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

We reviewed the clinical epidermiologic features and skin biopaies of 23 patients who were diagonosed with sparganosis. Clinically, the parasites were obtained from the lesions and confirmed histopathologically. The purpose of this study was to evaluate the elinical, epidemiological, and histopathological charaeteristics of sparganosis. The results are summurized as follows : 1. There were no difference between male (48%) and female (52%) patients. 2. Age distribution, at first visit, were variable, ranging from 7 to 75, with the mean age of 40 years-old. 3. Duration of symptoms were variable, ranging from 10 years to 15 years, with a mean duration of 3 years. 4. Frequency of clinical features were as follows; movable or fixed subcutaneous nodule (16 cases), subcutaneous nodule with pain & focal warmth t.o touch (6 cases), seizure (I case). 5. Number of parasites per lesion were single lesion with single, parasite (21 cases), single lesion with two parasites (2 cases) and three parasites (3 case). 6. Frequency of location of lesion were abdominal wall (8 cases), thigh (4 cases), breast (3 cases), scrotum (3 cases), arm (3 cases), buttoek (1 cases), ciiest wall (1 case), brain (1 case). 7. The histological change of the affected tissue were characterized as follows ; 1) necrotizing and granulomatous tissue with or without parasif os in the lesions. 2) some cases were associated with marked fibrosis or formation of lymphoid follicles. 3) There were many lympho-histocytes, eosinophils, giant cel1s and some plasma cells near the lesions.
Abdominal Wall ; Adult ; Age Distribution ; Arm ; Brain ; Breast ; Eosinophils ; Female ; Fibrosis ; Humans ; Male ; Parasites ; Plasma Cells ; Scrotum ; Seizures ; Skin ; Sparganosis* ; Thigh

PURPOSE: To quantify the cardiac dose reduction during breathing adapted radiotherapy using Real-time Position Management (RPM) system in the treatment of left-sided breast cancer. MATERIALS AND METHODS: Twenty-two patients with left-sided breast cancer underwent CT scans during breathing maneuvers including free breathing (FB), deep inspiration breath-hold (DIBH), and end inspiration breath-hold (EIBH). The RPM system was used to monitor respiratory motion, and the in-house self respiration monitoring (SRM) system was used for visual feedback. For each scan, treatment plans were generated and dosimetric parameters from DIBH and EIBH plans were compared to those of FB plans. RESULTS: All patients completed CT scans with different breathing maneuvers. When compared with FB plans, DIBH plans demonstrated significant reductions in irradiated heart volume and the heart V25, with the relative reduction of 71% and 70%, respectively (p < 0.001). EIBH plans also resulted in significantly smaller irradiated heart volume and lower heart V25 than FB plans, with the relative reduction of 39% and 37%, respectively (p = 0.002). Despite of significant expansion of lung volume using inspiration breath-hold, there were no significant differences in left lung V25 among the three plans. CONCLUSION: In comparison with FB, both DIBH and EIBH plans demonstrated a significant reduction of radiation dose to the heart. In the training course, SRM system was useful and effective in terms of positional reproducibility and patient compliance.
Breast Neoplasms* ; Cardiac Volume ; Feedback, Sensory ; Heart ; Humans ; Lung ; Patient Compliance ; Radiotherapy* ; Respiration* ; Tomography, X-Ray Computed

There are many known methods of dermo-epidermal separation for the investigation of autoimmune blistering diseases. Investigators should select a proper method since many differences exist preservation of antigenicity. In order to determine the stabilization of antigenirity by different separation methods, we have separated dermo-epidermal junction by means of 1M s;ilt, 56C PBS, 20mM EDTA and dispase. Indirect immunofluarescence and immunoblotting were performed on each specimen with sera of patients with pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, bullous pemphigoid and epidermolysis bullosa acquisita. The results are as follows : 1. In indirect immunofluorescence study of pemphigus group, best, result were obtained when normal skin without dermo-epidermal separation was used. Dispase well preserved antigenicity of pemphigus after dermo-epidermal separation, but no differences were noted in antigenicity stabilization among separation mehods by immunoblotting. 2. In indirect immunofluorecence study for differentiation of bullous pemphigoid and epidermolysis bullosa acquisita, we recommend EDTA and dispase methods in addition to 1M salt induced skin separation that have been most popularly used. 3. Results of the immunoblotting of bullous pemphigoid showed that 1M salt, EDTA and heat preserved the antigenicity well but the antigenicity was lost by dispase. 4. Results of the immunoblotting of epidermolysis bullosa acquisita she wed that antigen did not exist in epidermal extract. 5. Antigen preservation according to the different methods of demo-epidermal separation was not identical between indirect immunofluorescence and immunoblotting.
Blister* ; Edetic Acid ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Indirect ; Hot Temperature ; Humans ; Immunoblotting ; Pemphigoid, Bullous ; Pemphigus ; Research Personnel ; Skin

Cow milk allergy can be defined as an adverse immunologic reactions to cow milk protein. The term is often mistakenly applied to other causes of milk intolerence, such as lactase deficiency and galactosemia, which must be differenciated and excluded. We have experienced 10 children of cow milk allergy at neonatal onset who had suffered from G-I symtoms, such as diarrhea, irritability, weight loss, vomiting and abdominal distension. One half of this patients had family history of allergic diseas. All patients have positive specific IgE RAST to cow's milk protein, milk elimination test and milk challenge test. Brest milk feeding and soy bean formula feeding and soy bean formula feeding have started after diagnosis and then gastrointestinal symtoms and signs of all patients are improved but other allergic diseases are combined in 5 children.
Child ; Diagnosis ; Diarrhea ; Galactosemias ; Humans ; Hypersensitivity* ; Immunoglobulin E ; Infant* ; Lactase ; Milk ; Milk Hypersensitivity ; Milk Proteins ; Soybeans ; Vomiting ; Weight Loss

No abstract available.
Humans

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital pigmented nevi and a benign or maigrant pigment cell tumor of the leptomeninges. The s ndrome is thought to represent an erroi in the morphogenesis of the embryonal neuroectoderm. Even in the absence of melanorra, symptomatic neurocut.aneous melanosis has extremely poor prognosis. We report a case of neiirocutaneous melanosis in 38-year-old male. He was noted at birth to have a giant pigmented nevus on the bathing trunk area. He presneted with severe headache. Brain CT sca n was performed and 4 x 4cm sized well demarcated contrast enhanced mass was seeii in the right frontal lobe. Histopathologically, the tumor was composed of malignant melanoma cells. Multiple skin biopsies werperformed on the giant pigmented nevus and small nevi. All of the specimens revealed patterns typical of cornpound nevi. Dermatologists following patients with large or multiple congenial pigmented nevi should be aware of this condition to aid in prompt diagnosis.
Adult ; Baths ; Biopsy ; Brain ; Diagnosis ; Frontal Lobe ; Headache ; Humans ; Male ; Melanoma ; Melanosis* ; Morphogenesis ; Neural Plate ; Nevus ; Nevus, Pigmented ; Parturition ; Prognosis ; Skin

In a previous communication, we described a relatively simpli. method for assaying quantities of sweat using the skin surface hydrometer. In order to evaluate the pharmacologic response of weat glands in psoriasis, vitiligo and loalized scleroderma, we measured the basal skin conductance and the quantity of sweat induced by intradermal injections of pilocarpine nitrate in the lesional and per ilesional skin. The results were as follows : 1. In psoriatic plaques, based skin surface hydration and the sweat response to pilocarpine was markedly reduced (p<0.05), but there were no significant differences in normal perilesional skin. 2. In generalized vitiligo, basal skin surface hydration and the sweat gland response was normal. In the case of the segmental type, there was a slightly decreased sweat response in the lesion but, not significant statistically. 3. In the morphea plaque, basal skin surface hydration and the sw.at response to pilocarpine was markedly reduced but was normal in perilesional skin. This study presents evidence of the functional changes in the development of sweat secretion and surface hydration in the lesions of psoriasis, morphec and segmental type of vitiligo. The demonstration of ahnormalities in the pharmacolopic response to pilocarpine induction led us to suggest the possibility that some degree of sweat gland involvement is induced by the pathogenetic mechanisms of these diseases.
Injections, Intradermal ; Pilocarpine ; Psoriasis* ; Scleroderma, Localized ; Skin ; Sweat Glands* ; Sweat* ; Vitiligo*

This report is a clinical analysis and comparative study with previous report of bronchial asthma at National Medical Center on bronchial asthma in children who were admitted to the MP dept. of national Medical Center from January 1987 to December 1990. The results of the study were as follows: 1) The out-break ratio of bronchial asthma is 3.7% of hospitalized children which is no change that about 4% since 1980. 2) The children under 6 years of ge was 62.7% which among under 3 years was 33.1%. The rate of under 3 year children is increased as previous study. 3) The out-break of bronchial asthma was most common in September and October and so peak incidence was noted in fall and duration of hospitalization was most 2 week. 4) Familial allergic history was positive in 54.6% and bronchial asthma was most common. The presence of allergic past history was 52.2%) and atopic dermatitis was most common 5) Eosinophilia was found in 80.8% and elevated IgE was also in 61.6% of bronchial asthma. 6) We could get positive results of IgE RAST and skin test: D. pteronyssinus, D. farinae, Houst dust. 7) The most frequent findigs of the chest radiograph were within normal limits. 8) The clinical improvement rate of bronchial asthma is 52.5%.
Asthma* ; Child* ; Child, Hospitalized ; Dermatitis, Atopic ; Dust ; Eosinophilia ; Hospitalization ; Humans ; Immunoglobulin E ; Incidence ; Radiography, Thoracic ; Skin Tests

Subungual exostosis is a bony outgrowth occuring on the distal phalanx beneath the nail. This report concerns a case of subungual exostosis, accompanied with overlying myrmecia which developed in a 18-years-old man. Confirmatory X-ray showed a bony exostosis arising from the dorso-medial aspect of the distal end of the distal phalanx of the right great toe. The purpose of this artiicle is to direct attention to subungual existosis, the diagnosis of which may often be unsuspected in dermatology
Dermatology ; Diagnosis ; Exostoses* ; Toes