cerebral blood flow in the thalamus and cerebellum may be helpful in screening for the differential diagnosis of parkinsonian syndrome.

The purpose of the present study was to investigate the clinical significance of the highly sensitive fucosylated fraction of α-fetoprotein (hs-AFP-L3) in patients with chronic liver disease (CLD) and low serum α-fetoprotein (AFP) concentration.A total of 241 patients being treated at our institute with CLD and low serum AFP concentration (3-10 ng/mL) were investigated retrospectively. We measured total AFP and the percentage of AFP-L3 using a µTAS Wako i30 device. The possible presence of hepatocellular carcinoma (HCC) was thoroughly investigated by various examinations carried out from 1 month before to 1 month after measurements. In addition, hs-AFP-L3 elevated and non-elevated groups, divided by the cut-off value based on a receiver-operator characteristic (ROC) curve, were followed for possible future development of HCC.hs-AFP-L3 was above the detectable range in 60 patients (24.9%). Among those AFP-L3 positive cases, 20 (33.3%) were found to be HCC prevalent, whereas HCC was found in just 16 patients (8.8%) with undetectable hs-AFP-L3 levels. We determined the cut-off value of hs-AFP-L3%, which shows the proportion of AFP L3 in total AFP, to be 5.75%. During the follow-up period, HCC was newly detected in six patients (22.2%) in the hs-AFP-L3% elevated group and in 10 (5.6%) in the non-elevated group. Analysis using the Kaplan-Meier method showed the HCC-free rate of the hs-AFP-L3% elevated group was significantly lower than that of the non-elevated group (P=0.0038). Independent predicting variants were female sex (P=0.0024) and hs-AFP-L3% elevation (P=0.0036).Our results suggest hs-AFP-L3 level is a useful tumor marker for HCC in patients with CLD and low serum AFP concentration.

Detection of a hepatocellular carcinoma (HCC) in the early stage is critical, as clinical stage influences treatment selection and patient prognosis. Carcinogenetic development of an HCC is a multi-step process, and a differential diagnosis between a dysplastic nodule and a well-differentiated HCC is often difficult. A bright loop appearance is a significant finding that indicates disappearance of fatty deposition in the central area of the nodule during the progression toward HCC, however such a finding is rare in cases of sub-centimeter-sized HCCs. We encountered a case of HCC that developed a bright loop appearance on ultrasound (US) without enlargement approximately 2 years after diagnosis as a dysplastic nodule. Moreover, the hypoechoic area in the center of the nodule showed an HCC pattern in contrast enhanced US with Sonazoid™. Vascularity in the nodule could not be observed on dynamic contrast-enhanced CT or Gd-EOB-DTPA-enhanced MRI. When a change in the intranodular echo pattern is observed in sub-centimeter-sized nodules, examination of intranodular vascularity by contrast-enhanced US is important to evaluate borderline lesions.

BACKGROUND Thyrotropin releasing hormone (TRH) improves cerebellar ataxia and cerebellar perfusion in patients with spinocerebellar degeneration. It is not known whether TRH therapy can improve the cerebellar regional cerebral blood flow (rCBF) or not in patients with cerebellar variant of multiple-system atrophy (MSA-C). PATIENTS AND METHODS Seven patients with MSA-C received TRH intravenously (2 mg/day) for 14 days. Clinical efficacy was assessed using the International Cooperative Ataxia Rating Scale (ICARS) and brain perfusion single photon emission-computed tomography was performed before and after therapy. The rCBF in each region of interest (ROI) was calculated using 3DSRT, a fully automated the ROI technique. RESULTS The ICARS scores slightly improved in 6 of the 7 patients after TRH therapy, but this was not statistically significant. After TRH therapy, the cerebellar rCBF reduced in the 6 of 7 patients and the mean rCBF in cerebellum also significantly decreased (P= 0.029, paired t-test), whereas the rCBF in the precentral segment tend to increase (P= 0.048, paired t-test). CONCLUSION TRH therapy may be less effective on cerebellar ataxia and cerebellar rCBF in MSA-C. The 3DSRT program may be useful for the evaluation of the efficacy of TRH therapy on cerebral blood flow.

Objectives In the present study, we compared the patterns of regional cerebral blood flow (rCBF) between cerebellar variant of multiple system atrophy (MSA-C) and the late-onset cortical cerebellar ataxia (LCCA) using FineSRT. Patients and methods We performed 99mTc ethylcysteinate dimer (ECD) single-photon emission computed tomography in 11 patients with MSA-C (mean age 65.7 years), 7 patients with LCCA (mean age 62.0 years), and 12 age-matched healthy controls (mean age 64.1 years). The rCBF in the region of interest (ROI) was measured by the noninvasive Patlak plot method and calculated using FineSRT, which is a fully automated the ROI technique. Results All patients with MSA-C had various degrees of atrophy in the brainstem and middle cerebellar peduncles and 4 of 11 patients showed a clear ‘hot cross bun’ sign in pontine base on T2-weighted MR images. All patients with LCCA showed the various degrees of atrophy in cerebellum without brainstem. FineSRT revealed the significantly decreased rCBF in the caudate tail, fusiform, lingual, cerebellum, midbrain, and pons in MSA-C group compared with controls. Moreover, MSA-C group showed significantly decreased rCBF in the pons compared with LCCA group. Conclusion We suggest that a widespread brain involvement is present in patients with MSA-C and the decreased rCBF in the pons may support the differential diagnosis between MSA-C and LCCA.

Thyrotropin releasing hormone (TRH) therapy improves cerebellar ataxia in patients with spinocerebellar degeneration (SCD). We investigated the effect of TRH on regional cerebral blood flow (rCBF) using the fully automated region of interest (ROI) technique, 3DSRT. Ten patients with SCD received TRH intravenously (2 mg/day) for 14 days and underwent brain perfusion single photon emission computed tomography before and after therapy. Clinical efficacy was assessed using the International Cooperative Ataxia Rating Scale (ICARS). The rCBF in each ROI was measured using the noninvasive Patlak plot method and calculated using 3DSRT. TRH significantly improved the ICARS scores and increased rCBF in the callosomarginal segment and cerebellum. Cerebellar rCBF increased in 4 of 5 patients with improved ICARS scores and in 3 of 5 patients without improved ICARS scores after TRH therapy. The correlation between the change in cerebellar rCBF and the improved ICARS score, however, was not significant. These findings indicate that TRH therapy may increase cerebellar rCBF in some patients with cerebellar forms of SCD and that 3DSRT may be useful for evaluating the efficacy of TRH for increasing CBF. The beneficial effects of TRH may be due to increased cerebellar rCBF or the increased rCBF may be a secondary effect of TRH therapy.

A 76-year-old woman developed weakness and sensory loss in the lower limbs and urinary disturbance in four days. She had a history of operation for the ascending colon cancer and lung metastasis one year ago. Neurological examination revealed flaccid paraplegia, absent Achilles tendon reflex, severe disturbance of superficial and deep sensation below the L3 level, and vesicorectal abnormality. Magnetic resonance imaging (MRI) studies showed an intramedullary T1-iso, T2-low lesion with Gd-DTPA contrast enhancement in conus medullaris at LI level. The laboratory examination revealed the elevated level of serum FDP. D-dimer and TAT. She was diagnosed as hematomyelia, which may be caused by the activation of coagulation and fibrinolysis system. We suggested that the ascending colon cancer and lung metastasis may contribute to the coagulation-fibrinolysis abnormality.

Here we report two cases of pathologically confirmed tumor-like demyelinating lesions. In comparison with common primary demyelinating diseases, our cases demonstrated atypical radiologic features, such as a large monofocal lesion with mild brain edema, and open ring-like or focal enhancement on magnetic resonance images, suggesting brain tumors. The clinical manifestations included focal neurologic signs due to the lesions, monophasic episodes without relapse over a long follow-up period, and efficacy of oral corticosteroid therapy. Histological analysis of brain biopsy specimens showed the inflammatory demyelination and preserved axons without tumor cells. The present cases suggest the importance of considering inflammatory demyelinating disease in the different diagnosis of monofocal tumor-like lesion.

We investigated retrospectively 157 neurological patients who underwent percutaneous endoscopic gastrostomy (PEG) since 2003 May to 2006 January, focusing on their neurological diagnosis, complication, relation to ventilatory support and survival prognosis. Among 157 patients, 42 patients had amyotrophic lateral sclerosis, 39 Parkinson's disease, and 27 multiple system atrophy. The percentage for the neurodegenerative diseases was 68.0%. The most frequent complication during PEG procedure was transient hypoxia (8 patients), three of whom needed oxygen therapy, but no patients received emergent artificial ventilation. After PEG. six patients experienced accidental self-removal of the catheter, but there was no subsequent peritonitis. Seven patients were on tracheostomy positive-pressure ventilation during PEG. and two on non-invasive intermittent positive-pressure ventilation around the PEG period, in all of whom the PEG was carried out without any trouble. Investigation on survival after PEG showed that nine ALS patients died within six months because of respiratory failure. We conclude that PEG for neurological patients was generally performed safely. For ALS patients, however, respiratory function should be carefully monitored, and PEG should be performed in ALS patients before their respiratory function becomes worse.

Eighteen hybrid total hip arthroplasties were implanted in fifteen patients with rheumatoid arthritis and followed for a mean of 2.2 years. JOA score at the final follow-up evaluation was 72.2 points. Radiographic evaluation showed that 33% of the acetabular component had some radiolucent lines. There was migration of one cup and the remaining acetabular components were stable. The femoral components had no radiolucent lines and were stable. At this short-term follow-up period, hybrid total hip arthroplasties is a promising technique that is likely to be successful in patients with rheumatoid arthritis.

544 Background: Neurotoxicity is the most frequent dose-limiting toxicity of oxaliplatin. Cumulative sensory peripheral neurotoxicity results in discontinuation of chemotherapy for cancer and impairs neurological function irreversibly. To develop new strategies to continue chemotherapy according to the treatment plan without more severe neurotoxicity, objective assessment tools for oxaliplatin-induced peripheral neuropathy (OIPN) are needed. The clanging tuning fork test (CTFT) and the nerve conduction velocity test (NCVT) are widely used forbenign neuropathies. However, little is known about their clinical usefulness for OIPN. Methods: Colorectal cancer patients were prospectively monitored at baseline and during chemotherapy with modified FOLFOX6. The incidence and severity of OIPN were recorded using NCI-CTC grading (ver. 4.0). CTFT was conducted every 2 weeks and NCVT every 8 weeks. A generalized estimating equation model was used to examine the correlations between NCI-CTC grade and CTFT/NCVT. The accuracy of the assessment was evaluated using receiver operating characteristic (ROC) curve analysis. Results: Twenty-one patients without peripheral sensory neuropathy (PSN) were enrolled. Mean CTFT values were 13.4 ± 0.7 sec at baseline, 14.1 ± 0.4 sec with grade 0 PSN, 14.0 ± 0.4 sec with grade 1 PSN, 12.5 ± 0.7 sec with grade 2 PSN, and 7.5 ± 0.9 sec with grade 3 PSN. The mean CTFT value with grade 3 PSN was significantly shorter than with the other grades (p

ated with both adverse liver and cardiovascular outcomes. The highest quartile for the Goteborg University Cirrhosis Index did not predict adverse liver or cardiovascular outcomes. Of the 2437 patients, 273 (11.2%) had CT findings of greater than 30% steatosis, fewer than in other populations studied with different imaging methods. Review of 42 biopsy specimens showed strong correlation between histologic scoring of steatosis and the CT results. Patients with steatosis by CT were, on average, 7 years younger (58.5 vs 65.6, p

A S L D A b st ra ct s Method: Retrospective collection of safety and efficacy data in HCC patients older than 65 years compared with a matched group of patients younger than 65. Result: Study group (group 1) included 31 (18 males) patients >65 yrs who underwent a total of 62 procedures and control group (group 2) included 33 (27 males) patients

Purpose:Ultrasonography(US) has excellent spatial resolution, uses neither X-rays nor magnetic fields, is a portable machine that can be conveniently used at the bedside, and displays lesions in real time. The latter feature is convenient for curative percutaneous procedures such as radiofrequency ablation and percutaneous ethanol injection. However, US is not sensitive for the detection of small nodules in patients with advanced liver disease, and the ability to detect nodules varies depending on the experience and technical skill of the examiner. Real time virtual sonography(RVS) is the first system capable of demonstrating virtual multiplanar reconstructed images corresponding to ultrasonographic images in real time. The purpose of this study was to prospectively evaluate the diagnostic efficacy of RVS for detection of liver malignancy often overlooked by conventional US. Materials and Methods:Sixty-six patients with a presumptive diagnosis of hepatocellular carcinoma were assessed between January 2005 and December 2006 at Shimane University Hospital. All of the patients underwent CT angiography, either during arterial portography or hepatic arteriography. Patients with diffuse invasive hepatoma, 6 or more nodules, and nodules for which US-guided percutaneous therapy (>3 cm lesions) was not indicated were excluded. A final total of 59 patients with 140 nodules were included. After CT angiography, conventional US and RVS were performed. The number of nodules detected by these two methods as well as their characteristics (nodule diameter, location and echo pattern of the nodule, and the echo pattern of the liver parenchyma) were compared to determine which method was more sensitive and which characteristics had a stronger influence on sensitivity. Results:From a total of 140 nodules detected by CT angiography, 71 (50.7%) were detected by both conventional US and RVS, and 46 (32.9%) were detected by RVS but not by conventional US. Twenty-three nodules (16.4%) were not detected by either conventional US or RVS. Univariate analysis demonstrated that nodule size, location, and echo pattern of the nodule and the liver parenchyma, influenced the difference in detection ability between the two methods. Multivariate analysis suggested that the most important factor affecting the difference in detection ability was nodule size. Conclusion:The RVS system is useful for detection of non-hyperechoic nodules with 10 mm or smaller diameter located in the periphery of the liver.

AIM: To examine whether a dose-up to 900 mg of ursodeoxycholic acid (UDCA) decreases transaminases in hepatitis C patients. METHODS: From January to December 2007, patients with chronic hepatitis C or compensated liver cirrhosis with hepatitis C virus (HCV) (43-80 years old) showing positive serum HCV-RNA who had already taken 600 mg/d of UDCA were recruited into this study. Blood parameters were examined at 4, 8 and 24 wk after increasing the dose of oral UDCA from 600 to 900 mg/d. RESULTS: Serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), and gamma-glutamyl transpeptidase (GGT) levels were significantly decreased following the administration of 900 mg/d as compared to 600 mg/d. The decrease in ALT from immediately before the dose-up of UDCA to 8 wk after the dose-up was 14.3 IU/L, while that for AST was 10.5 IU/L and for GGT was 9.8 IU/L. Platelet count tended to increase after the dose-up of UDCA, although it did not show a statistically significant level (P = 0.05). Minor adverse events were observed in 3 cases, although no drop-outs from the study occurred. CONCLUSION: Oral administration of 900 mg/d of UDCA was more effective than 600 mg/d for reducing ALT, AST, and GGT levels in patients with HCV-related chronic liver disease.

We report herein an autopsy case of portal-systemic encephalopathy (PSE) presenting with diffuse tissue rarefaction in the cerebral deep white matter. Clinically, the patient showed recurrent episodes of unconsciousness, abnormal behavior and urinary incontinence, as well as flapping tremor. Cognitive impairment and peripheral neuropathy developed following recurrent episodes. Although conventional arterial portography revealed a small portal-systemic collateral vessel of a left gastro-renal venous shunt, abdominal CT and liver biopsy showed no evidence of liver cirrhosis and serum ammonia level showed a mild increase. T2-weighted MRI demonstrated symmetrical signal hyperintensities in the deep white matter. Neuropathological findings showed Alzheimer type II astrocytes in the deep layers of the cerebral cortices and severe tissue rarefaction with no or slight reactive astrocytosis in the subcortical and deep white matter. These white matter changes have been reported infrequently in patients with PSE. The present case suggests that chronic PSE without liver cirrhosis may develop diffuse white matter lesions.