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Health-related quality of Life Instrument in Neuromuscular Disorders

Kwon O

Medicine places its ultimate purpose at prolonging people’s healthy lives. Health-related quality of life (HRQoL) has emerged as one of the key measures of medical practice. HRQoL, the most important...
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Clinical Scales for the Evaluation of Myopathy Patients

Park HJ, Choi YC

With the rapid increase in the number of clinical trials in myopathy over the past decade, there is an increasing need for clinical scales to reflect patient’s clinical status. This...
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Pulmonary and Physical Rehabilitation in Critically Ill Patients

Jang MH, Shin MJ, Shin YB

Some patients admitted to the intensive care unit (ICU) because of an acute illness, complicated surgery, or multiple traumas develop muscle weakness affecting the limbs and respiratory muscles during acute...
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Basic concepts of needle electromyography

Kim JE, Seok JM, Ahn SW, Yoon BN, Lim YM, Kim KK, Kwon KH, Park KD, Suh BC, and on behalf of the Korean Society of Clinical Neurophysiology Education Committee

Clinical evaluations, nerve conduction studies, and electromyography play major complementary roles in electrophysiologic diagnoses. Electromyography can be used to assess pathologic changes and localize lesions occurring in locations ranging from...
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Critical Illness Neuromyopathy Complicating Cardiac Surgery

Baek WK, Kim YS, Kim JT, Yoon BN

Critical illness neuromyopathy (CINM) is a sporadically reported disease in the setting of an intensive care unit developing in the process of managing a critical illness. The disease primarily affects...
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Improvement of Peak Cough Flow After the Application of a Mechanical In-exsufflator in Patients With Neuromuscular Disease and Pneumonia: A Pilot Study

Jung JH, Oh HJ, Lee JW, Suh MR, Park J, Choi WA, Kang SW

OBJECTIVE: To investigate and demonstrate persistent increase of peak cough flow after mechanical in-exsufflator application, in patients with neuromuscular diseases and pneumonia. METHODS: A mechanical in-exsufflator was applied with patients in...
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Perioperative Anesthetic Management of a Patient with Dermatomyositis

Koo BS

Dermatomyositis is a relatively rare myopathy which is characterized by muscle weakness and chronic inflammatory changes in muscle and skin. Dermatomyositis typically presents with subacute, progressive proximal muscle weakness, a...
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Integrated diagnostic approach of pediatric neuromuscular disorders

Lee HN, Lee YM

Clinical and genetic heterogeneity in association with overlapping spectrum is characteristic in pediatric neuromuscular disorders, which makes confirmative diagnosis difficult and time consuming. Considering evolution of molecular genetic diagnosis and...
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Noninvasive Respiratory Management of Patients With Neuromuscular Disease

Bach JR

This review article describes definitive noninvasive respiratory management of respiratory muscle dysfunction to eliminate need to resort to tracheotomy. In 2010 clinicians from 22 centers in 18 countries reported 1,623...
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Clinical, Pathologic, and Genetic Features of Collagen VI-Related Myopathy in Korea

Lee JH, Shin HY, Park HJ, Kim SH, Kim SM, Choi YC

BACKGROUND AND PURPOSE: Mutations in collagen VI-related genes (COL6A1, COL6A2, and COL6A3) cause Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). These were previously believed to be separate disease...
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Heart Disease in Disorders of Muscle, Neuromuscular Transmission, and the Nerves

Finsterer , Stöllberger C

Little is known regarding cardiac involvement (CI) by neuromuscular disorders (NMDs). The purpose of this review is to summarise and discuss the major findings concerning the types, frequency, and severity...
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Comparison of Pulmonary Functions at Onset of Ventilatory Insufficiency in Patients With Amyotrophic Lateral Sclerosis, Duchenne Muscular Dystrophy, and Myotonic Muscular Dystrophy

Cho HE, Lee JW, Kang SW, Choi WA, Oh H, Lee KC

OBJECTIVE: To evaluate pulmonary functions of patients with amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and myotonic muscular dystrophy (MMD) at the onset of ventilatory insufficiency. METHODS: This retrospective study...
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Quantitative Analysis of Magnetic Resonance Imaging of the Neck and Its Usefulness in Management of Congenital Muscular Torticollis

Kim JW, Kim SH, Yim SY

OBJECTIVE: To quantify magnetic resonance imaging (MRI) findings of congenital muscular torticollis (CMT) and to demonstrate the usefulness of quantitative MRI findings in the management of CMT. METHODS: This was a...
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A Rare Manifestation of Hypothyroid Myopathy: Hoffmann's Syndrome

Lee KW, Kim SH, Kim KJ, Kim SH, Kim HY, Kim BJ, Kim SG, Choi DS

Hypothyroid myopathy is observed frequently and the resolution of the clinical manifestations of myopathy following thyroid hormone replacement is well known. However, a specific subtype of hypothyroid myopathy, Hoffmann's syndrome,...
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Efficacy of Entecavir Switching Therapy in Chronic Hepatitis B Patients with Clevudine-induced Myopathy

Lee JW, Lee YJ, Lee JJ, Kim JH, Jung YK, Kwon OS, Choi DJ, Kim YS, Kim JH

BACKGROUND/AIMS: Clevudine is a potent antiviral agent against HBV. However, long-term clevudine therapy may cause myopathy. This study was carried out to identify the efficacy of entecavir switching therapy in...
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Long-Term Treatment Efficacy and Safety of Clevudine Therapy in Naive Patients with Chronic Hepatitis B

Choung BS, Kim IH, Jeon BJ, Lee S, Kim SH, Kim SW, Lee SO, Lee ST, Kim DG

  • KMID: 2167421
  • Gut Liver.
  • 2012 Oct;6(4):486-492.
BACKGROUND/AIMS: Clevudine (CLV) has potent antiviral activity against chronic hepatitis B (CHB) virus infection. The long-term efficacy and safety of CLV therapy in naive patients with CHB were investigated. METHODS: In...
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Carrier Woman of Duchenne Muscular Dystrophy Mimicking Inflammatory Myositis

Yoon J, Kim SH, Ki CS, Kwon MJ, Lim MJ, Kwon SR, Joo K, Moon CG, Park W

Carrier woman of Duchenne muscular dystrophy (DMD) can mimic the inflammatory myositis in presenting symptoms. Two diseases should be differentiated by the clinical history, muscle biopsy and genetic study. There...
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Myopathy Following Ingestion of Ma-huang (Ephedra)-based Herbal Remedy

Baek JH, Suh BC, Kim YB, Chung PW, Moon HS, Jin DK, Kim BJ, Park YS

  • KMID: 2191931
  • J Korean Neurol Assoc.
  • 2009 Nov;27(4):424-427.
Ma-huang (Ephedra sinica) is a herb that exhibits sympathomimetic activity. We report a patient with acquired myopathy following ingestion of a Ma-huang-based herbal remedy for 6 months. A 50-year-old woman...
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Clinical and Genetic Analysis of Korean Patients with Miyoshi Myopathy: Identification of Three Novel Mutations in the DYSF Gene

Cho HJ, Sung DH, Kim EJ, Yoon CH, Ki CS, Kim JW

Miyoshi myopathy (MM) is an autosomal recessive distal muscular dystrophy caused by mutations in the dysferlin gene (DYSF) on chromosome 2p13. Although MM patients and their mutations in the DYSF...
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Tubular Aggregate Myopathy: A Case Report

Suh YL, Kim NR

We report a first Korean case of presumably dominantly inherited primary tubular aggregate myopathy in a 19-yr-old man, who presented with slowly progressive proximal muscle stiffness and weakness. In hematoxylin...
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