Bilateral traumatic carotid-cavernous fistulae (TCCFs) is rarely encountered neurovascular disease. For treatment of TCCF, detachable balloons have been widely used. Nowadays, transarterial and/or transvenous coil embolization with placement of covered stents is adopted as another treatment method. We experienced a patient with a bilateral TCCFs who was successfully treated with covered stents. However, cerebral hemorrhage occurred in the bed of previous infarction one day after treatment. Hyperperfusion syndrome was considered as a possible cause of the hemorrhage, so that barbiturate coma therapy was started and progression of hemorrhage was stopped. We emphasize that cerebral hyperperfusion hemorrhage can occur even after successful endovascular treatment of TCCF.
Barbiturates ; Caves ; Cerebral Hemorrhage ; Coma ; Fistula ; Hemorrhage ; Humans ; Infarction ; Stents

OBJECTIVE: There are few studies of osteoporotic burst fractures with spinal canal compromise that were treated with kyphoplasty. The purpose of this study was to assess the efficacy and the safety of kyphoplasty for the treatment of thoracolumbar spine osteoporotic burst fractures without neurological deficits and to investigate predictors that can improve the effectiveness of this treatment. METHODS: From October 2004 until December 2010, 79 osteoporotic burst fracture patients with asymptomatic spinal canal compromise were treated by kyphoplasty. Kyphoplasty involved injecting polymethylmethacrylate (PMMA) from the anterior one-third of the vertebral body into the posterior one-third of the vertebral body. PMMA powder and liquid were mixed in a ratio of 1:3 at 21-23degrees C. Preoperative and postoperative visual analog scores (VAS), kyphotic angles, the heights of the vertebral body, and the degrees of spinal canal compromise were analyzed. The relationships between the factors and the VAS difference between the preoperative and postoperative states were analyzed. RESULTS: The mean postoperative VAS, the postoperative kyphotic angle, the vertebral body height measurement from the anterior and the middle of the body were significantly improved after kyphoplasty (p<0.05). Postoperatively, the mean VAS was restored from 7.35 to 2.11, the postoperative kyphotic angle was restored from 18.21degrees to 12.08degrees, the vertebral body height measurement from the anterior of the body was from 18.56mm to 23.54mm and the middle of the body was from 24.81mm to 27.90mm. Among the variables, only the duration of symptoms before surgical treatment was implicated as a factor in the outcome (p<0.01). CONCLUSION: Kyphoplasty is a very effective and safe therapeutic modality for the treatment of osteoporotic burst fractures without neurological deficits. The standardization of this procedure helps to avoid complications. In the acute stages, early treatment is more effective for the reduction of pain. Further investigation is warranted in order to assess the effectiveness of early surgery in improving outcome.
Body Height ; Humans ; Kyphoplasty ; Osteoporotic Fractures ; Polymethyl Methacrylate ; Spinal Canal ; Spine

OBJECTIVE: There is a broad spectrum of compensated hydrocephalus. Various terms such as long-standing overt ventriculomegaly in adult (LOVA) has been coined, however, even such terms leave diverse aspect of this condition out of account. We have experienced compensated hydrocephalus cases which were considered to be activated after a long time period of quiescent state, and tried to compare their clinical characteristics with the relatively well described entity of LOVA. METHODS: We conducted a retrospective review of 206 patients who underwent ventriculoperitoneal shunt (VPS) between February 2001 and May 2012. Of these, 6 patients had chronic compensated hydrocephalus. The clinical and radiological characteristics are evaluated. RESULTS: Definite triventriculomegaly was observed in two patients. Macrocephaly was observed in two cases, one with aqueductal stenosis (AS), the other with unknown status of aqueduct. All of the cases with triventriculomegaly were normocephalic. Spinal causes were thought as aggravating factor in two. Two endoscopic third ventriculostomy and eight VPS were performed in five patients. Four patients responded well but one took a very complicated course. CONCLUSION: The relationships between macrocephaly, triventriculomegaly, and AS suggested in other studies were inconsistent. Blockage or narrowing of cerebrospinal fluid pathways were observed at various sites. Disturbances of spinal arachnoid pathways were related to the activation in some cases. Treatment is to be tailored individually considering various reigniting event. It is suggested that this entity is to be evaluated for better nomenclature reflecting diverse aspects of this condition. Further study is needed to elucidate underlying pathophysiology and effective management.
Adult ; Arachnoid ; Humans ; Hydrocephalus ; Macrocephaly ; Numismatics ; Retrospective Studies ; Ventriculoperitoneal Shunt ; Ventriculostomy

Aneurysms of the posterior inferior cerebellar artery (PICA) are rarely encountered. In particular, due to frequent anatomic complexity and the presence of nearby critical structures, PICA origin aneurysms are difficult to treat. However, recent reports of anecdotal cases using advanced endovascular instruments and skills have made the results of endovascular treatment rather outstanding. PICA preservation is the key to a successful endovascular treatment, based on the premise that a PICA origin aneurysm is well occluded. To secure PICA flow, stenting into the PICA would be the best method, however, it is nearly impossible technically via the ipsilateral vertebral artery (VA) if the PICA arose at an acute angle from the sac. In such a case, a bilateral approach for stent-assisted coiling can be a creative method for achievement of two goals of both aneurysm occlusion and PICA preservation: ipsilateral approach for coil delivery and contralateral cross-over approach for stent delivery via a retrograde smooth path into the PICA.
Achievement ; Aneurysm ; Arteries ; Intracranial Aneurysm ; Pica ; Stents ; Vertebral Artery

OBJECTIVE: After injury to the central nervous system (CNS), glial scar tissue is formed in the process of wound healing. This can be is a clinical problem because it interferes with axonal regeneration and functional recovery. It is known that intracellular proteins, including the glial fibrillary acidic protein (GFAP), nestin, and vimentin increase in the astrocytes after an injury to the CNS. By studying the time course and co-expression pattern of these intracellular proteins, this study will attempt to prove that these proteins are involved in the processes of glial scar formation. METHODS: Twenty-five male Sprague-Dawley rats were used in this study. Bregma of the cerebral cortex, an area was incised with a sharp blade, and perfusion was performed. The expressions of the intracellular proteins were assayed, while the co-localization of the intermediate filament (GFAP, nestin, and vimentin) and A2B5 were examined. RESULTS: At 12 hours, the GFAP was expressed in the white matter underlying the lesion, and in the cerebral cortex. Nestin was expressed in the astrocytes in the perilesional area after 3 days, while A2B5 was observed in the edge of the wound at 12 hours post-injury, with its expression reaching a peak at 7 days. Vimentin was detected in the white matter at 12 hours, and in the cortex, reaching a peak at 7 days. CONCLUSION: In the processes of glial scar formation, nestin, vimentin, and A2B5 were revealed in the astrocytes, and these factors may be involved in the division, proliferation, and transportation of the astrocytes.
Animals ; Astrocytes* ; Axons ; Brain* ; Central Nervous System ; Cerebral Cortex ; Cicatrix ; Glial Fibrillary Acidic Protein ; Gliosis ; Humans ; Intermediate Filaments ; Male ; Nestin ; Perfusion ; Rats* ; Rats, Sprague-Dawley ; Regeneration ; Transportation ; Vimentin ; Wound Healing ; Wounds and Injuries

Intravenous infusion flow regulators (IIFRs) are widely used devices but it is unknown how much the difference between the IIFR scale and the actual flow rate depends on the viscosity of the intravenous (IV) fluid. This study evaluated the effects of viscosity on the flow rate of five IV fluids (0.9% normal saline, Hartmann’s solution, plasma solution-A, 6% hetastarch, and 5% albumin) when using IIFRs. The viscosity of crystalloids was 1.07–1.12 mPa·s, and the viscosities of 6% hetastarch and 5% albumin were 2.59 times and 1.74 times that of normal saline, respectively. When the IIFR scales were preset to 20, 100, and 250 mL/hr, crystalloids were delivered at the preset flow rate within a difference of less than 10%, while 6% hetastarch was delivered at approximately 40% of the preset flow rates and 5% albumin was approximately 80% transmitted. When delivering colloids, IIFRs should be used with caution.

Familial hypercholesterolemia (FH) is a common autosomal dominant disorder caused by a defect in the low-density-lipoprotein (LDL) receptor, disrupting the normal control of cholesterol metabolism. We have collected 86 FH families for over 5 years who met following Dx criteria 1) hypercholesterolemia over 280 mg/dl 2) Achilles tendon xanthoma thicker than 9 mm, and 3) familial tendency, and characterized the pattern of mutations in Korea FH patients. METHOD: Mutation was screened with linkage analysis into two ways; large structural rearrangements were screened by genomic Southern blotting or long-PCR technique, and small structural rearrangements were screened by PCR of each exon followed by SSCP analysis. The exact mutation sites were confirmed by sequencing. RESULT: 1) Large mutation: Three different large deletions(FH110, FH29, FH32) were found in 7(11.5%) among 61 families screened. FH110 was a deletion of 5.7kb from intron 8 to 12, which was found in 5 unrelated families. FH29 was a deletion of 3.8kb from intron 6 to 8, and FH32 was a deletion of 2kb from intron 6 to 7. These three deletions have not been reported previously. The mechanism of deletion was unequal crossover from mispairing Alu-sequences. 2) Small or point mutations: Nineteen different small mutations were found in 19(31.4%) among 86 families screened . These mutations comprised 9 missense, 3 nonsense, 2 splicing mutations, 3 small deletions, and 2 small insertions. One missense mutation (Pro664Leu) was found in 6 unrelated families. Among these mutations, 12 have not been reported previously. CONCLUSIONS: LDL receptor gene mutations are heterogeneous in Korean FH patients. We could not observe founder mutation but detect common mutations.
Achilles Tendon ; Blotting, Southern ; Cholesterol ; Exons ; Humans ; Hypercholesterolemia ; Hyperlipoproteinemia Type II* ; Introns ; Korea ; Lipoproteins* ; Metabolism ; Mutation, Missense ; Point Mutation ; Polymerase Chain Reaction ; Polymorphism, Single-Stranded Conformational ; Receptors, LDL ; Receptors, Lipoprotein* ; Xanthomatosis

Familial hypercholesterolemia (FH) is a common autosomal dominant disorder caused by a defect in the low-density-lipoprotein (LDL) receptor, disrupting the normal control of cholesterol metabolism. We have collected 86 FH families for over 5 years who met following Dx criteria 1) hypercholesterolemia over 280 mg/dl 2) Achilles tendon xanthoma thicker than 9 mm, and 3) familial tendency, and characterized the pattern of mutations in Korea FH patients. METHOD: Mutation was screened with linkage analysis into two ways; large structural rearrangements were screened by genomic Southern blotting or long-PCR technique, and small structural rearrangements were screened by PCR of each exon followed by SSCP analysis. The exact mutation sites were confirmed by sequencing. RESULT: 1) Large mutation: Three different large deletions(FH110, FH29, FH32) were found in 7(11.5%) among 61 families screened. FH110 was a deletion of 5.7kb from intron 8 to 12, which was found in 5 unrelated families. FH29 was a deletion of 3.8kb from intron 6 to 8, and FH32 was a deletion of 2kb from intron 6 to 7. These three deletions have not been reported previously. The mechanism of deletion was unequal crossover from mispairing Alu-sequences. 2) Small or point mutations: Nineteen different small mutations were found in 19(31.4%) among 86 families screened . These mutations comprised 9 missense, 3 nonsense, 2 splicing mutations, 3 small deletions, and 2 small insertions. One missense mutation (Pro664Leu) was found in 6 unrelated families. Among these mutations, 12 have not been reported previously. CONCLUSIONS: LDL receptor gene mutations are heterogeneous in Korean FH patients. We could not observe founder mutation but detect common mutations.
Achilles Tendon ; Blotting, Southern ; Cholesterol ; Exons ; Humans ; Hypercholesterolemia ; Hyperlipoproteinemia Type II* ; Introns ; Korea ; Lipoproteins* ; Metabolism ; Mutation, Missense ; Point Mutation ; Polymerase Chain Reaction ; Polymorphism, Single-Stranded Conformational ; Receptors, LDL ; Receptors, Lipoprotein* ; Xanthomatosis

PURPOSE: We identified pediatric liver transplant recipients with successful withdrawal of immunosuppression who developed tolerance in Korea. MATERIALS AND METHODS: Among 105 pediatric patients who received liver transplantation and were treated with tacrolimus-based immunosuppressive regimens, we selected five (4.8%) patients who had very low tacrolimus trough levels. Four of them were noncompliant with their medication and one was weaned off of immunosuppression due to life threatening posttransplant lymphoproliferative disorder. We reviewed the medical records with regard to the relationship of the donor-recipients, patient characteristics and prognosis, including liver histology, and compared our data with previous reports. RESULTS: Four patients received the liver transplantation from a parent donor and one patient from a cadaver donor. A trial of withdrawal of the immunosuppressant was started a median of 45 months after transplantation (range, 14 months to 60 months), and the period of follow up after weaning from the immunosuppressant was a median of 32 months (range, 14 months to 82 months). None of the five patients had rejection episodes after withdrawal of the immunosuppression; they maintained normal graft function for longer than 3 years (median, 38 months; range, 4 to 53 months). The histological findings of two grafts 64 and 32 months after weaning-off of the medication showed no evidence of chronic rejection. CONCLUSION: The favorable markers for successful withdrawal of immunosuppression were 1) long-term (> 3 years) stable graft function, 2) no rejection for longer than 1 year after withdrawal of immunosuppression, 3) non-immune mediated liver diseases, and 4) pediatric patients.
Child ; Child, Preschool ; Female ; Humans ; Immunosuppressive Agents/*administration & dosage/therapeutic use ; Infant ; Korea ; Liver/pathology ; Liver Transplantation/*immunology/*methods ; Male ; Postoperative Complications/*drug therapy/immunology ; Tacrolimus/*administration & dosage/therapeutic use

Enteroviruses are the most common agents of myocarditis and have been implicates in the pathogenesis of dilated cardiopmyopathy. There are still discrepancies in the association of enterovirus and myocardial disease, partially due to lack of data on detection of virus antigen or viral culture in the tissue. For the treatment of fulminant myocarditis, aggressive hemodynamic support is warranted because of its excellent long-term prognosis. This 16 year-old girl was admitted because of anterior chest pain for a day. She had flu-like symptoms such as fever, sore throat and cough at 2 weeks ago. Electrocardiogram showed sinus tachycardia and ST segment elevations in lead II, III, aVF and V1-V4. Troponin T was positive and creatinine phosphokinase was elevated (1323 IU/L) at emergency room. On emergency echocardiogram, inferior wall motion was decreased and the ejection fraction (EF) was 70%. Coronary angiogram showed no thrombus and no significant stenosis in coronary artery, and spasm was not induced with ergonovine. Conventional treatment for congestive heart failure with digoxin (0.25 mg daily) and furosemide (20 mg t.i.d) was started under the impression of myocarditis. On the first hospital day, pulmonary edema and signs of shock were developed. The whole left ventricular(LV) wall motion were markedly decreased and EF was less than 20% on echocardiogram. Despite of intra-aortic balloon pump (IABP) for 4 hours, shock and pulmonary edema was progressed. Mechanical circulatory support was started with left ventricular assist device (LVAD, Bio-pump, Medtronic Bio-Medicus, USA). At the time of operation, central venous pressure was 24cmH20, systolic blood pressure was 75mmHg, left atrium(LA) and LV was dilated and the whole wall of LV showed almost akinesia , and LA appendage was biopsied. After 126 hours of LVAD, LV wall motion was restored and EF was 79% on echocardiogram. LVAD was removed 10 days after operation and she was discharged on 23 days of hospitalization without any heart failure symptoms. Immunohistochemistry of LA showed enteroviral VP1 capsid protein (primary antibody; NoVo Castra Laboratory, UK) over the entire LA wall. Her serum neutralized coxsackievirus B3 (CVB3, H3 variant of Woodruff strain) in neutralization test using horse anti-CVB3 (Nancy strain) antibody (ATCC, V030-501-560) as a positive control. The titer of neutralization Ab in her serum of 21 days increased more than 4 times than that of 2 days.
Adolescent ; Blood Pressure ; Capsid Proteins ; Cardiomyopathies ; Central Venous Pressure ; Chest Pain ; Constriction, Pathologic ; Coronary Vessels ; Cough ; Creatinine ; Digoxin ; Electrocardiography ; Emergencies ; Emergency Service, Hospital ; Enterovirus ; Ergonovine ; Female ; Fever ; Furosemide ; Heart Failure ; Heart-Assist Devices* ; Hemodynamics ; Horses ; Hospitalization ; Humans ; Immunohistochemistry ; Myocarditis* ; Neutralization Tests ; Pharyngitis ; Prognosis ; Pulmonary Edema ; Shock ; Spasm ; Tachycardia, Sinus ; Thrombosis ; Troponin T