In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), clinical presentation with movement disorders such as dystonia and progressive supranuclear palsy-phenotype are rarely reported. None of the CADASIL cases, to our knowledge, has been reported with chorea. Herein, we describe a Korean woman with CADASIL who had presented with chorea. 18F-fl uorodeoxyglucose positron emission tomography (FDG-PET) showed hypometabolism in the right basal ganglia. We found decreased FDG uptake of the right basal ganglia by SPM analysis.

No abstract available.
Carcinoma, Hepatocellular*

Rupture of anterior communicating artery aneurysm is one of the most important casuses of subarachnoid hemorrhage. Subarachnoid hemorrhage due to ruptured anterior communicating artery aneurysm shows characteristic CT findings, such as forntal hematoma or septum pellucidum hematoma. In some patients with the ruptured aneurysm, direct carotid angiography may be needed. particularly when transfemoral approach is not possible. However, not much have been known about the puncture site selection between the two carotid arteries in relation to the location of hematoma in CT scans. To determine the ideal puncture site of the direct carotid angiography, seventy-five cases of anterior communicating artery aneurysm confirmed by operation from May 1988 to April 1992, were analyzed in terms of relationship between location of hematoma on CT, direction of aneurysm and A1 artery on angiograms. Frontal lobar hematoma was more prevalent than septal hematoma, and was more common on the right side. In cases of lobar hematoma, aneurysm was directed to the ipsilateral side of the hematoma (67%) and was most commonly fed by contralateral A1 artery(75%). Septal bematoma consisted 36% of the cases and among there, midline directed aneurysm was most common, frequently fed by left A1 artery. In conclusion, when performing direct carotid angiography in patients with anterior communicating artery aneurysm, if forntal hematoma is observed on CT, it may be beneficial to puncture contralateral carotid artery. Meanwhile, if hemorrhage of septum pellucidum is observed, it may be better to puncture left carotid artery.
Aneurysm ; Aneurysm, Ruptured* ; Angiography ; Arteries* ; Carotid Arteries ; Hematoma ; Hemorrhage ; Humans ; Intracranial Aneurysm ; Punctures ; Rupture ; Septum Pellucidum ; Subarachnoid Hemorrhage ; Tomography, X-Ray Computed

PURPOSE: To investigate the efficacy of percutaneous embolization of hypervascular VX-2 carcinoma in the retroorbital area of the rabbits using Histoacrylic Blue(N-butyl 2-cyanoacrylate, NBCA) and to establish as an experimental model. MATERIALS AND METHODS: Retroorbital VX-2 carcinoma was made in 10 rabbits by injection of carcioma cells. At 2 and 4 weeks following the tumor cell injection, carotid angiography was performed before and after percutaneous embolization using 50% NBCA diluted with Lipiodol. Doppler ultrasonography was also performed to evaluate the vascularity of the mass. Light microscopic examination was used to evaluate the effect of embolization on the tumor mass. RESULTS: Angiography following percutaneous embolization showed filling of embolic materials in the tumor beds. Ultrasonographic findings showed tumor necrosis and increased Doppler signal in the tumor. Histopathological examinations showed extensive necrosis of tumor cells with remaining viable cells surrounding the capillaries. There were no NBCA emboli in arterioles and capillaries of tumor beds in multiple sections. CONCLUSION: Since 50% diluted NBCA used as percutaneous embolus into the retroorbital VX-2 carcinoma dose not get into small blood vessels of tumor beds. using NBCA in lower concentration in the hypervascular tumor would be the next step to figure out its usefulness. This study also serves as an experimental model of percutaneous embolization of hypervascular tumor.
Angiography ; Arterioles ; Blood Vessels ; Capillaries ; Embolism ; Enbucrilate* ; Ethiodized Oil ; Models, Theoretical ; Necrosis ; Orbit* ; Rabbits* ; Ultrasonography, Doppler

Here we report an autopsy case of diabetic ketoacidosis (DKA) with severe hypertriglyceridemia (12,900 mg/dl). A 29-year-old woman with a history of type 1 diabetes was found dead at a motel. There was no injury on external inspection, but a lump of cheese-like material was noted in the heart at autopsy and peripheral blood plasma had a creamy appearance. After postmortem biochemical analysis, we made a diagnosis of DKA with severe hypertriglyceridemia and concluded that these unusual autopsy findings were caused by DKA and postmortem change. Uncontrolled diabetes often causes DKA and hypertriglyceridemia. To the best of our knowledge, this is the first report in Korea of DKA with severe hypertriglyceridemia diagnosed by autopsy.
Adult ; Autopsy* ; Diabetic Ketoacidosis* ; Diagnosis ; Female ; Heart* ; Humans ; Hypertriglyceridemia* ; Korea ; Plasma ; Postmortem Changes

We report a 58-year-old man who developed synchronous gastric non-Hodgkin`s lymphoma (NHL) and renal cell carcinoma. He presented with epigastric discomfort for 2 months. Endoscopic finding of the stomach disclosed a large inegular ulceration with nodular margin on the upper body. Constrast enhanced CT scan of the abdomen showed an ulceration and focal wall thickening in the greater curvature side of stomach, and an enhanced bulging mass in the left kidney incidentally. The tissue obtained by radical proximal gastrectomy and nephrectomy showed diffuse large B-cell lymphoma on stomach and chromophobic type of renal cell carcinoma on kidney. To our knowledge, this is the first report of synchronous gastric NHL and renal cell carcinoma in Korea.
Abdomen ; Carcinoma, Renal Cell* ; Gastrectomy ; Hodgkin Disease* ; Humans ; Kidney ; Korea ; Lymphoma ; Lymphoma, B-Cell ; Middle Aged ; Nephrectomy ; Stomach ; Tomography, X-Ray Computed ; Ulcer

No abstract available.
Hepatitis B Surface Antigens* ; Humans ; Prevalence*

We report a case of idiopathic progressive atrophoderma of Pasini and Pierini in which the characteristics atrophic paches developed on the lumbar area and new lesions have a zosteriform distribution in lumbar area, scapular area and heels. This 32-year-old male patient who has had above lesions for 15 yeara, was first seen in September 1985. Histologic findings of this patient are atrophy in epidermis, tightly packed collagen bundles and moderatly decreased elastic fibers in the dermis. We observed this patient without treatment and 5 month later, lesions did not progress.
Adult ; Atrophy ; Collagen ; Dermis ; Elastic Tissue ; Epidermis ; Heel ; Humans ; Male

Histiocytosis X(Langerhans cell histiocytosis) is a rare prolifertive disorder of Langerhans cells that includes Lettere-Siwe disease, Hand-Shiiller-Christian his ase and eosinophilic granuloma. Since many authors have reported in anintermediate and poarl classified form histiocytosis X has a namenclatural had nosologic problem. We report a case benign cutaneous variant of histiocytisis X. A four-month-old boy had shown multiple skin-colour papules on the trunk, head and nik for one month. Extensive in- vestigations failed to detcct any systemic involvement. The clixron microscopic findings of the skin biopsy specimen were Qefinitely diagnostic for histiocytosi. X Since he did not sbow evidence of internal organ involvernent and any further progression of he skin lesion, no therapy was given. Over the next two months the disease underwent spontane us and complete remission.
Biopsy ; Eosinophilic Granuloma ; Head ; Histiocytosis* ; Histiocytosis, Langerhans-Cell* ; Humans ; Langerhans Cells ; Male ; Remission, Spontaneous* ; Skin

The authors underwent the serum lipid study, involving the lipoprotein fraction, and the HDL-cholesterol mean values in normal Korean adults visited to Hanyang University Hospital, and also compared with the previously reported serum lipid studies. The results are as follows; 1) The mean values of triglyceride, total cholesterol, and phospholipid were 103.3+/-17.1mg%, 188.9+/-18.2mg%, and 196.7+/-19.8mg%, respectively. 2) Compared with the past 7 years triglyceride showed increasing tendency with significance, and cholesterol and phospholipid showed slightly increasing tendency. 3) In the serum lipoprotein fraction, the mean values of alpha-lipoprotein, pre-beta-lipoprotein, and beta-lipoprotein were 37.2+/-5.9%, 16.0+/-4.2%, and 48.6+/-5.2%, respectively. 4) Compared with the past 10 years, the alpha-, and pre-beta-lipoprotein showed increasing tendency, meanwhile, beta-lipoprotein revealed decreasing tendency. 5) The mean value of HDL-cholesterol was 52.3+/-14.6mg%, and the ratio of HDL-cholesterol to total cholesterol(HDL-cholesterol/total cholesterol) was 28.2+/-7.9. 6) Compared with the past 2 years, HDL-cholesterol level showed slightly increasing tendency.
Adult ; Cholesterol ; Humans ; Lipoproteins ; Triglycerides