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Diagnostic challenge for the rare lysosomal storage disease: Late infantile GM1 gangliosidosis
Lee, Jin Sook,Choi, Jong-Moon,Lee, Moses,Kim, Soo Yeon,Lee, Sangmoon,Lim, Byung Chan,Cheon, Jung-Eun,Kim, In-One,Kim, Ki Joong,Choi, Murim,Seong, Moon-Woo,Chae, Jong-Hee Elsevier 2018 Brain and Development Vol.40 No.5
<P><B>Abstract</B></P> <P><B>Background</B></P> <P>GM1 gangliosidosis is a rare lysosomal storage disorder caused by GLB1 mutations. Because of its extreme rarity and symptoms that overlap with other neurodegenerative diseases, its diagnosis is sometimes challenging, especially in the late infantile form with less severe phenotype. We aim to expand the clinical and genetic spectrum of late infantile GM1 gangliosidosis.</P> <P><B>Methods</B></P> <P>We confirmed a diagnosis of GM1 gangliosidosis based on <I>GLB1</I> mutations and/or the deficiency of β-galactosidase activity. We identified the first two cases by whole-exome sequencing, and then the other six cases by direct sequencing of <I>GLB1</I> with enzyme analysis.</P> <P><B>Results</B></P> <P>All eight patients presented with developmental delay or regression during late infancy and later developed epilepsy, mostly intractable generalized tonic seizures. No clinical signs of storage disorders were noted except for skeletal abnormalities. Interestingly, we found aspartate transaminase (AST) elevations alone with normal alanine transaminase (ALT) levels in all patients. The recurrent mutation, p.D448V in GLB1, accounted for 50.0% of total alleles in our cohort.</P> <P><B>Conclusions</B></P> <P>With a high index of clinical suspicion, skeletal survey and AST level would be important for early diagnosis of GM1 gangliosidosis. In addition, we would highlight the clinical usefulness of whole-exome sequencing in the diagnosis of non-classical presentation of ultra-rare neurodegenerative disease in children.</P>
The Global Economy and Entrepreneurship
Lee, Sangmoon 明知大學校 經濟硏究所 1998 경영연구 Vol.17 No.1
The emergence of boundaryless global economy has had dramatic impact on the standard of living in almost every country around the globe. The growth rate of global income during the past five years is unprecedented in scope and scale. There is, however, the dark side of global economy. The global economy is totally ruthless to economies or organizations that do not provide values to the customer as the current economic crisis in Asia attests. In the turbulent and fast changing global economy, innovation and speed are important sustainable competencies. Consequently, creative entrepreneurs are becoming important ingredients of a healthy and growing economy.
Cleansed PHAT GCC based Sound Source Localization
Sangmoon Lee,Youngjin Park,Youn-sik Park 제어로봇시스템학회 2010 제어로봇시스템학회 국제학술대회 논문집 Vol.2010 No.10
Sound source localization (SSL) is one of the important techniques used in various engineering fields such as monitoring and surveillance system, and so on. When SSL is utilized in robot auditory system, it is essential for Human-Robot Interaction (HRI) in that robot can recognize its working environment and what direction a speaker is locating. Generalized cross correlation (GCC) function has been frequently used for SSL due to its fast computation and less requirement of high system resources. Artificial ear which is the robot auditory hardware we are dealing with consists of 2 microphones and a single pinna structure. This is locating on the both sides of robot head where free field condition can’t be applicable due to reflected wave caused by robot platform such as robot shoulder, etc. This paper has focused on modification of spatially mapped GCC function, which was proposed by B. Kwon, for application into artificial ear structure. We proposed cleansed PHAT GCC function for use in spatially mapped GCC method. Cleansed PHAT GCC function combines cleansing method and conventional PHAT GCC function for eliminating reflected wave which causes free field condition break down. Simulation results using proposed cleansed PHAT GCC function showed that 93% SSL performance is obtainable when a single sound source exists.
Quantised MPC for LPV systems by using new Lyapunov–Krasovskii functional
Lee, Sangmoon,Kwon, Ohmin IET 2017 IET CONTROL THEORY AND APPLICATIONS Vol.11 No.3
<P>This study deals with the problem of sampled-data model predictive control (MPC) for linear parameter varying (LPV) systems with input quantisation. The LPV systems under consideration depend on a set of parameters that are bounded and available online. To deal with a piecewise constant sampled-data and quantisation of the control input, the closed-loop system is modelled as a continuous-time impulsive dynamic model with sector non-linearity. The control problem is formulated as a minimisation of the upper bound of infinite horizon cost function subject to a sufficient condition for stability. The stability of the proposed MPC is guaranteed by constructing new Lyapunov-Krasovskii functional. Finally, a numerical example is provided to illustrate the effectiveness and benefits of the proposed theoretical results.</P>
H <SUB>∞</SUB> Filtering for a Class of Nonlinear Systems with Interval Time-varying Delay
Sangmoon Lee(이상문),Yajuan Liu(유아연) 대한전기학회 2014 전기학회논문지 Vol.63 No.4
In this paper, a delay-dependent H∞ filtering problem is investigated for discrete-time delayed nonlinear systems which include a more general sector nonlinear function instead of employing the commonly used Lipschitz-type function. By using the Lyapunov-Krasovskii functional approach, a less conservative sufficient condition is established for the existence of the desired filter, and then, the corresponding solvability condition guarantee the stability of the filter with a prescribed H∞ performance level. Finally, two simulation examples are given to show the effectiveness of the proposed filtering scheme.