• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.580-584
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• 2007

Multiple sclerosis (MS) is a demyelinating disorder that affects discrete areas of the CNS, including the optic nerves, in a quite variable relapsing-remitting fashion over a prolonged period of time. Although MS is usually considered to be a disease that affects peoples in early to middle adulthood, children do develop multiple sclerosis. The frequency of MS onset before the age of 15 years is 2.7-5% of all cases, while MS onset during infancy and early childhood was observed to be 0.2-0.7% of all cases. We report here on a Korean case of a relapsing-remitting MS female child who was treated with four rounds of intravenous methylpredinsolone pulse therapy and preventive Interferon-$\beta$-1b ($Betaferon^{(R)}$).

• 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.

• Journal of Acupuncture Research
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• v.30 no.5
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• pp.185-192
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• 2013

Objectives : Weight loss and loss of appetite in amyotrophic lateral sclerosis patients are common symptoms, these are associated with survival as well as nutritional condition. The changes in weight and appetite were reported after treatment using Megestrol acetate and Korean medical treatments. Methods : Amyotrophic lateral sclerosis patient with weight loss and loss of appetite were treated by administering megestrol acetate a known anti-malignant tumor agent and Korean medical treatments such as acupuncture, pharmacopuncture and herbal medicine. The changes in weight and appetite were checked using body mass index(BMI) and simplified nutritional appetite questionnaire(SNAQ). Results : There was a more than 17 kg increase in weight. BMI increased from 15.94 $kg/m^2$ to 21.97 $kg/m^2$ and SNAQ score increased from 7 to 16. Owing to several side effects, Megestrol acetate was stopped after which only Korean medical treatments were provided. After which the lasting effects in BMI and appetite could be seen. Conclusion : There are few studies on weight loss and loss of appetite in ALS patients. Using only megestrol acetate can cause several considerable side effects. Which respect to this, the findings in this study could open up new clinical guideline possibilities.

• Journal of Pharmacopuncture
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• v.13 no.4
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• pp.119-128
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• 2010

Objectives : The objective of this study is to report the change of progress in symptoms and various scales after treated with Mountain Ginseng Pharmacopuncture(MGP) on the patients of Amyotrophic Lateral Sclerosis(ALS). Methods : The three ALS patients who treated with MGP, were checked the change of progress by ALS Functional Rating Scale(ALSFRS), ALS Severity Score(ALSSS), grasping power on both arms and circumference of both thighs and calves. Results : After MGP treatment on three ALS patients, first case did not grow worse during MGP treated whereas she got worse rapidly during none treated period. Second case did not grow worse during treated period and third case got worse in progress by slow degrees despite of MGP treatment. Conclusions : Although MGP could not control the progress of ALS completely, MGP may help the improving of quality of life(QOL) in ALS patients and have the effect of delayed ALS progression.

• Korean Journal of Psychosomatic Medicine
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• v.5 no.2
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• pp.205-213
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• 1997

The aim of this study is to find the relationship between interictal psychiatric symptoms and seizure-related variables such as structural changes, regional interictal perfusion changes, the number of interictal epileptic discharges and the presence of accompanying generalized tonic clonic seizure(GTC). The subjects were 75 patients (47 males : mean age $28.3{\pm}7.7$) with intractable epilepsy, Interictal psychiatric symptoms were rated by Symptom Checklist-90-R(SCL-90-R). Each of 4 seizure variables was measured by MRI, $^{99m}TC-HMPAO$ SPECT and the prolonged EEG monitoring. The mean SCL-90-R subscale T-scores and the ratio of the patients with definite psychiatric symptoms(T-score > 65 at any one subscale of SCL-90-R) were compared among the groups with different seizure variables. Demographic variables and clinical variables were not statistically different among the groups with different seizure variables. The patients with right mesial temporal sclerosis had higher mean SCL-90-R scores for obsessive compulsive symptom, interpersonal sensitivity, depression, hostility and psychotic symptoms than the patients with left mesial temporal sclerosis, and they also showed higher ratio of definite psychiatric symptoms. The presence of interictal epileptic discharges was related with higher T-scores of paranoid subscale. The areas of hypoperfusion, the presence of the GTC, and other clinical seizure variables showed no significant influences on the mean SCL-90-R subscale T-scores. from these results, the authors speculated that interictal subictal epileptic dischrages may be related with psychiatric symptoms of the intractable epileptic patients, especially with right mesial temporal sclerosis.

• Tuberculosis and Respiratory Diseases
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• v.64 no.5
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• pp.369-373
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• 2008

Tuberous sclerosis (TS) is an autosomal dominant disorder that is characterized by cutaneous lesions, seizures, mental retardation and hamartomas in various organs including the skin, kidney and brain. Pulmonary involvement is extremely rare, and occurs in approximately 0.1 to 1% of TS cases. Recent reports have indicated multiple micronodular pneumocyte hyperplasia (MMPH) as another rare form of pulmonary involvement of tuberous sclerosis. We report a case of a 35 year-old-male patient who had no pulmonary symptoms but showed multinodular pulmonary shadows on his chest CT scan. The patient was finally diagnosed with TS with MMPH of the lung. MMPH does not appear to have any malignant potential but the clinical significance of MMPH in TS patients is unknown.

• Tuberculosis and Respiratory Diseases
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• v.83 no.4
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• pp.312-320
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• 2020

Background: Systemic sclerosis (SSc) involves multiple organ systems and has the highest mortality among connective tissue diseases. Interstitial lung disease is the most common cause of death among SSc patients and requires closer studies and follow-ups. This study aimed to identify lung function changes and predictors of progressive disease in systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: A retrospective study extracted SSc patients from an electronic database January 2002-July 2019. Eligible cases were SSc patients >age 15 diagnosed with SSc-ILD. Factors associated with progressive disease were analyzed by univariate and multivariate logistic regression analyses. Results: Seventy-eight SSc-ILD cases were enrolled. Sixty-five patients (83.3%) were female, with mean age of 44.7±14.4, and 50 (64.1%) were diffuse type SSc-ILD. Most SSc-ILD patients had crackles (75.6%) and dyspnea on exertion (71.8%), and 19.2% of the SSc-ILD patients had no abnormal respiratory symptoms but had abnormal chest radiographic findings. The most common diagnosis of SSc-ILD patients was non-specific interstitial pneumonia (43.6%). The lung function values of diffusing capacity of the lung for carbon monoxide (DLCO) and DLCO per unit alveolar volume declined in progressive SSc-ILD during a 12-month follow-up. Male and no previous aspirin treatment were the two significant predictive factors of progressive SSc-ILD with adjusted odds ratios of 5.72 and 4.99, respectively. Conclusion: This present study showed that short-term lung function had declined during the 12-month follow-up in progressive SSc-ILD. The predictive factors in progressive SSc-ILD were male sex and no previous aspirin treatment. Close follow-up of the pulmonary function tests is necessary for early detection of progressive disease.

• Journal of the Korea Convergence Society
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• v.8 no.6
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• pp.275-281
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• 2017

Human endogenous retroviruses (HERVs) are fossil viruses that began to be assimilated into the human genome some 30~40 million years ago, and now constitute nearly 8% of the human genome. These ancient retroviruses have since accumulated mutations that have rendered them defective; thus, they have been termed junk DNA. However, recent research indicates that not all HERVs remain silent passengers. Although they have not been shown to be causative of any human disease, endogenous retroviral sequences may become expressed under select pathological circumstances such as neurological disorders, including multiple sclerosis (MS), schizophrenia, and Amyotrophic Lateral Sclerosis (ALS); viral infections, including human immunodeficiency virus (HIV) and herpesvirus; and multiple types of cancers. This review focused on the possible interactions of HERVs and neurological diseases.

• Journal of the Korea Convergence Society
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• v.10 no.9
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• pp.193-198
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• 2019

This study analyzed the changes of vowel formant, voice intensity, and fundamental frequency of vowels for 11 months using acoustochemical spectrogram analysis of women diagnosed with amyotrophic lateral sclerosis (ALS). The test word was a vowel /a, i, u/ and a diphthong /h + ja + da/, /h + wi + da/, and /h +ɰi+ da/. Speech data were collected through the word reading task presented on the monitor using 'Alvin' program, and the recording environment was set to 5,500 Hz for the nyquist frequency and 11,000 Hz for the sampling rate. The records were analyzed by using spectrograms to vowel formants, voice intensity, and fundamental frequency. As a result of analysis, the fundamental frequency and intensity of the ALS process were decreased and the formant slope of the diphthong was decreased rather than the formant change in the vowel. This result suggests that the vowel distortion of ALS due to disease progression is due to the decrease of tongue and jaw co morbidity.

• Biomolecules & Therapeutics
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• v.29 no.5
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• pp.498-505
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• 2021

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 (CAT-1) activity in the transport of lysine and the pretreatment effect of lysine on pro-inflammatory states in an amyotrophic lateral sclerosis cell line. The mRNA expression of cationic amino acid transporter 1 was lower in NSC-34/hSOD1^G93A (MT) than the control cell line (WT), lysine transport is mediated by CAT-1 in NSC-34 cell lines. The uptake of [³H]L-lysine was Na⁺-independent, voltage-sensitive, and strongly inhibited by inhibitors and substrates of cationic amino acid transporter 1 (system y⁺). The transport process involved two saturable processes in both cell lines. In the MT cell line, at a high-affinity site, the affinity was 9.4-fold higher and capacity 24-fold lower than that in the WT; at a low-affinity site, the capacity was 2.3-fold lower than that in the WT cell line. Donepezil and verapamil competitively inhibited [³H]L-lysine uptake in the NSC-34 cell lines. Pretreatment with pro-inflammatory cytokines decreased the uptake of [³H]L-lysine and mRNA expression levels in both cell lines; however, the addition of L-lysine restored the transport activity in the MT cell lines. L-Lysine exhibited neuroprotective effects against pro-inflammatory states in the ALS disease model cell lines. In conclusion, studying the alteration in the expression of transporters and characteristics of lysine transport in ALS can lead to the development of new therapies for neurodegenerative diseases.