• Annals of Clinical Neurophysiology
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• v.17 no.1
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• pp.1-16
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• 2015

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is characterized by progressive death of motor neurons in the cortex, brainstem, and spinal cord. Until now, many treatment strategies have been tested in ALS, but so far only Riluzole has shown efficacy of slightly slowing disease progression. The pathophysiological mechanisms underlying ALS are multifactorial, with a complex interaction between genetic factors and molecular pathways. Other motor neuron disease such as spinal muscular atrophy (SMA) and spinobulbar muscular atrophy (SBMA) are also progressive neurodegenerative disease with loss of motor neuron as ALS. This common thread of motor neuron loss has provided a target for the development of therapies for these motor neuron diseases. A better understanding of these pathogenic mechanisms and the potential pathological relationship between the various cellular processes have suggested novel therapeutic approaches, including stem cell and genetics-based strategies, providing hope for feasible treatment of ALS.

• Annals of Clinical Neurophysiology
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• v.22 no.1
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• pp.33-36
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• 2020

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 familial and sporadic ALS. Here we report a Korean ALS patient who showed prominent upper motor-neuron-related symptoms with marked brain atrophy and neuropsychological deficits. The findings were highly suggestive of ALS in a patient with a likely pathogenic FIG4 variant.

• Journal of Dental Anesthesia and Pain Medicine
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• v.17 no.3
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• pp.235-240
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• 2017

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 cardiovascular insult (e.g., severe prolonged hypoxia or acidosis, extreme hypovolemia, or flow-restricting pulmonary embolus). Amyotrophic lateral sclerosis (ALS) is a disease that is characterized by progressive degeneration of all levels of the motor nervous system. Damage to the respiratory system and weakness of the muscles may increase the likelihood of an emergency situation occurring in patients with ALS while under general anesthesia. We report a case of PEA during the induction of general anesthesia in a patient with ALS who presented for dental treatment and discuss the causes of PEA and necessary considerations for general anesthesia in patients with ALS.

• Journal of muscle and joint health
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• v.15 no.2
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• pp.140-154
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• 2008

Purpose: The purpose of this study was to describe the adjustment process of patients with Amyotrophic Lateral Sclerosis(ALS). Method: The data were collected from May 2007 to February 2008 through individual in-depth interviews with 4 ALS patients. The data collection and analysis were performed according to grounded theory methodology, as suggested by Strauss and Corbin. Results: 'Accepting the sick role' was the central phenomenon, which was derived from 'perceiving an intial symptom'. Therefore, the adjustment pattern was represented by using the different strategies, which were 'raising hope' and 'hoping to dye comfortably'. For taking a view of the future, ALS patients adopted the strategies of 'living positively', 'being treated hardly', 'joining in the experience', 'depending on the absolute being', 'recognizing the dying process'. Conclusion: This study provides guidance for the development of nursing interventions for patients with ALS.

• Annals of Clinical Neurophysiology
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• v.5 no.1
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• pp.27-33
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• 2003

Background: Motor unit number estimation (MUNE) can directly assess motor neuron populations in muscle and quantify the degree of physiologic and/or pathologic motor neuron degeneration. A high degree of reproducibility and reliability is required from a good quantitative tool. MUNE, in various ways, is being increasingly applied clinically and statistical MUNE has several advantages over alternative techniques. Nevertheless, the optimal method of applying statistical MUNE to improve reproducibility has not been established. Methods: We performed statistical MUNE by selecting the most compensated compound muscle action potential (CMAP) area as a test area and modified the results obtained by weighted mean surface-recorded motor unit potential (SMUP). Results: MUNE measures in amyotrophic lateral sclerosis (ALS) patients showed better reproducibility with sizeweighted modification. Conclusions: We suggest size-weighted MUNE testing of "neurogenically compensated"CMAP areas present an optimal method for statistical MUNE in ALS patients.

• Annals of Clinical Neurophysiology
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• v.18 no.1
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• pp.18-20
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• 2016

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 neuron degeneration begins, and conflicting hypotheses have been suggested. Recent advanced radiological techniques enable us to look into ALS neuropathology in vivo. Herein, we report a case with upper motor neuron-predominant ALS in whom the results of brain magnetic resonance imaging (MRI) and myelin water fraction MRI suggest axonal degeneration.

• Annals of Clinical Neurophysiology
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• v.21 no.2
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• pp.113-116
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• 2019

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 neuromuscular junctions. However, on rare occasions, ALS develops after MG and vice versa. The coexistence of the two diseases represents a diagnostic challenge and requires thoughtful interpretation of clinical features. We present the case of a 53-year-old Korean male who developed ALS after MG, confirmed by clinical and electrophysiological follow-up.

• The Journal of Internal Korean Medicine
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• v.43 no.5
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• pp.891-900
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• 2022

Purpose: The purpose of this paper is to report the improvement of a patient with amyotrophic lateral sclerosis after long-term combined Korean medical treatment. Methods: A patient diagnosed with amyotrophic lateral sclerosis was treated with herbal medicine, acupuncture, pharmacopuncture, moxibustion, and rehabilitation for four separate hospital stays. To evaluate their respiratory discomfort and limb weakness, we used Manual Muscle Testing, the Pulmonary Function Test, and the Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised. Results: The weakness of the muscles of the lower extremities and respiratory function was improved. Conclusion: We consider that combined Korean medicine treatments might be an effective treatment for muscle weakness and respiratory discomfort of amyotrophic lateral sclerosis. To verify the effectiveness of these treatments, further research is required.

• Journal of Korean Academy of Nursing
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• v.45 no.2
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• pp.202-210
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• 2015

Purpose: The purpose of this study was to describe depression, caregiving burden and the correlation of the two variables in the families of patients with amyotrophic lateral sclerosis (ALS) and to clarify factors predicting caregiving burden. Methods: A descriptive and cross-sectional study was conducted with 139 family members who provided care to patients with ALS. The characteristics of patients and families, Korean-Beck Depression Inventory (K-BDI), Korean version of Zarit Burden Interview (K-ZBI) and Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (K-ALSFRS-R) were used as study measures. Results: The mean score for K-BDI was 19.39 out of 63 suggesting sub-clinical depression and 38.2% of the family members exhibited depression. The mean score for K-ZBI was 66.03 out of 88. The predictors for K-ZBI were K-BDI, age of family member, length of time spent per day in caring, relationship to patient and K-ALSFRS-R. Conclusion: The results of this study suggest that levels of depression and caregiving burden are high among family members caring for patients with ALS. As depression is associated with caregiving burden, screening and emotional supports should be provided to reduce the burden of care for these family. Support programs to alleviate the care burden are also needed, considering family demographics, time per day in caring giving and K-ALSFRS-R.

• Journal of Acupuncture Research
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• v.30 no.2
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• pp.73-79
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• 2013

Objectives : Sialorrhea in amyotrophic lateral sclerosis patients is a cause of death due to aspiration pneumonia as well as reduces the patient's quality of life. We report the changes that appeared in the sialorrhea treated by using trihexyphenidyl and Korean medical treatments. Methods : We treated amyotrophic lateral sclerosis patient with sialorrhea by using trihexyphenidyl a known antiparkinsonian agent and Korean medical treatments such as acupuncture, pharmacopuncture and herbal medicine. The salivation rate was checked with visual analogue scale(VAS). Results : There was a more than 50 % decrease in salivation in this case. Owing to the constipation, trihexyphenidyl was stopped after which only Korean medical treatments were provided. Over which a lasting decrease in salivation could be seen. Conclusions : Existing treatments would cause several considerable side effects and have difficulty in being applied in domestic clinics. In this respect, we suspect that our findings could open up new clinical guideline possibilities. We should solve the limitations of this case study and conduct more studies.