Spontaneous coronary artery dissection is a rarely identified entity whose exact incidence, etiology, pathogenesis, medium-term evolution and optimal treatment have not yet been firmly estabilished. The cause of spontaneous disection remains unclear but theories of etiology include a medial eosinophilic angiitis, pregnancy induced degeneration of collagen and rupture of the vasovasoum. Most paients die suddenly, but a clinical spectrum is seen including and unstable angina, myocardial infarction and cardiogenic shock. We experienced 4 cases with spontaneous coronary artery dissection found angiographically which caused myocardial infarction and unstable angina. Our patients were treated medically.
Angina, Unstable ; Collagen ; Coronary Vessels ; Eosinophils ; Humans ; Incidence ; Myocardial Infarction ; Myocardial Ischemia* ; Pregnancy ; Rupture ; Shock, Cardiogenic ; Vasculitis

Idiopathic restrictive cardiomyopathy is characterized by clinical and hemodynamic findings of restrictive cardiomyopathy in the absence of morphologic cause. The differential diagnosis between idiopathic restrictive cardiomyopathy and noncalcified constrictive pericarditis is difficult but the distinction is crucial because of the therapeutic implication. The diagnosis of idiopathic restrictive cardiomyopathy is aided by echocardiography and cardiac catheterization, cardiac magnetic resonance imaging. The patients should be approached systemically to differentiate from noncalcified constritive pericarditis.
Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Diagnosis ; Diagnosis, Differential ; Echocardiography ; Hemodynamics ; Humans ; Magnetic Resonance Imaging ; Pericarditis ; Pericarditis, Constrictive

BACKGROUND AND OBJECTIVES: Congenital nystagmus (CN) is an ocular oscillation that usually manifests during early infancy. To report a novel mutation in FERM domain containing 7 (FRMD7) gene in a Korean family with CN. MATERIALS AND METHODS:Genomic DNA was prepared from peripheral blood leukocytes and direct sequencing of the entire coding and adjacent intronic regions was performed to detect sequence variation of FRMD7 gene, where mutations were found recently in patients with familial CN. The family showed an X-linked pattern of inheritance without father-to-son transmission. RESULTS: Three family members with CN exhibited two sequence variations which were a novel mutation (c. 875T>C; Leu292Pro) and a polymorphism (c. 1403G>A; Arg468His, dbSNP rs#6637934). The proband was hemizygous for both variations and his mother and maternal grandmother were heterozygous carriers. CONCLUSION: This study provides an additional evidence for mutations in FRMD7 as a common cause of X-linked CN and expands its mutation spectrum.
Clinical Coding ; DNA ; Humans ; Introns ; Leukocytes ; Mothers ; Nystagmus, Congenital* ; Wills

Secondary intracranial hypertension (IH) is a syndrome with various etiologies, including a brain tumor, head trauma, hypoparathyroidism, hydrocephalus, meningitis, drug use, or venous obstruction. It is reasonably straightforward to diagnose secondary IH induced by these diseases. However, diagnosing secondary IH induced by unpredictable extracranial lesion is difficult, and is based largely on subtle neurological symptoms or signs and usually can be achieved by an extracranial evaluation. Here we describe four cases of secondary IH with unusual causes.
Brain Neoplasms ; Craniocerebral Trauma ; Headache ; Hydrocephalus ; Hypoparathyroidism ; Intracranial Hypertension ; Meningitis ; Spinal Cord Neoplasms

A 52-year-old woman presented with atypical chest pain. Coronary angiography demonstrated multiple microfistulae between left coronary artery and left ventricle (LV) cavity, extensive enough to produce an LV angiogram. This LV angiogram revealed apical hypertrophic cardiomyopathy (HCM) which was confirmed by echocardiography. Coronary steal phenomenon by coronary artery microfistulae and HCM might have a role for developing of angina in patient with apical HCM.
Cardiomyopathy, Hypertrophic* ; Chest Pain ; Coronary Angiography ; Coronary Vessels ; Echocardiography ; Female ; Fistula ; Heart Ventricles ; Humans ; Middle Aged ; Veins*

No abstract available.
Area Postrema ; Neuromyelitis Optica ; Vomiting

ST-elevation myocardial infarction (STEMI) caused by an acute aortic dissection is relatively rare. A diagnosis of dissection can be missed and the situation can become complicated. We report a patient who presented with acute aortic dissection responsible for STEMI related to a dissecting flap into the right coronary artery. This case emphasizes the need for careful assessment of the aorta in cases of atypical coronary occlusion in patients with STEMI without evidence of atherosclerosis in non-culprit coronary segments. The patient was discharged 7 days after primary percutaneous intervention for STEMI. However, she revisited the emergency department for recurrent chest pain and aortic dissection and was diagnosed and managed successfully with surgery.
Aorta ; Atherosclerosis ; Chest Pain ; Coronary Occlusion* ; Coronary Vessels ; Diagnosis ; Emergencies ; General Surgery ; Humans ; Masks* ; Myocardial Infarction* ; Myocardial Revascularization

ST-elevation myocardial infarction (STEMI) caused by an acute aortic dissection is relatively rare. A diagnosis of dissection can be missed and the situation can become complicated. We report a patient who presented with acute aortic dissection responsible for STEMI related to a dissecting flap into the right coronary artery. This case emphasizes the need for careful assessment of the aorta in cases of atypical coronary occlusion in patients with STEMI without evidence of atherosclerosis in non-culprit coronary segments. The patient was discharged 7 days after primary percutaneous intervention for STEMI. However, she revisited the emergency department for recurrent chest pain and aortic dissection and was diagnosed and managed successfully with surgery.
Aorta ; Atherosclerosis ; Chest Pain ; Coronary Occlusion* ; Coronary Vessels ; Diagnosis ; Emergencies ; General Surgery ; Humans ; Masks* ; Myocardial Infarction* ; Myocardial Revascularization

We report a case of recurrent Streptococcus Pneumoniae meningoencephalitis with a transethmoidal meningoencephalocele (TEME) but without cerebrospinal fluid (CSF) leakage. A 35-year-old man was admitted with S. pneumoniae meningitis. He had suffered from four episodes of recurrent pneumococcal meningitis during the previous 4 years. A computed tomography scan of the paranasal sinus showed the TEME protruding through a bony defect of the right frontal base. However, the patient did not have symptoms that could be attributable to CSF leakage, and radioisotope cisternography did not identify a leak. Brain magnetic resonance imaging revealed cortical lesions overlying the TEME, and electroencephalography revealed epileptiform discharges in frontal regions. Appropriate antibiotics therapy without steroids was given to improve his condition. The presented case suggests that even in the absence of clinically demonstrable CSF leakage, an occult skullbase defect and its associated meningoencephalocele should be considered in patients with recurrent bacterial meningitis.
Adult ; Anti-Bacterial Agents ; Brain ; Electroencephalography ; Humans ; Magnetic Resonance Imaging ; Meningitis ; Meningitis, Bacterial ; Meningitis, Pneumococcal ; Meningoencephalitis ; Pneumonia ; Steroids ; Streptococcus ; Streptococcus pneumoniae

Background: Many clinicians have experienced the difficulty of decision on termination of antituberculosis chemotherapy after the 6th month due to relapse of disease. There is still controversy in the effect of 2S(K)HRZ/4HRZ 6-month short course chemotherapy including pyrazinamide for 6 months in patients with pulmonary tuberculosis. And there is no long term follow-up study of 6-month short course chemotherapy for pulmonary tuberculosis in korea. So we had performed the study to find the result of 6-month antituberculosis chemotherapy for 4 years. Method: We studied prospectively the effect of 2S(K)HRZ/4HRZ in one hundred-fifty patients with pulmonary tuberculosis and followed up fifty-nine patients for more than 1 year to 4 years after the completion of 6-month short course therapy. Results: 1) Out of one hundred-fifty patients, seventy-two patients(48%) completed the prescribed 6-month chemotherapy. Sixty-eight patients(45.3%) have experienced premature discontinuation and the most common cause of premature discontinuation was drop-out against advice(thirty-six patients, 24%). Ten patients(6.7%) were treated beyond the 6 months mainly due to irregular treatment. 2) Fifty-nine patients(81.9%) among seventy-two patients with completed treatment have been followed up for more than 1 year and 32 patients(44.4%) for more than 4 years. There was three relapse patients of whom two patients have experienced relapse of pulmonary tuberculosis within 1 year after the termination of chemotherapy. 3) Among one hundred-thirty-four patients who have been assessible for more than two months of chemotherapy, including the patients who experienced within 2 months, there were eighty-two patients(61.2%) who have experienced adverse reactions and the treament regimen was changed only in thirteen patients(9.7%). The most frequent cause of adverse reactions was arthralgia and/or hyperuricemia, which had occurred in 33 patients(24.6%). Conclusion: In a university hospital in Korea, 6-month short course chemotherapy of 2S(K)HRZ/4HRZ had unnegligible relapses and premature discontinuation. Therefore, change of the regimen might be carefully considered by drug susceptibility results. Close monitoring of patients, retrial of sputum exam and radiologic evaluation during treatment might be required in the endemic area of drug resistant strains like in Korea. Further study about the effect of 6-month short course chemotherapy including pyrazinamide for 6-month might be needed.
Arthralgia ; Drug Therapy ; Follow-Up Studies* ; Humans ; Hyperuricemia ; Korea ; Prospective Studies ; Pyrazinamide ; Recurrence ; Sputum ; Tuberculosis, Pulmonary*