BACKGROUND: Severe bradycardia occurring in strabismus surgery is unpredictable and may lead to cardiac arrest. If we identify patients who have risk of severe bradycardia during strabismus surgery, it is helpful to provide a more meticulous anesthesia. In this study, we investigated the correlation between the degree of bradycardia and indices of power spectral analysis and nonlinear dynamic data analysis of preoperative ECG. METHODS: ECG was collected for twenty minutes from strabismus patients (n = 93). No premedicants were administered. After administration of anesthesia and traction of extraocular muscle, the lowest heart rate was recorded. We calculated the correlation coefficient between the power spectral density, approximate entropy and correlation dimension of preoperative ECG and the difference between preoperative and lowest HR. RESULTS: As the difference between the preoperative heart rate and the intraoperative bradycardia increased, the preoperative ECG showed a low total power (r = 0.27, P = 0.016), low low-frequency power (r = 0.31, P = 0.049), low high-frequency power (r = 0.30, P = 0.007) and high ratio of low to high-frequency power (r = 28, P = 0.03). There was no correlation between bradycardia and approximate entropy and correlation dimension, respectively. CONCLUSIONS: There was a correlation between indices of power spectral analysis of preoperative ECG and degree of bradycardia during strabismus surgery. Therefore we take into consideration preoperative ECG and its analytic indices in order to provide careful preparation for high risk patients who exhibit a tendency to severe bradycardia.
Anesthesia ; Bradycardia* ; Electrocardiography* ; Entropy ; Heart Arrest ; Heart Rate ; Humans ; Nonlinear Dynamics ; Reflex, Oculocardiac ; Statistics as Topic ; Strabismus* ; Traction

The posterior reversible encephalopathy syndrome(PRES) is characterized clinically by a combination of acute or subacute confusion, lethargy, visual disturbance, and seizures. PRES has been described in various clinical settings, including severe hypertension, chemotherapy, eclampsia, and seizure. We report a case of a 7-year-old girl who had taken cyclosporine for steroid resistant nephrotic syndrome. Twenty one days after the cyclosporine therapy, she was admitted due to generalized tonic clonic seizure and headache. Her blood pressure was 170/90 mmHg. Magnetic resonance(MR) imaging showed necrotic/cystic lesions involving the bilateral parieto-occipital region. After discontinuation of cyclosporine, and control of blood pressure, she had no more seizure and headache. The follow-up MR examination which was performed 6 months later showed the decreased extent of the lesion.
Blood Pressure ; Child* ; Cyclosporine* ; Drug Therapy ; Eclampsia ; Female ; Follow-Up Studies ; Headache ; Humans ; Hypertension ; Lethargy ; Nephrotic Syndrome* ; Posterior Leukoencephalopathy Syndrome* ; Pregnancy ; Seizures

PURPOSE: Systemic lupus erythromatosus (SLE) is an autoimmune disease which involves multiple organs, including the central nervous system. Neuropsychiatric (NP) involvement is one of the major causes of morbidity and mortality in children with SLE. The aim of this study was to evaluate neuropsychiatric manifestations and to find the clinical factors associated with the development of NP manifestation in children with SLE (NPSLE). METHODS: We retrospectively reviewed the medical records of 51 patients diagnosed with SLE at Asan Medical Center Children's hospital between April 1998 and January 2011. Clinical factors between patients with NP manifestations and patients without NP manifestations were compared. RESULTS: NPSLE developed in 41.2% (21/51) of the patients with SLE. The mean age of onset was 13.0 (8-19) years. Twelve patients (57.1%) developed NP manifestations within 1 month after the diagnosis of SLE. NP manifestations included seizures (47.6%), headaches (42.9%), movement disorders (9.5%), peripheral neuropathy/AIDP (9.5%), stroke/TIA (9.5%), psychosis (4.8%). As comparing clinical factors, hematological profile and immunologic profile between patients with NP manifestations and patients without NP manifestations, there were no significant differences between the two groups. CONCLUSION: NP symptoms are common in children with SLE, especially within 1 month after the diagnosis of SLE. However, there were no clinical factors or laboratory profiles that may predict the development of NPSLE. Therefore, careful monitoring of NP manifestations may be important in children with newly-diagnosed SLE and prompt immunomodulatory treatment should be considered.
Age of Onset ; Autoimmune Diseases ; Autoimmune Diseases of the Nervous System ; Central Nervous System ; Child ; Headache ; Humans ; Immunomodulation ; Lupus Erythematosus, Systemic ; Medical Records ; Movement Disorders ; Psychotic Disorders ; Retrospective Studies ; Seizures

We aimed to analyze characteristics of encephalopathy after both hematopoietic stem cell and solid organ pediatric transplantation. We retrospectively reviewed medical records of 662 pediatric transplant recipients (201 with liver transplantation [LT], 55 with heart transplantation [HT], and 67 with kidney transplantation [KT], 339 with allogeneic hematopoietic stem cell transplantation [HSCT]) who received their graft organs at Asan Medical Center between January 2000 and July 2014. Of the 662 patients, 50 (7.6%) experienced encephalopathy after transplantation. The incidence of encephalopathy was significantly different according to the type of organ transplant: LT, 16/201 (8.0%), HT, 13/55 (23.6%), KT, 5/67 (7.5%), and HSCT, 16/339 (4.7%) (P < 0.001). Drug-induced encephalopathy (n = 14) was the most common encephalopathy for all transplant types, but particularly after HSCT. Hypertensive encephalopathy was the most common after KT and HT, whereas metabolic encephalopathy was the most common after LT. The median time to encephalopathy onset also differed according to the transplant type: 5 days after KT (range 0–491 days), 10 days after HT (1–296 days), 49.5 days after HSCT (9–1,405 days), and 39 days after LT (1–1,092 days) (P = 0.018). The mortality rate among patients with encephalopathy was 42.0% (n = 21/50). Only 5 patients died of neurologic complications. Transplant-associated encephalopathy presented different characteristics according to the type of transplant. Specialized diagnostic approach for neurologic complications specific to the type of transplant may improve survival and quality of life in children after transplantation.
Brain Diseases* ; Brain Diseases, Metabolic ; Child* ; Chungcheongnam-do ; Heart ; Heart Transplantation ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Hypertensive Encephalopathy ; Incidence ; Kidney ; Kidney Transplantation ; Liver ; Liver Transplantation ; Medical Records ; Mortality ; Quality of Life ; Retrospective Studies ; Transplant Recipients ; Transplantation ; Transplants

PURPOSE: To compare prospectively and randomly tension-free vaginal tape(TVT) with transobturator vaginal tape inside-out(TVT-O) for the surgical treatment of female stress urinary incontinence(SUI). MATERIALS AND METHODS: One hundred twenty women with SUI were alternately assigned to either the TVT group(n=60) or TVT-O group(n=60). The preoperative evaluation included urodynamic study and a Korean version of the incontinence quality of life questionnaire(I-QoL). At 1-year after operation, surgical outcome, patient I-QoL parameters, long-term complications and uroflowmetry were evaluated in 2 groups. RESULTS: Preoperative patient characteristics including I-QoL and urodynamic study were comparable in the two groups. The rates of cure(86.8% for TVT vs. 86.8% for TVT-O), improvement(6.6% for TVT vs. 8.2% for TVT-O), and failure (6.6% for TVT vs. 5.0% for TVT-O) were similar for the two groups. The I-QoL parameters one year after surgery were improved significantly in both groups(p<0.001) and there was no difference between the two groups(p>0.05). The rates of the patient satisfaction with the procedure were 93.4% in the TVT group versus 95.0% in the TVT-O group(p>0.05). Mean operation time(11.5+/-1.4 min versus 15.2+/-1.8 min, p<0.05) was significantly shorter in the TVT-O than TVT. There were no long-term complications, such as vaginal erosion and prolonged voiding difficulty, in either group. CONCLUSION: TVT-O appears to be equally effective as TVT for the surgical treatment of stress urinary incontinence in women at a 1-year follow-up.
Female* ; Follow-Up Studies* ; Humans ; Patient Satisfaction ; Prospective Studies* ; Quality of Life ; Suburethral Slings* ; Urinary Incontinence* ; Urinary Incontinence, Stress ; Urodynamics

Duchenne and Becker muscular dystrophies (DMD and BMD, respectively) are X-linked neuromuscular disorders characterized by progressive muscle weakness and severe skeletal muscle degeneration. BMD is a milder form with a later onset. Patients with BMD tend to survive much longer than those with DMD. The differentiation between DMD and BMD is important in the genetic counseling of affected patients and their families. Since muscle biopsies are invasive procedures, the differential diagnosis of BMD and DMD is often dependent on the mutation identified in the DMD gene in affected patients. However, when a novel DMD mutation is identified, the differential diagnosis should be based on muscle biopsy findings with other clinical findings. Here we describe two Korean patients with BMD confirmed by muscle biopsy and genetic testing. Two novel exonic deletions in the DMD gene were identified.
Biopsy ; Diagnosis, Differential ; Exons ; Genetic Counseling ; Genetic Testing ; Humans ; Muscle Weakness ; Muscle, Skeletal ; Muscular Dystrophies* ; Muscular Dystrophy, Duchenne*

PURPOSE: This retrospective study was designed to know the relation between clinical features, genetics, and immunostaining findings among children with Duchenne muscular dystrophy (DMD)/Becker muscular dystrophy (BMD) and the validity of the diagnostic tools for muscular dystrophy. METHODS: The medical records and computerized databases of 93 patients diagnosed with DMD/BMD from June 1989 to December 2008 were reviewed retrospectively. Demographic characteristics including clinical features, serum creatinine kinase(CK) level, electromyogram(EMG) and nerve conduction velocity(NCV), muscle biopsy, immunochemical staining for dystrophin, and the deletion of dystrophin gene were analyzed. We calculate the concordance rate between type of frame (in or out of frame) and phenotype. RESULTS: 58(62%) children were diagnosed with DMD, 13(14%) BMD, 19(20%) unclassified dystrophy, and 3(3%) DMD/BMD carriers. The mean age of symptom onset was 5.0+/-3.5 years(range, 1-17). 46(49%) children presented gait disturbance and 35(37%) elevation of liver enzymes. The mean value of serum CK enzyme was 14,758+/-11,792 IU/L (range, 633-61,349). There was no dystrophin in the immunochemical stain among 48 DMD children and at least partial or incomplete dystrophin among 10 BMD children. 28/54(51%) children had dystrophin gene deletion in multiplex PCR and 13/14(92%) in Multiplex Ligation-dependent Probe Amplification(MLPA). The loss of heterozygosity was shown in 2 children by MLPA. The overall concordance rate between type of frame(in or out of frame) and phenotype was 95% in this study. CONCLUSION: Despite of small population, this finding indicates that the determination of type of frame (in or out of frame) by MLPA may be helpful in differential diagnosis of DMD/BMD. In addition, we surmise that the detection of carrier by MLPA is helpful in genetic counseling.
Biopsy ; Child ; Creatinine ; Diagnosis, Differential ; Dystrophin ; Gait ; Gene Deletion ; Humans ; Liver ; Loss of Heterozygosity ; Medical Records ; Multiplex Polymerase Chain Reaction ; Muscles ; Muscular Dystrophies ; Muscular Dystrophy, Duchenne ; Neural Conduction ; Phenotype ; Retrospective Studies

Primary intraspinal peripheral primitive neuroectodermal tumors (pPNET) are extremely rare. We report a 10-month-old girl presented with rapidly progressive paraparesis and raised cerebrospinal fluid protein. A magnetic resonance image demonstrated an intramedullary mass from C3 to T3 level and intradural extramedullary nodules in the lumbosacral area. Surgery was performed with partial tumor removal and histologic examination revealed a small round cell tumor and immunohistochemical characteristics of pPNET. She died due to tumor progression at four months after initial diagnosis. This case has been reported to raise awareness among clinicians to include the possibility of intraspinal tumors in the differential diagnosis of progressive neurological deficits mimicking Guillain-Barre syndrome.
Diagnosis, Differential ; Guillain-Barre Syndrome ; Humans ; Infant ; Magnetic Resonance Spectroscopy ; Neuroectodermal Tumors ; Neuroectodermal Tumors, Primitive ; Neuroectodermal Tumors, Primitive, Peripheral ; Paraparesis

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder that frequently involves the central nervous system (CNS). We compared the clinical characteristics, treatment, and prognosis of patients with HLH according to the degree of CNS involvement. METHODS: The clinical manifestations, initial laboratory data, treatment, and outcomes for 50 patients diagnosed with HLH and treated at Asan Medical Center between January 1995 and August 2011 were retrospectively reviewed and analyzed. CNS involvement was defined as the presence of neurological symptoms or an elevated white blood cell (WBC) count in the cerebrospinal fluid (CSF). RESULTS: Among these 50 patients, 23 (46%) developed CNS disease. Among patients with CNS disease, 19 had neurological symptoms, including seizures, altered consciousness, facial palsy, dysarthria, and dysphagia. Four patients had elevated CSF WBC counts without neurological symptoms. Twelve patients had abnormal brain imaging results, including high signal intensity lesions on T2-weighted magnetic resonance imaging (MRI) findings, ventriculomegaly, hemorrhage, atrophy, and leptomeningeal enhancement. Patients with CNS disease had lower ferritin, aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels as well as reduced 5-year survival as compared to those without CNS disease. CONCLUSION: CNS involvement is common among patients with HLH. Overall, patients with CNS disease achieve poorer outcomes than patients without CNS involvement. To improve outcomes, physicians must carefully monitor the neurological manifestations in patients with HLH and administer the appropriate course of intensified chemotherapy to patients with CNS disease.
Alanine Transaminase ; Aspartate Aminotransferases ; Atrophy ; Central Nervous System ; Central Nervous System Diseases ; Consciousness ; Deglutition Disorders ; Dysarthria ; Facial Paralysis ; Ferritins ; Hemorrhage ; Humans ; Leukocytes ; Lymphohistiocytosis, Hemophagocytic ; Magnetic Resonance Imaging ; Neuroimaging ; Neurologic Manifestations ; Organothiophosphorus Compounds ; Prognosis ; Retrospective Studies ; Seizures

Objective: : Stereoelectroencephalography (SEEG) is increasingly being recognized as an important invasive modality for presurgical evaluation of epilepsy. This study focuses on the clinical and technical considerations of SEEG investigations when using conventional frame-based stereotaxy, drawing on institutional experience and a comprehensive review of relevant literature. Methods: : This retrospective observational study encompassed the surgical implantation of 201 SEEG electrodes in 16 epilepsy patients using a frame-based stereotactic instrument at a single tertiary-level center. We provide detailed descriptions of the operative procedures and technical nuances for bilateral and multiple SEEG insertions, along with several illustrative cases. Additionally, we present a literature review on the technical aspects of the SEEG procedure, discussing its clinical implications and potential risks. Results: : Frame-based SEEG electrode placements were successfully performed through sagittal arc application, with the majority (81.2%) of cases being bilateral and involving up to 18 electrodes in a single operation. The median skin-to-skin operation time was 162 minutes (interquartile range [IQR], 145–200), with a median of 13 minutes (IQR, 12–15) per electrode placement for time efficiency. There were two occurrences (1.0%) of electrode misplacement and one instance (0.5%) of a postoperative complication, which manifested as a delayed intraparenchymal hemorrhage. Following SEEG investigation, 11 patients proceeded with surgical intervention, resulting in favorable seizure outcomes for nine (81.8%) and complete remission for eight cases (72.7%). Conclusion : Conventional frame-based stereotactic techniques remain a reliable and effective option for bilateral and multiple SEEG electrode placements. While SEEG is a suitable approach for selected patients who are strong candidates for epilepsy surgery, it is important to remain vigilant concerning the potential risks of electrode misplacement and hemorrhagic complications.