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Fused-in sarcoma (FUS) is genetically and clinicopathologically linked to frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). We have previously reported that intranuclear interactions of FUS and splicing factor, proline- and glutamine-rich (SFPQ) contribute to neuronal homeostasis. Under normal conditions, FUS forms a high-molecular-weight complex with SFPQ in the nucleus. However, disease-associated mutations in the FUS gene disrupt formation of the complex, resulting in unregulated alternative splicing of tau, a disproportional increase in the 4-repeat (4R)-tau/3-repeat (3R)-tau ratio, and eventual neurodegeneration. Disruption of the FUS-SFPQ interaction leads to an increase in the 4R-tau/3R-tau ratio, which manifests as FTLD-like phenotypes in mice. Here, we examined FUS-SFPQ interactions in 142 autopsied individuals with ALS/FTLD, progressive supranuclear palsy (PSP), cortico-basal degeneration (CBD), Alzheimer's disease (AD), or Pick's disease (PiD). Immunofluorescence imaging showed impaired intranuclear colocalization of FUS and SFPQ in the neurons in the ALS/FTLD, PSP, and CBD cases, but not in the AD and PiD cases. Furthermore, the ratio of 4R-tau/3R-tau was elevated in cases of ALS/FTLD and PSP but was largely unaffected in cases of AD. We concluded that impaired interactions between FUS and SFPQ and the subsequent increase in the 4R-tau/3R-tau ratio constitute a common pathogenesis pathway in FTLD spectrum diseases.

Japan has become a "super-aged" society, in which more than 20% of the population is over the age of 65 years. As the incidence rates of both dementia and epilepsy are high in elderly individuals, the prevalence rates of dementia and epilepsy have both increased in recent years. Dementia and epilepsy are often characterized by ambiguous symptoms, with numerous concomitant symptoms observed in these patients. Moreover, many reports indicate that the forgetfulness observed in dementia patients was actually due to the occurrence of epileptic seizures. Because Japan is currently a super-aged society, it is important to understand the characteristics of and relationships between dementia and epilepsy.

Japan is experiencing an increase in the number of patients with dementia, and the incidence of epilepsy is high among the elderly. A survey of patients with epilepsy who were admitted to our hospital's neurology department showed that new onset epilepsy occurred more frequently in elderly patients. In addition, a greater proportion of elderly patients had dementia as an underlying disease. Although the prevalence of epilepsy in dementia varies, higher brain dysfunctions are known to be related to epilepsy. Because a symptom of epilepsy may be mistaken for dementia, it is important to understand the association between epilepsy and dementia, including their tendencies and characteristics.

Background: Preoperative oral rehydration solution (ORS) prevents dehydration before surgery. Therefore taking enough ORS possibly reduces the hemodynamic changes during induction of anesthesia, and reduces the amount of fluid needed during anesthesia., Methods: Forty patients undergoing elective surgery were randomly assigned to two groups: drinking 1,500 ml ORS 6 to 2 hours before anesthesia (ORS group) and nothing by mouth from 6 hours before anesthesia (Control group). Anesthesia induction was performed using propofol and remifentanil. To evaluate the hemodynamic changes, hemodynamic parameters including heart rate, blood pressure, cardiac index (CI), and stroke volume variation (SVV) were recorded before induction, after propofol administration, and after remifentanil administration. Total urine volume and the amount of fluid were also recorded at the end of the anesthesia., Results: In ORS group, CI showed a significantly higher value after propofol administration (P = 0.046). SVV was significantly lower (P < 0.0001) and total amount of fluid during anesthesia was significantly reduced (P < 0.0001) in ORS group., Conclusions: Preoperative oral rehydration increases circulating blood volume, it keeps high CI during induction of anesthesia, and reduces the amount of intraoperative fluid.

Background: Preoperative oral rehydration solution (ORS) prevents hypovolemia. The aim of this study was to compare the effect of intubating dose of rocuronium in patients taking and those not taking preoperative ORS., Methods: Twenty patients, ASA I aged 20-50 years scheduled for elective surgery, were investigated and randomly assigned to two groups: drinking 1,500 ml ORS 6 to 2 hours before anesthesia (ORS group) and nothing by mouth from 6 hours before anesthesia (control group). Anesthesia was maintained with propofol and remifentanil, and rocuronium 0.6 mg x kg(-1) was administrated. To evaluate the effect of rocuronium, acceleromyography at the adductor pollicis was performed using 0.1 Hz stimulation. Cardiac index (CI) and stroke volume variation (SVV) from FloTra/Vigileo, times to 95% twitch depression as onset time (OT), and times to first twitch re-detection (TR) were recorded., Results: SVV was significantly lower in ORS group (P = 0.03), and CI showed no difference. In ORS group, TR was significantly shorter than that of control group (P=0.002), and OT tended to be prolonged (99.0 ± 36.3 s vs. 84.0 ± 37.5 s), but not significantly., Conclusions: Preoperative oral rehydration possibly increases circulating blood volume, and shortens the duration of rocuronium effect.

Japan is experiencing an increase in the number of dementia patients, and the incidence of epilepsy is high among the elderly. A survey, from 2007 to 2012, of 279 epilepsy patients admitted to our hospital's neurology department showed that newly-onset epilepsy occurred more frequently in elderly patients. Of 153 elderly patients with epilepsy, aged 65 years and older, 31(20.2%) had dementia as an underlying disease. Although the prevalence of epilepsy in dementia is varied, higher brain dysfunctions are known to occur as a result of epilepsy. Considering such symptoms as epilepsy with higher brain dysfunction (E-HBD) would enable clinical investigation without overlooking higher brain dysfunctions that have effective treatments.

Erythromelalgia is a rare condition characterized by constant or paroxysmal burning pain, erythema, and the elevation of skin temperature in the extremities. Recently, the impairment of C-fiber function due to autoimmune system involvement is considered as the primary cause of erythromelalgia. However, a successful treatment has yet not been established. We report a case of a 39-year-old woman with primary erythromelalgia accompanied by high cerebrospinal fluid protein concentration and axonal neuropathy. She received various antiepileptic and anti-inflammatory drugs, but failed to improve. She finally underwent high-dose intravenous immunoglobulin therapy, which dramatically improved her symptoms and normalized cerebrospinal fluid protein concentration. This result demonstrates the effectiveness of high-dose intravenous immunoglobulin therapy for the treatment of primary erythromelalgia and the possibility of autoimmune system involvement.

Acquired higher brain dysfunction is for the most part due to cerebral vascular disease, but epilepsy may also be a cause. In this study with five patients, we discuss the advantages of anti-epileptic drugs (AEDs) for persistent higher brain dysfunction. The patients showed chronic amnesia or acute aphasia, with associated symptoms like personality change. All five cases affected automatism or convulsive attack, though only after the emergence of higher brain dysfunction and administration of AEDs. There were underlying diseases like cerebral arteriovenous malformation in four cases, but the other patient had none. Electroencephalogram and single photon emission computed tomography revealed one case of aphasia epilepsy with higher brain dysfunction. These results suggest the potential therapeutic efficacy of AEDs for persistent higher brain dysfunction, and we must differentiate epilepsy with higher brain dysfunction from dementia or cerebral vascular disease.

A-63-year-old man was admitted to our hospital because of epigastralgia. On gastrointestinal fiberscopy, a complete obstruction of the pylorus was found, and endoscopic biopsy specimens from this region revealed gastric small cell carcinoma. CT scans showed the primary tumor at the pylorus with many regional lymph node metastases invading the pancreas. We performed gastrojejunostomy, followed by chemotherapy with irinotecan plus cisplatin (CDDP 30mg/m² and CPT-11 60mg/ m² on day 1 and 15). Three courses of treatment resulted in a marked reduction of both the primary tumor and regional lymph nodes. Subsequently, the patient underwent distal gastrectomy. Micro examination, revealed that the tumor had mostly changed to necrosis and fibrosis, and the pathological TNM grading was pT3 (SS) pN0M0, stage II A. Postoperatively, three courses of the same chemotherapy were performed. However, the patient died 5 months after the second operation, due to meningitis carcinomatosa without metastases of other organs.

Herein we describe a patient with established corticobasal degeneration with onset of nonfluent aphasia and showing symmetrical cerebral involvement. A 64-year-old man with a speech disorder for 2 years visited our hospital. He had nonfluent aphasia (reduced spontaneous speech, loss of intonation, anomia, repetition disorder, and difficulty in speaking short sentences). He also showed right-sided motor neglect, hypertonus of the left lower limb, a mask-like facial expression, and difficulty in closing his eyes. He was restless and walked around even during examination, suggesting frontotemporal dementia (FTD). Single-photon emission computed tomography (SPECT) revealed symmetrical reduction of cerebral blood flow in the bilateral fronto-temporo-parietal lobes. His neurological condition deteriorated gradually and a year later he could not speak comprehensive sentences. Magnetic resonance imaging (MRI) of the head at age 70 showed symmetrical atrophy of the bilateral fronto-temporal lobes. He died of respiratory failure after clinical problems lasting ten years. On pathological examination, the fixed brain weighed 1,010 g and showed bilateral symmetrical atrophy of the frontal lobes. Histopathological examination revealed neuronal loss and gliosis in the frontal lobes, especially in the frontal convexity, superior frontal gyrus and precentral gyrus. Gallyas-Braak silver staining showed astrocytic plaques, argyrophilic threads and coiled bodies mainly in the frontal lobes. The substantia nigra showed severe neuronal loss on both sides and presence of free melanin. Pathological diagnosis was corticobasal degeneration (CBD). We believe that the patient had nonfluent aphasia and FTD reflected in bilateral degeneration of the frontal lobes. Some cases of CBD may present with symmetrical degeneration of the brain, even though left-hemisphere symptoms such as aphasia reveal themselves at an early stage.

A 48-year-old woman, who had been suffering from systemic lupus erythematosus (SLE), developed normochromic normocytic anemia after receiving clomipramine hydrochloride. Her reticulocyte count was low, and a bone marrow aspirate revealed erythroid hypoplasia without involvement of other cell lines. Thus a diagnosis of pure red cell aplasia (PRCA) was made. The anemia gradually resolved following withdrawal of the drug. Although several drugs are known to cause PRCA, this is the first time that clomipramine hydrochloride has been reported to have such an effect. The underlying SLE in this case suggested the possible immunological pathogenesis of drug-induced PRCA.

We compared the Etest with a broth microdilution method (FP panel), performed according to the National Committee for modified Clinical Laboratory Standards (NCCLS) document M27-P guidelines, for determining the MICs of 81 clinical isolates of yeasts (7 Candida albicans, 8 Candida glabrata, 10 Candida parapsilosis, 6 Pichia anomala, 10 Candida tropicalis, 4 Candida guilliermondii, 4 Candida krusei, 6 Trichosporon cutaneum, 5 Candida ciferrii, 3 Candida famata, 4 Candida norvegensis, 2 Rhodotorula rubra, 3 Candida lusitaniae, 2 Candida curvata, 1 Candida inconspicua, 1 Candida intermedia, 1 Candida colliculosa, 1 Cryptococcus spp, 1 Tricosporon capitatum, 1 Pichia ohmeri, 1 Saccharomyces cerevisiae). The Etest results for 6 ATCC standard strains correlated well with reference MICs except those of flucytosine (5-FC) for C. krusei, which tended to be 1 to 2 log2 dilution higher than the MIC range determined by NCCLS guidelines. However, the best agreement between the results for clinical isolates was seen with 5-FC (100% agreement [Within +/- 2 log2 dilutions] between the results of the two methods). There was a 91.4% agreement between the results of the two methods with amphotericin B (Etest MICs tended to be 1 to 2 log2 dilution lower than those of the FP panel). The Etest results with litraconazole for clinical isolates except C. tropicalis were similar to MICs of the FP panel (Etest for C. tropicalis showed 1 to 2 log2 dilution lower than FP panel). Also, the Etest results with fluconazole for clinical isolates except C. tropicalis were similar of 1 log2 dilution higher than MICs of the FP panel (Etest for C. tropicalis showed more than 2 log2 dilution lower than FP panel). These results showed a good level of overall agreement between the Etest method and the broth microdilution test (FP panel). Since the Etest is a less laborintensive and much simpler method, it appears to be a useful procedure for testing the susceptibility of yeasts to antifungal agents.

We have evaluated changes in bone volume, bone marrow tissue, and the density of osteoclasts caused by intermittent administration of human parathyroid hormone (h-PTH) to experimental osteopenia induced in rat by ovariectomy (OVX) or by diabetes mellitus (use of streptozotocin: STZ). A bone and marrow histomorphometric study was performed on HE-stained and tartrate-resistant acid phosphatase-stained (TRAP-stain) tibial bone sections. Retired Wistar rats, 7-8 months old, were used. They were separated into the following nine groups; sham operated, base line control, vehicle administered, low or high dosage h-PTH administered OVX and STZ groups. 6.0 micrograms/kg/day of h-PTH (1-34) as a low dosage, and 60.0 micrograms/kg/day as a high dosage, was injected subcutaneously six times a week for 4 weeks from 9 weeks after ovariectomy or injection of streptozotocin. The bone volume decreased in both the OVX and STZ groups, while the fat tissue volume increased in the bone marrow in the OVX groups to compensate for this decrease, and the foamy marrow tissue volume increased in the STZ groups. The bone volume and the mean trabecular thickness in both the OVX and STZ groups increased by the intermittent administration of h-PTH, while the TRAP positive trabecular surface and the number of osteoclasts decreased. There was no significantly different bone changes between the low and high dosage groups. It is thought that the TRAP positive trabecular surface represented not only the active bone resorption surface but also the related contiguous uneroded surface.

A twenty-six-year-old male patient with advanced choriocarcinoma originated from mediastinum was treated by high-dose chemotherapy with peripheral blood stem cell (PBSC) transplantation. He had massive tumors in the cervical, mediastinal and bilateral lung fields. After 3 kur of PVeBV therapy (CDDP 60 mg x day 1-3, etoposide 160 mg x day 1-3, BLM 30 mg x day 1, VBL 12 mg x day 1), he obtained complete remission. PBSC was collected at the time of hematopoietic recovery and high-dose chemotherapy with PBSC transplantation was conducted because of the high possibility of recurrence. The hematopoietic recovery was rapid, and the patient remained well with no sign of recurrence for 24 months.

A 63-year-old male with refractory anemia with excess of blasts (RAEB) and hypoplastic marrow was treated with K-18 (240 mg/day P.O.). On admission, peripheral blood revealed pancytopenia. Bone marrow specimen revealed severe hypocellularity with 18.9% of the blast cells. Ten months later, the blast cells in the bone marrow decreased to 3.8%, and complete remission (CR) was obtained. CR was eight weeks. Duration of response (CR + PR) continued for about eight months. K-18 is an antitumor agent with minimal side effects, and seems to be effective for RAEB with hypoplastic marrow.

This article was aimed to propose a new concept on evaluating electromyographic activities of masticatory muscles during chewing movements viewed from the standpoint of motor skill. Correlation coefficients between the ratio of lateral distance to ten vertical level set at 0.5 mm to 5.0 mm with 0.5 mm step from the end of closing phase and activities of bilateral masseter, anterior and posterior temporalis in each chewing stroke were evaluated using raisin, peanut, soft and hard testing gum in five subjects. Habitual chewing side always demonstrated less numbers of subjects who showed high correlation coefficients especially in the case of soft testing gum.

Attempts were made to clarify the mechanism of platelet aggregation and to characterize the platelet aggregating material employing established human cancer cell lines. Eleven out of the nineteen human cancer cell lines investigated showed platelet aggregating activity. The existence of divalent cation was required for the platelet aggregation induced by HMV-1 tumor cells. The platelet aggregations induced by tumor cells (HMV-1, PC-10, 3LL) were not suppressed by specific thrombin inhibitor (MD-805). The platelet aggregating activities of tumor cells (HMV-1, M 7609) were diminished by treatment with trypsin but not with collagenase or neuraminidase. Aggregating activity was preserved with a preparation of membrane from these tumor cells, although it was abolished by heating(100 degrees C 15 min) or sonication. By SDS PAGE (autoradiography), membrane proteins with MW of 20,000 daltons which specifically bound to platelets were commonly found in cells with platelet aggregating activity (HMV-1, M 7609), but were absent in platelet non-aggregating cells (HGC-25). It is therefore concluded that platelet aggregation induced by human tumor cells does not require the coexistence of thrombin, but is evoked by direct interaction of platelets with aggregating proteins (MW 20,000 daltons) on the cell membrane.

This study aims to analyse how jaw-closing muscle activities are modulated during lateral jaw movement. Jaw movements were recorded by Sirognathograph (Siemens Co.) and electromyograms were simultaneously recorded from bilateral masseter, anterior temporal and posterior temporal muscles in 20 young adults without any stomatognathic dysfunction. Each subject sat upright in the chair with the head unsupported. The lateral jaw movements from the intercuspal position were guided three times for each lateral excurtion (right and left). The data were recorded on magnetic tape and later analysed by use of a computer. Following variables were analysed; the number of contacted teeth, the position of contacted teeth, the muscle activities during lateral jaw movement. The results can be summarised as follows; 1) The working-side posterior temporal muscle showed obvious activity in more than 90% subjects during lateral jaw movement. However, this muscle activity was not influence by the number or position of the contacted teeth. 2) The right side masseter muscle tended to show the obvious activity during both right and left lateral jaw movement. The results of this study suggest the difference in the activities between masseter and temporal muscle during lateral jaw movement.

The purpose of this study is to reveal the relationship between the head position, and the neck and head muscles. At 4 head positions, the activities of masseter, anterior temporal, anterior digastric, sternocleidomastoid and trapezius muscle of ten normal subjects standing straight were investigated electromyographically with surface electrodes during voluntary maximal clenching in centric occlusion. Head positions were right tilting, left tilting, up-right and natural head position. The results obtained were as follows; 1. During head tilting, the activities of anterior digastric and sternocleidomastoid muscle on the tilting side were increased, the activities of masseter and trapezius muscle on the opposite side of the tilting side were increased, the activity of the anterior temporal muscle did not vary from the activity during up-right head position. 2. During natural head position, only the activity of sternocleidomastoid muscle on the natural tilting side was increased.

For purpose of evaluating the characteristic of electromyographic activities of masticatory muscles with front or molar tooth loss, electromyographic activities of masticatory muscles and mandibular movements during tooth tapping and gum chewing were investigated in each group of tooth loss, and were compared with those of control group. The results were as follows. 1. In reference to the group with front tooth loss, 1) On tooth tapping, each S.D. of the time parameters of electromyographic activities of jaw closing muscles especially masseter and posterior temporalis was small, and that of jaw opening muscle was large. 2) On gum chewing, offset of jaw closing muscles' activities especially masseter and anterior temporalis were delayed. 3) On both tooth tapping and gum chewing, there was no difference in mandibular movements from control group. 2. In reference to the group with molar tooth loss, 1) On both tooth tapping and gum chewing, each S.D. of the time parameters of electromyographic activities of jaw closing muscles was large. 2) On tooth tapping, S.D. of opening ratio was large. 3) On gum chewing, both S.D. of chewing cycle and opening ratio were large.