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Change in Cationic Amino Acid Transport System and Effect of Lysine Pretreatment on Inflammatory State in Amyotrophic Lateral Sclerosis Cell Model

Latif S, Kang YS

Amyotrophic lateral sclerosis (ALS) is a lethal neurological disorder characterized by the deterioration of motor neurons. The aim of this study was to investigate alteration of cationic amino acid transporter...
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Clinical Scales Used in Motor Neuron Disease

Oh Si, Park JS, Sung JJ, Kim SH

Motor neuron diseases (MND) are heterogeneous spectra of disorders that that primarily affect the motor neurons (MN) resulting in motor nerve and muscle degeneration. The pathophysiological mechanisms of MN cell...
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Clinical Characteristics of Korean Juvenile Amyotrophic Lateral Sclerosis

Lee S, Park J, Oh KW, Kim SH

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron degeneration with phenotypic heterogeneity, including age at onset. Juvenile ALS (JALS) includes ALS patients aged less...
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Duodenoduodenal Intussusception due to a Migrated Percutaneous Radiologic Gastrostomy Tube: A Case Report

Hong JW, Kim SH, Yoon JA, Shin MJ, Shin YB

Patients with dysphagia often have difficulty in supplying adequate nutrition orally, and thus they often use gastrostomy for nutrition support. If the nutrition affects the deterioration of the disease, as...
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Motor dominant polyradiculopathy with Primary Sjögren's syndrome mimicking motor neuron disease

Ahn SW, Yoon BN

Sjögren's syndrome (SS)-associated polyradiculopathy is rarely reported. A 51-year-old woman presented with a history of gradual weakness in all four extremities for several months. Based on electrophysiological studies, spinal magnetic...
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Unilateral Hypoglossal Neuropathy in Multifocal Acquired Demyelinating Sensory and Motor Neuropathy: Differential Diagnosis of Motor Neuron Disease

Yoo SK, Kim MO, Kim CH, Kim YS, Kim SK, Kang H, Choi NC, Kwon OY, Lim BH, Park KH

Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is a peripheral neuropathy characterized by multifocal weakness and associated sensory impairment. MADSAM is associated with multifocal persistent conduction block and other...
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Applicability of the digital instrument to improve the reproducibility of motor unit number index

Ahn SW

BACKGROUND: The motor unit number index (MUNIX) and motor unit size index (MUSIX) refer to the electrophysiological measurement of the motor units using the surface electromyographic interference pattern (SIP) recorded...
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Percutaneous Endoscopic Gastrostomy Tube Insertion in Neurodegenerative Disease: A Retrospective Study and Literature Review

Sarkar P, Cole A, Scolding N, Rice CM

BACKGROUND/AIMS: With the notable exceptions of dementia, stroke, and motor neuron disease, relatively little is known about the safety and utility of percutaneous endoscopic gastrostomy (PEG) tube insertion in patients...
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Motor Neuron Disease Presenting With Acute Respiratory Failure: A Case Study

Oh H, Kang SW, Choi WA, Lee JW, Suh M, Kim EY

Motor neuron diseases (MNDs) refer to a heterogeneous group of progressive neurologic disorders caused by degeneration of motor neurons. The diseases affect either the upper motor neurons, lower motor neurons,...
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Frontotemporal Dementia with Motor Neuron Disease in a Patient with Antiphospholipid Syndrome: A Case Report

Jeong YC, Park JS, Kim SH, Choi H

BACKGROUND: Frontotemporal dementia (FTD) with motor neuron disease (MND) is a syndrome of progressive changes in behavior, language, muscle weakness and atrophy due to loss of function of neurons in...
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An Autopsy Case of Frontotemporal Dementia with Motor Neuron Disease

Kim EJ, Oh EH, Kim KT, Jung Y, Lee JH, Lee JH, Lee YM, Kim SJ, Shin JH, Shin MJ, Lee MJ, Ahn JW, Sung S, Choi KU, Jung DS, Seeley WW, Huh GY

Approximately 15% of patients with frontotemporal dementia (FTD) have co-occurring motor neuron disease (MND). FTD-MND cases have frontotemporal lobar degeneration (FTLD)-transactive response DNA-binding protein (TDP) pathology, which is divided into...
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Comparison between Flail Arm Syndrome and Upper Limb Onset Amyotrophic Lateral Sclerosis: Clinical Features and Electromyographic Findings

Yoon BN, Choi SH, Rha JH, Kang SY, Lee KW, Sung JJ

Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or...
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Diffusion Tensor Tractography Analysis of the Corpus Callosum Fibers in Amyotrophic Lateral Sclerosis

Kim JE, Oh JS, Sung JJ, Lee KW, Song IC, Hong YH

BACKGROUND AND PURPOSE: Involvement of the corpus callosum (CC) is reported to be a consistent feature of amyotrophic lateral sclerosis (ALS). We examined the CC pathology using diffusion tensor tractography...
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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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A Case of Motor Neuron Disease Presenting as Dyspnea in the Emergency Department

Park HS

Motor neuron disease encompasses a group of progressive neurologic disorders that destroy cells responsible for the control of essential muscles. The disorders are characterized by progressive weakness, muscle atrophy and...
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The Usefulness of Lumbar Spine MRI for Cauda Equina Syndrome

Moon TY, Baik SK, Lee IS

PURPOSE: To understand the usefulness of the lumbar MRI studies to establish therapeutic plans for cauda equina syndrome (CES) including the management of rectal and bladder dysfunction symptoms. MATERIALS AND METHODS:...
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Severe Amnestic Dementia With Motor Neuron Disease

Kim EJ, Ahn BY, Kim DS, Kim SJ, Na DL, Kim JS, Lee JH

  • KMID: 2343358
  • J Korean Neurol Assoc.
  • 2009 Feb;27(1):64-67.
Motor neuron disease (MND) and frontotemporal dementia often appear together. We report on a 74-year-old woman who presented with a 18-month history of memory deterioration and MND. Her initial clinical...
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General anesthesia for laparoscopic cholecystectomy in a patient with Kennedy's disease: A case report

Kim JH, Hwang JW, So YM, Ryu JH, Jeon YT, Do SH

Kennedy's disease is a rare lower motor neuron disease affecting the limbs and bulbar musculature. Regional anesthesia is generally the recommended anesthetic technique in patients with Kennedy's disease because of...
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Atypical Form of Motor Neuron Disease: Brachial Amyotrophic Diplegia

Park JY, Chun MH, You JY, Kim DY, You DS

  • KMID: 1481842
  • J Korean Geriatr Soc.
  • 2008 Jun;12(2):107-111.
Brachial amyotrophic diplegia is a rare disease presenting with severe weakness that is completely confined to the upper limbs over time without upper motor neuron sign. It might be a...
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A Case Report of Lou Gehrig's Disease Presenting with Rhinolalia Aperta in Its Early Stage

Kang HS, Yoon MH, Kim JP, Jeon SY

  • KMID: 2276976
  • Korean J Otorhinolaryngol-Head Neck Surg.
  • 2008 Dec;51(12):1151-1154.
Lou Gehrig's disease, also called amyotrophic lateral sclerosis, is a chronic progressive motor neuron disease of an unknown etiology, characterized by upper and lower motor neuron degeneration with a resultant...
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