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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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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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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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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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Edematous Hyponatremia Treated with Tolvaptan in a Patient with Amyotrophic Lateral Sclerosis

Kim GH

Amyotrophic lateral sclerosis (ALS) patients rarely present with either syndrome of inappropriate antidiuretic hormone secretion or generalized edema. Tolvaptan is a selective vasopressin V2 receptor antagonist that produces effective aquaresis,...
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Long-Term Outcome of Amyotrophic Lateral Sclerosis in Korean Subjects

Suh MR, Choi WA, Choi YC, Lee JW, Hong JH, Park J, Kang SW

OBJECTIVE: To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis. METHODS: Subjects who were diagnosed with ALS between January 2005 and December...
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KCHO-1, a novel herbal anti-inflammatory compound, attenuates oxidative stress in an animal model of amyotrophic lateral sclerosis

Kook MG, Choi SW, Seo Y, Kim DW, Song BK, Son I, Kim S, Kang KS

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective death of motor neurons in the central nervous system. The main cause of the disease remains elusive, but several...
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A case of celiac disease with neurologic manifestations misdiagnosed as amyotrophic lateral sclerosis

Ham H, Lee BI, Oh HJ, Park SH, Kim JS, Park JM, Cho YS, Choi MG

Celiac disease (CD) is an immune-mediated enteropathy and is a rare disease in Asia, including in Korea. However, the ingestion of wheat products, which can act as a precipitating factor...
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Reference Range of Respiratory Muscle Strength and Its Clinical Application in Amyotrophic Lateral Sclerosis: A Single-Center Study

Park KH, Kim RB, Yang J, Oh JH, Park SY, Kim DG, Shin JY, Sung JJ

BACKGROUND AND PURPOSE: Evaluating respiratory function is important in neuromuscular diseases. This study explored the reference ranges of the maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal...
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Impaired Perception of Emotional Expression in Amyotrophic Lateral Sclerosis

Oh SI, Oh KW, Kim HJ, Park JS, Kim SH

BACKGROUND AND PURPOSE: The increasing recognition that deficits in social emotions occur in amyotrophic lateral sclerosis (ALS) is helping to explain the spectrum of neuropsychological dysfunctions, thus supporting the view...
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Myelin Water Fraction MRI in a Case of Clinically Probable Amyotrophic Lateral Sclerosis

Yang J, Lee J, Kim E, Shin DH

Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron degenerative disease that clinically manifests both upper and lower motor neuron signs. However, it is unknown where and how the motor...
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Dissociation of Structural and Functional Integrities of the Motor System in Amyotrophic Lateral Sclerosis and Behavioral-Variant Frontotemporal Dementia

Bae JS, Ferguson M, Tan R, Mioshi E, Simon N, Burrell J, Vucic S, Hodges JR, Kiernan MC, Hornberger M

BACKGROUND AND PURPOSE: This study investigated the structural and functional changes in the motor system in amyotrophic lateral sclerosis (ALS; n=25) and behavioral-variant fronto-temporal dementia (bvFTD; n=17) relative to healthy...
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