Rett syndrome is a progressive encephalopathy in females that appears during the first 18 months of the life. A few neurophysiologic investigations of peripheral nerve and electrodiagnostic studies in Rett syndrome has proposed mild distal axonopathy. However, the electrodiagnostic study shows demyelination rather than axonopathy in presenting patient with Rett syndrome. An 11-year-old female had normal birth history, no perinatal problems and normal development until age of 18 months. Developmental regression was recognized by her parent and slowly progressed. At physical examination at her age of 7 years, stereotypic hand movements was present and she could walk with frequent fall. Brain Computed Tomography was not specific and electrodiagnostic study revealed slow conduction velocity in all nerves tested. After follow-up for 4 years, hand wringling was remained and she became to be unable to walk alone. Electrodiagnostic study revealed as follows; 1) Compound muscle action potentials and sensory nerve action potentials in all the nerves tested revealed prolonged distal latency with normal amplitude. 2) F waves were evoked with prolonged latency in the all the nerves tested. 3) H-reflex were absent, bilaterally. 4) Facial motor conduction study and blink reflex showed normal findings. 5) Auditory evoked potential and visual evoked potential studies showed normal findings. These electrodiagnostic study indicates demyelinating neuropathy.
Action Potentials ; Blinking ; Brain ; Child ; Demyelinating Diseases ; Electromyography ; Evoked Potentials, Auditory ; Evoked Potentials, Visual ; Female ; Follow-Up Studies ; H-Reflex ; Hand ; Humans ; Parents ; Peripheral Nerves ; Physical Examination ; Reproductive History ; Rett Syndrome

The study of blink reflexes was carried out to demonstrate the correlations, if there were, between the stage of diffuse axonal injury(DAI) and the abnormality of blink reflexes. The blink reflex was recorded in 20 healthy adult subjects and 22 patients with DAI who were classified according to Adams' classification(DAI I; 7, DAI II; 9 and DAI III; 6). The latencies and amplitudes of R1 and R2 in patients with DAI were compared with those of healthy subjects. The results were as follows; 1) In 20 subjects of patient group, the latencies of R1 were all within a normal range. In 2 subjects, the difference in latencies between the two sides was above 1.4 msec. 2) In 15 subjects, R2 was absent or delayed, and reduced in the size of amplitude in all. Nine were affected bilaterally, and 4 were unilaterally. 3) Seventy one percent of patients in each stage represented abnormal findings. 4) There were no correlations between the DAI stage and the blink reflex. This study demonstrated that the polysynaptic R2 was more profoundly suppressed than the oligosynaptic R1 in a diffuse axonal injury because of a loss or decrease of suprapontine facillitation, which influenced the trigeminal spinal complex and the interneuron of lateral reticular formation.
Adult ; Axons ; Blinking* ; Diffuse Axonal Injury* ; Humans ; Interneurons ; Reference Values ; Reticular Formation

Congenital melanocytic nevus (CMN) is a benign condition that either is present at birth or develops in the first weeks of life. Surgical removal is typically performed to improve cosmetic appearance and reduce the risk of malignant transformation. In this report, we present the case of a 26-year-old woman with a medium-sized CMN on her left breast. The nevus measured 14×8 cm, and the patient desired a single-stage excision. However, this approach would result in a large skin defect that would be challenging to reconstruct using a local flap or skin graft. Moreover, it could potentially compromise the maintenance of natural sagging and the contour of the breast. Consequently, we opted to place a thin thoracodorsal artery perforator free flap following the removal of the CMN. The patient was satisfied with the overall surgical results. By utilizing this free flap for reconstruction, we successfully preserved the natural shape and contour of the breast without complications such as postoperative hypertrophic scarring or contracture at the recipient site.

A 25-year-old woman was referred for discomfort when breathing through her left nose. The patient had undergone augmentation rhinoplasty 5 years ago, after which hypertrophic scarring occurred in the left nostril. Several corticosteroid injections were administered as the first line of treatment, but with no symptom improvement. Therefore, we proceeded with surgical scar removal, with the use of a nasal conformer. However, scarring in the left nostril recurred. Accordingly, we proceeded with further surgical treatment using the scar folding technique. After scar folding, neither scarring nor nostril stenosis recurred during 1 year of postoperative follow-up. To summarize, herein, we report a case of hypertrophic scarring in the nostril that was successfully treated with the scar folding technique.

Soybean polyunsaturated phosphatidylcholine (PC) is thought to exert anti-inflammatory activities and has potent effects in attenuating acute renal failure and liver dysfunction. The aim of this study was to investigate the effects of PC in protecting multiple organ injury (MOI) from lipopolysaccharide (LPS). Six groups of rats (N=8) were used in this study. Three groups acted as controls and received only saline, hydrocortisone (HC, 6 mg/kg, i.v.) or PC (600 mg/kg, i.p.) without LPS (15 mg/kg, i.p.) injections. Other 3 groups, as the test groups, were administered saline, HC or PC in the presence of LPS. Six hours after the LPS injection, blood and organs (lung, liver and kidney) were collected from each group to measure inflammatory cytokines and perform histopathology and myeloperoxidase (MPO) assessment. Serum cytokines (TNF-alpha, IL-6 and IL-10) and MPO activities were significantly increased, and significant histopathological changes in the organs were observed by LPS challenge. These findings were significantly attenuated by PC or HC. The treatment with PC or HC resulted in a significant attenuation on the increase in serum levels of TNF-alpha and IL-6, pro-inflammatory cytokines, while neither PC nor HC significantly attenuated serum levels of IL-10, anti-inflammatory cytokine. In the organs, the enhanced infiltration of neutrophils and expression of ED2 positive macrophage were attenuated by PC or HC. Inductions of MPO activity were also significantly attenuated by PC or HC. From the findings, we suggest that PC may be a functional material for its use as an anti-inflammatory agent.
Acute Kidney Injury ; Animals ; Cytokines ; Hydrocortisone ; Inflammation ; Interleukin-10 ; Interleukin-6 ; Kidney ; Liver ; Liver Diseases ; Lung ; Macrophages ; Neutrophils ; Peroxidase ; Phosphatidylcholines ; Rats ; Soybeans ; Tumor Necrosis Factor-alpha

Objective: This aim examined the outcomes of resuscitation and the clinical characteristics of patients with pre-hospitaltraumatic cardiac arrests (TCA). Methods: The charts of patients with pre-hospital TCA who visited the various emergency department (ED) in Gangwondofrom January 2013 to December 2017 were reviewed retrospectively. Results: TCA patients comprised 0.3% of patients transferred by 119. A total of 367 patients were enrolled in the study.Traffic accidents were the leading cause of the arrest. The initial field and ED rhythm were mostly asystole (field, 79.6%;ED, 82.3%). It took 11.24±9.95 minutes from the call to the field. From the field to ED, it took 22.87±15.37 minutes. Thetotal CPR time before ED arrival was 21.62±15.29 minutes. The causes of TCA were brain injury (35.7%), hypovolemicshock (29.2%), and severe lung injury (16.3%). Seventy TCA patients experienced at least one return of spontaneous circulation(ROSC). Twenty-six patients (7.14%) were admitted to the ward, and their average injury severity score was38.96. Eight patients expired before 12 hours after transient ROSC. Four more patients expired before 24 hours. Fourpatients were discharged alive among patients who lived for more than 24 hours. Conclusion In this study, 1.5% of patients were discharged alive. The possibility of ROSC was higher as the time to ED,and the cardiopulmonary resuscitation time of 119 was shorter. Pulseless electrical activity rather than asystole tends topromote ROSC. The survival rate increased when ROSC occurred before arriving at the ED.

BACKGROUND: It has been recognized that a defect in klotho gene expression accelerates the degeneration of multiple age-sensitive traits. Accumulating evidence indicates that aging is associated with declines in cognitive function and the activity of growth hormone (GH)/insulin-like growth factor-1 (IGF-1). METHODS: In this study, we examined whether a GH-releaser diet could be effective in protecting against cognitive impairment in klotho mutant mice. RESULTS: The GH-releaser diet significantly induced the expression of IGF-1 and IGF-1 receptors in the hippocampus of klotho mutant mice. Klotho mutant mice showed significant memory impairments as compared with wild-type mice. In addition, the klotho mutation significantly decreased the expression of cell survival/antiapoptotic factors, including phospho-Akt (p-Akt)/phospho-glycogen synthase kinase3beta (p-GSK3beta), phospho-extracellular signal-related kinase (p-ERK), and Bcl-2, but significantly increased those of cell death/proapoptotic factors, such as phospho-c-jun N-terminal kinase (p-JNK), Bax, and cleaved caspase-3 in the hippocampus. Treatment with GH-releaser diet significantly attenuated both decreases in the expression of cell survival/antiapoptotic factors and increases in the expression of cell death/proapoptotic factors in the hippocampus of klotho mutant mice. In addition, klotho mutation-induced oxidative stress was significantly attenuated by the GH-releaser diet. Consequently, a GH-releaser diet significantly improved memory function in the klotho mutant mice. GH-releaser diet-mediated actions were significantly reversed by JB-1, an IGF-1 receptor antagonist. CONCLUSION: The results suggest that a GH-releaser diet attenuates oxidative stress, proapoptotic changes and consequent dysfunction in klotho mutant mice by promoting IGF-1 expression and IGF-1 receptor activation.
Aging* ; Animals ; Caspase 3 ; Diet* ; Gene Expression ; Growth Hormone ; Hippocampus ; Insulin-Like Growth Factor I ; Memory ; Mice* ; Oxidative Stress ; Phosphotransferases ; Receptor, IGF Type 1