Purpose: To investigate the long-term clinical course and prognostic factors of branch retinal artery occlusion (BRAO). Methods: The medical records of patients diagnosed with BRAO were reviewed retrospectively. Visual acuity (VA) and central retinal thickness (CRT) at diagnosis were compared with those measured at the final visit. Patients with a decimal VA ≥ 0.6 (good prognosis group) were compared with those with a decimal VA ≤ 0.5 (poor prognosis group) at the final visit. Results: Fifty-five patients were enrolled and the mean follow-up period was 45.8 ± 27.8 months. The mean logarithm of minimum angle of resolution improved from 0.53 ± 0.57 at diagnosis to 0.36 ± 0.61 at the final visit (p = 0.026). The decimal VA was ≤ 0.1 in 13 (23.6%) patients, ≥ 0.2 and ≤ 0.5 in 16 (29.1%) patients, and ≥ 0.6 in 26 (47.3%) patients at diagnosis; the respective values were 9 (16.4%), 8 (14.5%), and 38 (69.1%) at the final visit. The mean CRT significantly decreased from 273.9 ± 34.7 µm at diagnosis to 248.9 ± 27.0 µm at the final visit (p < 0.001). The poor prognosis group (n = 17) was older (p = 0.044) and had a higher incidence of papillomacular bundle involvement (p < 0.001) than the good prognosis group (n = 38). Conclusions Patients with BRAO generally showed relatively favorable long-term outcomes. However, the final VA was ≤ 0.1 in 16.4% of them, suggesting the need for further treatment modalities to improve the outcome of patients with a poor prognosis.

The corpus callosum consists of white fibers connecting the cerebral hemispheres. Agenesis of the corpus callosum is an uncommon congenital anomaly which is easily diagnosed in the postnatal period by ultrasound and computed tomographic scan or MRI, but its prenatal sonographic diagnosis is difficult because of fetal head positioning and limiting trans-axial scans. We experienced a case of agenesis of the corpus callusum with chromosomal anomaly. The prenatal sonographic findings are ventricular abnormalities that demonstrated dilatation of lateral ventricles and disproportionate enlargement of the occipital horns, which were suggestive findings for the corpus callosal agenesis. We could confim the diagnosis of the corpus callosal agenesis with chromosome anomaly by postnatal MRI and chromosome analysis.
Agenesis of Corpus Callosum* ; Animals ; Cerebrum ; Corpus Callosum ; Diagnosis ; Dilatation ; Head ; Horns ; Lateral Ventricles ; Magnetic Resonance Imaging ; Ultrasonography

Background: and Purpose There is extensive literature on monogenic epilepsies caused by mutations in familiar channelopathy genes such as SCN1A. However, information on other lesscommon channelopathy genes is scarce. This study aimed to explore the genetic and clinical characteristics of patients diagnosed with unusual voltage-gated sodium and potassium channelopathies related to epilepsy. Methods: This observational, retrospective study analyzed pediatric patients with epilepsy who carried pathogenic variants of unusual voltage-gated sodium and potassium channelopathy genes responsible for seizure-associated phenotypes. Targeted next-generation sequencing (NGS) panel tests were performed between November 2016 and June 2022 at Severance Children’s Hospital, Seoul, South Korea. Clinical characteristics and the treatment responses to different types of antiseizure medications were further analyzed according to different types of gene mutation. Results: This study included 15 patients with the following unusual voltage-gated sodium and potassium channelopathy genes: SCN3A (n=1), SCN4A (n=1), KCNA1 (n=1), KCNA2 (n=4), KCNB1 (n=6), KCNC1 (n=1), and KCNMA1 (n=1). NGS-based genetic testing identified 13 missense mutations (87%), 1 splice-site variant (7%), and 1 copy-number variant (7%). Developmental and epileptic encephalopathy was diagnosed in nine (60%) patients. Seizure freedom was eventually achieved in eight (53%) patients, whereas seizures persisted in seven (47%) patients. Conclusions Our findings broaden the genotypic and phenotypic spectra of less-common voltage-gated sodium and potassium channelopathies associated with epilepsy.

OBJECTIVE: The purpose of this study was to determine whether funisitis is associated with changes in the umbilical cord plasma concentration of interleukin-6 (IL-6) and matrix metalloproteinase-8 (MMP-8), microbial invasion of the amniotic cavity and neonatal outcome. METHODS: The relationship among the presence of funisitis, IL-6 and MMP-8 concentrations in umbilical cord plasma at birth, the results of amniotic fluid culture performed within 5 days of birth was examined in 83 consecutive singleton births (20-35 weeks' gestation). Funisitis was diagnosed in the presence of neutrophil infiltration into the umbilical vessel wall or Wharton's jelly. The IL-6 and MMP-8 concentration was measured with a specific immunoassay. Amniocentesis was performed in 47 patients within 5 days of birth. RESULTS: (1) Funisitis was present in 21.7% of patients. (2) Patients with funisitis had a significant higher cord plasma IL-6 concentration, but had no significant difference in cord plasma MMP-8 concentration. (3) Clinical chorioamnionitis was more common in patients with funisitis than those without funisitis. (4) A cord plasma IL-6 > 6.34 pg/ml had a sensitivity of 77.8% and a specificity of 75.4% in the identification of funisitis. (5) No correlation between cord blood plasma IL-6 concentration and MMP-8 concentration was found. (6) There was no significant correlation between gestational age at birth and cord blood plasma MMP-8 concentrations, but there appeared to be a trend to increase of cord plasma MMP-8 concentrations as gestational ages at birth were increased. (7) Neonates with congenital sepsis had a significantly higher cord plasma IL-6 and MMP-8 concentration than those without congenital sepsis. CONCLUSION: In patient with funisitis, umbilical cord plasma IL-6 concentrations were higher than those without funisitis, but umbilical cord plasma MMP-8 concentrations had no significant difference in each group. The umbilical cord plasma IL-6 and MMP-8 can be useful as a predictor of the occurrence of congenital sepsis in preterm infant.
Amniocentesis ; Amniotic Fluid* ; Chorioamnionitis* ; Female ; Fetal Blood ; Gestational Age ; Humans ; Immunoassay ; Infant, Newborn ; Infant, Premature ; Interleukin-6* ; Matrix Metalloproteinase 8* ; Neutrophil Infiltration ; Parturition ; Plasma* ; Pregnancy ; Sensitivity and Specificity ; Sepsis ; Umbilical Cord* ; Wharton Jelly

PURPOSE: Retinal blood vessels and cerebral small vessels possess similar characteristics anatomically, physiologically and embryologically. We studied the availability of abnormal fundus findings of patients who had an atherothrombotic ischemic stroke and who have the risk factors. METHODS: Fundus photographs and brain images were taken in patients who had a first-ever symptomatic ischemic stroke of large artery atherosclerosis (LAA) or small vessel occlusion (SVO) from March 2004 to February 2005. We analyzed the association between fundus abnormalities and ischemic stroke subtypes. RESULTS: Based on brain MRI and MRA, a total of 47 patients were classified into SVO and LAA groups. The SVO group consisted of 27 patients (mean age: 69.7 years), and the LAA group consisted of 20 patients (mean age: 65.4 years). The control group comprised 15 patients (mean age: 64.9 years). The baseline characteristics were similar among the three groups. The severity of the retinal arteriolar narrowing and sclerosis were associated with hypertension. Compared to the control group, both the SVO and LAA groups showed more severe arteriolar sclerosis, the SVO group showed more severe arteriolar narrowing and the LAA group showed more frequent AV crossing and retinal exudate. CONCLUSIONS: Retinal arteriolar abnormalities such as arteriolar narrowing and sclerosis are more severe in atherothrombotic ischemic stroke patients. Indirectly, retinal microvascular changes may indicate the status of the cerebral vasculature. Thus, analysis of fundus findings is useful for predicting an atherothrombotic ischemic stroke and planning follow-up examinations.
Arteries ; Arterioles* ; Atherosclerosis ; Brain ; Exudates and Transudates ; Follow-Up Studies ; Humans ; Hypertension ; Magnetic Resonance Imaging ; Retinal Vessels ; Retinaldehyde* ; Risk Factors ; Sclerosis ; Stroke*

BACKGROUND: Milnacipran is a balanced serotonin norepinephrine reuptake inhibitor with minimal side effects and broad safety margin. It acts primarily on the descending inhibitory pain pathway in brain and spinal cord. In many animal studies, intrathecal administration of milnacipran is effective in neuropathic pain management. However, there is no study for the neurological safety of milnacipran when it is administered neuraxially. This study examined the neurotoxicity of epidural milnacipran by observing behavioral and sensory-motor changes with histopathological examinations of spinal cords in rats. METHODS: Sixty rats were divided into 3 groups, with each group receiving epidural administration of either 0.3 ml (3 mg) of milnacipran (group M, n = 20), 0.3 ml of 40% alcohol (group A, n = 20), or 0.3 ml of normal saline (group S, n = 20). RESULTS: There were no abnormal changes in the behavioral, sensory-motor, or histopathological findings in all rats of groups M and S over a 3-week observation period, whereas all rats in group A had abnormal changes. CONCLUSIONS: Based on these findings, the direct epidural administration of milnacipran in rats did not present any evidence of neurotoxicity in behavioral, sensory-motor and histopathological evaluations.
Animals ; Brain ; Cyclopropanes ; Injections, Epidural ; Neuralgia ; Norepinephrine ; Rats ; Serotonin ; Spinal Cord

BACKGROUND AND OBJECTIVES: It has been demonstrated that sleep apnea syndrome predisposes to cardiac rhythm disturbances and cardiovascular risks such as systemic hypertension. This study was conducted to investigate the types and frequency of cardiac arrhythmias which occurred during sleep and the effects of nasal continuous positive airway pressure (nCPAP) therapy in the patients with sleep apnea syndrome. SUBJECTS AND METHODS: The subjects were 197 patients who were referred to the Sleep Research Center of Korea University Medical Center for polysomnography due to snoring and sleep apnea from Jan. 1st 2000 to July 31st 2002. Of the 197 patients, 44 with severe sleep apnea syndrome, whose respiratory disturbance index (RDI) exceeded 40/hr, were enrolled. Their electrograms on polysomnography before and after nCPAP therapy were analyzed. RESULTS: Of the 44 subjects, 32 (72.8%) showed cardiac arrhythmias. The types of arrhythmias were atrial premature beats (APBs, n=17), premature ventricular complexes (PVCs, n=15), sinus bradycardia (heart rate less than 40 per minute, n=6), sinus pause (n=1), and sinoatrial block (n=5). No fatal arrhythmias were identified. Most, 93.2%, of these arrhythmias arose immediately after hypopneic or apneic episodes, and were accompanied by a significant decrease in SaO2, from 91.4% to 84.7% (p<0.05). After nCPAP therapy, these arrhythmias were completely disappeared in 11 patients (34.4%) and diminished in 15 (46.9%). Hypopneic or apneic episodes were preceded by cardiac arrhythmias in only 36.4% with nCPAP (p<0.05 vs. before). CONCLUSION: Cardiac arrhythmias were demonstrated in 72.8% of cases of severe sleep apnea syndrome, which were mostly benign and preceded by hypopneic or apneic episodes. nCPAP therapy decreased the frequency of hypopnea and apnea with elevated arterial O2 saturation, and effectively eliminated cardiac arrhythmias.
Academic Medical Centers ; Apnea ; Arrhythmias, Cardiac ; Bradycardia ; Cardiac Complexes, Premature ; Continuous Positive Airway Pressure ; Humans ; Hypertension ; Korea ; Polysomnography ; Positive-Pressure Respiration ; Sinoatrial Block ; Sleep Apnea Syndromes* ; Snoring ; Ventricular Premature Complexes

BACKGROUND: Ischemic stroke occurring during sleep is still an unexplored area of cerebrovascular event. As the exact onset time of stroke while sleeping (SWS) cannot be determined, these patients are generally excluded from the thrombolytic therapy of acute ischemic stroke. The aim of this study was to know whether differences in clinical features exist between patients suffering a SWS and those with stroke while awake (SWA). METHODS: We reviewed the medical records of acute ischemic stroke patients consecutively registered in Hallym Stroke Databank between January 1999 and June 2007. We compared the risk factors and clinical features between the SWS and SWA groups. RESULTS: A total of 2,962 patients were included in the study, of which 821 (27.7%) were SWS. No differences between SWS and SWA were identified with regard to baseline clinical characteristics and risk factors except a history of smoking. In stroke subtype, small vessel occlusions were more frequently in SWS group than SWA group. Intravenous rt-PA treatments were performed frequently in the SWA group. Clinical outcomes at discharge were better in SWA group than SWS group. CONCLUSION: This study suggest that no major differences were exist in clinical characteristics between SWS and SWA patients, except the history of smoking. Clinical outcomes of patients with ischemic stroke within 6 hours after stroke onset were poor in SWS group. In SWS group, relatively little chances of thrombolysis might be the explanation of these finding.
Glycosaminoglycans ; Humans ; Medical Records ; Risk Factors ; Smoke ; Smoking ; Stress, Psychological ; Stroke ; Thrombolytic Therapy

Percutaneous needle aspiration biopsy is a relatively simple and safe procedure for the diagnosis of lung and mediastinal lesions. Systemic air embolism during and after percutaneous needle aspiration biopsy of the lung is very rare; however, it is still a complication that can cause fatal outcomes, such as cerebral infarction and myocardial infarction. Here, we report a 72-year-old woman who suffered a change in consciousness immediately after receiving a percutaneous needle aspiration biopsy for the pathologic examination of pulmonary nodules found during a routine health medical examination. She had left side weakness and ST segment elevation on an electrocardiogram. After a high concentration of oxygen, she recovered from neurological symptoms and electrocardiographic abnormalities. The authors report a case of air embolism occurring simultaneously in the brain and coronary arteries after percutaneous needle aspiration biopsy.
Aged ; Biopsy ; Biopsy, Needle* ; Brain ; Cerebral Infarction ; Consciousness ; Coronary Vessels ; Diagnosis ; Electrocardiography ; Embolism ; Embolism, Air* ; Fatal Outcome ; Female ; Humans ; Intracranial Embolism ; Lung* ; Myocardial Infarction ; Needles* ; Oxygen

A 68-year old man diagnosed with Middle East Respiratory Syndrome-Coronavirus (MERS-CoV) presented with multiple pneumonic infiltrations on his chest X-ray, and the patient was placed on a mechanical ventilator because of progressive respiratory failure. Urinary protein excretion steadily increased for a microalbumin to creatinine ratio of 538.4 mg/g Cr and a protein to creatinine ratio of 3,025.8 mg/g Cr. The isotope dilution mass spectrometry traceable serum creatinine level increased to 3.0 mg/dL. We performed a kidney biopsy 8 weeks after the onset of symptoms. Acute tubular necrosis was the main finding, and proteinaceous cast formation and acute tubulointerstitial nephritis were found. There were no electron dense deposits observed with electron microscopy. We could not verify the virus itself by in situ hybridization and confocal microscopy (MERS-CoV co-stained with dipeptidyl peptidase 4). The viremic status, urinary virus excretion, and timely kidney biopsy results should be investigated with thorough precautions to reveal the direct effects of MERS-CoV with respect to renal complications.
Aged ; Biopsy ; Coronavirus Infections/*diagnosis/virology ; Creatinine/blood/urine ; Dipeptidyl Peptidase 4/metabolism ; Humans ; In Situ Hybridization, Fluorescence ; Kidney/metabolism/*pathology ; Male ; Microscopy, Confocal ; Microscopy, Electron ; Middle East Respiratory Syndrome Coronavirus/*genetics/isolation & purification ; RNA, Viral/genetics/metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Serum Albumin/analysis