Lingual dystonia is a rare type of dystonia, the main symptom of which varies from intermittent to sustained tongue fixation. Several studies have suggested that the cerebellum may be implicated in dystonia. There are several treatment options available for dystonia, including medication, botulinum toxin injection, and surgical intervention. We chose to inject botulinum toxin into the styloglossus muscle, and the symptoms of the lingual dystonia were improved. We report a case of lingual dystonia following a bilateral cerebellar stroke that responded to treatment with botulinum toxin.
Botulinum Toxins ; Cerebellum ; Dystonia ; Infarction ; Stroke ; Tongue

Increased expression of glucose transporter1 (GLUT1) has been reported in many human cancers. We hypothesized that the degree of GLUT1 might provide a useful biological information in gastric adenocarcinoma. RT-PCR and immunostaining were used to analyze GLUT1 expression in gastric cancer. RT-PCR showed GLUT1 expression was not largely detected in normal gastric tissue but was detected in cancerous gastric tissue of counterpart. By immunohistochemistry, GLUT1 protein was absent in normal gastric epithelium and intestinal metaplasia. 11 of 65 patients with gastric adenocarcinoma had specific GLUT1 immunostaining in a plasma membrane pattern with varied intensities. GLUT1 protein did not show any significant correlation with tumor stage and nodal metastasis (p+AD4-0.05 by Mann-Whitney test). However, the positive immunostaining for GLUT1 is associated with intestinal differentiation (p+AD0-0.003). Our results suggest that GLUT1 protein is associated with intestinal type of gastric cancer.
Adenocarcinoma/pathology ; Adenocarcinoma/chemistry+ACo- ; Adult ; Aged ; Female ; Gastric Mucosa/pathology ; Gastric Mucosa/chemistry+ACo- ; Human ; Intestines ; Male ; Metaplasia ; Middle Age ; Monosaccharide Transport Proteins/analysis+ACo- ; Neoplasm Proteins/analysis+ACo- ; Reverse Transcriptase Polymerase Chain Reaction ; Stomach Neoplasms/pathology ; Stomach Neoplasms/chemistry+ACo- ; Tumor Markers, Biological/analysis+ACo-

Increased expression of glucose transporter1 (GLUT1) has been reported in many human cancers. We hypothesized that the degree of GLUT1 might provide a useful biological information in gastric adenocarcinoma. RT-PCR and immunostaining were used to analyze GLUT1 expression in gastric cancer. RT-PCR showed GLUT1 expression was not largely detected in normal gastric tissue but was detected in cancerous gastric tissue of counterpart. By immunohistochemistry, GLUT1 protein was absent in normal gastric epithelium and intestinal metaplasia. 11 of 65 patients with gastric adenocarcinoma had specific GLUT1 immunostaining in a plasma membrane pattern with varied intensities. GLUT1 protein did not show any significant correlation with tumor stage and nodal metastasis (p+AD4-0.05 by Mann-Whitney test). However, the positive immunostaining for GLUT1 is associated with intestinal differentiation (p+AD0-0.003). Our results suggest that GLUT1 protein is associated with intestinal type of gastric cancer.
Adenocarcinoma/pathology ; Adenocarcinoma/chemistry+ACo- ; Adult ; Aged ; Female ; Gastric Mucosa/pathology ; Gastric Mucosa/chemistry+ACo- ; Human ; Intestines ; Male ; Metaplasia ; Middle Age ; Monosaccharide Transport Proteins/analysis+ACo- ; Neoplasm Proteins/analysis+ACo- ; Reverse Transcriptase Polymerase Chain Reaction ; Stomach Neoplasms/pathology ; Stomach Neoplasms/chemistry+ACo- ; Tumor Markers, Biological/analysis+ACo-

Osteoporosis is a known major health problem and a serious disease of the bone, there has been a great need to develop more and newer animal models for this disease. Among animal models used for testing drug efficacy, the minipig model has become useful and effective due to its close similarity with humans (validity), particularly with the pharmacokinetics of compounds via subcutaneous administration, the structure and function of the organs, the morphology of bone and the overall metabolic nature. Based on these advantages, we sought to develop a new animal model of osteoporosis using micropig, which differs from other miniature pigs in the genetic background. Female micropigs were used for the induction of a moderate osteoporosis model by bilateral ovariectomy (OVX) and compared with shamoperated animals. For osteoporosis evaluation, clinical biomarkers such as blood osteocalcin (OSC) and parathyroid hormone (PTH) levels were measured, as well as bone mineral density (BMD) using micro-computed tomography (micro-CT). Compared to sham, OVX animals have decreased blood OSC level, while the blood PTH level increased in blood sera. In addition, we observed the significantly decreased BMDs of tibia region in OVX animals. Based on these results, we report that the micropig model developed in this study can be used to develop a new and effective medical method for diagnosis and treatment of osteoporosis.
Animals ; Biomarkers ; Bone Density ; Female ; Humans ; Models, Animal ; Osteocalcin ; Osteoporosis ; Ovariectomy ; Parathyroid Hormone ; Salicylamides ; Swine ; Swine, Miniature ; Tibia

Diabetes, which has shown an explosive increase in terms of its incidence, is regarded as a serious disease that must be overcome for the sake of human life. Among animal models used for testing of drug efficacy, the mini-pig model has shown a rapid upload due to its many similarities with human, particularly concerning the pharmacokinetics of compounds after subcutaneous administration, the structure and function of the gastrointestinal tract, the morphology of the pancreas, and overall metabolic status. Based on these various advantages, we sought to develop an animal model of type II diabetic mellitus using the Micro-pig, which differs from other miniature pigs. We used six male Micro-pigs for induction of a moderate insulin deficient model with nicotinamide (NIA)/streptozotocin (STZ) treatment and three animals for control. For evaluation of incidence of type II diabetes, we measured blood glucose level, and performed oral glucose tolerance test and immunohistochemistry on pancreatic tissue using insulin antibody. Compared to control animals, all animals treated with NIA/STZ showed high levels of glucose and low levels of insulin. In addition, we observed the partially destroyed beta cell population from tissue of the pancreas in treated animals. Based on these results, we report that the Micro-pig model developed in this study can be used for testing of the efficacy of therapeutic agents for treatment of Type 2 diabetic mellitus.
Animals ; Blood Glucose ; Gastrointestinal Tract ; Glucose ; Glucose Tolerance Test ; Humans ; Immunohistochemistry ; Incidence ; Insulin ; Male ; Models, Animal ; Niacinamide ; Pancreas ; Swine

OBJECTIVE: This study was performed to evaluate the influence of maternal age on embryo quality and the frequency of multiple pregnancy in IVF-ET program. METHOD: 86 conventional IVF-ET cycles were divided into three groups according to the age by 5 year (group A: 26-30, group B: 3135, group C: 36-40 yrs). The in vitro fertilization and development outcome (fertilization, cleavage and high quality embryo rate) and the pregnancy outcome (pregnancy, implantation, G-sac/high quality embryo and multiple pregnancy rate) were examined. And then, these results were compared among the groups. RESULTS: The rates of fertilization (62.7, 68.5 and 65.4%, respectively) and cleavage (95.6, 97.6 and 98.0%, respectively) were not different among the groups. And the high quality embryo (HQE) rate also was not different among the groups (61.8, 62.9 and 62.8%, respectively). The pregnancy rate of group C (23.3%) was significantly lower than that of group A (41.2%) and B (48.7%). And the implantation rate was significantly decreased to group B (32.2%) and C (14.3%) when compared to group A (71.4%) and B (36.8%). CONCLUSION: The pregnancy rate was significantly decreased over 35 years. The G-sac/HQE and multiple pregnancy rate were significantly high below 31 years. Thus, these results suggest that the number of high quality embryo transferred should be limited by the age and another criteria for embryo quality evaluation were required for single embryo transfer.
Embryonic Structures* ; Female ; Fertilization ; Fertilization in Vitro ; Maternal Age* ; Pregnancy ; Pregnancy Outcome ; Pregnancy Rate ; Pregnancy, Multiple* ; Single Embryo Transfer

Corticobasal syndrome (CBS) is characterized by asymmetric dystonia, and myoclonus accompanied by higher cortical features including apraxia, alien limb phenomena, cortical sensory loss. Here, we report treatment course of 3 CBS patients. Asymmetric dystonia was seen in the first and second cases, a cortical sensory loss was seen in the third case and left lower limb apraxia was common in all cases. In the first and second cases, we performed an alcohol block on the obturator nerve and injected botulinum toxin into the lower leg to reduce dystonia. In the third case, patient was treated with a robotic assisted gait training, whole body therapeutic pool and gait training with laser pointer visual cueing. After appropriate treatment for patients, all 3 cases showed improvement in gait.
Apraxias ; Botulinum Toxins ; Cues ; Dystonia ; Emigrants and Immigrants ; Extremities ; Gait Apraxia ; Gait* ; Humans ; Leg ; Lower Extremity ; Myoclonus ; Nerve Block ; Neurological Rehabilitation ; Obturator Nerve ; Rehabilitation*

Hallervorden-Spatz disease (HSD) is a rare autosomal-recessive hereditary disorder characterized by the early onset of progressive movement alterations, including dystonia, rigidity, choreoathetosis, and mental deterioration. HSD is also associated with a variety of psychiatric symptoms, primarily depression and mental deterioration. However, psychosis has rarely been reported as a major symptom of HSD. We report two siblings who presented psychiatric symptoms as major clinical presentations, accompanied by ataxic and spastic gait, dysarthria, and typical neuroimaging findings of HSD. A 14-year-old girl presented complex motor tics, stereotypic behavior and anxiety symptoms. Her older brother, a 16-year-old boy, presented prominent auditory hallucinations, persecutory delusions and social withdrawal symptoms. Psychiatric symptoms were improved after atypical antipsychotic treatment. HSD is a rare disease but should be carefully considered in the diagnosis of patients with both motor disorder and various psychiatric symptoms.
Adolescent ; Anxiety ; Delusions ; Depression ; Dysarthria ; Dystonia ; Gait Disorders, Neurologic ; Hallucinations ; Humans ; Neuroimaging ; Pantothenate Kinase-Associated Neurodegeneration ; Psychotic Disorders ; Rare Diseases ; Siblings ; Substance Withdrawal Syndrome ; Tics

Orofacial dyskinesia is a condition caused by various diseases in which the tongue, lips, or jaws move involuntarily. Up to now, the exact mechanism for these degenerative changes in the brain remains unknown. Among various hypotheses, the most widely accepted hypothesis is that orofacial dyskinesia is caused by supersensitivity of the dopamine receptors. As a result, metoclopramide, a dopaminergic receptor blocking agent has been chosen as a treatment agent for our study. We used metoclopramide in seven stroke patients who displayed symptoms of orofacial dyskinesia following brain damage and observed an improvement in the symptoms from all patients. This case report represented new therapeutic methods and will aid in the treatment of orofacial dyskinesia.
Brain ; Dopamine ; Dyskinesias ; Humans ; Jaw ; Lip ; Metoclopramide* ; Movement Disorders* ; Receptors, Dopamine ; Stroke* ; Tongue

BACKGROUND: Acute erythroleukemia (AEL), FAB-M6 is a rare heterogenous disorder diagnosed by myeloblasts more than 30% of nonerythroid cells (NEC). Pure erythroleukemia (Di Guglielmo disease) with an excess of proerythroblasts can be classified as MDS or M0. An aberration of the p53 gene in acute myelogenous leukemia is rare, but related to complex karyotypes with poor prognosis. METHODS: To evaluate heterogenous features, 32 cases of AEL or suspicious AEL were categorized as consisting of more than 50% erythroblasts and M6a with more than 30% myeloblasts of NEC, M6b with more than 30% proerythroblasts of all erythroblasts, and M6c with more than 30% both myeloblasts and proerythroblasts. The relation of the p53 protein overexpression and chromosomal abnormalities to AEL and these subtypes was investigated. RESULTS: There were 18 M6a, 6 M6b, and 8 M6c. The percentage of erythroblasts was M6a 58.7%, 77.7% M6b, and 67.2% M6c. The percentage of myeloblasts in NEC was M6a 53.6%, M6b 4.3%, and M6c 39.2%. The percentage of proerythroblasts in all erythroblasts was M6a 5.6%, M6b 56.2%, and M6c 34.1%. Survivals of M6b and M6c were significantly shorter than M6a (12.0 vs. 2.0 vs. 2.0 months, P=0.003). Five of 11 cases showed complex karyotypes (1 M6a, 2 M6b, 2 M6c), of -5, 5q-, -7, 7q-, -17 and/or 17p-, with shorter survival and poor response. The p53 protein overexpression was M6a 27.3%, M6b 100%, and M6c 83.3%. The p53 protein overexpression was positive in all 5 cases of multiple complex karyotype with frequent treatment failure or shorter survival, but was negative in 5 normal karyotypes. CONCLUSTIONS: The occurrence of complex karyotypes and aberration of the p53 gene frequently observed in M6b and M6c subtypes of acute erythroleukemia would be considered in establishing a new and innovative treatment to target neoplastic proerythroblasts that are resistant to standard therapy for acute myelogenous leukemia.
Chromosome Aberrations ; Erythroblasts ; Genes, p53 ; Granulocyte Precursor Cells ; Karyotype ; Leukemia, Erythroblastic, Acute* ; Leukemia, Myeloid, Acute ; Prognosis ; Treatment Failure