The phenomenon of Myotonia consist in a failure of voluntary muscles to relax immediately when voluntary innervation ceases. The stiffness is accentuated by cold and relieved by exercise, while generalized muscle weakness and atrophy (or not commonly hypertrophy of muscle) is common. Myotonia is a feature of four principal clinical syndromes which have been classified myotonia congenita (Thomsens disease), myotonia atrophica (Steinerts disease), paramyotonia and myotonia and myotonia acquisita by Walton et al. We report here a forty six years old male of myotonia atrophica (Dystrophia myotonia, Steinerts disease) who presents bilateral cataracts, frontal baldness, gonadal atrophy, facial myopathy, sterno-cleidomastoid muscle atrophy and a progressive generalized myopathy of peripheral distribution in the limbs.
Alopecia ; Atrophy ; Cataract ; Extremities ; Gonads ; Humans ; Hypertrophy ; Male ; Muscle Weakness ; Muscle, Skeletal ; Muscular Atrophy ; Muscular Diseases ; Myotonia Congenita ; Myotonia ; Myotonic Dystrophy

The authores did clinical analysis of 150 cases of the Degenerative Joint Disease of the Knee and reviewed literature.
Joint Diseases ; Joints ; Knee

No abstract available.
Nipples*

BACKGROUND: Gamma linoleic acid (GLA, Epogam) is considered a safe and effective modality in the treatment of atopic dermatitis (AD) in which impaired function of the enzyme, delta-6-desaturase, has been reported to result in reduced levels of GLA, desaturated fatty acids. OBJECTIVE: We performed this study to observe the changes of skin surface conditions measured objectively by bioengineering methods in relation to clinical improvement after treatment with GLA (Epogam®) in children with AD. METHODS: Thirty-four children with AD were treated with GLA (Epogam®) and evaluated with clinical parameters.The changes of skin surface conditions were monitored by non-invasive experimental instruments. RESULTS: There was a significant decrease of transepidermal water loss (TEWL) and gradual improvements in clinical severity after 12 weeks of GLA (Epogam®) treatment. The change of skin surface pH was statistically significant on the antecubital fossa and abdomen except the popliteal fossa. The other parameters including skin surface hydration and skin surface lipid did not show consistent changes. CONCLUSION: Clinical improvement of AD with GLA (Epogam) seemed to be achieved by the reduction of TEWL.
Abdomen ; Bioengineering ; Child ; Dermatitis, Atopic* ; Fatty Acids ; Humans ; Hydrogen-Ion Concentration ; Linoleic Acid* ; Linoleoyl-CoA Desaturase ; Skin* ; Water

A hemangioma occurred in the bony epiphysis is extremly rare. A 5-year-old boy visited to our hospital with pain and flexion contracture on the right knee. MRI showed some lesions scattered in the epiphysis of the distal femur and the proximal tibia. Biopsy specimen from the distal femoral epiphysis revealed pathologic findings compatible with hemangioma. On 8 years follow-up, the lesion in the distal femoral epiphysis had been cured, and those in the proximal tibial epiphysis were spontaneously disappeared without surgery. The scanogram shows no leg length discrepancy and angular deformity. We reports a rare case of hemangioma occurred in the bony epiphysis with the results of 8 year follow-up with the review of literatures.
Biopsy ; Congenital Abnormalities ; Contracture ; Epiphyses ; Femur ; Follow-Up Studies ; Hemangioma ; Knee ; Leg ; Preschool Child ; Tibia

We describe a patient with overlap syndrome in whom systemic lupus erythematosus and scleroderma were combined. The patient expired due to severe central nervous system involvernent and pneumonia. Pertinent neuropsychiatric signs of the patient included generalied seizure, impairment of recent, memory, dysarthria, mental deterioration, Balints syndrome, right sided hemiparesis and right sided central type facial weakness. Hrain MRI and cerebral angiogram slowed cerebral vasculopathy. We suggest that a long-term follow-up is necessary for the confirmation of ciagnosis of a connective tissue disease, because the evolution, transition and overlapping features among the group of connective tissue diseases may commonly occur over time.
Central Nervous System ; Connective Tissue Diseases ; Dysarthria ; Follow-Up Studies ; Humans ; Lupus Erythematosus, Systemic ; Magnetic Resonance Imaging ; Memory ; Paresis ; Pneumonia ; Seizures

Angiogenesis, the induction of new capillaries and venules, is associated with tumor growth. This study was designed to determine whether cervical carcinomas are angiogenic, and to investigate whether tumor angiogenesis can serve as a prognostic factor in cervical carcinoma. Surgical specimens of 47 cervical carcinomas were immunohistochemically stained specifically for endothelial cells with factor VIII-related antigen to identify all vessels. Microvessels were counted from photographs of 200x microscopic fields. In addition, thirty-seven cases were studied by immunohistochemical means using the monoclonal antibodies for PCNA and for Ki-67 to determine tumor cell proliferation rates in cervical carcinomas. The microvessel count(MVC), the PCNA labelling index, and the Ki-67 index were calculated and compared with known prognostic factors and disease free survival rates in cervical carcinomas. A wide range in the MVC count(range 12-100 mean=38.2+/-19.2), the PCNA labeling index(8-69% mean=33.6+/-15.2%), and in the extent of Ki-67 staining(0-43% mean=10.3+/-10.5%) was observed, indicating considerable variation of tumor angiogenic activity and tumor growth rates. This study showed statistically significant correlations in disease free survival rates with both lymph node status and the microvessel count. However, there was no significant difference in disease free survival rates between tumor stage, age, the PCNA labelling index, and the Ki-67 index.
Antibodies, Monoclonal ; Capillaries ; Cell Proliferation ; Cervix Uteri* ; Disease-Free Survival ; Endothelial Cells ; Female ; Lymph Nodes ; Microvessels ; Proliferating Cell Nuclear Antigen ; Venules ; von Willebrand Factor

No abstract available.
Ultrasonics*

Herein we report an experience of the vaginal ureterolithotomy. The patient was a 48-year-old woman with a juxtavesical left ureteral stone, which was vaginally palpable. Although this operation has limited indications, it is a safe and easy method of removing stones from the lowermost ureter especially in the women. This method seems to have considerable value to be attempted in the carefully select patients.
Female ; Humans ; Middle Aged ; Ureter

No abstract available.
Neurofibroma*