• Title/Summary/Keyword: Neurological dysfunction

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Semi-Fixed Lip Bumper in Lesch-Nyhan Syndrome: An Interim Treatment Modality (반 고정식 립 범퍼를 이용한 Lesch-Nyhan 증후군 환아의 자해습관 차단)

  • Lee, Junhee;Lee, Eungyung;Shin, Jonghyun;Kim, Shin;Jeong, Taesung
    • Journal of the korean academy of Pediatric Dentistry
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    • v.47 no.1
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    • pp.93-98
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    • 2020
  • Lesch-Nyhan syndrome is a rare X-linked, recessively inherited disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. This syndrome is characterized by 3 major features: neurological dysfunction, hyperuricemia, and cognitive and behavioral disturbances (e.g., self-mutilation, which begins at 2 to 3 years of age). Uncontrollable self-mutilation begins with biting of the perioral tissues and extends into patterns such as finger biting and head hitting. This report describes the case of a 31-month-old boy who was diagnosed with Lesch-Nyhan syndrome with severe lip injuries caused by self-mutilative behaviors. The behaviors were blocked with a semi-fixed lip bumper for a short period. The device was applied to the patient on the day of the visit without the requirement for an oral impression. It was easy to manage oral hygiene and adjust the device because it was detachable by clinicians and guardians. Therefore, a semi-fixed lip bumper may be useful as an interim appliance to block selfmutilative behaviors in children with Lesch-Nyhan syndrome.

Rg3-enriched Korean Red Ginseng extract inhibits blood-brain barrier disruption in an animal model of multiple sclerosis by modulating expression of NADPH oxidase 2 and 4

  • Lee, Min Jung;Choi, Jong Hee;Oh, Jinhee;Lee, Young Hyun;In, Jun-Gyo;Chang, Byung-Joon;Nah, Seung-Yeol;Cho, Ik-Hyun
    • Journal of Ginseng Research
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    • v.45 no.3
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    • pp.433-441
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    • 2021
  • Background: Multiple sclerosis (MS) and its animal model, the experimental autoimmune encephalomyelitis (EAE), are primarily characterized as dysfunction of the blood-brain barrier (BBB). Ginsenoside-Rg3-enriched Korean Red Ginseng extract (Rg3-KRGE) is known to exert neuroprotective, anti-inflammatory, and anti-oxidative effects on neurological disorders. However, effects of Rg3-KRGE in EAE remain unclear. Methods: Here, we investigated whether Rg3-KRGE may improve the symptoms and pathological features of myelin oligodendroglial glycoprotein (MOG)35-55 peptide - induced chronic EAE mice through improving the integrity of the BBB. Results: Rg3-KRGE decreased EAE score and spinal demyelination. Rg3-KRGE inhibited Evan's blue dye leakage in spinal cord, suppressed increases of adhesion molecule platelet endothelial cell adhesion molecule-1, extracellular matrix proteins fibronection, and matrix metallopeptidase-9, and prevented decreases of tight junction proteins zonula occludens-1, claudin-3, and claudin-5 in spinal cord following EAE induction. Rg3-KRGE repressed increases of proinflammatory transcripts cyclooxygenase-2, inducible nitric oxide synthase, interleukin (IL)-1 beta, IL-6, and tumor necrosis factor-alpha, but enhanced expression levels of anti-inflammatory transcripts arginase-1 and IL-10 in the spinal cord following EAE induction. Rg3-KRGE inhibited the expression of oxidative stress markers (MitoSOX and 4-hydroxynonenal), the enhancement of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase 2 (NOX2) and NOX4, and NADPH activity in the spinal cord of chronic EAE mice. Furthermore, apocynin, a NOX inhibitor, mimicked beneficial effects of Rg3-KRGE in chronic EAE mice. Conclusion: Our findings suggest that Rg3-KRGE might alleviate behavioral symptoms and pathological features of MS by improving BBB integrity through modulation of NOX2/4 expression.

Ginsenoside Rg1 attenuates cerebral ischemia-reperfusion injury due to inhibition of NOX2-mediated calcium homeostasis dysregulation in mice

  • Han, Yuli;Li, Xuewang;Yang, Liu;Zhang, Duoduo;Li, Lan;Dong, Xianan;Li, Yan;Qun, Sen;Li, Weizu
    • Journal of Ginseng Research
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    • v.46 no.4
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    • pp.515-525
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    • 2022
  • Background: The incidence of ischemic cerebrovascular disease is increasing in recent years and has been one of the leading causes of neurological dysfunction and death. Ginsenoside Rg1 has been found to protect against neuronal damage in many neurodegenerative diseases. However, the effect and mechanism by which Rg1 protects against cerebral ischemia-reperfusion injury (CIRI) are not fully understood. Here, we report the neuroprotective effects of Rg1 treatment on CIRI and its possible mechanisms in mice. Methods: A bilateral common carotid artery ligation was used to establish a chronic CIRI model in mice. HT22 cells were treated with Rg1 after OGD/R to study its effect on [Ca2+]i. The open-field test and poleclimbing experiment were used to detect behavioral injury. The laser speckle blood flowmeter was used to measure brain blood flow. The Nissl and H&E staining were used to examine the neuronal damage. The Western blotting was used to examine MAP2, PSD95, Tau, p-Tau, NOX2, PLC, p-PLC, CN, NFAT1, and NLRP1 expression. Calcium imaging was used to test the level of [Ca2+]i. Results: Rg1 treatment significantly improved cerebral blood flow, locomotion, and limb coordination, reduced ROS production, increased MAP2 and PSD95 expression, and decreased p-Tau, NOX2, p-PLC, CN, NFAT1, and NLRP1 expression. Calcium imaging results showed that Rg1 could inhibit calcium overload and resist the imbalance of calcium homeostasis after OGD/R in HT22 cells. Conclusion: Rg1 plays a neuroprotective role in attenuating CIRI by inhibiting oxidative stress, calcium overload, and neuroinflammation.

Effects of Computerized Cognitive Training Program Using Artificial Intelligence Motion Capture on Cognitive Function, Depression, and Quality of Life in Older Adults With Mild Cognitive Impairment During COVID-19: Pilot Study (인공지능 동작 인식을 활용한 전산화인지훈련이 코로나-19 기간 동안 경도 인지장애 고령자의 인지 기능, 우울, 삶의 질에 미치는 영향: 예비 연구)

  • Park, Ji Hyeun;Lee, Gyeong A;Lee, Jiyeon;Park, Young Uk;Park, Ji-Hyuk
    • Therapeutic Science for Rehabilitation
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    • v.12 no.2
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    • pp.85-98
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    • 2023
  • Objective : We investigated the efficacy of an artificial intelligence computerized cognitive training program using motion capture to identify changes in cognition, depression, and quality of life in older adults with mild cognitive impairment. Methods : A total of seven older adults (experimental group = 4, control group = 3) participated in this study. During the COVID-19 period from October to December 2021, we used a program, "MOOVE Brain", that we had developed. The experimental group performed the program 30 minutes 3×/week for 1 month. We analyzed patients scores from the Korean version of the Mini-Mental State Examination-2, the Consortium to Establish a Registry for Alzheimer's Disease Assessment Packet for Daily Life Evaluation, the short form Geriatric Depression Scale, and Geriatric Quality of Life Scale. Results : We observed positive changes in the mean scores of the Stroop Color Test (attention), Stroop Color/Word Test (executive function), SGDS-K (depression), and GQOL (QoL). However, these changes did not reach statistical significance for each variable. Conclusion : The study results from "MOOVE Brain" can help address cognitive and psychosocial issues in isolated patients with MCI during the COVID-19 pandemic or those unable to access in-person medical services.

Prospero Homeobox 1 and Doublecortin Correlate with Neural Damage after Ischemic Stroke

  • Dong-Hun Lee;Eun Chae Lee;Sang-Won Park;Ji young Lee;Kee-Pyo Kim;Jae Sang Oh
    • Journal of Korean Neurosurgical Society
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    • v.67 no.3
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    • pp.333-344
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    • 2024
  • Objective : Markers of neuroinflammation during ischemic stroke are well characterized, but additional markers of neural damage are lacking. The study identified associations of behavioral disorders after stroke with histologic neural damage and molecular biological change. Methods : Eight-week-old, 25 g male mice of the C57BL/6J strain were subjected to middle cerebral artery occlusion (MCAO) to induce ischemic stroke. The control group was a healthy wild type (WT), and the experimental group were designed as a low severity MCAO1 and a high severity MCAO2 based on post-stroke neurological scoring. All groups underwent behavioral tests, realtime polymerase chain reaction, triphenyltetrazolium chloride (TTC) staining and Hematoxylin and Eosin staining. One-way analysis of variance was used to analyze statistical significance between groups. Results : In TTC staining, MCAO1 showed 29.02% and MCAO2 showed 38.94% infarct volume (p<0.0001). The pro-inflammatory cytokine interleukin (IL)-1β was most highly expressed in MCAO2 (WT 0.44 vs. MCAO1 2.69 vs. MCAO2 5.02, p<0.0001). From the distance to target in the Barnes maze test, WT had a distance of 178 cm, MCAO1 had a distance of 276 cm, and MCAO2 had a distance of 1051 (p=0.0015). The latency to target was 13.3 seconds for WT, 27.9 seconds for MCAO1, and 87.9 seconds for MCAO2 (p=0.0007). Prospero homeobox 1 (Prox1) was most highly expressed in MCAO2 (p=0.0004). Doublecortin (Dcx) was most highly expressed in MCAO2 (p<0.0001). Conclusion : The study demonstrated that histological damage to neural cells and changes in brain mRNA expression were associated with behavioral impairment after ischemic stroke. Prox1 and Dcx may be biomarkers of neural damage associated with long-term cognitive decline, and increased expression at the mRNA level was consistent with neural damage and long-term cognitive dysfunction.

Correlations of Cerebellar Function with Psychotic Symptoms and Cognitive Function in Schizophrenic Patients (남자 정신분열병 환자의 소뇌기능과 정신증상 및 인지기능간의 연관성)

  • Kim, Seo Young;Jun, Yong Ho;Kwon, Young Joon;Jeong, Hee Yeon;Hwang, Bo Young;Shim, Se Hoon
    • Korean Journal of Biological Psychiatry
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    • v.14 no.3
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    • pp.184-193
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    • 2007
  • Objectives:There is increasing evidence that the cerebellum plays an important role in cognition and psychiatric symptoms as well as motor coordination. The concept of cognitive dysmetria has been making cerebellar function in schizophrenia the focus of current studies. In other words, disruption in the corticocerebellum-thalamic -cortical circuit could lead to disordered cognition and clinical symptoms of schizophrenia. The purposes of this study were to determine cerebellar dysfunction in male schizophrenic patients semiquantitatively with ICARS and to investigate the clinical and cognitive correlates of ICARS in patients. Methods:We compared the scores of cerebellar neurologic sign using ICARS in 47 male patients with a DSM-IV-TR diagnosis of schizophrenia with 30 gender and age-matched healthy control subjects. The semiquantitative 100-point ICARS consists of 19 items divided into 4 unequally weighted subscores:posture and gait disturbances, kinetic functions, speech disorders and oculomotor disorders. All subjects were also assessed with cognitive function test. Cognitive functions were evaluated by Korean-Mini Mental Status Examination (K-MMSE), Verbal fluency test, and Clock drawing test. The patients were administered Korea version of Positive and Negative Symptom Scale(K-PANSS) to assess the symptom severity. Results:Schizophrenic patients had significantly higher scores on the ICARS than control subjects with posture and gait disturbances, kinetic functions, and oculomotor disorders. They also showed more significant impairments in cognitive function tests than control subjects. There was a significant correlation between ICARS and negative symptoms of patients. In cognitive function test, Clock drawing test was significantly associated with negative symptoms. In addition, Clock drawing test was negatively correlated with the total score of ICARS. Conclusion:In this study, we confirmed that schizophrenic patients have significant impairments in cognitive and cerebellar function, and that those were related with negative symptoms of schizophrenic patients. These results support a role of the cerebellum in schizophrenia. It is meaningful that we used a structured, and reliable procedure for rating neurological soft signs, ICARS. We hope that future prospective studies using a similar design help that rate of neurological sign should have been visible with the progression of illness.

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The clinical study on 44 cases of patient with Thoracolumbar Compression Fracture (흉(胸)·요추(腰椎) 압박골절(壓迫骨折) 환자(患者)에 대한 임상적(臨床的) 고찰(考察))

  • Lim, Jeoung-Eun;Kim, Kee-Hyun;Hwang, Hyeon-Seo
    • Journal of Acupuncture Research
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    • v.17 no.2
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    • pp.41-51
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    • 2000
  • Clinical observation was made on 44 cases of stable thoracolumbar compression fracture that were confirmed through simple x-ray and neurological examination. They were hospitalized and treated by acupuncture and moxibustion, bed rest, herb medicine and physical therapy. If necessary, patient was given an enema. The results obtained are as follows. 1. The patient distribution ratio, in regard to sex, was shown to be 1: 13.7 for males to females. In regard to age, it was shown that people in their 60's was the most predominant case, followed by people in their 70's, 80's, 50's and 40's, respectively. 2. In regard to contributing factors, it was observed that accidental falls were most frequent, followed by reasons unknown, repetitive lifting of heavy objects, overlaboring and bruise in that order. 3. In regard to duration of illness before treatment, it was found that treatment within 1 week was most predominant, followed by over 4 weeks, and 1-2 weeks, respectively. 4. With regard to the duration of hospitalization, hospitalization within 2 weeks was found to be most predominant, followed by 2-4 weeks. 5. In regard to the level of the affected vertebral body; The affected vertebral bodies distributed between T3 to L5 except for T7; T12 was found to be most predominant, followed in turn by L1 and L5. 6. In regard to the number of affected vertebral bodies, 2 was the most frequent followed by 1 and 3, in that order. The average of the number of affected vertebral bodies was observed to be 2.8 and single vertebral body compression fracture was shown to have no clinical and statistical difference as compared with multiple vertebral body compression fractures. 7. In regard to the grade of the seriousness of symptoms, it was found that Grade IV was most predominant, followed by Grade III. 8. With regard to signs at the first medical examination, low back pain was seen in the highest number, and followed in order by gait disturbance, flank pain, flexion-extension disturbance, disturbance of rotation to right or left, and bowel dysfunction. 9. Concerning the effect of treatment, good results were most predominantly seen, and 95.5% of total patients showed fair results. 10. The duration of admission treatment due to the grade of clinical symptoms was as follows; In the case of the Grade IV, it was observed that within 2 weeks was most predominant and for Grade III was 2-4 weeks. It was also found that the grade of clinical symptoms was not in proportion to the duration of admission treatment. 11. In regard to the result of treatment due to the grade of clinical symptoms; It was found that in the case of Grade IV, within 2 weeks was most predominant, and for Grade III, it was found to be 2-4 weeks. 12. Intestinal obstruction was shown in 50% of total patients; In the case of duration of constipation, more than 1 week was found to be most predominant, followed by 2 and 4 days respectively. 13. With regard to the treatment of intestinal obstruction, using acupuncture and moxibustion, herb - medicine and enema together were found to be most effective. 14. Intestinal obstruction was mostly seen in the case of Grade IV.

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Does the Gut Microbiota Regulate a Cognitive Function? (장내미생물과 인지기능은 서로 연관되어 있는가?)

  • Choi, Jeonghyun;Jin, Yunho;Kim, Joo-Heon;Hong, Yonggeun
    • Journal of Life Science
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    • v.29 no.6
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    • pp.747-753
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    • 2019
  • Cognitive decline is characterized by reduced long-/short-term memory and attention span, and increased depression and anxiety. Such decline is associated with various degenerative brain disorders, especially Alzheimer's disease (AD) and Parkinson's disease (PD). The increases in elderly populations suffering from cognitive decline create social problems and impose economic burdens, and also pose safety threats; all of these problems have been extensively researched over the past several decades. Possible causes of cognitive decline include metabolic and hormone imbalance, infection, medication abuse, and neuronal changes associated with aging. However, no treatment for cognitive decline is available. In neurodegenerative diseases, changes in the gut microbiota and gut metabolites can alter molecular expression and neurobehavioral symptoms. Changes in the gut microbiota affect memory loss in AD via the downregulation of NMDA receptor expression and increased glutamate levels. Furthermore, the use of probiotics resulted in neurological improvement in an AD model. PD and gut microbiota dysbiosis are linked directly. This interrelationship affected the development of constipation, a secondary symptom in PD. In a PD model, the administration of probiotics prevented neuron death by increasing butyrate levels. Dysfunction of the blood-brain barrier (BBB) has been identified in AD and PD. Increased BBB permeability is also associated with gut microbiota dysbiosis, which led to the destruction of microtubules via systemic inflammation. Notably, metabolites of the gut microbiota may trigger either the development or attenuation of neurodegenerative disease. Here, we discuss the correlation between cognitive decline and the gut microbiota.