Dystrophic epidermolysis bullosa is a rare, chronic non-inflammatory bullous disease, which easily forms bullae by minor mechanical trauma or spontaneously, is inherited either in an autosomal dominant or autosomal recessive fashion. We report herein two cases which presented with bullae, erosions and ulcers on extremities, buttock, chest, abdomen and face and loss of all nail since birth in two sisters. Bulla occured bencath the basal lamina histopathologically, anchoring fibrils were almost absent on electron miaoscopy in both cases. The two sisters represented dystrophic epidermolysis bullosa considering the absence of family history inheritcd in an autosomal dominant fashion and the clinical, histological and electronmicroscopic findings.
Abdomen ; Basement Membrane ; Buttocks ; Epidermolysis Bullosa Dystrophica* ; Extremities ; Humans ; Parturition ; Siblings* ; Thorax ; Ulcer

PURPOSE: We investigated the causes and mechanisms driving acute border zone infarctions using diffusion-weighted imaging (DWI). MATERIALS AND METHODS: We analyzed DWI in 104 patients (male: 72 years, female: 32 years, age range: 44 to 84 years) with acute border zone infarction. The DWI patterns were classified as follows: pattern A- An acute border zone infarction combined with multiple small disseminated cortical infarctions, pattern B- An acute border zone infarction only. RESULTS: The most common cause of acute border zone infarctions was extracranial internal carotid artery (ICA) stenosis (45 cases, 43%). Other causes included middle cerebral artery stenosis (22 cases, 21%), intracranial ICA stenosis (14 cases, 13%), unknown, (12 cases 12%), iatrogenic (6 cases, 6%) and cardiogenic (5 cases, 5%), respectively. The most common pattern for DWI was pattern A (83 cases, 80%). We performed a transcranial Doppler in 7 of 75 cases (11%), and found at least 1 embolic pulse. CONCLUSION: The most common pattern of DWI for acute border zone infarctions was pattern A. We propose that the mechanisms driving acute border zone infarctions are emboli coupled with hypoperfusion.
Brain Infarction ; Carotid Artery, Internal ; Cerebral Infarction ; Constriction, Pathologic ; Diffusion ; Diffusion Magnetic Resonance Imaging ; Humans ; Infarction ; Middle Cerebral Artery

BACKGROUND: Improvements in catheter equipments and increasing experience of the operators have brought about a broadening of the indications and applications of percutaneous transluminal coronary angioplasty(PTCA). In particular, coronary angioplasty has been employed in total occlusions.We evaluated the initial success rate and safety of PTCA total occulsive coronary artery disease. METHODS: To assess the success rate and safety of PTCA total coronary artery occlusion, the data of 24 patients(male 19, female 5, mean age 55+/-9 years), in whom PTCA for total occlusion were performed, were examined. RESULTS: Primary success rate of procedure was 66.7%(16 out of 24 lesions).The success rate according to the duration of total occlusions was 8 out of 11(72.7%) with occlusions<4 weeks duration and 3 out of 7(42.9%) with occlusions>4 weeks duration(p=0.07). There was no difference in success rate according to vassel dilate(left anterior descending 75.0%, right coronary 44.4%) and length of occluded lesion(0.93%+/-0.47%cm, p=0.35). Eight procedural failures included inability to cross the lesion with a guide wire in 6 and inability to dilate the lesion in 2, but there was no emergency coronary bypass surgery or cardiac death. CONCLUSION: Even though the recanalization of occluded coronary arteries has a lower initial success rate than angioplasty for stenotic arteries, PTCA in total occlusion can be performed as a safe and effective therapeutic modality in selective patients.
Angioplasty ; Angioplasty, Balloon, Coronary* ; Arteries ; Catheters ; Coronary Artery Disease ; Coronary Vessels* ; Death ; Emergencies ; Female ; Humans

BACKGROUND: Indications and applications of percutaneous transluminal coronary angioplasty(PTCA) have been broaden recent years. We evaluated the initial success rate and safety of PTCA in mulitiple lesions and vessels. METHODS: To assess the success rate and safety of PTCA In mulitiple lesions and vessels, the data of 60 lesions from 28 patients(male 23, female 5, mean age 56+/-12 years), in whom PTCA for multiple lesions and vessels were performed, were examined. Initial results and complications were compared in 37 lesions undergoing multivessel and 23 lesions undergoing multilesion PTCA. RESULTS: Overall primary success rate of procedure was 82%(49 out of 60 lesions). Angioplasty was attempted in mean 2.1 stenotic lesions per patient. Primary success rate per lesion was 84%(31 of 37) among those who underwent multivessel and 78%(18 of 23) among those who underwent multilesion PTCA(p>0.05). Success rate according to the combination of dilated vessels was 85.7%(12 of 14) in left anterior descending(LAD) and left circumflex(LCX), 83.3%(10 of 12) in LAD and right coronary artery(RCA), 100.0%(4 of 4) in LAD and diagonal branch, RCA and LCX in 75.0%(3 of 4), and 66.7%(2 of 3) in LAD, LCX and diagonal branch. Eleven failures(18.3%) included inability to pass the guide wire cross the lesion or inability to locate the balloon catheter in 5(8,3%), abrupt closure in 2(3,3%), coronary spasm in 1(1.7%) and major branch occlusion in 3(5.0%), but there was no emergency coronary bypass surgery or cardiac death. CONCLUSION: Coronary angioplasty in selected patients with multivessel and multilesion coronary artery disease might be useful and have relatively good initial results, but the long-term efficacies with other forms of treatment must be evaluated prospectively.
Angioplasty* ; Catheters ; Coronary Artery Disease ; Death ; Emergencies ; Female ; Humans ; Spasm

We present a series of 10 Patients(Seven men and three women with an average age of 53 years) who underwent decompressive craniectomy for treatment to massive brain swelling following acute cerebral infarction. Clinical signs of cerebral herniation(anisocoria or fixed and dilated pupil, and/or hemiplegia with decerebrate righidity) were present in all patients. Computed tomography and magnetic resonance imaging showed the mass effect by cerebral edema through midline shift. All patients were treated with an extensive craniectomy and duroplasty. Among them, one recovered without neurological deficit, three were moderately disabled but functionally dependent, three remained in a persistent vegetative state and three died within 9 days after surgery(good recovery=1, moderate disability=3, persistent vegetative state=3, death=3). The results suggest that decompressive craniectomy can be an useful lifesaving procedure for massive cerebral edema following widespread hemispheric infarction.
Brain Edema ; Cerebral Infarction* ; Decompressive Craniectomy* ; Female ; Hemiplegia ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male ; Persistent Vegetative State ; Prognosis ; Pupil

No abstract available.
Thanatophoric Dysplasia*

No abstract available.

Marchiafava-Bignami disease is a rare complication of chronic alcoholism and this malady typically manifests as callosal lesion. I report here on one patient with Marchiafava-Bignami disease (MBD) who has symmetric restricted diffusion in both lateral-frontal cortices, in addition to the callosal lesion.
Alcoholism ; Corpus Callosum ; Diffusion ; Humans ; Marchiafava-Bignami Disease*

PURPOSE: To determine the magnetic resonance (MR) imaging findings of tuberculous arthritis, and the frequency-in such cases-with which tuberculoma occurs. MATERIALS AND METHODS: MR images of 26 patients (M:F, 14:12; mean age, 46.2 years) with pathologically proven tuberculous arthritis were retrospectively reviewed. The presence of joint effusion, subchondral erosion, synovial proliferation and soft tissue abscess, and whether the inner wall of this abscess was smooth, were assessed. In particular, we determined whether a nodular lesion which showed low SI on T1WI, central low SI with peripheral high SI on T2WI, and rim enhancement on contrast study, was a tuberculoma. RESULTS: The joints involved were those of the knee (n=7), hip (n=7), shoulder (n=4), sacroiliac region (n=3), elbow (n=3), and ankle (n=2). Joint effusion was noted in 15 cases (58%), and subchondral erosion in 24(92%). synovial proliferation was found in 23 cases (88%), and soft tissue abscess in 24 (92%). The inner wall of this abscess was irregular in 17 cases (71%). A tuberculoma was present in intra- or extra-articular soft tissue in 18 cases (69%). CONCLUSION: The MR findings of tuberculous arthritis were subchondral erosion, synovial proliferation, and soft tissue abscess. The presence of a tuberculoma in intra- or extra-articular soft tissue, a specific finding in tuberculous arthritis, was noted in 69% of our cases.
Abscess ; Ankle ; Arthritis* ; Elbow ; Hip ; Humans ; Joints ; Knee ; Retrospective Studies ; Shoulder ; Tuberculoma*

No abstract available.
Animals* ; Fever* ; Hantaan virus*