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

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

OBJECTIVE: The purpose of this study was to investigate the clinical features and outcomes of pediatric cavernous malformation (CM) in the central nervous system. METHODS: Twenty-nine pediatric patients with supratentorial CM underwent microsurgical excision. In selected cases, transparent tubular retractor system (TTRS) was used to reduce retraction injury and intraoperative neuromonitoring (IONM) was held to preserve functioning cortex. Patients' demographics and symptoms were reviewed and surgical outcomes were discussed. RESULTS: The main initial clinical manifestations included the following : seizures (n=13, 45%), headache (n=7, 24%), focal neurological deficits (n=3, 10%), and an incidental finding (n=6, 21%). Overt hemorrhage was detected in 7 patients (24%). There were 19 children (66%) with a single CM and 10 (34%) children with multiple CMs. In 7 cases with deep-seated CM, we used a TTRS to minimize retraction. In 9 cases which location of CM was at eloquent area, IONM was taken during surgery. There was no major morbidity or mortality after surgery. In the 29 operated children, the overall long-term results were satisfactory : 25 (86%) patients had no signs or symptoms associated with CMs, 3 had controllable seizures, and 1 had mild weakness. CONCLUSION: With the assistance of neuronavigation systems, intraoperative neuromonitoring, and TTRS, CMs could be targeted more accurately and excised more safely. Based on the satisfactory seizure outcome achieved, complete microsurgical excision in children is recommended for CMs presenting with seizures but removal of hemosiderin-stained areas seems to be unnecessary.
Central Nervous System ; Child ; Demography ; Headache ; Hemangioma, Cavernous, Central Nervous System* ; Hemorrhage ; Humans ; Incidental Findings ; Mortality ; Neuronavigation ; Seizures

We present an unusual case of peritoneal catheter migration following a ventriculoperitoneal shunt operation. A 7-month-old infant, who had suffered from intraventricular hemorrhage at birth, was shunted for progressive hydrocephalus. The peritoneal catheter, connected to an 'ultra small, low pressure valve system' (Strata(R); PS Medical,Gola, CA, USA) at the subgaleal space, was placed into the peritoneal cavity about 30 cm in length. The patient returned to our hospital due to scalp swelling 21 days after the surgery. Simple X-ray images revealed total upward migration and coiling of the peritoneal catheter around the valve. Possible mechanisms leading to proximal upward migration of a peritoneal catheter are discussed.
Catheters ; Hemorrhage ; Humans ; Hydrocephalus ; Infant ; Parturition ; Peritoneal Cavity ; Scalp ; Ventriculoperitoneal Shunt

No abstract available.
Aged* ; Humans