A 31-year-old female with paroxysmal nocturnal hemoglobinuria (PNH) presented with left hemiparesis. One year prior to this presentation she had been diagnosed with bihemispheric cerebral infarctions associated with moyamoya syndrome. During her current presentation, MRI revealed acute infarction of the right hemisphere and MRA revealed occlusion of the right internal carotid artery (ICA), and aggravation of the preexisting stenosis of the contralateral ICA and ipsilateral posterior cerebral artery compared to the previous MRA. We report herein a case of progressive moyamoya syndrome associated with PNH.
Adult ; Carotid Artery, Internal ; Cerebral Infarction ; Constriction, Pathologic ; Female ; Hemoglobinuria, Paroxysmal* ; Humans ; Infarction ; Magnetic Resonance Imaging ; Moyamoya Disease* ; Paresis ; Posterior Cerebral Artery

Hemangiopericytoma is uncommon vascular neoplasm that arises from pericytes arround the capillary walls. It was first described as a distinct vascular neoplasm by Stout and Murray in 1942 The anatomic distribution is widespread throughout the body, with approximately one third occur in the head and neck. No sex predilection has been found. Although middle age appears to be the most prevalent time of onset, this neoplasm has been found in all age groups. The differentiation between benign and malignant hemangiopericytoma can be difficult. Although the majority of these tumors are benign, there are malignant variants that can metastasize. Metastasis of seemingly benign tumors may appear year of decade later, so long term close follow-up is needed The treatment of choice is complete surgical excision of the tumor. Despite their vascular origin, these tumors are relatively radioresistant. Radiation therapy is reserved for inoperable metastases or treatment of postoperative surgical fields. Here we present a case of hemangiopericytoma occuring on the Lt. buccal mucosa.
Capillaries ; Follow-Up Studies ; Head ; Hemangiopericytoma* ; Humans ; Middle Aged ; Mouth Mucosa* ; Neck ; Neoplasm Metastasis ; Pericytes ; Vascular Neoplasms

It is believed that migrainous aura is correlated with cortical spreading depression and spreading benign oligemia. A 54-year-old female with migraine with aura presented with left hemianopia preceding pulsating headache. Perfusion-weighted images revealed delayed contrast arrival to the right occipital lobe and nearly normal relative cerebral blood volume images, indicating benign oligemia. Follow-up perfusion-weighted images revealed resolution of the perfusion abnormalities. We report herein a case of migraine with aura presenting with reversible delayed perfusion in the right occipital lobe on perfusion-weighted images.
Blood Volume ; Cortical Spreading Depression ; Epilepsy ; Female ; Follow-Up Studies ; Headache ; Hemianopsia ; Humans ; Middle Aged ; Migraine Disorders ; Migraine with Aura* ; Occipital Lobe ; Perfusion*

OBJECTIVE: To investigate the synovial mRNA expression of receptor activator of NFkappaB (RANK), RANK ligand (RANKL), osteoprotegerin (OPG) and RANKL/OPG mRNA expression ratio, and to evaluate the effects of IL-17 in experimental rheumatoid arthritis (RA) model. METHODS: After induction of collagen-induced arthritis (CIA) by type II collagen in DBA1 mice, mice were anesthetized at day 28 and a small aperture in the skin of the knee was performed. Mice, in which arthritis of knee was present, were selected and divided into 3 groups, and phosphate-buffered saline (PBS group), IL-17 (IL-17 group) or anti-IL-17 monoclonal antibody (anti-IL-17 group) was injected to both knee joint at day 28 and 32. At day 35, mice were sacrificed and synovium of knee joints were isolated. Synovial mRNA expression of RANKL, RANK and OPG was assessed by real-time RT-PCR and immunohistochemical stain. RESULTS: Synovial RANKL and RANK mRNA expressions were significantly different among IL-17, PBS, anti-IL-17 and normal group (IL-17>PBS>anti-IL-17>normal group), and synovial OPG mRNA expressions in PBS, IL-17 and anti-IL-17 group were significantly high than those in normal group, however, there was no significant difference among IL-17, PBS and anti-IL-17 group. RANKL/OPG mRNA ratio was significantly different among these groups (IL-17>PBS>anti-IL-17>normal group). In immunohistochemical stain, RANKL, RANK and OPG-positive cells were expressed at synovium. CONCLUSION: Synovial RANKL/OPG mRNA ratio was enhanced in CIA, and IL-17 induced higher RANKL/OPG ratio in the synovium of CIA, which was blocked by anti-IL-17 antibody. These results suggest that RANKL/OPG mRNA ratio play an important roles on bone destruction, and IL-17 may be actively involved in bone destruction by enhancing RANKL/OPG ratio in CIA model.
Animals ; Arthritis ; Arthritis, Experimental ; Arthritis, Rheumatoid* ; Collagen Type II ; Interleukin-17* ; Knee ; Knee Joint ; Mice ; Osteoprotegerin ; RANK Ligand ; RNA, Messenger* ; Skin ; Synovial Membrane

PURPOSE:Carotid endarterectomy may benefit patients with bilateral carotid stenosis by improving cerebrovascular hemodynamics of ipsilateral as well as contralateral cerebral hemispheres. We investigated cerebrovascular hemodynamics after carotid endarterectomy in patients with contralateral carotid occlusion by acetazolamide stress brain SPECT. MATERIALS AND METHODS: Subjects were 14 symptomatic patients (all men, mean age 66 yrs) with carotid stenosis (> 50%) with contralateral carotid occlusion. Acetazolamide stress Tc-99m ECD brain SPECTs were performed within 2 weeks before and after carotid endarterectomy using one day protocol. Cerebral blood flow (CBF) and cerebrovascular reserve (CVR) were assessed visually. In 12 patients, correlation between the patency of proximal anterior cerebral or anterior communicating arteries (A1/A-comm) and the improvement of CBF or CVR after endarterectomy was assessed. RESULTS: Preoperative SPECT showed reduced CBF in 2 ipsilateral and 10 contralateral hemispheres. CVR was reduced in 4 ipsilateral and 9 contralateral hemispheres. Of 12 hemispheres with reduced CBF, 2 hemispheres (16.7%) showed improvement of CBF after endarterectomy. However, reduced CVR was improved in all 4 ipsilateral and 7 of 9 (78%) of contralateral hemispheres after endarterectomy. Three of 4 with stenotic A1/A-comm and 4 of 8 with patent A1/A-comm had reduced contralateral CVR. Reduced contralateral CVR improved in all 3 patients with stenotic A1/A-comm and 3 of 4 with patent A1/A-comm. CONCLUSION: Acetazolamide stress brain SPECT demonstrated improvement of compromised cerebrovascular reserve in not only ipsilateral but also contralateral hemispheres of patients with contralateral carotid occlusion after carotid endarterectomy, and may, therefore, be useful for evaluating cerebral blood flow and cerebrovascular reserve after carotid endarterectomy.
Acetazolamide* ; Arteries ; Brain Ischemia ; Brain* ; Carotid Stenosis* ; Cerebrum ; Endarterectomy ; Endarterectomy, Carotid* ; Hemodynamics ; Humans ; Male ; Tomography, Emission-Computed, Single-Photon*

Phenylpropanolamine is a sympathomimetic amine used widely as a decongestant or appetite suppressant. Reports of the myocardial injury from the use of phenylpropanolamine are rare and the mechanism of the myocardial injury is not known clearly. We experienced a case of myocardial injury after ingestion of phenyl-propanolamine. A 46-year-old woman was admitted because of chest pain and dyspnea after ingestion of 5 tablets of anorectic pill containing phenylpropanolamine 75 mg per tablet. The serum creatine kinase MB isoenzyme levels were elevated and electrocardiographic abnormalities suggesting myocardial infarction were seen in the precordial lead. In echocardiograpy, left ventricular anteroseptal wall motion was nearly akinetic but coronary angiography showed normal coronary arteries except sluggish blood flow in left anterior descending artery.
Appetite ; Arteries ; Chest Pain ; Coronary Angiography ; Coronary Vessels ; Creatine Kinase ; Dyspnea ; Eating* ; Electrocardiography ; Female ; Humans ; Middle Aged ; Myocardial Infarction ; Phenylpropanolamine* ; Tablets

Right sided aortic arch is an uncommon congenital anomaly. It can be classified into three types, depending on the left aortic arch's degenerating pattern and the branching pattern of the great vessels. It can be associated with major congenital heart disease, depending on the type of right sided aortic arch. We report a case of an 18-years-old female who has right sided aortic arch with atrial septal defect (ASD). In our case, the patient had a right sided aortic arch and aberrant left subclavian artery, also she had ASD (ostium secundum) and moderate tricuspid regurgitation with pulmonary hypertension. The patient was successfully performed patch closure of ASD and tricuspid valve annuloplasty via midline sternotomy. The patient had uneventful postoperative course.
Aneurysm ; Aorta, Thoracic ; Cardiovascular Abnormalities ; Deglutition Disorders ; Female ; Heart Diseases ; Heart Septal Defects, Atrial ; Humans ; Hypertension, Pulmonary ; Sternotomy ; Subclavian Artery ; Tricuspid Valve ; Tricuspid Valve Insufficiency

PURPOSE: To evaluate the effectiveness of percutaneous transluminal angioplasty (PTA) and pulsed-spraypharmacomechanical thrombolysis (PSPMT) using urokinase for the management of insufficient hemodialysis access. MATERIALS AND METHODS: Between September 1996 and May 1998, 21 insufficient hemodialysis accesses were treated in 16 patients (3 artificial arteriovenous fistulae, AVF ; and 13 arteriovenous graft, AVG). PTA and PSPMT were performed in 6 and 15 and 15 cases, respectively, and success and long-term patency rates were evaluated. RESULTS: The overall success rate of PTA and PSPMT for insufficient hemodialysis access was 76.2%(16/21). The success rates of PTA and PSPMT were 83.3%(5/6) and 73.3%(11/15), respectively. the primary patency rates of PSPMT were 69+/-12.8% at 6 months and 38+/-18.6% at 12 months. One of the two initially successful PTAs had been patent for 7months, and the second PTA was performed at that time due to venous stenosis. The other was patent for 15 months throughout the follow-up period. CONCLUSION: PTA and PSPMT are effective primary methods for the treatment of insufficient hemodialysis access ; success and patency rates were high, and the procedures can be performed repeatedly.
Angioplasty ; Arteriovenous Fistula ; Constriction, Pathologic ; Endometriosis* ; Female ; Follow-Up Studies ; Humans ; Renal Dialysis ; Transplants ; Urokinase-Type Plasminogen Activator

OBJECTIVE: To evaluate the expression of Toll-like receptor (TLR) 2, 4 and 9 and investigate the effects of IL-17 on the expression of TLRs in experimental rheumatoid arthritis (RA) model. METHODS: After induction of collagen-induced arthritis (CIA) by type II collagen in DBA1 mice, phosphate-buffered saline (PBS, PBS group) or IL-17 (IL-17 group) was injected to both knee joint at day 28 and 32. At day 35, mice were sacrificed and knee joints were isolated. Synovial mRNA expressions of TLR-2, 4 and 9 determined by real-time RT-PCR were compared among normal DBA1 mice (normal group), PBS and IL-17 group. RESULTS: Synovial TLR-2, 4, and 9 mRNA expressions of IL-17 and PBS group were significantly higher than normal group, and those of IL-17 group were higher than PBS group. CONCLUSION: Synovial TLR-2, 4 and 9 expression was enhanced in CIA and up-regulated by local overexpression of IL-17. These results suggest that TLRs play a roles on CIA and IL-17 induced aggravation of arthritis in CIA.
Animals ; Arthritis ; Arthritis, Experimental* ; Arthritis, Rheumatoid ; Collagen Type II ; Interleukin-17 ; Knee Joint ; Mice ; RNA, Messenger ; Toll-Like Receptors*

Protective effect of human cerebrospinal fluid antioxidants against enzyme inactivation caused by metal-catalyzed oxidation systems were investigated. When purified glutamine synthetase(GS) was incubated with human cerebrospinal fluid(CSF), the enzyme was progressively inactivated. Catalase and EDTA could inhibit the enzyme inactivation by 50-80%. Small-molecular(Mr<-10,000) fraction of CSF inactivated the exogenous GS, but large-molecular(Mr>-10,000) fraction did not. The GS inactivation by the small-molecular fraction was also markedly inhibited by catalase and EDTA. These results suggested that metal-catalyzed oxidation is involved in the GS inactivation by the small-molecular fraction of CSF. Dithiothreitol(DTT)was shown to inhibit almost completely the oxidative inactivation of GS by CSF. However, DTT inhibited only partially the oxidative inactivation of GS caused by small-molecular fraction of CSF. When large-molecular fraction of CSF was separated by anion-exchange HPLC chromatography, there was a peak of antioxidant activity inhibiting the small-molecular fraction-induced GS inactivation in the presence of DTT. The antioxidant activity was neutralized by monoclonal antibodies to transthyretin. Purified transthyretin was found to efficiently inhibit ascorbate/Cu2+-induced GS inactivation in the presence of DTT. Uric acid and glucose did not shoe any protective effect on the GS inactivation in the same condition. The above results suggest that metal-catalyzed oxidation occurs normally in human CSF, and the transthyretin may play an important role as a CSF antioxidant in protecting proteins from metal-catalyzed oxidation.
Antibodies, Monoclonal ; Antioxidants ; Catalase ; Cerebrospinal Fluid* ; Chromatography ; Chromatography, High Pressure Liquid ; Edetic Acid ; Glucose ; Glutamine ; Humans* ; Prealbumin* ; Shoes ; Uric Acid