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

Though the incidence of paragonimiasis has been remarkably decreased since 1970, it is still not a rare disease in Korea. Major problems in the diagnosis of pulmonary paragonimiasis on chest radiography are its differentiation from pulmonary tuberculosis and lung cancer. Chest radiographic findings have been described in detail, but little have been reported on CT findings. We reviewed CT findings of 10 patients with pulmonary paragonimiasis. The characteristic CT findings were similar to those on chest radiography, such as air-space consolidation (70%), nodular mass (50%), pleural effusion (40%), cystic lesion (30%), small low density within the mass (30%), linear density (20%), pneumothorax(20%), and burrow track (20%). CT depicted the cystic lesions and the burrow tracks more clearly and showed the small worm-retaining cysts within the mass that were not detectable on chest radiography. In conclusion, all of these CT findings are useful in the diagnosis of pulmonary paragonimiasis especially when differentiation from tuberculosis or lung canceris difficult on chest radiography.
Diagnosis ; Humans ; Incidence ; Korea ; Lung ; Lung Neoplasms ; Paragonimiasis* ; Pleural Effusion ; Radiography ; Radiography, Thoracic ; Rare Diseases ; Thorax ; Tuberculosis ; Tuberculosis, Pulmonary

No abstract available.
Dermatitis, Atopic ; Pyroglyphidae

For the evaluation of exercise induced R wave amplitude changes in lead V5 (big up tri, openR) and multiple leads(summation operatorR), we studied 20 normal subjects and 21 ischemic heart disease patients with positive exercise test. All underwent submaximal, multiple leads multistage treadmill exercise testing. 14 to 20 normal subjects(70%) there were decreased R wave amplitude in multiple leads on treadmill exercise testing. 16 of 21 patients(76%) there were increased R wave amplitude on treadmill exercise testing. In normal group, R wave amplitude between rest and exercise was significantly decreased in lead V5 and multiple leads. In patients grous, R wave amplitude between rest and exercise was significantly increased in lead V5 and multiple leads. It is suggested exercise induced R wave amplitude changes probably enhance the diagnostic reliability by ST segment changes on treadmill exercise testing.
Exercise Test* ; Humans ; Myocardial Ischemia

We present three neuropathologically-verified and two clinically-probable cases of Creutfeldt Jakob disease. All five had nonspecific prodromal complaints or symptoms prior to overt neurological signs and showed striking progressive neurologic deterioration, especially cognitive decline and cerebellar dysfunction. Myoclonic involuntary movements and complete decapitated states followed in one or two months. The characteristic even pathognomonic in proper clinical settings, features of electroencephalography, magnetic resonance imaging and positron emission tomography and pathologic findings are presented.
Cerebellar Diseases ; Creutzfeldt-Jakob Syndrome* ; Dyskinesias ; Electroencephalography ; Magnetic Resonance Imaging ; Positron-Emission Tomography ; Strikes, Employee

Gastric bezoars are collection of indigestible material in the stomach, and these can be classified into five broad categories: phytobezoar, trichobezoar, pharmacobezoar, lactobezoar and miscellaneous materials such as sand, stone and concrete (gypsum). The treatment of gastric bezoars has recently changed from surgical management to such nonsurgical interventions as enzymatic dissolution, nasogastric suctioning and endoscopic removal by polypectomy snare and basket. Up to the present, electrohydraulic lithotripsy has been a well established method for the treatment of urinary and hepatobiliary stones. We report here on a patient who had a huge gastric bezoar that was formed by the ingestion of gypsum, and this was treated with endoscopic electrohydraulic lithotripsy.
Bezoars* ; Calcium Sulfate* ; Eating* ; Endoscopy ; Humans ; Lithotripsy* ; Silicon Dioxide ; SNARE Proteins ; Stomach ; Suction

BACKGROUND AND OBJECTIVES: A rotation chair test has been used to evaluate the function of the horizontal semicircular canals. Currently, two chair systems according to the presence of cylindrical darkroom are used in a clinic setting. However, it has not been thoroughly investigated whether one system is superior to the other system or not. In this study, we aimed to compare test outcomes and subject convenience between two systems. SUBJECTS AND METHODS: Twenty subjects with no history of otologic disease were enrolled. Subjects were tested with two systems: system [A] with a cylindrical chamber and system [B] with no chamber. The results of sinusoidal harmonic acceleration (SHA), step velocity (SV), and visual fixation (VFX) tests were compared between the systems. Subject convenience was assessed with a questionnaire survey and results were compared between the systems. RESULTS: There were no significant differences in gain or asymmetry in SHA test between the systems. However, the phase of system [A] was significantly lower than that of system [B] at 0.16 Hz. There was no significant difference between the systems in directional preponderance (DP) gain or DP time constant. Regarding the VFX test, gain was higher in system [A] than system [B]. Subjects reported less stuffiness and less anxiety with system [B] than system [A], while preferring the system [A] goggles. CONCLUSIONS: A rotation chair system without a darkroom can provide a more comfortable experience for subjects in terms of stuffiness and anxiety, while showing comparable results in SHA and SV tests with a darkroom system.
Acceleration ; Anxiety ; Ear Diseases ; Eye Protective Devices ; Semicircular Canals

BACKGROUND AND PURPOSE: The occurrence of PWD in neurodegenerative disease is very rare, and this is the first report of it being related to early-onset AD. We describe a patient with early-onset Alzheimer's disease (AD) who presented with pure word deafness (PWD). CASE REPORT: The patient had experienced PWD for 2 years, followed by other cognitive deficits suggestive of parietotemporal dysfunction. Brain imaging including 18FDG-PET and [11C] PIB-PET supported the diagnosis of AD. CONCLUSIONS: Our case highlights the clinical variability that characterizes early-onset AD.
Alzheimer Disease ; Aphasia, Primary Progressive ; Deafness ; Humans ; Neurodegenerative Diseases ; Neuroimaging

BACKGROUND/AIMS: To evaluate the therapeutic efficacy of interferon in chrcnic viral hepatitis, interferon was administered to 222 patients with biopsy proven chronic viral hepatitis. METHODS: 32 patients were excluded and the 190 patients was included, 149 men and 41 women. Average age was 34.4+-8.93 (14-67) years. 161 cases had HBsAg and HBeAg, and 29 cases had anti-HCV Ab. Three millicn units of interferon beta were given to 37 patients with chronic B hepatitis, daily for one month Six million units of interferon alpha were given to 124 patients with chronic B hepatitis and 29 patients with chr onic C hepatitis, three times a week for six months. RESULTS: 1) Out of 124 patients with clronic hepatitis B treated with a-interferon, HBeAg negativity for more tban six months was observed in 25 patients (20.2%), and defined as responder group. The 23 patients (18.5%) were defined as probable responder, because of persistent seroregativity of HBeAg for the last 6 months, despite of fluctuation of sercangativity during the follow-up. The 29 patients (23.4%) were defined as probable non-responder because of recurrence of HBeAg, which once was cleared but reappeared during last 6 months. But there was no seroconversion in 47 cases (37.9%). The overall response rate was 38.7%. 2) Out of 37 patients with chronic hepatitis B treated with B-interferon, 7 patients (18.9%) were responder, 6 patients (16.2%) probable responder, 12 patients (32.4%) probable non-responder, 12 patients (32.4%) non-responder. The overall response rate was 35.1%. 3) Out of 29 patients with chronic hepatitis C treated with a-interferon, normalization of alanine aminotransferase (ALT) level for mcrre than six months was observed in 9 patients (31.0%), and defined as responder group. The 3 patients (10.3%) were defined as probable responder, because ALT levels fluctuated but wes normalized during the last 6 months. The 5 patients (17.2%) were defined as pobable ncn-mponder, because of persistent fluctuation of ALT levels during the last 6manths, which once were normalized but reelevated. In 12 patients (41.4%), there had never been a normalization of ALT level. The overall resporate was 41.1%. 4) The period of HBeAg seropositivity was 1.33 times longer than the period of seronegativity. The faster the seroconvmion, the more the tendency to be a respander for the patients with chronic hepatitis B. CONCLUSION: Interferon therapy may be effective in some cleonic viral hepatitis.
Alanine Transaminase ; Biopsy ; Female ; Follow-Up Studies* ; Hepatitis B ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic ; Hepatitis C, Chronic ; Hepatitis* ; Humans ; Interferon-alpha ; Interferon-beta ; Interferons* ; Male ; Recurrence

We report a 52-yr-old Korean woman with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) whose diagnosis was confirmed by skin biopsy and the presence of a novel mutation in the NOTCH3 gene. The patient's clinical features were rather unusual in that 1) clinical presentations were only two episodes of stroke and mild dementia unaccompanied by mood disturbances or migraine, and 2) there was no family history. Brain MRI showed T2 hyperintensities in both temporal pole areas in line with the recent suggestion by O'Sullivan et al. that the abnormality could be a radiologic marker of CADASIL. FDG-PET also showed a hypometabolism in the temporal pole areas with an abnormal finding on MRI in addition to the hypometabolism in cortical and subcortical regions. We could learn from this case that CADASIL may be included in the differential diagnoses in patients with vascular dementia associated with a small vessel disease, even in the absence of a family history, especially when there are no known stroke risk factors and when the MRI shows T2 hyperintensity in the temporal pole regions.
Amino Acid Substitution ; Biopsy ; Brain/pathology ; Codon/genetics ; Dementia, Multi-Infarct/diagnosis ; Dementia, Multi-Infarct/genetics* ; Dementia, Multi-Infarct/pathology ; Dementia, Multi-Infarct/radionuclide imaging ; Female ; Human ; Korea ; Magnetic Resonance Imaging ; Middle Aged ; Mutation, Missense* ; Neuropsychological Tests ; Point Mutation* ; Proto-Oncogene Proteins/genetics* ; Skin/pathology ; Tomography, Emission-Computed