• Journal of Life Science
• /
• v.31 no.2
• /
• pp.237-247
• /
• 2021

Immune responses in the central nervous system (CNS) function as the host's defense system against pathogens and usually help with repair and regeneration. However, chronic and exaggerated neuroinflammation is detrimental and may create neuronal damage in many cases. The NOD-, LRR-, and pyrin domain―containing 3 (NLRP3) inflammasome, a kind of NOD-like receptor, is a cytosolic multiprotein complex that consists of sensors (NLRP3), adaptors (apoptosis-associated speck like protein containing a caspase recruitment domain, ASC) and effectors (caspase 1). It can detect a broad range of microbial pathogens along with foreign and host-derived danger signals, resulting in the assembly and activation of the NLRP3 inflammasome. Upon activation, NLRP3 inflammasome leads to caspase 1-dependent secretion of the pro-inflammatory cytokines IL-1β and IL-18, as well as to gasdermin D-mediated pyroptotic cell death. NLRP3 inflammasome is highly expressed in CNS-resident cell types, including microglia and astrocytes, and growing evidence suggests that NLRP3 inflammasome is a crucial player in the pathophysiology of several neuroinflammatory and psychiatric diseases, such as Alzheimer's disease, Parkinson's disease, multiple sclerosis, stroke, traumatic brain injury, amyotrophic lateral sclerosis, and major depressive disorder. Thus, this review describes the molecular mechanisms of NLRP3 inflammasome activation and its crucial roles in the pathogenesis of neurological disorders.

• Clinical and Experimental Pediatrics
• /
• v.50 no.6
• /
• pp.580-584
• /
• 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 rehabilitation welfare engineering & assistive technology
• /
• v.7 no.2
• /
• pp.85-90
• /
• 2013

Core muscle is the most crucial for rehabilitation of patients with musculoskeletal and nervous problem, there is pain, imbalance and functional movement disorder of upper and lower extremities. In this study, low-frequency electrical stimulator was developed for the purpose increasing the activity of rectus abdominis (RA) and the thickness of transverse abdominis (TrA), internal obliquus abdominis (IO), and external obliquus abdominis (EO). Fifteen female in their 20's was divided into a experiment groups (traditional electrical stimulation group, developed electrical stimulation group) and control group. Low-frequency electrical stimulation was performed in experimental groups for 4 times a week, 4 weeks. The muscle activities (RA) by EMG and muscle thickness (TrA, IO, EO) by Ultrasonogrphy imaging were measured, respectively. In the result, the muscle activity of RA and muscle thickness of TrA and EO were significantly increased at developed electrical stimulation group (p<.05). Therefore, the developed low-frequency electrical stimulation is useful for rehabilitation with CNS and PNS subjects.

• Korean Journal of Psychosomatic Medicine
• /
• v.7 no.2
• /
• pp.241-246
• /
• 1999

Delirium is a syndrome characterized by impairement of consciousness, disorientation, disturbance of sleep-wake cycle, memory impairement, disturbance of perception. It is induced by many causes, which are CNS diseases(head trauma, vascular disease, brain tumor, etc), medical diseases(metabolic disorder, endocrine disturbance, cardiovascular disease) and drugs(anticholinergics, anticonvulsant, antipsychotics, cimetidine etc). Transdermal scopolamine which is usually used to prevent motion sickness has anticholinergic property, and so it can induce delirium. The authors report two cases of delirium induced by transdermal scopolamine. The cases shared common characteristics which were as follows : 1. All of two patients were elderly women. 2. Delirium symptom was abruptly occurred during trip after attaching scopolamine patches. 3. Delirium symptom was rapidly improved within 2-3 days. It is important to educate for both users and managers about directions for transdermal scopolamine patch usage to prevent delirium. And careful history taking is needed to diagnose delirium induced by transdermal scopolamine accurately.

• Journal of mucopolysaccharidosis and rare diseases
• /
• v.4 no.1
• /
• pp.26-36
• /
• 2018

Mucopolysaccharidosis IIIA is a heritable neurodegenerative disorder resulting from the dysfunction of the lysosomal hydrolase sulphamidase. This leads to the primary accumulation of the complex carbohydrate heparan sulphate in a wide range of tissues and CNS degeneration. Characterization of animal model is the beginning point of the therapeutic clinical trial. Mouse model has a limitation in that it is not a human and does not have all of the disease phenotypes. Therefore, delineate of the phenotypic characteristics of MPS IIIA mouse model prerequisite for the enzyme replace treatment for the diseases. We designed 6-month duration of phenotypic characterization of MPS IIIA mouse biochemically, behaviorally and histologically. We compared height and weight of MPS IIIA mouse with wild type from 4 weeks to 6 months in both male and female. At 6 months, we measured GAG storage in urine kidney, heart, liver, lung and spleen. The brain GAG storage is presented with Alcian blue staining, immunohistochemistry, and electron-microscopy. The neurologic phenotype is evaluated by brain MRI and behavioral study including open field test, fear conditioning, T-maze test and Y-maze test. Especially behavioral tests were done serially at 4month and 6month. This study will show the result of the MPS IIIA mouse model phenotypic characterization. The MPS IIIA mouse provides an excellent model for evaluating pathogenic mechanisms of disease and for testing treatment strategies, including enzyme or cell replacement and gene therapy.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.18 no.3
• /
• pp.388-395
• /
• 1996

Congenital muscular torticollis(CMT) is a disorder characterized by shortening of at least one of the cervical muscles and tilting of the head to opposite side. The most commonly affected muscle is the sternocleidomastoid muscle. Pathogenesis and etiology of congenital muscular torticollis were not clearly identified, but considered as fetal malposition, birth trauma, vascular accident, heredity, infection and CNS pathology. Untreated congenital muscular torticollis often causes facial asymmetry and This is the rasult of tensional rotation of the face toward affected side. So early treatment may prevent facial and neck asymmetry and limitation of neck movement. There are many treatment methods in CMT, including conservative and operative method, but presently Bipolar release and Z-Plasty of SCM muscle has been introduced when the conservative treatment had failed. The benefits of this method are to preservation of the normal Neck V-contour and improvement of the neck motion. We treated CMT using Bipolar release and Z-plasty in two patients. After that the patients improved on the range of neck motion and maintained the normal V-conture of the neck, so we report two cases of CMT with literatures.

• Proceedings of the Korean Environmental Health Society Conference
• /
• 2005.12a
• /
• pp.87-94
• /
• 2005

Results from previous studies revealed that metal level in the body is related to certain types of diseases. For example. serum copper level with chronic heart failure, iron and transferrin in the blood serum with acute cerebral vascular diseases, Zn in the CNS, lead with neurotoxicity, hypertension, genetic damage, arsenic with cancer skin lesion, Al with neurobehavioral function (cognitive impairment and memory disorder), and etc. The rate of stroke has increased in recent years and several metals were found to be responsible for causing stroke. This study compared several blood metal concentrations between stroke and non-stroke patients. Patients with stroke (116 samples) and non-stroke (111 samples including lowback pain and others) participated in this study. Total of 227 blood samples were collected and participants completed questionnaires regarding age, gender, occupation, residence, alcohol, smoking, and etc. To be qualified into the stroke group, patients have never experienced stroke previously. Subjects only included ischemic stroke and intracerebral hemorrhage patients diagnosed by brain CT and brain MRI. Patients with high risk of metal exposure such as herbal intake and job related exposure were excluded. 10ml of blood samples were analyzed by ICP-MS method at the Center of Nature and Science at Sangji University. Metal geometric mean (SD) concentrations in blood of study subjects showed higher values, 2.64-36.12%, than WHO reference values in Mn, Ni, Hg, Se, and As. Metal concentration in blood of stroke patients non-adjusted for potential confounders was higher except for Hg and also higher except for Ni in adjusted for potential confounders. Co was significantly higher in stroke patients (p=0.002) than non-stroke patients adjusted for potential confounders. Regression coefficient values of stroke patients was 0.17-8.25 in each metals. Odd ratio of stroke patients had 0.96 (Ni)-2.68 (Co) compared to non-stroke cases. This result means that Co increase of 1 raises the risk ratio of stroke by 2.86 times. Based on the results, metal concentration in blood seems to affect incidence of stroke.

• Journal of Life Science
• /
• v.22 no.8
• /
• pp.1057-1063
• /
• 2012

Epilepsy is the most prevalent chronic neurological disorder and can be controlled by antiepileptic drugs (AEDs) in up to 70% of patients. We performed an association study between adverse drug reactions and the genetic polymorphisms of CYP2C9, CYP2C19, ABCB1, and SCN1A. The clinical data of 83 epilepsy patients who had received AEDs containing carbamazepine (CBZ) were collected. We extracted genomic DNA from peripheral blood and then genotyped CYP2C9 ($CYP2C9^*2$, $CYP2C9^*3$), CYP2C19 ($CYP2C9^*2$, $CYP2C9^*3$), ABCB1 (C3435T), and SCN1A (IVS5N+5 G>A) using direct sequencing. The allele frequencies of $CYP2C9^*3$, $CYP2C9^*2$, $CYP2C9^*3$, ABCB1 (3435C>T), and SCN1A (IVS5N+5 G>A) were 0.93, 0.72, 0.91, 0.61, and 0.55, respectively. Statistically significant differences were indicated from the data obtained. Patients with SCN1A genotype CC or CT were compared with patients with SCN1A genotype TT while using more than 500mg of carbamazepine. We have associated functional polymorphisms with the dose used in regular clinical practice for Korean epilepsy patients who had received antiepileptic drugs (AEDs) containing carbamazepine. For AEDs, we found that one of the SCN1A genotypes is associated with a 500 mg dose. There was no association found with CNS ADR caused by AEDs.