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Shen-Yang LIM,Ai Huey Tan,Azlina Ahmad Annuar,Susanne A. Schneider,Ping Chong Bee,Jia Lun Lim,Norlisah Ramli,Mohamad Imran Idris 대한파킨슨병및이상운동질환학회 2018 Journal Of Movement Disorders Vol.11 No.2
We present a case of beta-propeller protein-associated neurodegeneration, a form of neurodegeneration with brain iron accumulation. The patient harbored a novel mutation in the WDR45 gene. A detailed video and description of her clinical condition are provided. Her movement disorder phenomenology was characterized primarily by limb stereotypies and gait dyspraxia. The patient’s disability was advanced by the time iron-chelating therapy with deferiprone was initiated, and no clinical response in terms of cognitive function, behavior, speech, or movements were observed after one year of treatment.
Purposeless Groaning in Parkinson's Disease
Shen-Yang LIM,Ai Huey Tan,Jia Lun Lim,Azlina Ahmad Annuar 대한파킨슨병및이상운동질환학회 2018 Journal Of Movement Disorders Vol.11 No.2
Purposeless groaning has been reported in advanced progressive supranuclear palsy. We present a case of purposeless groaning occurring as a primary complaint in a patient with advanced Parkinson's disease. Purposeless groaning is thought to be a manifestation of disinhibition and perseveration, due to frontal-subcortical dysfunction. Proper recognition of this phenomenon will help clinicians to avoid unnecessary investigations and treatment. (e.g., prescription of opioid medications).
Ai Huey Tan,Shen-Yang LIM 대한파킨슨병및이상운동질환학회 2023 Journal Of Movement Disorders Vol.16 No.2
Patients with Parkinson’s disease (PD) face a multitude of gastrointestinal (GI) symptoms, including nausea, bloating, reduced bowel movements, and difficulties with defecation. These symptoms are common and may accumulate during the course of PD but are often under-recognized and challenging to manage. Objective testing can be burdensome to patients and does not correlate well with symptoms. Effective treatment options are limited. Evidence is often based on studies in the general population, and specific evidence in PD is scarce. Upper GI dysfunction may also interfere with the pharmacological treatment of PD motor symptoms, which poses significant management challenges. Several new less invasive assessment tools and novel treatment options have emerged in recent years. The current review provides an overview and a practical approach to recognizing and diagnosing common upper and lower GI problems in PD, e.g., dyspepsia, gastroparesis, small bowel dysfunction, chronic constipation, and defecatory dysfunction. Management aspects are discussed based on the latest evidence from the PD and general populations, with insights for future research pertaining to GI dysfunction in PD.
임대윤(Dae Yun Lim),김주영(Ju Young Kim),신윤덕(Yun De Shen),황인철(In Choul Whang),양성모(Sung Mo Yang),기창두(Chang Doo Kee) 대한기계학회 2006 대한기계학회 춘추학술대회 Vol.2006 No.11
The bio-wrap used in our experiments has many good properties including high tensile strength and ductility than widely used polyethylene wrap. However, the cost of the wrap material is very expensive and it requires very precise control to get uniform-thin film. In general, PID control does work for this kind of wrap machine, but very thin film is very sensitive to the environmental changes of temperature and humidity, etc, and has highly nonlinear characteristics. This paper suggests Hybrid-fuzzy controller which controls the system in the every possible case and make a database to the temperature and humidity of the wrap.
Alfand Marl F. Dy Closas,Shen-Yang LIM 대한파킨슨병및이상운동질환학회 2023 Journal Of Movement Disorders Vol.16 No.1
KMT2B-linked dystonia (DYT-KMT2B) is a childhood-onset dystonia syndrome typically beginning in the lower limbs and progressing caudocranially to affect the upper limbs with eventual prominent craniocervical involvement. Despite its recent recognition, it now appears to be one of the more common monogenic causes of dystonia syndromes. Here, we present an atypical case of DYT-KMT2B with oromandibular dystonia as the presenting feature, which remained restricted to this region three decades after symptom onset. This appears to be the first reported case of DYT-KMT2B from Southeast Asia and provides further supporting evidence for the pathogenic impact of the KMT2B c.6210_6213delTGAG variant.
Sharifah Azira Taufik,Norlina Ramli,Ai Huey Tan,Shen-Yang Lim,Mohd Taufiq Abdul Ghani,Nortina Shahrizaila 대한신경과학회 2024 Journal of Clinical Neurology Vol.20 No.3
Background and Purpose There is increasing evidence that the anterior visual pathways are involved in neurodegenerative diseases including amyotrophic lateral sclerosis (ALS) and Parkinson’s disease (PD). This study investigated longitudinal changes in retinal nerve fiber layer (RNFL) thickness in patients with ALS and PD with the aim of better understanding their roles as biomarkers of disease progression. Methods This study recruited 21 ALS patients, 19 age-matched PD patients, and 21 agematched healthy controls. Patient demographics and clinical scores relating to the respective diseases were documented. The RNFL thickness was measured using optical coherence tomography at baseline and after 6 months. Results At baseline, the RNFL in the superior quadrant was significantly thinner in the patients with ALS than in healthy controls (109.90±22.41 μm vs. 127.81±17.05 μm [mean±standard deviation], p=0.008). The RNFL thickness did not differ significantly between the ALS and PD patients or between the PD patients and healthy controls. At 6 months, there was further significant RNFL thinning in patients with ALS, for both the overall thickness (baseline: median=94.5 μm, range=83.0–106.0 μm; follow-up: median=93.5 μm, range=82.5–104.5 μm, p=0.043) and the thickness in the inferior quadrant (median=126 μm, range=109.5–142.5 μm; and median= 117.5 μm, range=98.5–136.5 μm; respectively, p=0.032). However, these changes were not correlated with the ALS functional scores. In contrast, the patients with PD did not demonstrate a significant change in RNFL thickness between the two time points. Conclusions The RNFL thickness is a promising biomarker of disease progression in patients with ALS but not in those with PD, which has a slower disease progression.
Historical and More Common Nongenetic Movement Disorders From Asia
Norlinah Mohamed Ibrahim,Priya Jagota,Pramod Kumar Pal,Roongroj Bhidayasiri,Shen-Yang LIM,Yoshikazu Ugawa,Zakiyah Aldaajani,Beomseok Jeon,Shinsuke Fujioka,Jee-Young Lee,Prashanth Lingappa Kukkle,Huifa 대한파킨슨병및이상운동질환학회 2023 Journal Of Movement Disorders Vol.16 No.3
Nongenetic movement disorders are common throughout the world. The movement disorders encountered may vary depending on the prevalence of certain disorders across various geographical regions. In this paper, we review historical and more common nongenetic movement disorders in Asia. The underlying causes of these movement disorders are diverse and include, among others, nutritional deficiencies, toxic and metabolic causes, and cultural Latah syndrome, contributed by geographical, economic, and cultural differences across Asia. The industrial revolution in Japan and Korea has led to diseases related to environmental toxin poisoning, such as Minamata disease and β-fluoroethyl acetate-associated cerebellar degeneration, respectively, while religious dietary restriction in the Indian subcontinent has led to infantile tremor syndrome related to vitamin B12 deficiency. In this review, we identify the salient features and key contributing factors in the development of these disorders.
Si Lei Fong,Ai Huey Tan,Kar Foo Lau,Norlisah Ramli,Shen-Yang LIM 대한파킨슨병및이상운동질환학회 2019 Journal Of Movement Disorders Vol.12 No.3
Hemichorea-hemiballismus is characterized by unilateral, brief, unpredictable involuntary movements of one side of the body (upper and lower limbs and sometimes also affecting the face). The most common causes for this condition are strokes, hyperglycemia-associated hemichorea-hemiballismus, and other focal lesions affecting the basal ganglia