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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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Percutaneous radiologic gastrostomy in patients with amyotrophic lateral sclerosis: A safe and effective technique

Xu C, Yang ZQ, Liu S, Yang W, Shi HB, Zhou WZ

Background: Percutaneous radiologic gastrostomy (PRG) was considered as an alternative technique for long-term enteral nutrition, and the current study is aimed to evaluate the feasibility and safety of this technique...
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Clinical Course of Amyotrophic Lateral Sclerosis according to Initial Symptoms: An Analysis of 500 Cases

Lee JW, Kang SW, Choi WA

Purpose: One obstacle in early diagnosis of amyotrophic lateral sclerosis (ALS) is its vague initial presentation, which is generally classified into limb- and bulbar-dominant types and may be mistaken for...
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Ocular Involvement Occurs Frequently at All Stages of Amyotrophic Lateral Sclerosis: Preliminary Experience in a Large Italian Cohort

Cozza F, Lizio A, Greco LC, Bona S, Donvito G, Carraro E, Tavazzi S, Ticozzi N, Poletti B, Sansone VA, Lunetta C

Background and Purpose The study aimed to obtain optometric findings of amyotrophic lateral sclerosis (ALS) patients in different stages of the disease, and to determine the relation between ocular data...
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The Role of the SLC Transporters Protein in the Neurodegenerative Disorders

Aykaç A, Şehirli A

The solute carrier (SLC) superfamily is one of the major sub-groups of membrane proteins in mammalian cells. The solute carrier proteins include more than 400 different membrane-spanning solute carriers organized...
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Likely pathogenic FIG4 related amyotrophiclateral sclerosis patient whocorrelated with clinical, imaging andneuropsychological studies

Ko PW, Min YS, Park JS

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with numerous causes that include genetic factors. Efforts to reveal the genetics of ALS have identified several candidate genes that are associated with...
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Proper chest compression depth for cardiopulmonary resuscitation in patients with amyotrophic lateral sclerosis: a retrospective study

Kwon D, Oh J, Lee H, Kang H, Lim TH, Ko BS, Cho Y, Kim H

  • KMID: 2501770
  • J Korean Soc Emerg Med.
  • 2020 Feb;31(1):10-16.
Objective: The chest compression depth recommended in the current guidelines for average adult cardiopulmonary resuscitation is approximately 1/4-1/5 depth of the external chest anteroposterior (AP) diameter and at least 50...
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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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Molecular factors related to skeletal muscle atrophy in a mouse model of amyotrophic lateral sclerosis

Cai M, Yang EJ

Amyotrophic lateral sclerosis (ALS) is progressive neurological disease that results in the death of motor neurons in the brain and spinal cord, leading to a decrease in skeletal muscle size...
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Assisted Breathing with a Diaphragm Pacing System: A Systematic Review

Woo Al, Tchoe Hj, Shin Hw, Shin Cm, Lim CM

Purpose: Patients with respiratory failure associated with neurological dysfunction often require mechanical ventilator support, which poses increased economic burden and ventilator-associated complications. A diaphragm pacing system (DPS) is an implanted...
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Shape Analysis of the Subcortical Nuclei in Amyotrophic Lateral Sclerosis without Cognitive Impairment

Tae WS, Sung JH, Baek SH, Lee CN, Kim BJ

Background and Purpose: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that mainly affects the pyramidal motor system. However, recent studies have suggested that degeneration of the extramotor system plays...
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Riluzole Selective Antioxidant Effects in Cell Models Expressing Amyotrophic Lateral Sclerosis Endophenotypes

Sala G, Arosio A, Conti E, Beretta S, Lunetta C, Riva N, Ferrarese , Tremolizzo L

OBJECTIVE: Until recently, riluzole was the only drug licensed for amyotrophic lateral sclerosis (ALS). In spite of its efficacy, the mechanism of action remains elusive, and both blocking of glutamate...
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Uncommon coexistence of myasthenia gravis and amyotrophic lateral sclerosis

Cho EB, Yang TW, Jeong H, Yoon C, Jung S, Park KJ

Myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) are distinct disorders. ALS affects motor neurons that control muscle movement, while MG controls communication between neurons and muscles, which occurs at...
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Improved Bulbar Function in Amyotrophic Lateral Sclerosis after Nuedexta (Dextromethorphan and Quinidine) Treatment

Cho HJ, Park JM, Park JS

Nuedexta (dextromethorphan and quinidine) is an Food and Drug Administration approved medication for pseudobulbar affect. Interestingly, this drug was recently reported to improve speech, swallowing, and the ability to handle...
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Impaired Nucleoporins Are Present in Sporadic Amyotrophic Lateral Sclerosis Motor Neurons that Exhibit Mislocalization of the 43-kDa TAR DNA-Binding Protein

Aizawa H, Yamashita , Kato H, Kimura T, Kwak S

BACKGROUND AND PURPOSE: Disruption of nucleoporins has been reported in the motor neurons of patients with sporadic amyotrophic lateral sclerosis (sALS). However, the precise changes in the morphology of nucleoporins...
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Factors Influencing Psychosocial Well-Being in Family Caregivers of People with Amyotrophic Lateral Sclerosis

Chu HS, Tak YR, Kim SH

PURPOSE: The purpose of this study was to identify factors influencing psychosocial well-being in family caregivers of patients with amyotrophic lateral sclerosis (ALS). METHODS: A descriptive correlational design was used. The...
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Usefulness of phrenic nerve conduction study in early diagnosis of isolated respiratory onset amyotrophic lateral sclerosis

Lee DE, Park D, Kim JK, Park JS

Isolated respiratory onset amyotrophic lateral sclerosis (ALS) is a rare clinical manifestation and the diagnosis can be challenging. A 72-year-old man presented with dyspnea and hypercapnia that had started 11...
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Successful Management of Acquired Tracheomalacia of Patients With Amyotrophic Lateral Sclerosis: A Report of Three Cases

Yang JH, Kim TW, Lee BJ, Yoon JA, Shin MJ, Shin YB

Tracheomalacia is characterized by weakness of the tracheal walls and supporting cartilage. It results in dynamic compression of the airway, where the cross-sectional area of the trachea is reduced by...
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A De Novo RAPGEF2 Variant Identified in a Sporadic Amyotrophic Lateral Sclerosis Patient Impairs Microtubule Stability and Axonal Mitochondria Distribution

Heo K, Lim SM, Nahm M, Kim YE, Oh KW, Park HT, Ki CS, Kim SH, Lee S

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is frequently linked to microtubule abnormalities and mitochondrial trafficking defects. Whole exome sequencing (WES) of patient-parent trios has proven to...
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