Search

Intense negative ion source producing multimegawatt hydrogen/deuterium negative ion beams has been developed for the neutral beam injector (NBI) in TOKAMAK thermonuclear fusion machines. Negative ions are produced in a cesium seeded multi-cusp plasma generator via volume and surface processes, and accelerated with a multistage electrostatic accelerator. The negative ion source for JT-60U has produced 18.5 A/360 keV (6.7 MW) H− and 14.3 A/380 keV (5.4 MW) D− ion beams at average current densities of 11 mA/cm2 (H−) and 8.5 mA/cm2 (D−). A high energy negative ion source has been developed for the next generation TOKAMAK such as the International Thermonuclear Experimental Reactor (ITER). The source has demonstrated to accelerate negative ions up to 1 MeV, the energy required for ITER. Higher negative ion current density of more than 20 mA/cm2 was obtained in the ITER concept sources. It was confirmed that the consumption rate of cesium is small enough to operate the source for a half year in ITER-NBI without maintenance.

Anisotropic resistivities of Bi_2Sr_2Ca_{1-x}Er_xCu_2O_8 single crystals were measured and analyzed from 4.2 to 500 K with special interest in the parent antiferromagnetic insulator of x=1.0. Although the resistivity is semiconducting along both the in- and out-of-plane directions, the temperature dependence is found to be significantly different. As a result, the resistivity ratio for x=1.0 takes a broad maximum near room temperature. The electric conduction in parent antiferromagnetic insulators is different from other semiconductors, and is as unconventional as that in high-temperature superconductors.

Development of the negative-ion sources has been conducted to realize a high power neutral beam injector for International Thermonuclear Experimental Reactor (ITER). A high negative-ion current density of 31 mA/cm2 (H−) at a very low pressure of 0.1 Pa has been produced in a cesium seeded multicusp plasma generator which has the same concept of the ITER source. For a vacuum insulated accelerator, a voltage holding experiment of long distance vacuum gaps up to ∼1.8 m has been performed. It was clarified that the transition region of product pressure distance (pd) from the vacuum breakdown to the gas discharge is about 0.2 Pa m which is high enough from the operating region of the ITER source. A prototype vacuum insulated accelerator was fabricated based on the experiment and tested. A high-energy H− beam acceleration up to 970 keV, 37 mA, and 1 s has been successfully demonstrated.

The influence of a dipole magnetic field in the extractor on the beamlet deflection has been investigated using a JAERI 400 keV H− ion source. The beam deflection angle decreased from 10.2 to 7.0 mrad when the integrated value of the magnetic field was changed from 2530 to 910 G cm. The measured deflection angle was larger than the estimated value from the ion trajectory considering the magnetic field. To understand the dominant factor that enhances the beam deflection angle, three-dimensional trajectory simulation was performed. It was confirmed that the axis of the beamlet deflected by the magnetic field in the extractor is displaced from the center of the aperture at the grounded grid (GRG). This displacement enhances the beam deflection angle due to the effect of the electrostatic lens at the GRG. This phenomenon is similar to the beam deflection by aperture displacement of the GRG.

Metastasis to the uterine cervix from non-gynecologic neoplasms is rare. However, metastatic tumors sometimes precede the diagnosis of a primary tumor, and may lead to diagnosis of the primary tumor.A 50-year-old woman was referred to us complaining of increasing right flank pain. Computed tomography scan demonstrated an enlarged uterus with right-sided hydronephrosis and hydroureter. Cervical cytology revealed adenocarcinoma. She was considered to have a Stage IIIB cervical adenocarcinoma. Although no cervical lesion was seen colposcopically, histopathology from biopsies of the uterine cervix revealed poorly differentiated adenocarcinoma infiltrating around the normal endocervical glands. A metastasis from the gastrointestinal tract was suspected. The patient underwent gastroscopy and was found to have Borrmann type IV gastric cancer. Biopsies confirmed a poorly differentiated adenocarcinoma with signet ring cells.Physicians should bear in mind that metastatic tumors may precede the diagnosis of a primary tumor and could manifest by mimicking advanced cervical cancer.

It is known that an anti-angiogenic compound AGM-1470 inhibits cells in vitro and in vivo, on mitogen-induced cell growth in capillary endothelial cells. In monolayer cultures, 1 ng/ml AGM-1470 completely inhibited both basic fibroblast growth factor (bFGF) induced cell growth and vascular endothelial growth factor (VEGF) induced cell growth in a cytostatic manner. IC50 was 85 pg/ml and 55 pg/ml for bFGF and VEGF induced cell growth, respectively. Moreover, in collagen gels, AGM-1470 suppressed the colony formation induced by bFGF and by VEGF in a dose dependent manner. Few colonies appeared 25 days after co-culuture with I ng/ml AGM-1470 and either 1 ng/ml bFGF or 5 ng/ml VEGF. It is suggested that a potent; inhibition of growth signals of more than one growth factor for endothelial cells might be involved in anti-angiogenic activity of AGM-1470.

AGM-1470 is known to inhibit angiogenesis. It inhibited the mitogen induced cell growth in microvascular endothelial cells in vitro. Ten ng/ml AGM-1470 completely inhibited both basic fibroblast growth factor induced cell growth, and interleukin-4 induced cell growth. In an in vivo study, we invesigated the antitumor effect of AGM-1470 in dimethylbenz[a]anthracene (DMBA) induced rat mammary tumors. A dose dependent growth inhibition was seen between administered doses 10 mg/kg and 40 mg/kg. Forty mg/kg 2-week administration of AGM-1470 totally suppressed the tumor growth. In addition, an additive inhibitory effect of AGM-1470 with an antiestrogen tamoxifen was found (p

To develop a high power negative ion source/accelerator for 1 MeV class neutral beam injector, hydrogen negative ion beam acceleration has been studied using a five-stage, multiaperture electrostatic accelerator. After conditioning each accelerator stage, the negative ions are accelerated to 1 MeV successfully with a drain current of 25 mA for 1 s. Cs was introduced into the ion source to produce higher current density. The highenst acceleration current density of 15 mA/cm2 was successfully accelerated up to an energy of 700 keV for 1 s, keeping the optimum perveance. The total acceleration current of 200 mA was extracted from nine central apertures 14 mm diameter each. A preliminary measurement of the heat load in the accelerator showed that the direct interception of the beam for the first grid and the third grid was negligibly small. The highest heat load was 4.5% of the input power at the second grid.

Installation of the kicker magnets in the extraction section of the rapid cycling synchrotron in the J-PARC facility has been completed. They succeeded in extracting the proton beam accelerated up to 3 GeV in the first commissioning with beam. The operation parameters of the kicker system agreed well with the parameters, which were estimated from the magnetic field measurement and the current test of each power supply. The distribution of the integrated magnetic field is flat enough to meet the beam requirements. The beam based measurement of the flattop of the magnetic field was performed with the beam position monitors. Although a ringing structure was observed, which exceeded the specified limit, the degree of the flattop distortion could be corrected by the timing adjustment of the output current. The stability of the power supply is on the order of � 0:2%, which is sufficient to achieve stable beam extraction. The rise time of the output current largely depends on the gas pressure in the thyratrons. Dependence of the rise time on the gas pressure was strictly measured and the number of unwanted prefires could be reduced.

The 3 GeV Rapid Cycling Synchrotron (RCS) at Japan Proton Accelerator Research Complex is nearly at the operational stage with regard to the beam commissioning aspects. Recently, the design painting injection study has been commenced with the aim of high output beam power at the extraction. In order to observe the phase space footprint of the painting injection, a method was developed utilizing a beam position monitor (BPM) in the so-called single pass mode. The turn-by-turn phase space coordinates of the circulating beam directly measured using a pair of BPMs entirely positioned in drift space, and the calculated transfer matrices from the injection point to the pair of BPMs with several successive turns were used together in order to obtain the phase space footprint of the painting injection. There are two such pairs of BPMs placed in two different locations in the RCS, the results from which both agreed and were quite consistent with what was expected.

The large-bore magnets in the injection system of a high-intensity proton synchrotron are placed close to each other as compared to their bore sizes. In such cases, the magnetic cores interfere with the fringe fields of adjacent magnets. Moreover, the incoming beam passes through several kinds of time-varying fields along the injection line and the nonlinear field region of the ring quadrupole magnet. The beam behavior under these conditions is analyzed by using the Runge-Kutta method. Although the interference of the magnetic fields does not result in emittance growth, it produces a closed-orbit distortion. Field fluctuations of the time-varying field cause an emittance growth and the nonlinear field deforms the beam profile in phase space of the incoming beam. These effects lead to the modification of the injection scheme. This study focuses on the particle tracking with magnetic field interferences during the early stage of beam commissioning, when the space charge force is not important.

A 19-year-old male with pernicious anemia and hypogammaglobulinemia (common variable immunodeficiency: CVID) is reported in comparison with classical pernicious anemia. This case was characterized by an earlier onset of anemia, the absence of autoantibodies to intrinsic factor or gastric parietal cells and involvement of the pyloric antrum as well as the gastric corpus. It is suggested that dysregulation of cellular immunity produces the autoimmune lesion in the gastric mucosa, including the pyloric antrum, in a patient with CVID, and that some of such cases develop pernicious anemia.

Despite the involvement of cerebellar ataxia in a large variety of conditions and its frequent association with other neurological symptoms, the quantification of the specific core of the cerebellar syndrome is possible and useful in Neurology. Recent studies have shown that cerebellar ataxia might be sensitive to various types of pharmacological agents, but the scales used for assessment were all different. With the long-term goal of double-blind controlled trials-multicentric and international-an ad hoc Committee of the World Federation of Neurology has worked to propose a one-hundred-point semi-quantitative International Cooperative Ataxia Rating Scale (ICARS). The scale proposed involves a compartimentalized quantification of postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders, in order that a subscore concerning these symptoms may be separately studied. The weight of each symptomatologic compartment has been carefully designed. The members of the Committee agreed upon precise definitions of the tests, to minimize interobserver variations. The validation of this scale is in progress.

The kicker magnets are installed in the vacuum chamber at the extraction section of the RCS (Rapid Cycling Synchrotron) in J-PARC (Japan Proton Accelerator Research Complex) facility. They kick the 3GeV proton beam to the extraction septum magnets followed by a downstream beam transport line. The RCS is designed to achieve the 1MW beam power with minimum loss. Therefore, the kicker is required to have a wide aperture, UHV (ultra-high vacuum) in its chamber, and uniformity of magnetic field. The wide aperture requires large exciting current and the use environment of the thyratron is very tough. Therefore aging run was found out to be needed. And the uniformity of the magnetic field was improved by changing length of the conductors. In order to achieve the UHV, we succeeded in reducing outgas from components of the magnet. In this paper, we will introduce the specification of the extraction kicker system in the RCS, and report countermeasure against the technical challenge described above.

The construction of J-PARC(Japan proton accelerator research complex) is in a rapid progress, where the first beam commissioning of 3-GeV RCS(rapid cycling synchrotron) is expected to be start on May, 2007. The present work concerns the very final design as well as the calculation of the injection line until the beam dump. This includes an accurate and safe aperture list of all elements in the injection-dump line as well as an accurate estimation of the uncontrolled beam losses so as to optimize them concerning mainly the radiation issues and moreover for the hands-on maintenance.

The pulse bending magnets for the injection system of the 3-GeV RCS in J-PARC [1] [2] has been designed. The injection system consists of the pulsed bending magnets, which are four horizontal bending magnets (shift bump) and four horizontal painting magnets (h-paint bump) for the injection bump orbit, and two vertical painting magnets (v-paint magnet). The injection beam energy and the extraction beam power are 400 MeV and 1 MW at 25 Hz repetition rate, respectively. The acceptance to include the injection beam, the painting beam and the circulating beam at the shift bump points is a 388 mm wide and a 242 mm high. The shift bump has been designed using a 3D magnetic analysis code [3], which accomplished less than 0.4 % field deviation under 0.26 T excitation level.

A processor core, originally designed in a 0.5/spl mu/m Al process, is redesigned for a 0.13/spl mu/m Cu process to create a dual-core processor with 1MB integrated L2 cache, offering an efficient performance to power ratio for compute-dense server applications. Circuit design challenges, including negative bias temperature instability (NBTI), leakage, coupling noise and intra die process variation are discussed.

The J-PARC Project 3-GeV proton synchrotron is designed to accelerate 8.3 /spl times/ 10/sup 13/ protons per pulse at a 25 Hz repetition rate for the injection energy of 400 MeV. The incoming beam emittance of the 400-MeV linac is 4/spl pi/ mm mrad and the acceptance in the 3-GeV synchrotron is 324/spl pi/ mm mrad in both the horizontal and vertical planes. Painting injection system is designed to fit in the FODO structure, which has rather short drift space. The bump orbit for painting injection is designed to have a full acceptance of the circulating orbit through the injection period. A full-acceptance bump orbit will enable both correlated and anti-correlated painting injection.

Although the study of quantum mechanical tunneling has a long history, the importance of tunneling in chemical reactions has been the subject of misunderstanding. Traditionally, the curvature in an Arrhenius plot of reaction rate constants has been explained by tunneling. However, it is generally very difficult to experimentally observe such clear curvature in Arrhenius plots for reactions in the gas phase. On the other hand, a clear curvature and low temperature limits were observed in many condensed phase reactions at low temperature below 100 K. Nevertheless, it has been theoretically pointed out that tunneling still plays a very important role even for reactions in the gas phase, for which the corresponding Arrhenius plots show nearly linear behavior. For example, Schatz (1987, 1988) showed that tunneling contributes more than 75% of the total rate constants for the simplest H + H2 → H2 + H reaction and its isotopic variants even at room temperature, where no significant curvature in Arrhenius plots is seen (cf. Sect. 12.1.1). We should notice that this important conclusion was derived from the accurate three-dimensional quantum reactive scattering calculations on a very accurate potential energy surface. In other words, it is usually difficult to conclude, only from the experimental results, whether tunneling is playing a significant role in the reaction studied. Thus, quantum mechanical theory is very important for understanding the importance of tunneling in chemical reactions in the solid phase.

Human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome (AIDS) have been very important problems all over the world. The progression of HIV infection into AIDS is controlled with anti-HIV drugs, but the treatments with the anti-HIV drugs have some problems. Hence, the research and development of more effective anti-HIV treatments have been performed. As we consider it important to understand the dynamics of HIV infection, we propose a new mathematical model of HIV infection. The model is characterized by the calculations of responses against stimuli; that is, the experimental phenomena (when the values of responses are plotted against the logarithm of the values of stimuli, a sigmoid curve is obtained) are incorporated into the model. By using the calculations of the model, we obtain the simulation results which show a slow increase in the viral load and a slow decrease in non-infected CD4/sup +/ T cells after the acute phase.

Service dogs have received much attention as effective choices for promoting participation in society of disabled persons. The purpose of this study was to obtain the fundamental data to prepare guidelines regarding service dogs use in community life, by making clear the problems in terms of public health and community welfare through a survey of attitudes towards service dogs in community residents.Study 1 was conducted as follows. The subjects were 423 community residents between their teens and seventies who participated in health and welfare lectures in 4 metropolitan areas. A questionnaire survey was conducted just after watching a promotion video about service dogs, including several items on aspects not accept, were anxious about or were unpleasant in the case of community life of a disabled person and a service dog. Study 2 was as follows. A questionnaire survey was conducted inside a supermarket in the central part of Tokyo. One group of subjects were 103 shopping customers who witnessed a shopping scene featuring a disabled person with her service dog, and the other subjects were 84 customers who were not witnessed to the scene. The same survey was simultaneously performed for impression about the shopping scene.Study 1 showed that 31.8% of all the respondents replied that they had some knowledge of service dogs before watching the video. A total of 20.4% (32.5% of the elderly) replied that there were places where service dogs should not enter. Concretely speaking, these included restaurants for 7.3-22.5% and medical institutions for 8.5-12.5% of the respondents. A third of all the respondents (51.3% of older persons) replied they could not endure some actions by service dogs, concretely "carrying food in the mouth" was highly pointed out by 27.3%. Zoonosis (15.3-19.0%), hair scattering (9.5-21.3%), injuries (4.4-7.4%) were also pointed out as main sources of anxiety or uncomfortable feelings. Such negative opinions were especially apparent among those aged 60 years and older. Also, 44.1% knew some disabled person. Cross tabulation showed those who had experience of taking care of a dog were significant less anxious about "vague anxiety or discomfort," and "zoonosis" than those who had not (P0.01). On the other hand, most of both witness (79.6%), and non-witness groups (81.0%) replied that it was heartwarming that the service dog assisted with shopping. None expressed discomfort about the presence of "service dog" inside the supermarket. In terms of carrying a commodity in the mouth, 92.2% and 89.1% in both groups replied that they don't mind, especially if safety was guaranteed for public health, and 91.3% and 95.2% in both groups replied that they had no other problems. Moreover, none of them would not entering stores. Significant differences were seldom in replies between the witness and non-witness groups.Although the respondents were extremely affirmative regarding the image of service dogs, it became clear that anxiety about public health aspects such as "zoonosis" or "utilization of medical institutions" was persisting. On the whole, negative opinions were more apparent in the elderly.

We reported a 68-year-old man with anti-phospholipid antibody syndrome who presented slowly progressive pure motor monoparesis(PMM) in left upper extremity as a sign of cerebral infarction. He had history of hypertension and hyperlipidemia. He first noticed clumsiness in left fingers, then weakness of left fingers with drop hand developing gradually in 2 to 6 weeks. He began to feel difficulty in raising left upper arm in 8 weeks and was admitted to our hospital. On admission, he exhibited severe weakness in distal portion and moderate weakness in proximal portion of left upper extremity. Deep tendon reflexes were slightly hyperactive in left side. Muscle strength of right upper extremity and bilateral lower extremities were normal. There was no sensory and autonomic abnormality. Laboratory examination revealed high titer of anti-cardiolipin IgM antibody. Brain MRI demonstrated a small cortical infarction in the right precentral gyrus. Cerebral angiography revealed severe stenosis in right common carotid artery. Other examinations including EMG were unremarkable. PMM in left upper extremity was considered to be caused by the ischemic lesion in the precentral motor cortex. Slowly progressive course might be explained by the hypovolemic factor due to the marked stenosis in right common carotid artery, poor collateral circulation, and abnormal coagulation caused by anti-phospholipid antibody syndrome.

The out-of-plane dielectric constant of the parent insulator of the high-temperature superconductor Bi_2Sr_2(Dy,Er)Cu_2O_8 was measured and analysed from 80 to 300 K in the frequency range of 10^6-10^9 Hz. All the samples were found to show a fairly large value of 10-60, implying some kind of charge inhomogeneity in the CuO_2 plane. Considering that the superconducting sample Bi_2Sr_2(Ca,Pr)Cu_2O_8 also shows a similar dielectric constant, the charge inhomogeneity plays an important role in the insulator-superconductor transition., Comment: RevTex4 format, 5 pages, 3 figures, submitted to J. Phys. Condens. Mat

This report describes a case of a 17-year-old girl with Charcot-Marie-Tooth disease (CMT) representing rigid spine and respiratory failure. At age 11, she tended to walk on her toes and had difficulty in getting up from the floor without support. She became aware of flexion limitation of the neck at the age of 12. At 15 years of age, She began to have dyspnea on effort. When she was 17 years old, neurological examination revealed mild weakness of the upper extremities and severe weakness of the distal lower extremities, generalized wasting and areflexia. Superficial sensation was mildly impaired distally, and vibration sensation was severely impaired in the lower extremities. Motor and sensory nerve conduction velocities were mildly reduced, and compound muscle action potential of the tibial and peroneal nerves and sensory nerve action potential on ulnar and sural nerves were absent. Electromyography showed neurogenic changes with denervation potentials. Sural nerve biopsy revealed severe loss of myelinated fibers without any onion-bulb formation. As for family history, her elder sister showed moderate loss of vibration sensation in the lower extremities. On the basis of these findings, she was diagnosed as having CMT type 2, though a mode of inheritance was uncertain. She also had peculiar findings of flexion limitation of the spine (rigid spine), contracture of the hip joint, and fatty degeneration of paraspinal muscles on CT. Percent vital capacity (VC) was 22.5%, and arterial blood gas analysis showed PaO2 of 60.5 mmHg and PaCO2 65.0 mmHg. To our knowledge, this is the first case of CMT accompanied by rigid spine and respiratory failure. Motor and sensory neuropathy combined with rigid spine also have not been reported previously. The relationship between rigid spine syndrome with neurogenic muscular atrophy and CMT type 2C with the clinical characteristics of diaphragm and vocal cord paresis is discussed.

A 15-year-old boy developed ataxic gait, diplopia and hoarseness. Within 3 days after the onset, he had additional symptoms of dysphagia and dysarthria. He was admitted to our hospital 7 days after the onset of the disease. On admission, he had total ophthalmoplegia, ataxia, areflexia, facial diplegia, bulbar palsy and weakness of the neck and upper arms. Serum anti-GQ 1 b and anti-GT 1 a antibodies were significantly elevated. A diagnosis of Fisher syndrome associated with pharyngeal-cervical-brachial weakness was made. He was placed on a high dose of intravenous immunoglobins (12.5 g/day x 2 days) and had steroid pulse therapy (methylprednisolone 1 g x 3 days), which resulted in an almost complete recovery. There have been no reports of Fisher syndrome associated with brachio-pharyngeal-palsy. As in the case of the pharyngeal-cervical-brachial variant of Guillain Barré syndrome, anti-GT 1 a antibodies may be associated with Fisher syndrome with pharyngeal-cervical-brachial weakness.

The resistivity and the thermopower for Bi2Sr2Ca1-x R x Cu2O8 single crystals are measured and analyzed with a special interest in the parent antiferromagnet insulators. Above room temperature, the parent insulators show an electric conduction confined in the CuO2 plane and a decreasing thermopower with temperature, which are very similar to those for high-T c cuprates. These data strongly suggest that essential anomalies of high-T c cuprates inheres in the parent insulators.

The reaction of excited nitrogen atoms, N(2D), with the simplest alkyne, C2H2, was investigated for the first time under single-collision conditions in crossed beam experiments with mass spectrometric detection. The experimental results combined with electronic structure calculations and RRKM predictions allow us to identify cyanomethylene (HCCN) as the main primary reaction product and to establish its formation dynamics. The identification of the N/H exchange channel suggests that the title reaction is a good candidate to explain formation of nitriles in the upper atmosphere of Titan.

The anisotropic dielectric constants of the parent antiferromagnet Bi2Sr2 MCu2O8 (M=Dy, Y and Er) single crystals were measured from 80 to 300 K. The in-plane dielectric constant is found to be very huge (104-105). This suggests a remnant of the Fremi surface of the parent antiferromagnet. The out-of-plane dielectric constant is 50–200, which is three orders of magnitude smaller than the in-plane one. A significant anomaly is that a similar out-of-plane dielectric constant is observed in superconducting samples.

A 69-year-old woman developed paraplegia and hypesthesia on upper extremities and below T4 level. Examination of cerebrospinal fluid showed increased protein levels and pleocytosis. MRI of the cervical spinal cord revealed syrinx formation from C3 to upper thoracic cord. A diagnosis of acute transverse myelitis was made. A high dose of corticosteroid including pulse therapy did not improve her symptoms and signs of myelopathy, but the syrinx could not be found thereafter. One year later, she developed severe visual loss due to bilateral optic neuritis which was improved spontaneously. The clinical course and MRI findings were similar to those of the optic-spinal form of multiple sclerosis (MS). The presence of anticardiolipin antibodies, lupus anticoagulant and perinuclear anti-neutrophil cytoplasmic antibodies, however, strongly suggested that vasculitic and/or ischemic mechanisms induced by these autoantibodies might play a role on the development of the disease. We conclude that our case should be distinguished from MS.

BMT was carried out on a patient with DiGeorge syndrome who suffered recurrent infections after birth. At 13 months of age, 8.0 x 10(8)/kg of bone marrow nuclear cells were infused from an HLA-identical sibling using only anti-thymocyte globulin to prevent rejection. Donor DNA was not detected on microsatellite polymorphism by PCR. At 19 months of age, a second BMT from the same donor was carried out using busulfan and cyclophosphamide as conditioning. DNA examination of bone marrow showed chimerism at day 18 and complete donor origin at day 28. Seven months post-BMT, the numbers of CD3-, CD4- and CD8-positive cells were in the normal range. BMT is thus an effective therapy for DiGeorge syndrome.

The report deals with the first description of two siblings, a 43-year-old woman and 39-year-old man, who have developed cerebellar ataxia, choreoathetosis, dementia, epilepsy, hyperelasticity of the skin, and hypermobility and dislocation of joints. The frequent dislocations of joints sometimes could not be corrected surgically. Their mother and maternal uncle have the same neurological signs and symptoms as the siblings do. However, they do not present with the signs of skin and joints. Gene analysis of peripheral blood lymphocytes from these 4 patients revealed that the CAG repeat length of the dentato-rubro-pallido-luysian atrophy (DRPLA) gene is increased in all four. On the contrary, their father has displayed only hyperelasticity of the skin. From the clinical signs, family history and skin biopsy, we consider that the siblings and their father have Ehlers-Danlos syndrome (EDS) type III. In conclusion, the previously unreported coincidental development of maternal DRPLA and paternal EDS observed in two siblings deserves to be described.

We report a case of 45-year-old women with Bartter's syndrome and concomitant renal dysfunction. In 1986, the patient demonstrated muscle weakness and serum potassium levels as low as 1.1 mEq/l. She was suspected of having Bartter's syndrome because of hypokalemia, metabolic alkalosis, hyperreninemia, hyperaldosteronism and normotension. Pretibial edema developed in 1989 for which she received 40 to 100 mg/week of furosemide intermittently for the next 5 years. Her serum potassium level ranged from 1.5 to 3.9 mEq/l. In 1991, her serum creatinine level rose to 2.1 mg/dl, then continued to increase gradually. She was admitted to our hospital in 1994 for evaluation of the renal dysfunction. Decreased creatinine clearance (44 ml/min) and a defect in urinary concentrating capacity (Fishberg's test, 370 mOsm/kg.H2O) were detected. Renal biopsy revealed juxtaglomerular cell hyperplasia. These findings resulted in the diagnosis of Bartter's syndrome. The renal biopsy also showed diffuse interstitial fibrosis and marked tubular atrophy. We postulate in this case that long-term hypokalemia due to Bartter's syndrome and the administration of furosemide led to chronic interstitial nephritis and renal dysfunction.

The patient was a 25-year-old male with acute multifocal motor neuropathy with conduction block (MMNCB) after Campylobacter jejuni enteritis. After having suffered from diarrhea for 3 days, he rapidly developed asymmetrical distal-dominant muscle weakness in all extremities. Sensory disturbance was unremarkable except for slight disturbance in deep sensation. Deep tendon reflexes were normal throughout the course of present illness. CSF analysis revealed increased protein up to 66 mg/dl without pleocytosis. In electrophysiological examinations, persistant multifocal conduction blocks in the motor nerves were predominantly noted in the distal part of the extremities. Serum titers of anti-Campylobacter jejuni antibody, anti-GM1 antibody and anti-GalNAc-GD1a antibody were elevated. Muscle weakness resolved completely within 7 weeks. The sural nerve biopsy did not reveal either axonal degeneration, nor demyelination. These clinical and laboratory findings suggested that this case was most likely an acute type of MMNCB after Campylobacter jejuni enteritis.

Pharmacokinetic and clinical evaluation of an injectable cephem antibiotics, cefozopran (SCE-2787, CZOP), was conducted in newborn patients and the following results were obtained: 1. Clinical results The clinical efficacy of CZOP was evaluated in one each patient with intrauterine infection and suspected septicemia. The efficacy was "excellent" in both patients. No clinically serious adverse drug reactions of signs and symptoms and abnormal alterations of the laboratory test values were recognized. 2. Pharmacokinetics CZOP was intravenously given to newborn patients at doses of 25.0, 20.0, and 18.75 mg/kg. The blood CZOP concentrations were 44.7 +/- 7.0 micrograms/ml (n = 3), 48.3 micrograms/ml and 48.2 micrograms/ml at one hour after administration, respectively. The elimination half life (T 1/2) was 4.22 +/- 1.17 hours (n = 3) in the patients given 25.0 mg/kg and 2.74 hours in the patient given 20.0 mg/kg. The urinary drug excretion rate was 44.5 +/- 8.7% and 31.3 +/- 9.7% of dose within 8 hours after administration of 25.0 mg/kg and 20.0 mg/kg, respectively.

We reported a case of idiopathic hypereosinophilic syndrome with psychiatric symptoms and abnormal areas in subcortical white matter on brain MRI. The 25-year-old man was referred to us because of fever, confusional state and emotional incontinence. He soon became apathetic and emotionless. While he was confused, we studied brain CT twice and brain MRI four times. In deep white matter around the lateral ventricles and the cortices of parietal lobe, occipital lobe and cerebellar hemispheres, T1-weighted MRI revealed low intensity areas and T2-weighted MRI showed high intensity areas. After gadolinium infusion, some above-stated areas were enhanced. The lesions in the deep white matter disappeared when symptoms diminished, while lesions in the occipital cortex remained unchanged. Brain MRI studies were beneficial and useful to analyse the rare idiopathic hypereosinophilic syndrome with psychic disorders.

We reported a sporadic case with late onset SCA1. There was no family history of neurological diseases. His parents had been healthy until they died at the age of 77 and 89 years, respectively. The patient noticed gait disturbance at age of 60. Thereafter, he gradually developed cerebellar ataxia, hyporeflexia, mild atrophy of the facial and limb muscles and moderate deep sensory disturbance. MRI of the brain showed moderate atrophy of the cerebellum and brainstem. Sequencing analysis of SCA1 gene demonstrated that the patient had an expanded allele with 40 CAG repeats and no CAT interruption. Consequently, he was diagnosed as having SCA1. These results suggest the possibility that among apparently sporadic cases with cerebellar ataxia, there are some cases of SCA1 with mild CAG repeat expansion.

We report a case of dystrophia myotonica (DM) in a 38-year-old man with homozygous DM kinase abnormality. His distal muscle strength was reduced moderately; muscle biopsy showed type 1 predominance and type 1 fiber atrophy. The patient's WAIS total IQ score was under 60. Since his childhood, his cognitive deficit has been more severe than his muscle weakness. MRI demonstrated many abnormal changes in the brain of this patient, but these changes were mild in comparison to the severity of his reduced cognition and low IQ. The relationship between mental dysfunction and DM kinase abnormalities is quite different from that seen in cases of heterozygous DM kinase abnormalities. This case demonstrated severe mental changes in spite of mild DM kinase abnormalities. We suspect the homozygous DM kinase abnormality to be a cause of the different clinical presentations of this patient.

We describe the relation of the CAG repeat length to the genetic anticipation in a Japanese family with dentato-rubro-pallido-luysian atrophy (DRPLA). The proband, a 21-year-old woman, developed epilepsy at age 19. Her mother has displayed cerebellar ataxia, choreoathetosis, and dementia since age 40, and the grandmother has shown cerebellar ataxia since age 52. So a genetic anticipation was observed. The CAG repeat sizes of peripheral blood lymphocytes from the proband, mother, and grandmother were found to be 61, 59, and 60, respectively. Thus, the mother showed earlier onset but a smaller CAG repeat length as compared to the grandmother. This case is thought to be rare and valuable in respect that the intergenerational contraction of the CAG repeat was shown in spite of the genetic anticipation was observed.