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A laser-driven relativistic electron beam (REB) has a large beam divergence. For the efficient heating of material by REB, for example, core heating in fast ignition, beam guiding from its generation point to the heating material to be heated by suppressing spatial divergence in a perpendicular direction to keep its intensity sufficiently high is indispensable. We evaluated the dependences of the REB guiding performance in the resistivity gradient guiding scheme [A. P. L. Robinson and M. Sherlock, “Magnetic collimation of fast electrons produced by ultraintense laser irradiation by structuring the target composition,” Phys. Plasmas 14, 083105 (2007)] on laser intensity and duration, from the present petawatt laser parameters to the fast ignition-relevant laser parameters, in terms of the temperature dependence of resistivity and energy deposition of the REB. The REB transport simulations in solid materials show that for a fast ignition-relevant laser condition, a nickel cylinder works as a guiding material; an REB transport efficiency of 40% is obtained for a 100- μm propagation. The simulation results show that the resistive gradient guiding scheme is one of possible candidates for improving core heating efficiency.

Recent progress in the physics of fast ignition of fusion targets is reviewed here. Fundamental studies on hot electron energy transport show that the scheme looks promising if the heating pulse can be guided close enough to a compressed core. The idea of using cone-guided compression was first demonstrated experimentally under a Japan-UK collaboration. The use of the gold cone was extremely successful and showed a 10 3 neutron increase out of CD target implosion with a 300 J/0.5 ps enforced heating laser pulse. The heated temperature was close to 1 keV. In order to increase the temperature to 10 keV, a 10 kJ PW- laser system is necessary. Osaka University has started constructing such a laser system.

Target design and the fabrication scheme are discussed aiming an application for the future laser fusion reactor KOYO-F that has 4 modular chambers with a wet wall operated with fast ignition scheme. Gain estimation and load of fusion output from the non-symetric burn on the first wall are shown.

The fast ignition is a new scheme in laser fusion, in which higher energy gain is expected with smaller laser pulse energy. The cone target has been introduced for realizing higher coupling efficiency. At ILE, Osaka University, the laser with 4 beams and the total output of 10kJ/ps: LFEX has been built and we have started the integrated experiments. The experiments showed that the coupling efficiency is degraded because of the laser pre‐pulse. Namely, the main pulse is absorbed in a long scale pre‐plasma produced by the pre‐pulse and hot electron energy is higher than that for a clean pulse. Furthermore, the distance between the hot electron source and the core plasma is long. So, we are exploring how to overcome the pre‐pulse effects on the cone target. The next series of experiments is planned for this fall. In these experiments, the LFEX pre‐pulse level will be reduced and advanced targets for mitigating the pre‐pulse effects will be introduced.In this paper, it is proposed that a thin foil cover the l...

Thermonuclear ignition and subsequent burn are key physics for achieving laser fusion. In fast ignition, a highly compressed fusion fuel generated with multiple ns‐laser beams is rapidly heated with a large energy, ps‐laser pulse in prior to core disassembly. This scheme has a high potential to achieve ignition and burn since driver energy required for high fusion gain is predicted to be about one tenth of that needed for the central ignition scheme. In Japan, Fast Ignition Realization Experiment (FIREX) project has been started to clarify the physics of energy transport and deposition in the core plasma and to demonstrate fuel temperature of above 5 keV. After the success, FIREX‐I will be followed by the second phase of the project (FIREX‐II) to demonstrate ignition and burn. LFEX laser, designed to deliver a laser pulse of 10 kJ in 10 ps, are operational and the first phase of FIREX experiments has been stated. A new target is proposed to attain dense compression of fuel and improve laser‐core coupling efficiency by adopting double‐cone structure, a low‐density inner liner, low‐Z outer coating, and Br‐doped fuel shell. In this paper, present status and near term prospects of the FIREX‐I project will be reported together with activities on target designing, laser development, and plasma diagnostics.

In fast ignition research, the transport dynamics of fast electrons is one of the critical issues. Fast electrons generated by an intense laser pulse show a highly anisotropic velocity distribution. To gain insight into the anisotropy of the velocity distribution of fast electrons, polarized x‐ray spectroscopy has been proposed. The polarization spectroscopy of Cl Heα radiation was experimentally demonstrated at 1017 W/cm2 (∼100 mJ in 130 fs), and a new time‐dependent atomic population kinetics code was also developed. It predicts that the high polarization arises only in a low‐density region of the target plasma. Additional x‐ray polarization measurements were done at 101717–18 W/cm2 (∼10 J in ∼1 ps). Polarization was measured as a function of the overcoat thickness of a target. The polarization is negative in the shallow region near the target surface, and becomes near zero at the laser intensity of ∼1018 W/cm2. At ∼1017 W/cm2, the polarization varies from negative to positive, and finally zero along wi...

X‐ray polarization spectroscopy was studied to derive directly the velocity distribution function (VDF) of hot electrons propagating in plasma created with a high intensity laser pulse. Polarization measurement was made at around 1018 W/cm2 using a laser pulse (∼10 J in ∼1 ps) from Alise facility at CEA/CESTA. Chlorinated triple‐layer targets were irradiated, and Cl Heα line was observed with an x‐ray polarization spectrometer. Polarization degrees were measured as a function of the target overcoat thickness, corresponding to the depth along pre‐formed plasma. It is found that the polarization is weakly negative for thin coating, but becomes positive, and finally zero for thick coating. This result is consistent with predictions made with a time‐dependent atomic kinetics code developed to gain an insight into the generation of polarized Cl Heα radiation. The de‐polarization on the surface is attributed to excessive bulk electron temperature and that in the deep region to elastic‐scattering processes by th...

Protons having energies up to 3.8 MeV are experimentally generated by the p‐polarized, 45 fs, ∼1019 W/cm2 laser pulse interacting with a near‐critical plasma cloud produced by the irradiation of the amplified spontaneous emission (ASE) pedestal at an intensity of ∼1013 W/cm2 onto a 7.5‐μm‐thick polyimide foil target. The emission angle of the high‐energy protons is shifted away from the target normal toward the laser‐propagation direction of 45°. The mechanism which explains the proton generation from the near‐critical plasma cloud is discussed using the two‐dimensional (2D) particle‐in‐cell (PIC) simulation.

金沢大学先端科学・社会共創推進機構, Highlly collimated energetic protons whose energies are up to 4 MeV are generated by an intense femtosecond Titanium Sappheire laser pulse interacting with a 7.5, 12.5, and 25 μm-thick Polyimide tape target and 5 μm-thick copper target. We find no clear difference on the proton spectra from 7.5, 12.5, and 25 μm Polyimide tape target. The highest conversion efficiency from laser energy into protons of ∼3% is observed with a 7.5 μm thick Polyimide target. The quality of the proton beam is good enough to obtain a clear projection image of a mesh having 10 μm line and space structure, installed into the passage of the beam. We obtain clear vertical lines on the proton intensity profiles from the copper target, which are considered to be transferred from the surface of the copper target. From it, we can restrict the size of the proton emitting region to be ∼20μm. © 2008 American Institute of Physics., Embargo Period 12 months

The fast ignition scheme is one of the most fascinating and feasible ignition schemes for the inertial fusion energy. At ILE Osaka University, FIREX (Fast Ignition Realization Experiment) project is in progress. Implosion experiments of the cryogenic target are scheduled in near future. There are two key issues for the fast ignition. One is controlling the implosion dynamics to form high density core plasma in non‐spherical implosion, and the other is heating the core plasma efficiently by the short pulse high intense laser. The time and space scale in the fast ignition scheme vary widely from initial laser irradiation to solid target, to relativistic laser plasma interaction and final fusion burning. The numerical simulation plays an important role in demonstrating the performance of the fast ignition, designing the targets, and optimizing laser pulse shapes for the scheme. These all the physics are desired to be self‐consistently described. In order to study these physics of FI, we have developed “Fast Ignition Integrated Interconnecting code” (FI3), which consists of collective Particle‐in‐Cell (PIC) code (FISCOF1D/2D), Relativistic Fokker‐Planck with hydro code (FIBMET), and 2‐dimensional Arbitrary‐Lagrangian‐Eulerian (ALE) radiation hydrodynamics code (PINOCO). Those codes are sophisticated in each suitable plasma parameters, and boundaries conditions and initial conditions for them are imported/exported to each other by way of DCCP, a simple and compact communication tool which enable these codes to communicate each others under executing different machines. We show the feature of the FI3 code, and numerical results of whole process of fast ignition. Individual important physics behind the FI are explained with the numerical results also.

Hot electron transport in a high density plasmas has been studied using a two dimensional hybrid code. The results show that the initially cylindrical solid beam evolves into a hollow, annular beam due to the Weibel instability and generates strong magnetic fields on both sides of the annular ring. The annular structure subsequently breaks up into several beamlets due to a mechanism similar to a tearing instability. It is found that the magnetic fields parallel to the direction of beam propagation also grow during the tearing process. These beamlets are attract each other and finally they merged into a single beam which carries a net electric current. The tearing and merging processes have also been analyzed by the hybrid code for a uniform initial condition to determine an asymptotic behaviors of the total number of hot electron beams. We also have performed a three dimensional particle-in-cell (PIC) simulation to compare with the results of 2D hybrid code. It shows that the spatial variation of cold electron density is much more uniform than that obtained by 2D code.

To investigate the usefulness of expectorated sputum in the assessment of airway inflammation by smoking. I examined cytokine expression in the sputum of healthy smokers and non-smokers, and compared them before, and at 8,12 and 16 weeks after, cessation of smoking. Four cytokines, interleukin (IL) 8, interferon (IFN)-gamma, tumor necrosis factor (TNF)-alpha, and IL-10, were assessed by immunohistochemistry. In smokers, the percentage of bronchial epithelial (BE) cells and macrophages with high intracellular contents of the pro-inflammatory cytokines IL-8, IFN-gamma and TNF-alpha, were significantly increased in comparison with non-smokers. In contrast, expression of IL-10 was significantly decreased in smokers. After smoking cessation, expression of IL-8 was significantly decreased by the 16th week, that of IFN-gamma was significantly decreased by the 12th to the 16th week, but that of TNF-alpha was unchanged even at 16 weeks post-cessation, and that of IL-10 was significantly increased at that time. Cytokine expression in BE cells was similar to that in macrophages. From the data, we concluded that expectorated sputum is useful for assessing airway inflammation due to smoking and for following the series of changes that follow smoking cessation.

To explore the optimum dose of intravenous immunoglobulin (i.v.Ig) for treating patients with chronic inflammatory demyelinating polyrneuropathy and multifocal motor neuropathy, we compared the usefulness of i.v.Ig among 3 treatment doses. Fifty-nine patients were randomly divided into three treatment dosage groups: 20 patients for Group I using 50 mg/kg/day x 5 days, 19 patients Group II using 200 mg/kg/day x 5 days, and 20 patients Group III using 400 mg/kg/day x 5 days. We assessed clinically and electrophysiologically the effectiveness of the treatment at 5 weeks after the initial infusion. For patients in Group I and II who had not improved (or worsened) with the first treatment, we gave a one-step larger dose in the second treatment (i.e. 200 mg/kg/day x 5 days for those who had been given 50 mg/kg/day x 5 days, 400 mg/kg/day x 5 days for those who had been given 200 mg/kg/day x 5 days) after more than 9 weeks. We found that 15% of the patients in Group I, 21% in Group II and 60% in Group III improved dose-dependently with the first intravenous immunoglobulin treatment. Seven (47%) of 16 patients in Group I and 4 (40%) of 11 patients in Group II improved after the second treatment with larger doses. Adverse reactions including chill sensation, fever, skin eruption and increase in blood GOT and GPT levels were transient and mild. One patient in Group III developed left hemiparesis showing the small infarction in the right thalamus during the course of the treatment, but the symptom was mild. In conclusion, the high-dose intravenous immunoglobulin therapy (400 mg/kg/day x 5 days) is useful for treating patients with CIDP and MMN, although care must be taken of the risk of causing cerebral infarctions.

A novel magnetic head with thin-film magnetic yokes and thin-film coils for VCR systems has been developed. The head has 25- mu m-thick CoNbZr (B/sub s/=0.9 T) magnetic yokes with round edges. The lower yoke is buried in the groove with a round bottom formed by a combination of photolithographic and mechanical processing, while the upper yoke is formed by ion etching. The head with a 150 mu m gap depth has excellent recording performance on high H/sub c/ media (H/sub c/=1500 Oe). Its contour-signal level is below -45 dB from the main signal ( lambda =2.5 mu m). >

We report a 55-year-old woman who developed symptoms resembling parkinsonism. Her psychiatric symptoms in the early stage, cervical dystonia and tremor increasing on movement were consistent with manganese poisoning. Manganese levels were elevated to 1.5 micrograms/l in the serum (normal; 0.3-1.1 micrograms/l) and to 1.4 micrograms/l in the urine (normal; less than 1.2 micrograms/l). Intravenous infusion of calcium disodium editate (CaEDTA; chelating agent) was followed by the marked excretion of manganese (27.3 micrograms/l) in the urine. These findings support manganese poisoning. Manganese poisoning is a disease which results from chronic exposure to manganese, but the source of manganese exposure remained obscure in this patient. T1-weighted MRI of the brain showed symmetric high signal intensity in the globus pallidus without any abnormality on T2-weighted images. There is a report that manganese induced brain lesions in Macaca fascicularis as revealed by MRI and the fascicularis developed signs of unsteady gait and hypoactivity. The patient responded to treatment with CaEDTA and the second MRI demonstrated regression of abnormal signal intensity. This may be due to enhanced manganese excretion. To our knowledge, this is the first case of probable manganese-induced human parkinsonism whom changes in MRI were noted after treatment with CaEDTA.

A 38-year-old man presented with personality change, postural tremor of the right arm and leg, and right hemidysesthesia. MR imaging (MRI) revealed left hemisphere dominant brain atrophy and leuko-araiosis in the deep white matter. Serological test of serum and cerebrospinal fluid demonstrated high titers of antibodies to Treponema pallidum. He was diagnosed as Lissauer's general paresis based on the clinical symptoms and signs, and MRI findings. This disorder is characterized by focal cerebral atrophy, corresponding to focal neurologic signs. Neuropathological features in Lissauer's general paresis are spongiform atrophy of the cerebral cortex and demyelination of the white matter. Leuko-araiosis on MRI in our case may represent demyelination of the white matter. Although MRI findings in general paresis are usually nonspecific, this is, as far as we know the first case report of general paresis with MRI showing focal cerebral atrophy and leuko-araiosis.

We describe two brothers with early onset cerebellar ataxia associated with hypoalbuminemia (EOCAH). Choreo-athetoid movements preceded the cerebellar ataxia, and serum pseudocholinesterase elevation preceded the hypoalbuminemia. The parents are first cousins. Patient 1, the 22-year-old elder brother, developed choreoathetoid movements of the neck and extremities at the age of eighteen months. He later developed slowly progressive cerebellar ataxia with decreased tendon reflexes. The choreoathetoid movements ceased at about 16 years of age. A recent examination revealed cerebellar ataxia, action myoclonus of the neck and upper limbs, diminished tendon reflexes, mild sensory disturbance in the lower extremities, and very slight amyotrophy of the feet. Patient 2, the 18-year-old younger brother, developed choreo-athetoid movements at the age of 6 years, followed by slowly progressive cerebellar ataxia with disminished tendon reflexes. No scoliosis, ECG abnormalities, or edema was detected. Serum biochemistry studies revealed elevated pseudocholinesterase (6,226 to 2,390 IU) in the patient's early teens. Serum albumin levels tended to be low (3.7 to 4.1 g/dl). Serum triglyceride and beta-lipoprotein levels were elevated in the patients' late teens. Genetic studies showed slight linkage of D9S15. The maximum lod score was 0.289 (recombination fraction rate was 0.14).

A 29-year-old man was referred to our hospital because of exertional dyspnea and progressive eruption on the buttocks and the lower extremities. Chest roentgenograms and computed tomograms taken at that time revealed diffuse fibrotic changes accompanied by multiple cavities and bullae in the lungs. There were no signs of mediastinal or hilar lymphadenopathy. A chest roentgenogram taken 7 years before admission showed no abnormalities. Serum ACE and lysozyme levels were high: 29.9 IU/l and 14.1 micrograms/ml, respectively. 67Ga scintigraphy showed diffuse uptake in both lung fields. The PPD skin test was negative, and repeated sputum smears and cultures were negative for pyogenic bacteria and acid-fast bacilli. Examination of transbronchial lung biopsy and skin biopsy specimens confirmed the diagnosis--they showed noncaseating epithelioid granulomas with giant cells and a negative reaction of the stain to acid-fast bacilli, which are compatible with sarcoidosis. The patient was given 30 mg/d of prednisone orally. The dyspnea and eruption were clearly alleviated, although there was little roentgenographic regression of cystic or fibrotic changes. There have been only a few reports of cystic and fibrotic changes early in the course of sarcoidosis. The cystic lesions in this case were probably secondary pulmonary cavities caused by the contracting and obstructive changes related to pulmonary fibrosis.

A 52-year-old right-handed man presented progressive dystonia and apraxia of his right hand of five years' duration. He also suffered from parkinsonian features such as rigidity or impaired postural reflexes. Serial investigation of brain MRI revealed progressive cerebral atrophy, which started in the left parietal lobe, and subsequently extended to both hemispheres. He was clinically diagnosed as corticobasal degeneration. He could not point at any part of his own body in response to verbal or visual commands. On the other hand, he could point at every part of the examiner's body or of the illustrated body image. Deep sensations and linguistic functions were not involved. This cognitive impairment was regarded as autotopagnosia. In contrast with inability to recognize any part of the own body in response to the commands, he could name every part of his body as soon as the examiner touched there. Moreover, his symptoms of autotopagnosia were ameliorated by looking at himself in a mirror; he could point at any part of his own body. Disconnection between primary proprioceptive sensory area and the center of body schema was thought to be the mechanism of autotopagnosia in this patient, because the impairment improved with the aid of visual or tactile informations. We speculated the lesion was the left parietal lobe.

The authors demonstrated the functionality of a circularly arranged submicron scale optical cavity with a GaInNAs gain medium and an AlOx cladding layer within a two-dimensional photonic crystal slab. The GaInNAs gain has negligible optical degradations due to process damage, probably stemming from the suppressed diffusion carriers and the small surface recombination velocity of the material. The calculated spectrum when using a finite different time domain simulation provides a cavity mode containing a whispering gallery mode at a wavelength close to 1.3 μm. An observation of the tuned spectral peaks related to the cavity mode shows this cavity has promise for future device applications.

A 36-year-old man with hemophilia A was admitted to hospital because of otalgia, hearing loss, nasal obstruction, nonproductive cough, and high fever. His laboratory data showed high-grade acute inflammatory reactions. His chest X-ray and CT films showed multiple cavitary masses in the right lower lung field. Bronchoscopy performed at our institution revealed bronchial nodules in the intermediate truncus, and BAL revealed increases in the neutrophils and an IgG index (BAL IgG/albumin divided by serum IgG/albumin). Biopsy specimens obtained from nasal mucosa showed epithelioid granulomas with Langerhans' giant cells and necrotizing vasculitis. Antineutrophil cytoplasmic antibodies were also positive, but no evidence of glomerulonephritis was observed. The diagnosis of limited Wegener's granulomatosis was thus made. He was treated with standard therapy (daily cyclophosphamide and glucocorticoids), but within 1 month he had complications of empyema with herpes zoster, and bronchopleural fistula. The complications resolved with appropriate treatment.

We report three patients with slowly progressive spastic paraplegia and dementia; MRI on these patients revealed hypoplasia of the corpus callosum. The mode of inheritance was supposed to be autosomal recessive. Patient 1 (26-year-old man) is an elder brother of patient 2 (21-year-old man). Their parents are first cousins. Patient 3 (woman), a sporadic case, died of pneumonia at the age of 44. Their motor development after the birth was normal, but patient 3 was mildly mentally retarded. Gait disturbance due to spastic paraplegia developed at the age of nine (patient 2), fifteen (patient 1) and nineteen (patient 3), respectively. They also showed slowly progressive mental deterioration. Patient 1 has also suffered from mild amyotrophy and sensory disturbance in the distal part of the extremities since the age of 25. Patient 3 was bed-ridden at the middle of her thirty's because of generalized amyotrophy and sensory disturbance in addition to spastic quadriplegia and profound dementia. Their MRI reveal the thinning of the corpus callosum. We think the thinning must be hypoplasia of the corpus callosum, because the cerebrum showed normal appearance on MRI in patient 1 and patient 2. These clinical findings and imaging studies are essentially similar to those of the cases reported by Iwabuchi et al (1991). We propose autosomal recessive HSP associated hypoplasia of the corpus callosum as a new type of HSP.

Cerebellar ataxia with hypogonadotropic hypogonadism is a rare condition. Both hypothalamic LHRH and pituitary gonadotropin deficiency may appear with cerebellar ataxia. In this report, a 19-year-old man who presented with progressive cerebellar ataxia and mental disturbance was found to have low plasma gonadotropin levels. No rise in gonadotropin was demonstrated after repeated stimulation with LHRH. Our findings suggested that the cause of hypogonadism was pituitary gonadotropin deficiency, or both hypothalamic and pituitary dysfunction. The endocrinological data and clinical features of our patient and all other reported cases suggest that the age of onset of the illness is related to the type of endocrinological abnormality: young-onset patients may have endocrine disturbances involving both the hypothalamus and the pituitary gland, which may be affected secondary to the hypothalamic disturbance, whereas adult-onset patients may show only hypothalamic disturbance.

Lower lung field tuberculosis has been considered as rare disease, but it was observed about ten percent in the literature. It seemed that the important problems in lower lung field tuberculosis are delay and difficulty of diagnosis. The materials for this report was accumulated over a period of 10 years, from January 1983 to December 1992 at the National Sanatorium Fukuoka-Higashi Hospital. The criteria for lower lung field tuberculosis are: (1) disease confined to lower lobe except for S6a, middle lobe and lingular segment, (2) bacteriologically and/or histologically proved diagnosis of tuberculosis, (3) clinically considered as tuberculosis for which improved by only anti-tuberculous drugs. During this period, 672 new patients suffering from active pulmonary tuberculosis were admitted to the sanatorium. Of the total of 672 patients, 57 patients were selected as the criteria for lower lung field tuberculosis. The incidence of them was 8.5 percent of the total new admissions. Of these 57 patients, 30 were females and 27 males. The mean age of these 57 patients was 59.4 years ranging between 19 and 86 years. The incidence of them obtained through ten years did not show any tendency to increase or decrease. The respiratory and general symtoms most commonly observed were cough and chest pain. The average duration of used for diagnosis of the lower lung field tuberculosis over 65 years old was 71.7 days and it seemed to show the delay and difficulty of diagnosis.

Recent progress in neurology has revealed that hereditary olivo-ponto-cerebellar atrophy (OPCA) is in fact three different diseases. These are Menzel's disease, in which degeneration in the olivopontocerebellar (OPC) system is quite severe and similar to that of the patient described by Menzel in 1891, spinocerebellar ataxia 1 (SCA 1), in which the gene locus exists in the short arm of chromosome 6, and hereditary OPCA with retinal degeneration. We present a family with Menzel's disease, some of whom showed dementia and various extrapyramidal symptoms including tremor, myoclonus, and choreoathetoid involuntary movement.This dominant hereditary cerebellar ataxia family had five affected members in four generations. Neuropathological examination of one member (Case 3) revealed Menzel's disease. There was severe degeneration in the OPC system, the substantia nigra, Clarke's column, the posterior column, and the anterior horn of the spinal cord, and slight-to-moderate degeneration in the globus pallidus and subthalamic nucleus. However, the dentate nucleus, spinocerebellar tracts, and oculomotor nucleus including the medial longitudinal fasciculus were spared. The brain weight was 990 g. Case 1 (Case 3's grandmother) developed slowly progressive ataxia at the age of 55. She showed no involuntary movement or dementia. She died at 63 years of age. Case 2 (Case 3's mother) developed ataxia at 42 years of age, followed by tremor of the hands and head, and died at age 57. She did not show dementia. Case 3 (the autopsied case) developed progressive ataxia at 27 years of age, followed by mental deterioration, tremor, myoclonus, and generalized amyotrophy and sensory disturbance during her fifth decade.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors present the clinico-pathological findings in a member of a family residing in Akita Prefecture located in the north-eastern region of Japan. Four members in three generations of the family developed ataxia. The autopsied patient was a 42-year-old woman, who, at the age of 25, had developed progressive cerebellar ataxia with pyramidal spasticity and increased deep tendon reflexes predominant in the lower extremities. However, she retained fine movement of the hands and fingers and showed no dysarthria until the age of 35. She could no longer walk unassisted at 38 years old. She showed cerebellar ataxia in both hands and legs, dysarthria, bulging eyes, progressive extraoculomotor palsy with nystagmus, bradykinesia, sensory disturbance, and dystonia in the face, upper extremities, and fingers. Deep tendon reflexes were decreased, especially in the lower extremities. Subacute generalized muscular atrophy developed at the age of 39. She became bedridden and died of pneumonia. The clinical diagnosis was Type-2 of the entity known in Japan as Machado-Joseph disease. At neuropathological examination, the brain weight was 1,250 g. The spinocerebellar system including Clarke's column and the spinocerebellar tracts were degenerated, but the cerebellar cortex and inferior olivary nucleus were spared. Slight-to-moderate degeneration was observed in the pontocerebellar system. In the dentate nucleus, most of the neurons showed what is known in Japan as "grumose degeneration", but there was no neuronal loss or gliosis. The hilus of the dentate nucleus and the superior cerebellar peduncle were intact.(ABSTRACT TRUNCATED AT 250 WORDS)

A 54-year-old woman was hospitalized because of paraparesis, sensory disturbance of the lower extremities, and dysuria. Laboratory data included an erythrocyte sedimentation rate (ESR) of 16 mm/h and lactate dehydrogenase (LDH) level of 459 IU/l. Myelogram showed an enlarged spinal cord at the L1 level, and spinal MRI revealed a high signal area on T2-weighted images. Spinal cord biopsy by thoraco-lumbar laminectomy showed thickening of the pia mater and neovascularization, but no malignant cells. Immediately after the operation, the patient displayed progressive mental deterioration. Cranial MRI showed widespread high signal areas in the cerebral white matter on T2-weighted images which resembled those of leukoencephalopathy. Steroid therapy was tried but was ineffective. Ten months after the onset of symptoms, a brief period of regression of both clinical symptoms and MRI appearances occurred following the administration of Inosiplex, subsequent progressive deterioration led to death 14 months after the onset of symptoms. LDH, C-reactive protein (CRP), and cerebrospinal fluid (CSF) protein were all elevated during the deterioration of her general status. Postmortem examination revealed a large tumor mass of the left adrenal gland which extended to the Th12, L1, and L2 vertebrae and remarkable brain edema. Microscopic mononuclear tumor cells were widespread but confined to the lumens of small vessels of the brain, spinal cord, spinal root and right adrenal gland, almost as if they were occluded. Cerebral white matter presented widespread multiple small infarcts and extensive myelin thinning. In addition to the left adrenal gland, interstitial invasions of tumor cells were discovered in the kidneys, iliopsoas muscles, oviducts, myocardium, lymph nodes, and thyroid gland.(ABSTRACT TRUNCATED AT 250 WORDS)

We report a patient whose clinicopathological findings are compatible with those of the case reported by Menzel in 1891. This case was briefly reported by Kinoshita et al in 1967 as hereditary ataxia of Menzel type. But the concept of this disease has been confused, especially in the early investigations for the hereditary olivo-ponto-cerebellar atrophy (OPCA) in Japan. The Kinoshita's patient should be considered as the first Japanese case whose findings are identical with Menzel's report. This report represents a precise study of the case reported by Kinoshita et al. The patient was a 42-year-old Japanese woman. Her mother and one of her brothers suffered from the same disease. She began to experience progressive ataxia at the age of 30. At age 42, she was admitted to another hospital because of inability to walk and mental deterioration. Neurological examination revealed cerebellar ataxia in the extremities and trunk, childish personality change, dementia, diminished deep tendon reflexes with extensor plantar response bilaterally, slowness and hypokinesia in the movement, generalized muscular atrophy, and sensory disturbance prominent in deep sensory. She had no involuntary movement and dysautonomia. She had no retinal degeneration, nystagmus, nor progressive nuclear oculomotor palsy. She died of pneumonia. Neuropathological findings revealed brain weight of 850g.(ABSTRACT TRUNCATED AT 250 WORDS)

This report is to investigate neuropathological changes of the thalamus observed in progressive supranuclear palsy. Six autopsy cases were examined. They were 5 males and a female, whose age was between 57 and 72 years old. They developed dementia in various degrees and three of the cases showed periodic stuporous state and akinetic mutism in the last stage. The paraffined thin-sections were stained with hematoxylin-eosin, luxol-fast-blue, Holzer, modified Bielschowsky, Gallyas and anti-tau stainings. The histological changes were evaluated in the aspects of neuronal loss, fibrillary gliosis and neurofibrillary tangles. In conclusion, there were three types in six cases. The first was seen in two cases and that there appeared many Alzheimer's neurofibrillary tangles (NFTs) in medial nuclei, lateral and ventral nuclei as well as zona incerta and reticular nucleus. The second was observed in two cases and that the loss of nerve cells was severe, especially in medial nuclei and the dorsal part of lateral and ventral nuclei and fibrillary gliosis was observed beyond the lesions of neuronal loss. The third was that the appearance of NFTs was moderate in number and neuronal loss was minimal. Moreover, neuronal loss and NFTs of zona incerta were common in these cases. Argentophilic fibrillary inclusions were observed in the neuropil and astrocytes in all cases. It is important to investigate the correlations between neuropathological changes and clinical manifestations, especially in the peculiar type of dementia observed in PSP. The thalamic changes of PSP may play an important role in causing stuporous state and akinetic mutism as well as subcortical dementia.

Point mutation of mitochondrial DNA has been described in the blood from a MELAS patient. The 39-year-old patient developed progressive dementia, stroke-like episodes, heart conduction defect (Lown-Ganong-Levin syndrome) and cortical blindness. CT scan revealed brain atrophy and low density areas in the bilateral occipital lobes. Laboratory tests showed hyperglycemia and lactic acidosis. Muscle biopsy showed ragged red fibers on Gomori trichrome staining. He was clinically diagnosed as having MELAS and insulin-dependent diabetes mellitus. Onset of diabetes mellitus and MELAS was almost same. Family history showed his mother's brother and sisters had also insulin-dependent diabetes mellitus. We amplified the leucine (UUR) tRNA gene from the patient's blood with polymerase chain reaction (PCR) and analysed it by restriction enzyme analysis and sequencing. Genetic analysis showed A-to-G substitution at the nucleotide position 3243 in the leucine (UUR) tRNA gene. This substitution made a new restriction site Apa I. Mutant DNA coexisted with wild type DNA (heteroplasmy). It is shown that in some types of mitochondrial encephalomyopathies, especially patients of Kearns-Sayre syndrome (KSS), diabetes mellitus is often complicated. And in KSS patients insulin receptor in normal, but insulin secretion from beta cells of pancreas is decreased. In MELAS patients, however, has diabetes mellitus been reported to be rarely complicated and relationship between MELAS and diabetes mellitus is not done. As far as we know, two cases, including ours, with genetically diagnosed MELAS have been reported to have diabetes mellitus.(ABSTRACT TRUNCATED AT 250 WORDS)

A 48-year-old housewife was hospitalized because of spastic paraparesis accompanied by dysesthesia and amyotrophy in her lower extremities, and by urinary disturbances. At the age of 35 years she noticed tingling sensations in her right foot and gradually her gait became spastic. In a few years lumbago and the spinal form of intermittent claudication developed. When she was 41 years old muscle wasting was noticed in her right leg, and in four years the same feature was also seen in her lower left limb. Plain X-ray films showed a wide cervical canal with no signs of basilar impression. On MRI we could see a polycyst-like syrinx that extended over the entire spinal cord. In addition the surfaces of the lower thoracic cord seemed irregular, but arteriovenous malformation itself was not visualized. Neither hydrocephalus nor Chiari malformation was accompanied. Needle EMG examination revealed some neurogenic changes in the muscles of both lower limbs. Cystometry proved uninhibited contractions of the detrusor muscle and the existence of detrusor-sphincter dyssynergia. Myelography showed an enlarged spinal cord above the D11-12 level with a shadow of worm-like appearance between the D7-12 level. CSF protein was elevated. Spinal angiography confirmed the presence of single coiled vessels with a nidus at D8-10 that were fed by bilateral D10 intercostal arteries and drained to D7, D9 or D12. Embolization of these abnormal vessels was conducted, and since then the patient's condition has remained stable. Vascular malformation is a rare cause of secondary syringomyelia. Clinical manifestations are determined mainly by the level of vascular malformation and are not reflective of the level of syrinx.(ABSTRACT TRUNCATED AT 250 WORDS)

Acupuncture has been practiced in the treatment of many diseases in Japan. "Okibari" is one of the procedures in acupuncture treatment: a fine stainless steel or silver needle is inserted into the subcutaneous tissue through the skin, to remain in the subcutaneous tissue. A 57-year-old pharmacist was knocked down by a motorcycle in 1971, since then moderate weakness of left extremities and stiffness of muscles have remained as sequelae. She was consequently treated with acupuncture. Many small needles were inserted permanently in the nuchal, occipital and other areas of the body ten to twelve years before she developed gradual clumsiness and dysesthesia in her right hand in 1984. When she was admitted for the first time in 1985, neurological examination revealed left Horner's syndrome and diminished deep sensation in her right extremities with pseudo-athetosis of her right hand, along with spastic paresis of left extremities and right carpal tunnel syndrome. An old needle which had strayed into left dorsal medulla was considered to be responsible for these symptoms. In 1988 loss of pain and temperature sensation in the right side of her body below the shoulder, and diminished deep sensation of left extremities were appended, and weakness of her left extremities became aggravated. Pseudo-athetosis of her right hand was seen less prominently. In plain X-ray films many needle shadows were visualized. On CT scan needle shadows could be seen also in the left dorsal medulla, right cerebellum and in the subarachnoid space of left dorsal C1-C2 level.(ABSTRACT TRUNCATED AT 250 WORDS)

Idiopathic spasmodic torticollis is a type of focal dystonia. Major muscles which rotate the neck are M. sternocleidomastoideus (SCM) and M. splenius capitis (Spl) on both sides. In torticollis patients, its clinical characteristics could be understood as a vectorial summing up of tonus in bilateral SCM and Spl at rest. There is not any curative treatment for dystonia yet. A variety of medications and many types of surgical interventions have been tried without consistent or satisfactory results. In recent days local injection of botulinum-A toxin has shown to be effective in weakening focal dystonias. We used pure ethanol for local injection. Fourteen patients aged between 20-77 years (mean 48.9) were treated by alcoholisation. Disease duration ranged from 5 months to 12 years (mean 4.9 years). All had torticollis alone or had segmental dystonia containing spasmodic torticollis. Patients were recorded electromyographically using surface electrodes to make sure which neck muscles were hypertonic, and were rated before and after treatment according to the stages (0; normal-5; most severe). On the bases of these recordings the two most active muscles were selected for injection. Into the motor point of these muscles 1 ml of 1% lidocaine, and then the same dose of 99% ethanol were injected. This procedure was repeated on the mean ten times (6-14 times) every other week. The number of times of injection was decided in each case. Using the paired Student t test, there was a significant (p less than 0.01) improvement of the stage for the patients after injection, with a mean of 3.7 before treatment and 2.3 after treatment.(ABSTRACT TRUNCATED AT 250 WORDS)