| 1. |
- Saito, T. R., et al.
(författare)
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The WASA-FRS project at GSI and its perspective
- 2023
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Ingår i: Nuclear Instruments and Methods in Physics Research Section B. - : Elsevier. - 0168-583X .- 1872-9584. ; 542, s. 22-25
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Tidskriftsartikel (refereegranskat)abstract
- A novel technique to study bound states of exotic hadrons in subatomic nuclei, such as hypernuclei and mesic nuclei, has been developed by employing the Fragment Separator FRS and the WASA central detector at GSI. Two experiments, S447 for studying light hypernuclei, especially hypertriton and a Ann bound state, and S490 for searching for ri' mesic-nuclei, were recently performed. Data analyses are currently in progress, and light charged particles such as protons and x & PLUSMN; are clearly observed and identified in the both experiments. For S447, light nuclear fragments that can also be residual nuclei from decays of hypernuclei of interests have been analysed by the FRS, and a momentum resolution, Ap/p, of 5 x 10-4 has been achieved. Further data analyses are to be completed. The WASA-FRS project will be continued and extended with the FRS at FAIR Phase 0, and upgrading of the WASA magnet and detectors is currently in progress. Furthermore, construction of a larger detector system with the Super-FRS at FAIR Phase 1 is also under consideration.
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| 2. |
- Tanaka, Y. K., et al.
(författare)
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WASA-FRS EXPERIMENTS IN FAIR PHASE-0 AT GSI
- 2023
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Ingår i: ACTA PHYSICA POLONICA B PROCEEDINGS SUPPLEMENT. - : Jagiellonian University.
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Konferensbidrag (refereegranskat)abstract
- We have developed a new and unique experimental setup integrating the central part of the Wide Angle Shower Apparatus (WASA) into the Fragment Separator (FRS) at GSI. This combination opens up possibilities of new experiments with high-resolution spectroscopy at forward 0 and measurements of light decay particles with nearly full solid-angle acceptance in coincidence. The first series of the WASA-FRS experiments have been successfully carried out in 2022. The developed experimental setup and two physics experiments performed in 2022 including the status of the preliminary data analysis are introduced.
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| 3. |
- Dulski, J., et al.
(författare)
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Clinical variability of neuroacanthocytosis syndromes : A series of six patients with long follow-up
- 2016
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Ingår i: Clinical Neurology and Neurosurgery. - : Elsevier BV. - 0303-8467. ; 147, s. 78-83
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Tidskriftsartikel (refereegranskat)abstract
- Objective To provide clinical clues to differential diagnosis in patients with chorea and other movement disorders with blood acanthocytes. Methods We present a long-term video accompanied follow-up of six Caucasian patients with neuroacanthocytosis from several centers, three diagnosed with chorea-acanthocytosis (ChAc): 34-y.o.(no.1), 36-y.o.(no.2), 43-y.o.(no.3), two diagnosed with McLeod Syndrome (MLS): 52-y.o.(no.4), 61-y.o.(no.5) and one 63-y.o.(no.6), a brother of no.5, with clinical suspicion of MLS. Additionally we report pathological findings of the mother of two brothers with MLS reported in our series with acanthocytes on peripheral blood smear Results The patients had an unremarkable family history and were asymptomatic until adulthood. Patients no. 1,2,4,5,6 developed generalized chorea and patient no. 3 had predominant bradykinesia. Patients no. 1,2,3 had phonic and motor tics, additionally patients no. 1 and 2 exhibited peculiar oromandibular dystonia with tongue thrusting. In patients no. 2 and 3 dystonic supination of feet was observed, patient no. 3 subsequently developed bilateral foot drop. Patients no. 2 and 4 had signs of muscle atrophy. Tendon reflexes were decreased or absent and electroneurography demonstrated sensorimotor neuropathy in patients no. 1,2,3,4,5, except no. 6. Generalized seizures were seen in patients no. 2,3,5,6 and myoclonic jerks in patient no. 1. Cognitive deterioration was reported in patients no. 1,2,3,5,6. Serum creatine kinase levels were elevated in all six patients. Conclusion We highlight the variability of clinical presentation of neuroacanthocytosis syndromes and the long time from the onset to diagnosis with the need to screen the blood smears in uncertain cases, however, as in one of our cases acanthocytes may even be not found. Based on our observations and data from the literature we propose several red flags that should raise the suspicion of an NA syndrome in a patient with a movement disorder: severe orofacial dyskinesia with tongue and lip-biting (typical of ChAc), feeding dystonia, psychiatric and cognitive disturbances, seizures, peripheral neuropathy, elevation of creatine kinase, elevation of transaminases, hepatosplenomegaly, cardiomyopathy and arrhythmias, and an X-linked pattern of inheritance (McLeod Syndrome, MLS).
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| 4. |
- Hashimoto, T., et al.
(författare)
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Beamline test of a transition-edge-sensor spectrometer in preparation for kaonic-atom measurements
- 2017
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Ingår i: IEEE Transactions on Applied Superconductivity. - 1051-8223. ; 27:4
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Tidskriftsartikel (refereegranskat)abstract
- We are developing a new technique to apply transition-edge sensors (TESs) to X-ray spectroscopy of exotic atoms, especially of kaonic atoms. To demonstrate the feasibility of this pioneering project, performance of a TES-based X-ray detector was evaluated in pion- and kaon-beam environments at particle accelerators.We successfully observed X-rays from pionic-carbon atoms with a resolution as good as 7 eV FWHM at 6 keV. Also at a kaon beamline, we confirmed that the TES spectrometer will be able to achieve our resolution goal, 6 eV, in our first scientific campaign to measure X-rays from kaonic-helium atoms.
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| 5. |
- Hashimoto, T., et al.
(författare)
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Kaonic-Atom X-ray Spectroscopy with Superconducting Microcalorimeters
- 2017
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Ingår i: Proceedings of the 12th International Conference on Hypernuclear and Strange Particle Physics (HYP2015). - 9784890271221 ; 17
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Konferensbidrag (refereegranskat)abstract
- We will measure kaonic helium X-rays using transition-edge-sensor microcalorimeters, TES, in the J-PARC hadron experimental facility. To demonstrate the feasibility of the experiment, we performed a measurement of pionic carbon X-rays at PSI, where an excellent FWHM energy resolution of 7 eV at 6.4 keV was achieved. We also evaluated the expected TES performance in the kaon beam at J-PARC. The simulation results show that the TES spectrometer would work with a good energy resolution, and clear peaks of kaonic helium X-rays would be observed on a reasonably suppressed background.
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| 6. |
- Sekiya, R., et al.
(författare)
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Search for η'-mesic nuclei in 12C(p, dp) reaction with the WASA detector at GSI-FRS
- 2024
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Ingår i: Nuovo cimento della societa italiana de fisica. C, Geophysics and space physics. - : Società Italiana di Fisica. - 1124-1896 .- 1826-9885. ; 47:4
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Tidskriftsartikel (refereegranskat)abstract
- We conducted an experimental search for η′-mesic nuclei, bound systems of an η′ meson and a nucleus, in 12C(p, dp) reactions. We measured the missing mass in the (p, d) reaction to obtain the mass spectrum of the reaction product near the η′ emission threshold. Forward-emitted deuterons were momentum-analyzed in the FRS of GSI. We installed a nearly 4π detector WASA near the 12C target to effectively select formation and decay of the η′-mesic nuclei. We are presently finalizing the analysis.
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| 7. |
- Sundal, Christina, et al.
(författare)
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Parkinsonian features in hereditary diffuse leukoencephalopathy with spheroids (HDLS) and CSF1R mutations
- 2013
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Ingår i: Parkinsonism & Related Disorders. - : Elsevier BV. - 1353-8020. ; 19:10, s. 869-877
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Tidskriftsartikel (refereegranskat)abstract
- Atypical Parkinsonism associated with white matter pathology has been described in cerebrovascular diseases, mitochondrial cytopathies, osmotic demyelinating disorders, leukoencephalopathies leukodystrophies, and others. Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is an autosomal dominant disorder with symptomatic onset in midlife and death within a few years after symptom onset. Neuroimaging reveals cerebral white matter lesions that are pathologically characterized by noninflammatory myelin loss, reactive astrocytosis, and axonal spheroids. Most cases are caused by mutations in the colony-stimulating factor 1 receptor (CSF1R) gene. We studied neuropathologically verified HDLS patients with CSF1R mutations to assess parkinsonian features. Ten families were evaluated with 16 affected individuals. During the course of the illness, all patients had at least some degree of bradykinesia. Fifteen patients had postural instability, and seven had rigidity. Two patients initially presented with parkinsonian gait and asymmetrical bradykinesia. These two patients and two others exhibited bradykinesia, rigidity, postural instability, and tremor (two with resting) early in the course of the illness. Levodopa/carbidopa therapy in these four patients provided no benefit, and the remaining 12 patients were not treated. The mean age of onset for all patients was about 45 years (range, 18-71) and the mean disease duration was approximately six years (range, 3-11). We also reviewed HDLS patients published prior to the CSF1R discovery for the presence of parkinsonian features. Out of 50 patients, 37 had gait impairments, 8 rigidity, 7 bradykinesia, and 5 resting tremor. Our report emphasizes the presence of atypical Parkinsonism in HDLS due to CSF1R mutations.
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| 8. |
- Tanaka, Y. K., et al.
(författare)
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Search for eta '-mesic nuclei using (p,d) reaction with FRS/Super-FRS at GSI/FAIR
- 2020
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Ingår i: Journal of Physics, Conference Series. - : IOP Publishing. - 1742-6588 .- 1742-6596. ; 1643
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Tidskriftsartikel (refereegranskat)abstract
- We plan a semi-exclusive measurement of the C-12(p,dp) reaction to search for eta'-mesic nuclei, aiming at investigating in-medium properties of the eta'-meson. We employ a 2.5 GeV proton beam impinging on a carbon target to produce eta'-mesic C-11 nuclei via the C-12(p,d)eta'circle times C-11 reaction. Using coincidence measurements of the forward going deuterons, important for missing-mass spectroscopy, and decay protons emitted from the eta'-mesic nuclei for event selection will provide a high experimental sensitivity to observe eta'-mesic nuclei. We will perform the measurements by combining the WASA detector system with the fragment separator FRS at GSI and also with the Super-FRS at FAIR in the future. The plan of the experiments and the present status are reported.
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| 9. |
- Dulski, J., et al.
(författare)
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Neuroacanthocytosis - Clinical variability (a report on six cases)
- 2014
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Ingår i: Movement Disorders. - : Wiley. - 0885-3185. ; 29:Suppl 1, s. 194-194
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Konferensbidrag (refereegranskat)abstract
- Objective: To provide clinical clues to differential diagnosis in patients with chorea and other movement disorders with blood acanthocytes. Background: Neuroacanthocytosis (NA) is an umbrella term for neurological conditions associated with acanthocytosis. Core NA syndromes, with basal ganglia involvement and in which acanthocytosis is a frequent finding, include autosomal recessive choreaacanthocytosis (Ch-Ac) and X-linked McLeod syndrome (MLS). Due to the very low prevalence, scarcity of data and high clinical variability they may be underdiagnosed. Methods: Six male patients (pts), three diagnosed with Ch-Ac: 33-y.o.(no.1), 35-y.o.(no.2), 42-y.o.(no.3), two diagnosed with MLS: 52-y.o.(no.4), 60-y.o.(no.5) and one 62-y.o.(no.6), a brother of no.5, with clinical suspicion of MLS. The patients had an unremarkable family history and were asymptomatic until adulthood. Pts no.1,2,4,5,6 developed generalized chorea and patient no.3 had predominant bradykinesia. Pts no.1,2,3 had phonic and motor tics, additionally pts no.1 and 2 exhibited peculiar oromandibular dystonia with tongue thrusting. In pts no.2 and 3 dystonic supination of feet was observed, patient no.3 subsequently developed bilateral foot drop. Pts no. 2 and 4 had signs of muscle atrophy. Tendon reflexes were decreased or absent and electroneurography demonstrated sensorimotor neuropathy in patients no. 1,2,3,4,5, except no. 6. Generalized seizures were seen in patients no.2,3,5,6 and myoclonic jerks in patient no 1. Cognitive deterioration was reported in patients no.1,2,3,5,6. Serum creatine kinase levels were elevated in all six patients. Results: Peripheral blood smears revealed acanthocytosis in patients no.1,2,3,5,6, except no. 4. In patients no. 1 and 3 reduced expression of chorein was detected on Western blot. In patient no. 2 genetic testing showed mutations in VPS13A gene and in no.4 and 5 genetic analysis confirmed mutations in XK gene (MLS). The time from the onset of symptoms till establishing the diagnosis in patients no. 1,2,3,4,5 was 11,5,7,6,32 years respectively. Patient no.4 suddenly developed multiple organ failure and died of cardiac arrhythmia at the age of 52. Conclusions: We highlight the variability of clinical presentation of NA syndromes and the long time from the onset to diagnosis with the need to screen the blood smears in uncertain cases, however, as in one of our cases acanthocytes may even be not found.
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| 10. |
- Labbé, Catherine, et al.
(författare)
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Role for the microtubule-associated protein tau variant p.A152T in risk of α-synucleinopathies.
- 2015
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Ingår i: Neurology. - 1526-632X. ; 85:19, s. 1680-1686
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Tidskriftsartikel (refereegranskat)abstract
- Objective:To assess the importance of MAPT variant p.A152T in the risk of synucleinopathies. Methods:In this case-control study, we screened a large global series of patients and controls, and assessed associations between p.A152T and disease risk. We included 3,229 patients with clinical Parkinson disease (PD), 442 with clinical dementia with Lewy bodies (DLB), 181 with multiple system atrophy (MSA), 832 with pathologically confirmed Lewy body disease (LBD), and 2,456 healthy controls. Results:The minor allele frequencies (MAF) in clinical PD cases (0.28%) and in controls (0.2%) were not found to be significantly different (odds ratio [OR] 1.37, 95% confidence interval [CI] 0.63-2.98, p = 0.42). However, a significant association was observed with clinical DLB (MAF 0.68%, OR 5.76, 95% CI 1.62-20.51, p = 0.007) and LBD (MAF 0.42%, OR 3.55, 95% CI 1.04-12.17, p = 0.04). Additionally, p.A152T was more common in patients with MSA compared to controls (MAF 0.55%, OR 4.68, 95% CI 0.85-25.72, p = 0.08) but this was not statistically significant and therefore should be interpreted with caution. Conclusions:Overall, our findings suggest that MAPT p.A152T is a rare low penetrance variant likely associated with DLB that may be influenced by coexisting LBD and AD pathology. Given the rare nature of the variant, further studies with greater sample size are warranted and will help to fully explain the role of p.A152T in the pathogenesis of the synucleinopathies
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