Creutzfeldt-Jakob disease(CJD) is characterized clinically by rapidly progressive dementia with pyramidal, extrapyramidal, and cerebellar symptoms and signs, and histologically by spongiform change, neuronal loss and reactive gliosis. We have experienced 2 cases of CJD. Case 1 was a 36-year-old male who had suffered from myoclonus and cerebellar symptoms including sluggish speech, gait and balance disturbance. Case 2 was a 70-year-old female who had showed cognitive dysfunction, ataxic gait and disturbance of extraocular movement. Both patients, underwent brain biopsy. Case 1 revealed marked cortical atrophy, 2mm in thickness, with neuronal loss and astrocytic proliferation extending into white matter. The spongiform change, made up of many small, usually rounded or oval, vacuoles was noted mainly in the neuropil. Case 2 revealed remarkable spongiform change throughout the cortex and cytoplasmic vacuoles compressing the nuclei of neuronal cells were numerous. Neuronal loss and gliosis were also found without considerable change in the white matter. On double immunostaining against GFAP and PrP(Prion Protein), there was a weak positive reaction for PrP in the perinuclear cytoplasm in case 1, and a strongly positive reaction in case 2. The electron microscopic examination showed numerous membrane-bound vacuoles in neuropil and perikarya of neurons. The majority of the vacuoles were multiseptated by thin membranous structures. They demonstrated curled, or disrupted membrane, that had foldings and protrusions into the vacuolar clear spaces. There were neither identifiable virus-like particles nor amyloid deposition.
Female ; Male ; Humans

No abstract available.
Allografts* ; Anemia, Hemolytic* ; Cadaver* ; Transplantation*

A 23-year-old woman had solar urticaria that was activated by visible light and UVA, but the intensity of the urticarial reaction of visible light was more pronounced than that of UVA. The results of passive and reverse passive transfer studies were negative, We report a case of solar urticaria which is characterized by overlapping features of 60th type Il and Il[ in the classification of Harber.
Classification ; Female ; Humans ; Light ; Urticaria* ; Young Adult

Through the clinicopathologic study of pathergy test sites of 16 patients with Behrets disease in the active or inactive stage, the following results were obtained; 1. In the active stage, six of 16 patients (37.5%) showed positive reactions in clinical pathergy tests, which are much higher than that in the inactive stage when only one patient showed a positive reaction. The severity of the ciniral pathergy reaction was directly related to the activity of the disease. 2. The characteristic histopathologic finding in the active stag was a dermal inflammatory cellular infiltration composed mainly of polymorphonuclear lukocytes and of lymphomononuclear cells accompanied by leukoctocylasia. Two of 16 patients showed true leukocytoclastic vasculitis. 3. Although the type of disease and the reactivity of the clinical pathergy test were related in terms of the severity of the histopathologic findings, there were some notible histopathologic changes even in the cases with negative clinical pathergy reactions. We concluded that the histopathologic findings of the pathergy test sites were more reliable for the diagnosis of Behcet's disease than the clinical findinigs.
Diagnosis ; Humans ; Vasculitis

No abstract available.
Neurilemmoma*

No abstract available.
Chordoma*

Hypophosphatasia is a rare disease characterized by defective bone mineralization due to deficiency of tissue-nonspecific alkaline phosphatase. The patient was an 8-day-old male infant who presented with seizure since that day. Other symptoms included res piratory failure, requiring the use of a mechanical ventilator. Physical exami na tion revealed a large bulging anterior fontanelle, soft skull bone, and radial devia tion of both wrists. Laboratory examination showed normal serum calcium, low para thyroid hormone, normal 25-hydroxy vitamin D, and severely low alkaline phos phatase levels. Skeletal X-ray revealed dysplasia of the skull and cupping of the epiphysis of the limbs. Two heterozygous mutations (c.1052A>G, c.1559delT) of the ALPL gene were identified by Sanger sequencing. Thus, we report a case of confirmed lethal perinatal hypophos phatasia in Korea.

It is obvious that adequate nutrition is essential for growth and development of school children, and many elementary schools in this country have already practiced it. Therefore, it would seem apparent that the school feeding program would have a significant effect on the growth and development of school children. This paper presents a two-year experimental school-feeding program from 1973 to 1974, and attempts to evaluate its effects by before-and-after nutrition surveys conducted in two elementary schools, one experimental and the other as a control. The two schools are both located in the same county (Yongin-Kun, Kyunggi-Do), and the families of their students are presumed to share the same socio-economic level. To assess the effect of school-feeding, we measured height, weight, chest circumference and grasping power. Physical examination was done foresigns of nutritional deficiency. A stool examination for parasites and blood examinations for hemoglobin, hematocrit and serum protein were included. Analysis were done for 150 students selected randomly at the beginning of the program. These students attended the school throughout the program period. Results are as follows: 1. The amount of increase of height, weight, chest circumference and grasping power were greater in the experimental school than in the control school, but the differences are not statistically significant. 2. Signs of vitamin deficiency decreased in both experimental and control schools during the two-year program period. 3. At the time of the 1974 post-survey, values of Hb. & Hct. revealed no significant differences between the two schools, but serum protein level was a little higher than that of general Korean rural children of the same age. 4. Infestation rate of parasites had increased in both schools during the two-year program period. 5. Each student of the two schools was classified into three major classes, according to the level of economic condition of his or her parents, namely higher, middle and lower. The results of each class of the experimental school was compared with that of the corresponding class of the control school, expecting the relative magnitude of change largest in the lower economic class of the experimental school. However, change was greatest in the middle class, still not being statistically significant. Finally, the authors concluded that the two-year period for such a program is not sufficiently long for its beneficial effects to be demonstrated and measured. As long as the growth and development of children are concerned, planning with a more distant perspective is required, as well as the development of new methods of evaluation.
Avitaminosis ; Child ; Growth and Development ; Hand Strength ; Hematocrit ; Humans ; Lunch* ; Malnutrition ; Nutrition Surveys ; Parasites ; Parents ; Physical Examination ; Thorax

No abstract available.
Animals ; Killer Cells, Natural* ; Mice*

No abstract available.
Hair ; Survival Rate ; Transplants