Authors report a typical case of congenital fiber type disproportion (CFTD) with unique clinicopathologic characteristics. The patient was a 13-year-old boy who presented with weakness of lower extremities, especially proximal muscle, since his infancy. He has suffered from severe scoliosis which got worse since the age of 12. He showed mild dysarthria, high arched palate, and fish face. All routine laboratory data were within normal limits. EMG findings suggested myopathy. The muscle biopsy revealed fiber type disproportion with type 1 predominance. While most of the type 1 myofibers were atrophic or normal in size, the type 2 fibers showed universal hypertrophy. The difference of mean diameter between the larger and the smaller fibers was 27.9%. The patient's clinicopathologic settings fulfilled the criteria of CFTD.
Adolescent ; Biopsy ; Dysarthria ; Humans ; Hypertrophy ; Lower Extremity ; Male ; Muscular Diseases* ; Myopathies, Structural, Congenital* ; Palate ; Scoliosis

No abstract available.
Korea* ; Lung Neoplasms* ; Lung*

Primary sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) of the thyroid gland is a very rare disease. We present the clinical and histopathologic findings of a 37-year-old woman recently diagnosed with SMECE of the thyroid gland. The patient, clinically euthyroid, who presented with a neck swelling since last 2 years along. Fine needle aspiration cytology suggested thyroid papillary carcinoma. Total thyroidectomy, central neck dissection and right selective neck dissection were performed. Although SMECE is considered to be a relatively slow growing and non-aggressive tumor, occasional metastasis does occur. We report an additional case of SMECE, with metastasis to regional lymph nodes. Physicians should be aware of extended operation, including total thyroidectomy and/or neck node dissection for metastatic lesion of the neck node. More standardized treatment is likely to evolve in the future.
Adult ; Biopsy, Fine-Needle ; Carcinoma, Mucoepidermoid* ; Carcinoma, Papillary ; Eosinophilia* ; Female ; Humans ; Lymph Nodes ; Neck ; Neck Dissection ; Neoplasm Metastasis ; Rare Diseases ; Thyroid Gland* ; Thyroidectomy

It has not been elucidated whether the initiation of condylar development of the mandible is related with the periosteum of the mandible, or if it derives from a separate programmed blastema not related with the mandible. Also, although the mandibular condylar cartilage is known to promote growth, few studies have dealt with molecular-biologic mechanisms such as the expression of specific genes according to the differentiation of the mandibular condyle. To elucidate the unique cellular characteristics, development, and differentiation process of the mandibular condyle, an examination of expressions of genes characteristic of cartilage and bone were carried out using RT-PCR and mRNA in situ hybridization. 1. Type?collagen mRNA was detected with type II collagen mRNA in the differentiation and growth process of the cartilage of the mandibular condyle. Type II collagen mRNA was demonstrated in the whole resting and upper part of the proliferative zone, whereas type II collgen mRNA was observed in the resting, proliferative and upper hypertrophic cartilage zone of the mandibular condyle. 2. The condylar cartilage rapidly increased in size due to the accumulation of hypertrophic chondrocytes as characterized by the expression of type II collagen mRNA during postnatal development. 3. BMP-4 mRNA was present in the anlage of the future condylar process and also in the ossifying mandibular body. 4. IHH mRNA was limited exclusively to the lower part of the proliferative zone and the upper part of the hypertrophic cartilage zone during condylar development. These findings were different from those in the growth-plate cartilage of the long bone, indicating a characteristic feature of the differentiation of the chondrocytes in the condylar cartilage present in prenatal and postnatal development. Furthermore, it was also suggested that chondroblasts of condylar cartilage rapidly differentiate into hypertrophic chondrocytes with increased functional Load force such as muscle activity and mastication.
Animals ; Cartilage* ; Chondrocytes ; Collagen ; Collagen Type II ; In Situ Hybridization ; Mandible ; Mandibular Condyle* ; Mastication ; Mice* ; Periosteum ; RNA, Messenger*

The lesions on computerized tomography (CT) scan were evaluated in 7 cases of conduction aphasia. On Modified Westem Aphasia Battery (MWAB), all the patients showed fluent aphasia with some paraphasic errors, good comprehension, marked repetition dificit and variable degree of impairment in naming. The three most common lesions on CT scan were supramarginal gyrus and it's white matter, primary auditory cortex, and insular region, which were involved in 6,5,4 cases out of 7 respectively. Of the three lesions, one or variable combinations of them may be the critical structures for repetition difict in these patients, as has been reported in previous studies. In 5 cases Wermicke area was relatively intact and prominent involvement was seen in only 2 cases, which is thought to be associated with relative preseration foaural comprehension in conduction aphasia.
Aphasia ; Aphasia, Conduction* ; Aphasia, Wernicke ; Auditory Cortex ; Comprehension ; Humans ; Tomography, X-Ray Computed

Although light microscopic features of muscle are not pathognomonic in most cases of myasthenia gravis (MG), careful examination of neuromuscular junction by electron microscopy (EM) can reveal important clues for this disease. We report here a case of MG confirmed by EM study to emphasize that tissue diagnosis is still the best adjuvant to confirm the diagnosis. An 18-year-old female visited our hospital complaining of progressive muscle weakness for 3 years. She had difficulty in running, going upstairs and doing routine activities. Symptoms were aggravated with continuous work and resolved after rest. She had weakness of bilateral masseter and facial muscles and proximal portions of extremities without definite diurnal variation. Electromyography showed myopathic changes in proximal muscles of extremities. MG was considered but tensilon test was equivocal. Repetitive nerve stimulation tests revealed 20-30 percent decrease in responses to low and high rate stimulation. Muscle biopsy revealed selective type 2 atrophy. Ultrastructurally, abnormalities of neuromuscular junctions, i.e., wide primary synaptic cleft, and wide and shallow secondary synaptic clefts with mild myopathic features were present. These findings were pathognomonic for MG. Later, her symptoms were improved completely 3 months after thymectomy. The histologic finding of thymus was follicular hyperplasia.
Adolescence ; Biopsy ; Case Report ; Female ; Human ; Microscopy, Electron ; Mitochondria/ultrastructure ; Mitochondria/pathology ; Muscle, Skeletal/ultrastructure ; Muscle, Skeletal/pathology ; Muscle, Skeletal/enzymology ; Myasthenia Gravis/pathology* ; Myofibrils/ultrastructure ; Myofibrils/pathology ; Myosin ATPase/analysis ; Neuromuscular Junction/ultrastructure* ; Neuromuscular Junction/pathology*

Although light microscopic features of muscle are not pathognomonic in most cases of myasthenia gravis (MG), careful examination of neuromuscular junction by electron microscopy (EM) can reveal important clues for this disease. We report here a case of MG confirmed by EM study to emphasize that tissue diagnosis is still the best adjuvant to confirm the diagnosis. An 18-year-old female visited our hospital complaining of progressive muscle weakness for 3 years. She had difficulty in running, going upstairs and doing routine activities. Symptoms were aggravated with continuous work and resolved after rest. She had weakness of bilateral masseter and facial muscles and proximal portions of extremities without definite diurnal variation. Electromyography showed myopathic changes in proximal muscles of extremities. MG was considered but tensilon test was equivocal. Repetitive nerve stimulation tests revealed 20-30 percent decrease in responses to low and high rate stimulation. Muscle biopsy revealed selective type 2 atrophy. Ultrastructurally, abnormalities of neuromuscular junctions, i.e., wide primary synaptic cleft, and wide and shallow secondary synaptic clefts with mild myopathic features were present. These findings were pathognomonic for MG. Later, her symptoms were improved completely 3 months after thymectomy. The histologic finding of thymus was follicular hyperplasia.
Adolescence ; Biopsy ; Case Report ; Female ; Human ; Microscopy, Electron ; Mitochondria/ultrastructure ; Mitochondria/pathology ; Muscle, Skeletal/ultrastructure ; Muscle, Skeletal/pathology ; Muscle, Skeletal/enzymology ; Myasthenia Gravis/pathology* ; Myofibrils/ultrastructure ; Myofibrils/pathology ; Myosin ATPase/analysis ; Neuromuscular Junction/ultrastructure* ; Neuromuscular Junction/pathology*

PURPOSE: The purpose of this study was to compare the fracture strength of the IPS Empress ceramic crown according to the incisal reduction (2.0mm, 2.5mm, 3.0mm) and axial inclination (4degrees., 8.degrees, 12degrees.) of the upper canine. MATERIALS AND METHODS: After 10 metal dies were made for each group, the IPS Empress ceramic crowns were fabricated and each crown was cemented on each metal die with resin cement. The cemented crowns mounted on the testing jig were inclined 30 degrees and the universal testing machine was used to measure the fracture strength. RESULTS: 1. The fracture strength of the ceramic crown with 3.0mm depth and 12degrees inclination was the highest (839N). Crowns of 2.0mm depth and 12degrees inclination had the lowest strength (559N). 2. There was no significant difference in the fracture strength by axial inclination in the same incisal reduction group. 3. The fracture mode of the crowns was similar. Most of fracture lines began at the loading area and extended through proximal surface perpendicular to the margin irrespective of incisal reduction.
Ceramics ; Crowns* ; Resin Cements

Little is known about processing mechanism of sensory input from the periodontal ligaments to the trigeminal motor nucleus for the control of chewing force and modulation of chewing pattern. Low threshold mechanoreceptive periodontal afferent was labeled with horseradish peroxidase by use of intra-axonal injection technique and investigated with electron microscopy. Quantitative ultrastructural analysis was performed on the 39 serially reconstructed labeled boutons in the trigeminal motor nucleus in cat. Labeled bouton contained clear spherical vesicles and one or two large dense cored vesicles. Most of labeled boutons were dome or round shape. All the analysed labeled boutons were presynaptic to dendritic shaft or distal dendrite and those presynaptic to soma or proximal dendrite were not observed. A large number of labeled boutons (46.2%) were postsynaptic to one or two presynaptic pleomorphic vesicle containing endings. Synaptic triad, in that a presynaptic ending which is presynaptic to the labeled bouton, in turn, is presynaptic to dendrite that is postsynaptic to the labeled bouton, was observed in 10.3% of the labeled boutons. Most of the labeled boutons showed simple synaptic organization, in that 64.1% of the labeled boutons made synaptic contacts with one or two neuronal profiles. One (2.6%) of the 39 analyzed labeled boutons showed synaptic contacts with 5 or more neuronal profiles. Labeled bouton volume, mitochondrial volume, apposed surface area and active zone area showed wide variation. These ultrastructural parameters were positively correlated with bouton volume. The values for apposed surface area and active zone area with presynaptic p-endings, in contrast to those with postsynaptic dendrites, showed narrow range and had little correlation with bouton volume. The present study revealed characteristic features on ultrastructural parameters of labeled boutons from periodontal afferent which is involved in periodontal masseteric reflex, and that influence on the postsynaptic trigeminal motoneurons showed wide variability.
Animals ; Carisoprodol ; Cats ; Dendrites ; Horseradish Peroxidase ; Mastication ; Microscopy, Electron ; Mitochondrial Size ; Neurons ; Periodontal Ligament ; Reflex ; Synapses

Objective: To investigate the suitability of citrus-press cakes, by-products of the juice industry as a source for the whitening agents for cosmetic industry.
Methods:Ethylacetate extracts of citrus-press cakes (CCE) were examined for their anti-melanogenic potentials in terms of the inhibition of melanin production and mechanisim of melanogenesis by using Western Blot analysis with tyrosinese, tyrosinase-related protein-1 (TRP-1), TRP2, and microphthalmia-associated transcription factor (MITF) proteins. To apply the topical agents, citrus-press cakes was investigated the safety in human skin cell line. Finally flavonoid analysis of CCE was also determined by HPLC analysis.
Results: Results indicated that CCE were shown to down-regulate melanin content in a dose-dependent pattern. The CCE inhibited tyrosinase, TRP-2, and MITF expressions in a dose-dependent manner. To test the applicability of CCE to human skin, we used MTT assay to assess the cytotoxic effects of CCE on human keratinocyte HaCaT cells. The CCE exhibited low cytotoxicity at 50 μg/mL. Characterization of the citrus-press cakes for flavonoid contents using HPLC showed varied quantity of rutin, narirutin, and hesperidin.
Conclusions:Considering the anti-melanogenic activity and human safety, CCE is considered as a potential anti-melanogenic agent and may be effective for topical application for treating hyperpigmentation disorders.