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Characteristics of South Korean Patients with Hereditary Transthyretin Amyloidosis

Choi K, Seok JM, Kim BJ, Choi YC, Shin HY, Sunwoo IN, Kim DS, Sung JJ, Lee GY, Jeon ES, Kim NH, Min JH, Oh J

BACKGROUND AND PURPOSE: This retrospective cross-sectional study included 18 patients from unrelated families harboring mutations of the transthyretin gene (TTR), and analyzed their characteristics and geographical distribution in South Korea. METHODS:...
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Repetitive Nerve Stimulation in MuSK-Antibody-Positive Myasthenia Gravis

Kim SW, Sunwoo MK, Kim SM, Shin HY, Sunwoo IN

BACKGROUND AND PURPOSE: Responses to repetitive nerve stimulation (RNS) in patients with muscle-specific tyrosine kinase (MuSK) antibody (Ab)-positive myasthenia gravis (MG) vary depending on the muscles tested. We analyzed the...
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Muscle-specific receptor tyrosine kinase (MuSK) myasthenia gravis associated with castleman disease

Oh J, Yang WI, Cho JH, Sunwoo IN

Muscle specific tyrosine kinase (MuSK) myasthenia gravis (MG) is a rare subtype of MG, which is immunologically distinct and differential therapeutic response. Though MG is often associated with other autoimmune...
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Decremental Responses to Repetitive Nerve Stimulation in X-Linked Bulbospinal Muscular Atrophy

Kim JY, Park KD, Kim SM, Sunwoo IN

BACKGROUND AND PURPOSE: X-linked bulbospinal muscular atrophy (X-BSMA) is characterized by bulbar and spinal muscular weakness and fasciculations. Although X-BSMA is a motor neuronopathy, there are several reports of myasthenic...
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Erratum: Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

Kim YH, Chung HK, Park KD, Choi KG, Kim SM, Sunwoo IN, Choi YC, Lim JG, Lee KW, Kim KK, Lee DK, Joo IS, Kwon KH, Gwon SB, Park JH, Kim DS, Kim SH, Kim WK, Suh BC, Kim SB, Kim NH, Sohn EH, Kim OJ, Kim HS, Cho JH, Kang SY, Park CI, Oh J, Shin JH, Chung KW, Choi BO

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.
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Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

Kim YH, Chung HK, Park KD, Choi KG, Kim SM, Sunwoo IN, Choi YC, Lim JG, Lee KW, Kim KK, Lee DK, Joo IS, Kwon KH, Gwon SB, Park JH, Kim DS, Kim SH, Kim WK, Suh BC, Kim SB, Kim NH, Sohn EH, Kim OJ, Kim HS, Cho JH, Kang SY, Park CI, Oh J, Shin JH, Chung KW, Choi BO

  • KMID: 2425232
  • J Clin Neurol.
  • 2012 Jun;8(2):139-145.
BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities...
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Heterogeneous Characteristics of Korean Patients with Dysferlinopathy

Park HJ, Hong JM, Suh GI, Shin HY, Kim SM, Sunwoo IN, Suh BC, Choi YC

Dysferlinopathy is caused by mutations in the DYSF gene. To characterize the clinical spectrum, we investigated the characteristics of 31 Korean dysferlinopathy patients confirmed by immunohistochemistry. The mean age of...
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A Case of Acute Polyneuropathy with Nephrotic Syndrome Showing Transient Proximal Sensory Conduction Defects

Oh J, Kim SM, Sunwoo IN

Acute sensorimotor polyneuropathy that resembles Guillain-Barre syndrome (GBS) is rarely accompanied with nephrotic syndrome, and its underlying immunological mechanisms are unclear. A 56-year-old man presented with simultaneous acute progressive symmetric...
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Sequential Involvement of the Nervous System in Subacute Combined Degeneration

Minn YK, Kim SM, Kim SH, Kwon KH, Sunwoo IN

PURPOSE: Subacute combined degeneration (SCD) involves progressive degeneration of the spinal cord, optic nerve, and peripheral nerves. Vitamin B12 (VB12) is a co-factor in myelin synthesis. Because each cell that...
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A Case of Lambert-Eaton Myasthenic Syndrome with Small-Cell Lung Cancer and Transient Increase in Anti-Acetylcholine-Receptor-Binding Antibody Titer

Lee JH, Shin HY, Kim SM, Sunwoo IN

BACKGROUND: Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic neuromuscular junction disorder that is most frequently associated with small-cell lung cancer (SCLC). The titers of antibodies against voltage-gated calcium channels are...
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Myopathy With Anti Signal Recognition Particle Antibody

Hong HJ, Sunwoo IN, Shin HY, Kim SH, Cho JH, Kim SM

  • KMID: 2343411
  • J Korean Neurol Assoc.
  • 2011 Aug;29(3):231-233.
Myopathies associated with anti-signal-recognition particle (SRP) antibodies usually present with severe muscle weakness and exhibit necrotizing myopathy with little inflammation pathologically. Here we report a case of a 61-year-old man...
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Repetitive Nerve Stimulation Test in Amyotrophic Lateral Sclerosis with Predominant Oropharyngeal Manifestations

Kim JY, Park KD, Kim SM, Sunwoo IN

BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis (ALS) patients display easy fatigability and abnormal decrements on repetitive nerve stimulation (RNS) test of clinically involved limb muscles, which can result in ALS...
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Kennedy Disease Showing Prominent Remyelinating Features of Sural Nerve

Kim JY, Kim SM, Park KD, Kim TS, Sunwoo IN

  • KMID: 2065726
  • J Korean Neurol Assoc.
  • 2010 May;28(2):115-118.
We report a 55-year-old man with chronic weakness of both legs with recently experienced nasal voice. Despite the absence of sensory symptoms, electrophysiologic studies revealed the presence of sensorimotor polyneuropathy....
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Clinical Heterogeneity in Korean Patients with Nemaline Myopathy

Hong JM, Kim SM, Sunwoo IN, Kim SH, Kim TS, Shim DS, Choi YC

PURPOSE: Nemaline myopathy (NM) is a clinical heterogeneous congenital myopathy characterized by the presence of subsarcolemmal or cytoplasmic rod-like structures that call nemaline bodies in the muscle fibers. The purpose...
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Intravascular Lymphomatosis of the Central Nervous System Presenting Progressive Paraparesis

Lee JJ, Sunwoo IN, Park B, Kim EH, Koh HS, Kim SM

  • KMID: 1454837
  • J Korean Neurol Assoc.
  • 2010 Feb;28(1):70-72.
No abstract available.
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Iatrogenic Botulism After Intramuscular Injection of Botulinum Toxin Type A

Yoo JS, Sunwoo IN, Shin HY, Koh HS, Ye BS, Kim SM

  • KMID: 1454829
  • J Korean Neurol Assoc.
  • 2010 Feb;28(1):47-49.
Botulinum toxin type A (BTA) is widely used for both medical treatment and cosmetic purposes. A 46-year-old woman presented with progressive generalized weakness and dysphagia. The patient had injected BTA...
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Clinical Characteristics and Outcomes of Juvenile and Adult Dermatomyositis

Na SJ, Kim SM, Sunwoo IN, Choi YC

Dermatomyositis (DM) is an idiopathic inflammatory myopathy with bimodal onset age distribution. The age of onset is between 5-18 yr in juvenile DM and 45-64 yr in adult DM. DM...
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Clinical Features and Genetic Analysis of Fascioscapulohumeral Muscular Dystrophy

Hong JM, Kim SM, Sunwoo IN, Seo KD, Shim DS, Suh BC, Kim DS, Cho JH, Choi YC

  • KMID: 2343353
  • J Korean Neurol Assoc.
  • 2009 Feb;27(1):42-48.
BACKGROUND: Facioscapulohumeral muscular dystrophy (FSHD) is associated with contractions of the polymorphic D4Z4-repeat array in 4q35 and has the distinctive clinical presentation of an initial involvement of the facial, shoulder-girdle,...
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Two Cases of Delayed Post-radiation Lower Cranial Nerve Palsies

Park HJ, Kwon KH, Min YK, Suh BC, Shim DS, Kim SM, Sunwoo IN

  • KMID: 2343311
  • J Korean Neurol Assoc.
  • 2008 Feb;26(1):55-58.
Radiation-induced lower cranial neuropathy shows a clinical presentation similar to tumor recurrence or amyotrophic lateral sclerosis. We experienced two patients with bulbar palsies several years after radiotherapy for nasopharyngeal cancer....
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The Clinical and Pathological Characteristics of Polymyositis Using ENMC Diagnostic Criteria

Oh SH, Kim SM, Sunwoo IN, Lee DH, Kim TS, Kim SH, Choi YC

OBJECTIVE: Polymyositis (PM) has known to be the most common type of idiopathic inflammatory myopathy (IIM). However, recent immunopathological studies demonstrated that PM was overdiagnosed previously due to...
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