The nonprogressive unilateral intracranial arteriopathy known as transient (focal) cerebral arteriopathy is not a well-recognized arteriopathy among practitioners of Korea and Japan, although it cannot be easily differentiated from early moyamoya disease. This review summarizes the nomenclature, pathophysiology, diagnostic evaluation, clinico-radiological features, and management of nonprogressive (reversible or stable) unilateral arteriopathy based on the relevant literature and our own experiences. Nonprogressive unilateral arteriopathy should be strongly suspected in children presenting with basal ganglia infarction and arterial beading. The early identification of patients likely to have nonprogressive or progressive arteriopathy would ensure proper management and guide further research for secondary stroke prevention.
Basal Ganglia ; Child* ; Humans ; Infarction ; Japan ; Korea ; Moyamoya Disease ; Stroke*

A case of congenital tectal lipoma found incidentally at an autopsy is reported. This female fetus was a product of pregnancy termination at 24 weeks of gestational age after sonographic detection of hydrocephalus. Autopsy revealed a small mass in the periaqueductal portion. The mass was composed of adipose tissue, cartilage, and mature bony tissue with hematopoiesis. The resultant diagnosis was tectal lipoma with osteocartilagenous element. It is uncertain whether the lesion represents a teratoma or hamartoma or mesenchymal metaplasia. The osteocartilagenous component suggests the latter.
Adult ; Autopsy ; Bone and Bones/pathology ; Brain Neoplasms/*congenital/pathology ; Cartilage/pathology ; Case Report ; *Corpora Quadrigemina/pathology ; Female ; Human ; Infant, Newborn ; Lipoma/*congenital/pathology ; Pregnancy

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

Cryopyrin-associated periodic syndrome (CAPS) is a hereditary autoinflammatory syndrome caused by mutations in NLRP3 (encoding cryopyrin), which presents with fever, fatigue and arthralgia. Thus far, however there have been no reports of CAPS in Korea. Herein, we report 3 cases of CAPS for the first time in Korea. The first case, a 28-year-old man with recurrent urticaria, arthralgia and fever induced by cold, all of which were observed in his father, showed elevated erythrocyte sedimentation rate and C-reactive protein. He exhibited a p.Gly303Asp variant of the NLPR3 gene. The second case, a 2-year-old girl who had recurrent urticaria, arthritis and oral and genital ulcers, was positive for HLA B51 and a p.Glu569Lys mutation in exon 3 of the NLRP3 gene. Administration of anakinra greatly improved her symptoms. The third case, a 4-year-old boy who presented with recurrent urticaria, arthralgia, and fever, exhibited a p.Val72Met mutation in exon 1 of the NLRP3 gene. Administration of tocilizumab improved all of his symptoms. This small case series suggests that clinicians consider CAPS and conduct genetic studies when arthralgia and fever are accompanied by urticaria in Korea.
Adult ; Arthralgia ; Arthritis ; Blood Sedimentation ; C-Reactive Protein ; Child, Preschool ; Cryopyrin-Associated Periodic Syndromes ; Exons ; Fathers ; Fatigue ; Female ; Fever ; Humans ; Interleukin 1 Receptor Antagonist Protein ; Korea ; Male ; Ulcer ; Urticaria

OBJECTIVE: To evaluate the efficacy of clinical use of a intrauterine Sengstaken-Blakemore tube (S-B tube) in postpartum hemorrhage not controlled with medication and conservative treatment. METHODS: Retrospective review was done in 18 women with insertion of S-B tube into intrauterine cavity who did not controlled with medication and conservative treatment for postpartum hemorrhage. After third stage of labor, women were treated with prophylactic intramuscular Methylergonovine 0.2 mg and intravenous infusions of oxytocin routinely. If the postpartum bleeding continued, Misoprostol 800 microgram (per rectal) and intravenous infusions of Dinoprost 2 mg mixed with 5% D/S 500 cc were employed. And then women were examined for retained placenta and laceration of birth canal. Where necessary, retained placenta was removed and lacerations were sutured. If the postpartum bleeding did not controlled despite all of procedures, we decided use of intrauterine S-B tube. RESULTS: The mean age of the patients was 30.9+/-4.4 (27-39) years, their mean body weights and parities were 67.8+/-8.2 (56.2-85.7) kg, 1.8+/-0.8 (1-4) and mean gestational age was 38.5+/-2.9 (37-42) weeks respectively. The mean time from delivery to insertion of S-B tube was 107.6+/-94.0 (24-360) minutes. The mean filled normal saline amount was 190.5+/-35.2 (120-230) cc. Of 18 who was inserted of S B tube, 15 cases needed not additional surgical therapy. So success rate was 83.3%. CONCLUSION: Insertion of intrauterine S-B tube appears as a simple and effective means of treating postpartum hemorrhage not controlled with medication and conservative treatment.
Body Weight ; Dinoprost ; Female ; Gestational Age ; Hemorrhage ; Humans ; Infusions, Intravenous ; Lacerations ; Methylergonovine ; Misoprostol ; Oxytocin ; Parturition ; Placenta, Retained ; Postpartum Hemorrhage* ; Postpartum Period* ; Retrospective Studies

The coexistence of moyamoya disease (MMD) with an arteriovenous malformation (AVM) is exceedingly rare. We report two cases of AVM associated with MMD. The first case was an incidental AVM diagnosed simultaneously with MMD. This AVM was managed expectantly after encephalo-duro-arterio-synangiosis (EDAS) as the main feeders stemmed from the internal carotid artery, which we believed would be obliterated with the progression of MMD. However, the AVM persisted with replacement of the internal carotid artery feeders by new external carotid artery feeders from the EDAS site. The AVM was eventually treated with gamma knife radiosurgery considering an increasing steal effect. The second case was a de novo AVM case. The patient was initially diagnosed with MMD, and acquired an AVM eight years later that was slowly fed by the reconstituted anterior cerebral artery. Because the patient remained asymptomatic, the AVM is currently being closely followed for more than 2 years without further surgical intervention. Possible differences in the pathogenesis and the radiologic presentation of these AVMs are discussed with a literature review. No solid consensus exists on the optimal treatment of MMD-associated AVMs. Gamma knife radiosurgery appears to be an effective treatment option for an incidental AVM. However, a de novo AVM may be managed expectantly considering the possible risks of damaging established collaterals, low flow characteristics, and probably low risks of rupture.
Anterior Cerebral Artery ; Arteriovenous Malformations ; Carotid Artery, External ; Carotid Artery, Internal ; Child ; Consensus ; Humans ; Intracranial Arteriovenous Malformations* ; Moyamoya Disease* ; Radiosurgery ; Rupture

Abdominal lymphangiomas are uncommon angiomatous tumor occurring mainly in childhood. This is a retrospective clinicopathologic study of 17 cases of abdominal lymphangioma. The patients included are five children and 12 adults, with a mean age at initial presentation of 30.7 years (age ranges 3-63). The locations of the tumors were mesentery (5), retroperitoneum (4), colon (3), omentum (3), mesocolon (1) and gallbladder (1). Infiltrative growth was more common pattern than entirely circumscribed pattern. Masses were mostly multilocular cysts and contained chyle or serous fluid. On immunohistochemical staining, 16 cases were reactive for either CD31 or factor VIII-related antigen. These fact would suggest that intra-abdominal lymphangiomas simulate the immunohistochemical features of collecting lymphatics. Follow up was possible in 12 cases for 3-50 months (mean 19 months) and only one patient showed local recurrence. Although abdominal lymphangiomas are rare in adulthood and correct preoperative diagnosis is difficult, awareness of such a possibility in adulthood will contribute to make a correct preoperative diagnosis.
Abdominal Neoplasms/physiopathology ; Abdominal Neoplasms/pathology* ; Abdominal Neoplasms/metabolism ; Adult ; Antigens, CD31/biosynthesis ; Child ; Child, Preschool ; Factor VIII/biosynthesis ; Female ; Human ; Lymphangioma/physiopathology ; Lymphangioma/pathology* ; Lymphangioma/metabolism ; Male ; Middle Age ; Retrospective Studies

This retrospective study describes and evaluates the effectiveness of a modified technique of conventional endoscopic dacryocystorhinostomy (DCR) that minimizes the obstruction of a neo-ostium by creating an enlarged marsupialized lacrimal sac using mucosal flaps. Forty-two patients who had undergone 46 endoscopic DCR at a tertiary medical center, from 2002 to 2004, for correction of lacrimal system obstruction were investigated. The surgical technique involves elevation of a nasal mucosal flap, full sac exposure using a power drill, and shaping of the mucosal flap to cover denuded bone and juxtapose exposed sac mucosa. Postoperative symptoms and endoscopic findings of the neo-ostium were evaluated. Mean duration of follow-up was 5.9 months. An eighty-three percent primary success rate was observed, without any serious complications. Obstruction of the neo-ostium with granulation tissue was observed in eight cases, among which six underwent revision with success in all cases. Overall, 44 (96%) of 46 cases experienced surgical successes. Endoscopic DCR, a procedure in which a large marsupialized lacrimal sac is created from mucosal flaps, yields a very satisfactory success rate with straightforward and highly successful revision available for those in whom the primary procedure yields a substandard result.
Treatment Outcome ; Surgical Flaps ; Nasal Mucosa/surgery ; Middle Aged ; Male ; Lacrimal Duct Obstruction/*surgery ; Lacrimal Apparatus/pathology/surgery ; Humans ; Follow-Up Studies ; Female ; Endoscopy/*methods ; Dacryocystorhinostomy/*methods ; Child ; Aged ; Adult ; Adolescent

OBJECTIVES: Previous studies have suggested that some personality characteristics are associated with the onset, prognosis and social function in schizophrenia patients. However few is known about the personality and affective characteristic of genetic high risk group (GHR) for schizophrenia. This study aimed to investigate the personality and the affective characteristic of GHR group for schizophrenia. METHODS: Participants were 54 healthy controls (HC), 26 subjects with GHR for schizophrenia and 28 subjects with first episode psychosis (FEP). We performed three self-report questionnaires; NEO-Personality Inventory-Revised, State and Anger Expression Inventory and Positive Affect and Negative Affect Schedule. RESULTS: The GHR group showed higher score in agreeableness item than the HC (p=0.028). In extraversion item, the FEP group showed significantly lower scores than the HC (p=0.001). The GHR group showed lower scores in neuroticism item compared with FEP group in trend level. The FEP group showed higher trait-anger, lower positive affect and higher negative affect compared with the others. CONCLUSION: The GHR group seem to share certain vulnerable personality and affective characteristics for schizophrenia with the FEP group. On the other hands, the GHR group appeared to be more agreeable than the other groups, which might act as the compensation for other impaired functions.
Anger ; Anxiety Disorders ; Compensation and Redress ; Extraversion (Psychology) ; Hand ; Humans ; Polytetrafluoroethylene ; Prognosis ; Psychotic Disorders ; Schizophrenia