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SUMMARY A new method of system identification of high-rise buildings is proposed in which high-rise buildings are represented by a shear-bending model. The method is devised to find the story shear and bending stiffnesses of a specific story from the horizontal floor accelerations. The special characteristic of the proposed method is to use a previously derived set of closed-form expressions for the story shear and bending stiffnesses in terms of the limited floor accelerations and to utilize a reduced shear-bending model with the same number of elements as the vibration recording points. In the proposed method, a difficulty of prediction of an unstable specific function in a low-frequency range can be overcome by introducing an ARX model. It is demonstrated that the shear-bending model can simulate the vibration records with a reasonable accuracy. It is shown further that the vibration records at two super high-rise buildings during the 2011 Tohoku (Japan) earthquake can be simulated with the proposed method including a technique of adding degrees of freedom between the vibration recording points. Copyright © 2012 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Abstract: In the afternoon of March 11, 2011, the eastern Japan was severely attacked by the 2011 off the Pacific coast of Tohoku earthquake (the Great East Japan earthquake). Nearly 30,000 people were killed or are still missing by that earthquake and the ensuing monster tsunami as of April 11, 2011. This paper reports some aspects of this devastating earthquake which hit an advanced country in seismic resistant design. It has been reported that long-period ground motions were induced in Tokyo, Nagoya and Osaka. The properties of these long-period ground motions are discussed from the viewpoint of critical excitation and the seismic behavior of two steel buildings of 40 and 60 stories subjected to the long-period ground motion recorded at Shinjuku, Tokyo is determined and discussed. This paper also reports the effectiveness of visco-elastic dampers like high-hardness rubber dampers in the reduction of responses of super high-rise buildings subjected to such long-period ground motions. The response reduction rate is investigated in detail in addition to the maximum response reduction. In December 2010 before this earthquake, simulated long-period ground motions for earthquake resistant design of high-rise buildings were provided in three large cities in Japan (Tokyo, Nagoya and Osaka) and nine areas were classified. Two 40-story steel buildings (slightly flexible and stiff) are subjected to these long-period ground motions in those nine areas for the detailed investigation of response characteristics of super high-rise buildings in various areas. [Copyright &y& Elsevier]

Objective: To unveil current medical and psychosocial conditions of patients with West syndrome in Japan., Methods: A cross-sectional analysis was performed in patients with West syndrome registered in the Rare Epilepsy Syndrome Registry (RES-R) of Japan. Furthermore, new-onset patients registered in the RES-R were observed prospectively and their outcomes after one and two years of follow-up were compared with data at onset., Results: For the cross-sectional study, 303 patients with West syndrome were included. Seizures (such as spasms, tonic seizures and focal seizures) occurred daily in 69.3% of the patients at registration. Seizure frequency of less than one per year was observed in cases of unknown etiology (22.6%), genetic etiology (23.8%) and malformation of cortical development (MCD; 19.1%). Neurological findings were absent in 37.0%, but a high rate of abnormality was seen in patients with Aicardi syndrome, hypoxic-ischemic encephalopathy (HIE), genetic etiology and MCD other than focal cortical dysplasia, accompanied by a >50% rate of bedridden patients. Abnormal EEG was found in 96.7%, and CT/MRI was abnormal in 62.7%. Treatments included antiepileptic drug therapy (94.3%), hormonal therapy (72.6%), diet therapy (8.3%) and surgery (15.8%). Intellectual/developmental delay was present in 88.4%, and was more severe in patients with Aicardi syndrome, genetic etiology and HIE. Autism spectrum disorder was found in 13.5%. For the longitudinal study, 27 new-onset West syndrome patients were included. The follow-up study revealed improved seizure status after two years in 66.7%, but worsened developmental status in 55.6%, with overall improvement in 51.9%., Significance: The study reveals the challenging neurological, physical and developmental aspects, as well as intractable seizures, in patients with West syndrome. More than a half of the children showed developmental delay after onset, even though seizures were reduced during the course of the disease.

Although there are many known Mendelian genes linked to epileptic or developmental and epileptic encephalopathy (EE/DEE), its genetic architecture is not fully explained. Here, we address this incompleteness by analyzing exomes of 743 EE/DEE cases and 2366 controls. We observe that damaging ultra-rare variants (dURVs) unique to an individual are significantly overrepresented in EE/DEE, both in known EE/DEE genes and the other non-EE/DEE genes. Importantly, enrichment of dURVs in non-EE/DEE genes is significant, even in the subset of cases with diagnostic dURVs (P = 0.000215), suggesting oligogenic contribution of non-EE/DEE gene dURVs. Gene-based analysis identifies exome-wide significant (P = 2.04 × 10 -6 ) enrichment of damaging de novo mutations in NF1, a gene primarily linked to neurofibromatosis, in infantile spasm. Together with accumulating evidence for roles of oligogenic or modifier variants in severe neurodevelopmental disorders, our results highlight genetic complexity in EE/DEE, and indicate that EE/DEE is not an aggregate of simple Mendelian disorders.