• Journal of Microbiology and Biotechnology
• /
• v.28 no.9
• /
• pp.1413-1425
• /
• 2018

Shiga toxins (Stxs) are the main virulence factors expressed by the pathogenic Stx-producing bacteria, namely, Shigella dysenteriae serotype 1 and certain Escherichia coli strains. These bacteria cause widespread outbreaks of bloody diarrhea (hemorrhagic colitis) that in severe cases can progress to life-threatening systemic complications, including hemolytic uremic syndrome (HUS) characterized by the acute onset of microangiopathic hemolytic anemia and kidney dysfunction. Shiga toxicosis has a distinct pathogenesis and animal models of Stx-associated HUS have allowed us to investigate this. Since these models will also be useful for developing effective countermeasures to Stx-associated HUS, it is important to have clinically relevant animal models of this disease. Multiple studies over the last few decades have shown that mice injected with purified Stxs develop some of the pathophysiological features seen in HUS patients infected with the Stx-producing bacteria. These features are also efficiently recapitulated in a non-human primate model (baboons). In addition, rats, calves, chicks, piglets, and rabbits have been used as models to study symptoms of HUS that are characteristic of each animal. These models have been very useful for testing hypotheses about how Stx induces HUS and its neurological sequelae. In this review, we describe in detail the current knowledge about the most well-studied in vivo models of Stx-induced HUS; namely, those in mice, piglets, non-human primates, and rabbits. The aim of this review is to show how each human clinical outcome-mimicking animal model can serve as an experimental tool to promote our understanding of Stx-induced pathogenesis.

• Advances in Traditional Medicine
• /
• v.10 no.4
• /
• pp.239-253
• /
• 2010

Coenzyme $Q_{10}$ ($CoQ_{10}$, or ubiquinone) is an electron carrier of the mitochondrial respiratory chain (electron transport chain) with antioxidant properties. In view of the involvement of $CoQ_{10}$ in oxidative phosphorylation and cellular antioxidant protection a deficiency in this quinone would be expected to contribute to disease pathophysiology by causing a failure in energy metabolism and antioxidant status. Indeed, a deficit in $CoQ_{10}$ status has been determined in a number of neuromuscular and neurodegenerative disorders. Primary disorders of $CoQ_{10}$ biosynthesis are potentially treatable conditions and therefore a high degree of clinical awareness about this condition is essential. A secondary loss of $CoQ_{10}$ status following HMG-CoA reductase inhibitor (statins) treatment has been implicated in the pathophysiology of the myotoxicity associated with this pharmacotherapy. $CoQ_{10}$ and its analogue, idebenone, have been widely used in the treatment of neurodegenerative and neuromuscular disorders. These compounds could potentially play a role in the treatment of mitochondrial disorders, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, Friedreich's ataxia, and other conditions which have been linked to mitochondrial dysfunction. This article reviews the physiological roles of $CoQ_{10}$, as well as the rationale and the role in clinical practice of $CoQ_{10}$ supplementation in different neurological diseases, from primary $CoQ_{10}$ deficiency to neurodegenerative disorders. These will help in future for treatment of patients suffering from neurodegenerative disease.

• Journal of Periodontal and Implant Science
• /
• v.34 no.2
• /
• pp.357-366
• /
• 2004

Chronic exposure to high levels of manganese leads a pronounce and debilitating disorder known as manganism. Research on the toxic manifestation of manganese have focused primarily on its neurological effects because exposure to high levels of the metal produces a distinct and irreversible extrapyramidal dysfunction resembling the dystonic movements associated with Parkinson's physiological and biochemical systems in the body. The purpose of this study was to evaluate the effect of manganeses on primary rat calvarial cell growth and toxicity. The experimental groups were in concentration of 0, 10, 30, 60, 100, 300 ${\mu}M$. Cell activity was assessed at day 1 and day 3 using a fluorescent molecular probe. Cell proliferation was evaluated at day 1 and day 3 by MTT assay. The amount of total protein synthesis was measured at day 3 and day 7. The results were as follows: The proliferation of primary rat calvarial cells were inhibited by $MnCl_2$ in the concentration exceeding $100{\mu}M$. The primary rat calvarial cells treated with $MnCl_2$ showed similar protein synthesis to the control group except in 100 ${\mu}M$. These result suggest that manganese suppress the viability and protein synthesis of primary rat calvarial cells in concentration exceeding $100{\mu}M$.

• Journal of Periodontal and Implant Science
• /
• v.34 no.4
• /
• pp.771-780
• /
• 2004

Chronic exposure to high levels of manganese (Mn) leads a pronounced and debilitating disorder known as manganism. Research on the toxic manifestation of manganese have focused primarily on its neurological effects because exposure to high levels of the metal produces a distinct and irreversible extrapyramidal dysfunction resembling the dystonic movements associated with Parkinson's physiological and biochemical systems in the body. The purpose of this study is to determine the effects of Mn on mineralization in primary rat calvarial cells. The experimental groups were in concentration of 0, 10, 30 and 60 ${\mu}M$. The results were as follows: 1. ALP activity was decreased in concentration of 30 and 60 ${\mu}M$ (p<0.01). 2. Bone nodule formation was depressed in concentration of 30 and 60 ${\mu}M$ at day 14 and 21 (p<0.01). 3. RT-PCR results showed an altered expression of bone matrix proteins. These result suggested that manganese might decrease or alter the expression of the osteoblast phenotype.

• The Journal of Korean Academy of Sensory Integration
• /
• v.6 no.1
• /
• pp.25-33
• /
• 2008

Objective : To identify the effectiveness of a short-term intensive intervention program on the improvement of adaptive response of a child with mixed sensory integration disorder. Method : Four and half years old boy who has been diagnosed of PDD received 40 min of one intervention session and 50 min of 3 intervention sessions. The intervention was a part of the 2008 Sensory Integration Treatment Course developed by the Korean Academy of Sensory Integration (KASI) and all sessions were implemented under supervision by experts. Result : Adaptive responses of the child were enhanced throughout the intervention process in terms of postural response and peer interaction. His oral defensiveness is improved. As the intervention progressed, he exhibited more active movements, louder voice, and coherence within peer group. Conclusion : This case report demonstrates effectiveness of a short-term intensive intervention program in terms of improving adaptive response. To enrich the effectiveness, tt is suggested to educate parents about neurological base of the child's behaviors so the they understand the importance of various sensory experience within play.

• The Journal of Korean Physical Therapy
• /
• v.21 no.1
• /
• pp.65-71
• /
• 2009

Purpose: Numerous investigators demonstrated that adaptative changes were induced by motor skill acquisition in the central nervous system. We investigated the changes of neuroelectric potential following motor learning with serial reaction time task in young healthy subjects, using electroencephalography (EEG). Methods: Twelve right-handed normal volunteers were recruited, who have no history of neurological dysfunction and were given to written the informed consent. All subjects were assigned to flex to extend the wrist joint or flex the thumb for pressing the matched button as quickly and accurately as possible, when one of five colored lights was displayed on computer screen (red, yellow, green, blue, white). EEG was measured, whenfive types simulations ware presented randomly with equal probabilities of 20% in total 200 times at the pre and post test. And they were scheduled for 30 minutes practice session during two consecutive days in the laboratory. Results: The results showed that the reaction time at the post test was significantly reduced, compared to one of the pre test in serial reaction time task. In EEG map analysis, the broaden bilateral activation tended to be changed to the focused contralateral activation in the frontoparietal area. Conclusion: These findings showed that acquisition of motor skill led to product more fast motor execution, and that motor learning could change cortical activation pattern, from the broaden bilateral activation to the focused contralateral activation. Thus we concluded that the adaptative change was induced by motor learning in healthy subjects.

• Journal of Korean Neurosurgical Society
• /
• v.46 no.2
• /
• pp.99-102
• /
• 2009

Objective: Cardiac dysfunction after aneurysmal subarachnoid hemorrhage (SAH) is associated with elevation of serum cardiac troponin I (cTnl) levels. Elevation of cTnl predicts cardiopulmonary and neurological complications, and poor outcome. Methods: We retrospectively reviewed the medical and radiologic records of 114 (male: 30, female: 84) patients who developed aneurysmal SAH between January 2006 and June 2007 and had no history of previous cardiac problems. We evaluated their electrocardiography and cTnl level, which had been measured at admission. A cTnl level above 0.5 $\mu$g/L was defined as an indicator of cardiac injury following SAH. We examined various clinical factors for their association with cTnl elevation and analyzed data using chi-square test, t-test and logistic regression test with SPSS version 12.0. The results were considered significant at p< 0.05. Results: The following parameters shows a correlation with cTnl elevation: higher Hunt-Hess (H-H) grade (p = 0.000), poor Glasgow Outcome Scale (GOS) score (p = 0.000), profound pulmonary complication (p = 0.043), higher heart rate during initial three days following SAH (p = 0.029), ruptured aneurysm on communicating segment of internal carotid artery (p = 0.025), incidence of vasospasm (p = 0.421), and duration of hyperdynamic therapy for vasospasm (p = 0.292). A significant determinants for outcome were cTnl elevation (p = 0.046) and H-H grade (p = 0.000) in a multivariate study. Conclusion: A cTnl is a good indicator for cardiopulmonary and neurologic complications and outcome following SAH. Consideration of variable clinical factors that related with cTnl elevation may be useful tactics for treatment of SAH and concomitant complications.

• Therapeutic Science for Rehabilitation
• /
• v.2 no.1
• /
• pp.5-12
• /
• 2013

The purpose of this review is to address the flow of current neuroscientific researches and to provide for the clinicians with therapeutic evidences for schizophrenia which can help them clinical decision making. Since the very beginning, a lot of scientific studies about schizophrenia have been undertaken. In this review, I describes the evidences focused on development of schizophrenia including neurobiological dysfunction, neurodevelopmental model, Kalirin, and Brain-Derived Neurotrophic Factor(BDNF) and neuroanatomic abnormalities based on neuroimaging studies. In conclusion, schizophrenia influencing on broad impairment of human function such as activities of daily life, occupations, and relationships has been studied underlying causes and treatments, but still remained uncertainty. However, there are plenty of useful evidences available for the clinicians to make a good therapeutic choice.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.11 no.1
• /
• pp.88-98
• /
• 2011

Lesch-Nyhan syndrome is a X-linked recessive disorder caused by a deficiency of the enzyme hypoxanthine-guanidine phosphoribosyltransferase (HPRT), enzyme to recycle purines. Case history: born induced vaginal delivery at 40 weeks complicated by premature membrane ruputure, body weight 2.820 gm. He showed failure to thrive showing severe protein aversion like milk products and pink daper. Developmental delay revealing rolling over at 10.5 month, followed by regression. Seizure at 2 months, His poor oral feeding was lifelong problem. Weak crying, spastic, choreoathetoid movement. Self mutilating behavior noted and diagnosed at age 3 years. No family history of consanguinity and neurological disorders. Method: Laboratory test, physical exam, imaging study and molecular. Clinical follow up Treat ment with allopurinol. Result: uric acid 10.5 mg/dL (N 3.5-7.9), APRT 151.1uM/ min/ml pro(25.7-101), HPRT 7.6 (N 233.5-701) and c.151C>T hemizygote (p,Arg51X). Abdominal sonogram showed staghorn calculi in both kidneys, brain MRI brain atrophy. Clinical follow up showed, seizure at 2 mo, developmental delay (head control and, rolling over at at 11mo, pointing body part at 2 yr 7 mo, eye hand coordination at 2 y 11mo,creeping at 3 y 7 mo, speaking words at 6 y 6 mo ),and developmental regression at 3 yr of age. Sleeping problem including insomnia and severe constipation. Self mutilating behavior (lip bite) started at 2.5 yr, neurologic sx including intermittent upward gaze accompanied by swallowing difficulty at 3 y 7 mo grand mal seizure at 4.5 yr and spastic extremity and trunchal hypotonia and choleoathetoid movement and ataxia at 6.5 yr. Scoliosis with severe spasticity at 9 yr 9 mo. Acute life threatening episode with irregular breathing at 9 yr and 9 mo, Emaciation and nephrolithiasis and recurrent pneumonia. Died suddenly at 10 yr 3 mo. Conclusion: life long feeding problem, chronic gut motility dysfunction, sleeping difficulty and progressing neurologic deterioration and nephrolithiasis despite normal serum uric acid maintence by allopurinol treatment.

• Annals of Clinical Neurophysiology
• /
• v.1 no.2
• /
• pp.118-125
• /
• 1999

Background and Aims : Nerve conduction study is invaluable in clinical neurology, especially for assessing peripheral neuropathies. Abnormal nerve conduction studies may result not only from peripheral nerve dysfunction itself, but also from other various mechanical, technical, and physiological factors such as age, sex, height and temperature. So we conducted this study to establish the our own normal values. Methods : In this study, from March. 1997 to July. 1998, 40 Korean adults among person came to Health Promotion Center over the age of 20 without any suspicion of neurological deficits were analysed to determine the effect of compound effects of several physiological factors. Results : The nerve conduction velocities of the upper extremity and proximal segments were faster than those of the lower extremity and distal segments. Physiological factors such as age, height and temperature affect the results of nerve conduction studies in multiple regression analysis. The sex difference is recognized over peroneal motor nerve. There are no sex differences in amplitude transformed into normal distribution. The significant physiological factor affecting the amplitude of nerve conduction is age, whereas height and temperature play no role. Conclusions : In multiple regression analysis, height is widespread variable for the nerve conduction velocities and temperature is important variable for lower extremities. The parametric statistical analysis cannot be applied to the amplitude of the compound muscle or nerve action potentials because of marked left shift in distribution. Sqareroot transformation of the CMAP and CNAP may be useful in normalizing the distribution. The most significant physiological factor affection the amplitude is age. Sex differences are not seen in nerve conduction study.