Murat Mengi

Treatment of Homocystinuria-Related Dystonia with Deep Brain Stimulation: A Case Report

A 23-year-old woman with the medical history of homocystinuria that had been diagnosed at the age of 14 has been non-responsive to treatment. The patient presented with the symptoms of dysphonia, dysarthria and severe dystonia of the neck and left extremities. Blood and urine biochemistry revealed high levels of homocystine. Brain magnetic resonance imaging was normal with no detectable pathologies. Medical treatment strategies were used and repeated injections of botulinum toxin A were administered, but the symptoms showed no significant improvement. The patient was then operated, and deep brain stimulators targeting the bilateral globus pallidus internus were implanted. After the activation of the electrodes, dystonia symptoms showed a remarkable improvement. Good outcome was documented during the follow-up period of 7 months. To our best knowledge, this is the first reported case of homocystinuria-related dystonia symptoms that were successfully treated with deep brain stimulation.

Pallidal deep brain stimulation in a 5-year-old child with dystonic storm: case report.

A 5-year-old child had a medical history of epilepsy and a newly presented mental retardation with a life-threatening dystonic storm. Neuroimagings showed bilateral calcification of the pallidum. Several treatment modalities were performed, but the symptoms showed no significant improvement. The patient was operated on in order to place a deep brain stimulation (DBS) targeting bilateral globus pallidum internus (GPi). The dystonia showed a remarkable improvement after surgery, with 81% reduction of dystonia severity after 15 months. To our best knowledge, this is the youngest patient mentioned in the literature to be treated with DBS, which was also life-saving in this case.

Regular Aerobic Training Combined with Range of Motion Exercises in Juvenile Idiopathic Arthritis

Objective. To assess the effects of regular aerobic training combined with range of motion (ROM) exercises on aerobic capacity, quality of life, and function in children with juvenile idiopathic arthritis (JIA). Methods. Thirty patients with JIA and 20 healthy age-matched controls (mean age ± SD, 11.3 ± 2.4 versus 11.0 ± 2.3, resp.; P > 0.05) were included. All patients performed aerobic walking (4 days a week for 8 weeks) and active and passive ROM exercises of involved joints. All patients completed the childhood health assessment questionnaire (CHAQ) and the child health questionnaire. ROM measurements of joints were performed by using universal goniometer. Aerobic capacity was determined by measuring peak oxygen uptake (VO2peak) during an incremental treadmill test. Results. Peak oxygen uptake and exercise duration were significantly lower in JIA group than in controls (32.5 ± 6.6 versus 35.9 ± 5.8 and 13.9 ± 1.9 versus 15.0 ± 2.0, resp.; P < 0.05 for both). Eight-week combined exercise program significantly improved exercise parameters of JIA patients (baseline versus postexercise VO2peak and exercise duration, 32.5 ± 6.6 to 35.3 ± 7.9 and 13.9 ± 1.9 to 16.3 ± 2.2, resp.; P < 0.001 for both). Exercise intervention significantly improved CHAQ scores in JIA patients (0.77 ± 0.61 to 0.20 ± 0.28, P < 0.001). Conclusion. We suggest that regular aerobic exercise combined with ROM exercises may be an important part of treatment in patients with JIA.

Influence of Intermittent Hypobaric Exposure on SOD and TBARS Levels in Trained Rats

Live high train low (LHTL) is a well-known training model for preparation of competitions. In this study, the thiobarbituric acid reacting substances (TBARS) levels and superoxide dismutase (SOD) activity were determined in heart, lung and muscle tissues of rats. They were intermittently exposed to hypobaric pressure of 523 mmHg, corresponding to an altitude of 3,000 m, and they performed swim training at sea level. Two groups of male rats were trained to swim for thirty minutes a day and 4 days a week, lasting 9 weeks. Two groups were exposed to hypobaria for 120 min a day and 4 days a week for 9 weeks in pressure cabin. In heart tissue, TBARS levels of normobaric trained (NbT) group was higher (P < 0.05) than those of the normobaric sedentary (control) group. TBARS levels of hypobaric trained (HbT) group was higher than those of the control and hypobaric sedentary (Hb) groups (P < 0.001; P < 0.01, respectively). TBARS levels of lung tissue of HbT group was also higher than those of the same groups (control; P < 0.01, Hb; P < 0.05, respectively). In muscle tissue, TBARS levels of HbT group was higher than those of the sedentary groups (control; P < 0.001, Hb; P < 0.05, respectively). SOD activity of heart tissue of HbT group was higher (P < 0.001) than that of the other groups. In lung tissue, SOD activity of control group was lower than that of the other groups (HbT; P < 0.001, NbT; P < 0.01, Hb; P < 0.01, respectively). In muscle tissue, SOD activity of HbT group was higher (P < 0.01) than that of the control group. The results of this study suggest that intermittent hypobaric exposure may augment exercise-induced oxidative stress in heart, lung and muscle of trained rats.

Intraventricular albumin: an optional agent in experimental post-traumatic brain edema

Abstract Hypothesis: Human albumin may be effective in the treatment of posttraumatic brain edema due to its hyperoncotic features. Therefore, the aim of the experimental study presented in this paper has two points: the first is to evaluate the efficacy of intraventricular hyperoncotic human albumin on post-traumatic brain edema and the second is to try to show the appropriate posttraumatic time window for albumin administration. Method: Traumatic brain injury and subsequent edema was formed by a model of impact acceleration injury in rats. Human albumin was administered via intraventricular route by using a stereotactic head holder. All animals in each group were decapitated 24 hours after the procedure and the effect of albumin was evaluated by measurement of tissue specific gravity. Results: Tissue specific gravity decreased in edematous tissue (trauma indicator), increased after albumin administration at the 12th (p<0.001), and both at the 1st and 12th hour of the trauma (edema treatment; p<0.001). On the other hand, albumin administered at the 12th, and at both the 1st and 12th hours in the rats without trauma has caused the formation of the brain edema. Conclusion: We conclude that human albumin is effective in cytotoxic, but not in vasogenic edema and exerts its best anti-edematous effect at the 12th hour of severe head trauma and this study may help future studies that will try to show the effects of albumin with different time modalities after severe head injury.

Age-related changes in rat prostate tissue; perspective of protein oxidation.

Abstract Background: Increased systemic oxidative stress is considered as an important risk factor for prostate cancer occurrence; however, the relationship between impaired redox homeostasis of prostate tissue and aging remains unclear. Objective: In our study, we hypothesized that age-related deterioration of redox homeostasis in prostate tissue may be considered as a predisposing factor for prostate cancer occurrence. Methods: Sprague–Dawley rats were divided into two groups as young control (5 months) and naturally aged (24 months). We investigated the levels of oxidant and antioxidant parameters in prostate tissue. Results: Advanced oxidation protein products, protein carbonyl, non-protein thiol and lipid hydroperoxides levels of aged rats were significantly higher than in the young control rats (p < 0.01, p < 0.05, p < 0.001, p < 0.05, respectively). Additionally, antioxidant activity of Cu-Zn-superoxide dismutase in elderly group was significantly lower than young controls (p < 0.05). Conclusions: We suggest that increased non-protein thiol levels found in aged rats may prevent further dissemination of oxidative protein damage. We also propose that the increased levels of oxidative protein damage markers and decreased Cu-Zn superoxide dismutase activity in aged prostate may be considered as a predisposing factor for prostate cancer. Further studies are warranted to clarify all these oxidative changes as initiation factors for prostate cancer in the association of aging with prostate cancer.

The effects of lipoic acid on redox status in brain regions and systemic circulation in streptozotocin-induced sporadic Alzheimer’s disease model

AbstractWhile the deterioration of insulin-glucose metabolism (IGM), impaired redox homeostasis (IRH), β-amyloid accumulation was reported in Sporadic Alzheimer’s Disease (SAD) model, aforementioned factors related to lipoic acid administration and anthropometric indexes (AIs) are not yet studied with integrative approach. β-amyloid accumulation, redox homeostasis biomarkers and AIs are investigated in SAD model. Streptozotocin-induced inhibition of insulin-signaling cascade but not GLUT-2 and GLUT-3 transporters takes a role in β-amyloid accumulation. Inhibition types are related to IRH in cortex, hippocampus and systemic circulation. Lipoic acid (LA) shows both antioxidant and prooxidant effect according to the anatomical location. LA administration also leads to improved AIs during GLUT-2 inhibition and cortical redox status in GLUT-3 inhibited group. Optimal LA action could be possible if its redox behavior is balanced to antioxidant effect. Diagnostic usage of systemic IRH parameters as biomarkers and their possible correlations with deteriorated IGM should be investigated. Graphical abstractᅟ

Interaction of Cocaine- and Amphetamine-regulated Transcript and Neuropeptide Y on Behavior in the Central Nervous System.

INTRODUCTION In the central nervous system, cocaine- and amphetamine-regulated transcript (CART) 55-102 peptide is localized in areas, such as the ventral tegmental area, amygdala, hypothalamus, and hippocampus, where emotional activity is regulated. Studies on the effects of the intracerebroventricular (ICV) administration of CART peptide on behavior remain limited. The findings from these studies suggest that this neuropeptide has anxiogenic-like effects. In the central nervous system, neuropeptide Y (NPY) has similar localization as CART. Previous behavioral studies have demonstrated that the ICV administration of NPY has anxiolytic-like effects. METHODS In our study, we established five experimental groups of male Wistar rats to study the competitive effects of NPY and CART peptide. These groups were sham (n=10), CART (n=10), NPY (n=10), CART-NPY (n=10), and NPY-CART (n=10). The open field test, elevated plus maze test, and Porsolt swim test were performed for behavioral analyses. Moreover, the rats were decapitated after the behavioral tests, and the amount of these two peptide in their brains was quantified. RESULTS Our study revealed that the ICV administration of CART peptide is anxiogenic and inhibits animals undergoing learned helplessness in the Porsolt swim test. When we evaluated the results of our study with respect to NPY, we observed its anxiolytic-like effects; in the Porsolt swim test, although it reduced the duration of immobilization, it did not affect the period of struggle. CONCLUSION Our results revealed that during the competitive interaction of these two peptides, anxiogenic CART peptide suppressed the anxiolytic effects of NPY.