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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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Korean Medical Professionals' Attitudes and Experiences on Advance Care Planning for Noncancerous Disease

Lee JE, Goo A, Shin DW, Yoo JH, Korean Geriatrics Society End-of-Life Research Group

BACKGROUND: Advance care planning (ACP) allows patients to declare their preferences for life-sustaining and hospice palliative care. However, the perception of ACP remains low in Korea. The present study assessed...
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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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Exercise and Neuroinflammation in Health and Disease

Seo DY, Heo JW, Ko JR, Kwak HB

Neuroinflammation is a central pathological feature of several acute and chronic brain diseases, including Alzheimer disease (AD), Parkinson disease (PD), amyotrophic lateral sclerosis (ALS), and multiple sclerosis (MS). It induces...
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Cholesterol Metabolism in the Brain and Its Association with Parkinson's Disease

Jin U, Park SJ, Park SM

Parkinson's disease (PD) is the second most progressive neurodegenerative disorder of the aging population after Alzheimer’s disease (AD). Defects in the lysosomal systems and mitochondria have been suspected to cause...
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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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Pseudobulbar Affect in Parkinsonian Disorders: A Review

Hakimi M, Maurer CW

Pseudobulbar affect (PBA) is a neurological symptom of inappropriate and uncontrollable laughter or crying that occurs secondary to a variety of neurological conditions, including parkinsonian disorders. PBA is a socially...
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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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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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Genetic Ablation of EWS RNA Binding Protein 1 (EWSR1) Leads to Neuroanatomical Changes and Motor Dysfunction in Mice

Yoon Y, Park H, Kim S, Nguyen PT, Hyeon SJ, Chung S, Im H, Lee J, Lee SB, Ryu H

A recent study reveals that missense mutations of EWSR1 are associated with neurodegenerative disorders such as amyotrophic lateral sclerosis, but the function of wild-type (WT) EWSR1 in the central nervous...
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Coexistence of Amyotrophic Lateral Sclerosis in the Proband of an X-Linked Charcot-Marie-Tooth Disease Type 1 Pedigree in China

Feng SY, Feng SM, Li LY, Zou ZY

No abstract available.
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Current Clinical Applications of Diffusion-Tensor Imaging in Neurological Disorders

Tae WS, Ham BJ, Pyun SB, Kang SH, Kim BJ

Diffusion-tensor imaging (DTI) is a noninvasive medical imaging tool used to investigate the structure of white matter. The signal contrast in DTI is generated by differences in the Brownian motion...
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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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Abnormal Oculomotor Functions in Amyotrophic Lateral Sclerosis

Kang BH, Kim JI, Lim YM, Kim KK

BACKGROUND AND PURPOSE: Although traditionally regarded as spared, a range of oculomotor dysfunction has been recognized in amyotrophic lateral sclerosis (ALS) patients. ALS is nowadays considered as a neurodegenerative disorder...
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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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Pulseless electrical activity during general anesthesia induction in patients with amyotrophic lateral sclerosis

You TM, Kim S

Pulseless electrical activity (PEA) is a clinical condition characterized by unresponsiveness and lack of palpable pulse in the presence of organized cardiac electrical activity and is caused by a profound...
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Effects of a Home Respiratory Management Program for Patients with Amyotrophic Lateral Sclerosis

Hwang MS, Lee MK, Song JR

PURPOSE: The purpose of this study was the evaluation of a Home Nursing Care based Respiratory Management Program (HHNbRMP) on health care ability, respiratory and psychological status and quality of...
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