Background: and Purpose: The Movement Disorder Society-Sponsored Revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) is widely used for estimating the symptoms of Parkinson’s disease. Translation and validation of the MDS-UPDRS is necessary for non-English speaking countries and regions. The aim of this study was to validate the Korean version of the MDS-UPDRS. Methods: Altogether, 362 patients in 19 centers were recruited for this study. We translated the MDS-UPDRS to Korean using the translation-back translation method and cognitive pretesting. We performed both confirmatory and exploratory factor analyses to validate the scale.We calculated the comparative fit index (CFI) for confirmatory factor analysis, and used unweighted least squares for exploratory factor analysis. Results: The CFI was higher than 0.90 for all parts of the scale. Exploratory factor analysis also showed that the Korean MDS-UPDRS has the same number of factors in each part as the English version. Conclusions The Korean MDS-UPDRS has the same overall structure as the English MDSUPDRS. Our translated scale can be designated as the official Korean MDS-UPDRS.

The original version of this article contained wrong informations of some authors which should be changed.

OBJECTIVE: Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea. METHODS: We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN). RESULTS: Four subtypes of NBIA including PKAN (n = 30), PLA2G6-related neurodegeneration (n = 2), beta-propeller protein-associated neurodegeneration (n = 1), and aceruloplasminemia (n = 1) have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG). Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN. CONCLUSIONS: We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.
Adult ; Age of Onset ; Alleles ; Basal Ganglia ; Brain ; Dystonia ; Freezing ; Gait ; Gene Frequency ; Genetic Association Studies ; Humans ; Iron ; Korea ; Movement Disorders ; Neurodegenerative Diseases ; Pantothenate Kinase-Associated Neurodegeneration* ; Parkinsonian Disorders ; Phenotype ; Population Characteristics* ; Referral and Consultation ; Weather

BACKGROUND AND PURPOSE: Nonmotor symptoms (NMS) in Parkinson's disease (PD) have multisystem origins with heterogeneous manifestations that develop throughout the course of PD. NMS are increasingly recognized as having a significant impact on the health-related quality of life (HrQoL). We aimed to determine the NMS presentation according to PD status, and the associations of NMS with other clinical variables and the HrQoL of Korean PD patients. METHODS: We surveyed patients in 37 movement-disorders clinics throughout Korea. In total, 323 PD patients were recruited for assessment of disease severity and duration, NMS, HrQoL, and other clinical variables including demographics, cognition, sleep scale, fatigability, and symptoms. RESULTS: In total, 98.1% of enrolled PD subjects suffered from various kinds of NMS. The prevalence of NMS and scores in each NMS domain were significantly higher in the PD group, and the NMS worsened as the disease progressed. Among clinical variables, disease duration and depressive mood showed significant correlations with all NMS domains (p<0.001). NMS status impacted HrQoL in PD (rS=0.329, p<0.01), and the association patterns differed with the disease stage. CONCLUSIONS: The results of our survey suggest that NMS in PD are not simply isolated symptoms of degenerative disease, but rather exert significant influences throughout the disease course. A novel clinical approach focused on NMS to develop tailored management strategies is warranted to improve the HrQoL in PD patients.
Cognition ; Demography ; Humans ; Korea ; Movement Disorders* ; Parkinson Disease* ; Prevalence ; Quality of Life*

No abstract available.
Myocardial Infarction

BACKGROUND: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. MATERIAL AND METHOD: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was 17.3+/-10.8 days (range: 9~36 days) at the 1st staged operation, 8.9+/-7.1 months (4.6~23.3 months) at the 2nd staged operation (the Bidirectional Glenn procedure) and 32.4+/-9.8 months at the final staged operation (the Fontan procedure). During the 2nd staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. RESULT: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was 19.6+/-14.9 months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). CONCLUSION: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.
Aspirin ; Echocardiography ; Follow-Up Studies ; Fontan Procedure ; Hemodynamics ; Humans ; Hypoplastic Left Heart Syndrome ; Medical Records ; Norwood Procedures ; Polytetrafluoroethylene ; Retrospective Studies ; Tricuspid Valve ; Tricuspid Valve Insufficiency ; Ventricular Function

BACKGROUND: Surgery for mitral valve disease in children carries both technical and clinical difficulties that are due to both the wide spectrum of morphologic abnormalities and the high incidence of associated cardiac anomalies. The purpose of this study is to assess the outcome of mitral valve surgery for treating congenital mitral regurgitation in children. MATERIAL AND METHOD: From 1997 to 2007, 22 children (mean age: 5.4 years) who had congenital mitral regurgitation underwent mitral valve repair. The median age of the patients was 5.4 years old and four patients (18%) were under 12 months of age. 15 patients (68%) had cardiac anomalies. There were 13 cases of ventricular septal defect, 1 case of atrial septal defect and 1 case of supravalvar aortic stenosis. The grade of the preoperative mitral valve regurgitation was II in 4 patients, III in 15 patients and IV in 3. The regurgitation was due to leaflet prolapse in 12 patients, annular dilatation in 4 patients and restrictive leaflet motion in 5 patients. The preoperative MV Z-value and the regurgitation grade were compared with those obtained at follow-up. RESULT: MV repair was possible in all the patients. 19 patients required reduction annuloplasty and 18 patients required valvuloplasty that included shortening of the chordae, papillary muscle splitting, artificial chordae insertion and cleft closure. There were no early or late deaths. The mitral valve regurgitation after surgery was improved in all patients (absent=10, grade I=5, II=5, III=2). MV repair resulted in reduction of the mitral valve Z-value (2.2+/-.1 vs. 0.7+/-.3, respectively, p<0.01). During the mid-term follow-up period of 3.68 years, reoperation was done in three patients (one with repair and two with replacement) and three patients showed mild progression of their mitral regurgitation. CONCLUSION: Our experience indicates that mitral valve repair in children with congenital mitral valve regurgitation is an effective and reliable surgical method with a low reoperation rate. A good postoperative outcome can be obtained by preoperatively recognizing the intrinsic mitral valve pathophysiology detected on echocardiography and with the well-designed, aggressive application of the various reconstruction techniques.
Aortic Stenosis, Supravalvular ; Child ; Dilatation ; Echocardiography ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Mitral Valve ; Mitral Valve Insufficiency ; Papillary Muscles ; Prolapse ; Reoperation

BACKGROUND: As the patients who undergo heart transplantation have achieved better survival in recent years, a growing number of recipients are at a risk for experiencing surgical complications in addition to rejection and infection. In this paper, we report on our experience with the surgical complications that occurred in heart transplant recipients. MATERIAL AND METHOD: From April 1994 to September 2003, 37 heart transplantations were performed at our center by a single surgeon. The indications for transplantation were dilated cardiomyopathy, ischemic cardiomyopathy, valvular cardiomyopathy and familial hypertrophic cardiomyopathy. RESULT: Twenty postoperative complications required surgeries in 15 patients (41%). The types of operations required were; redo-sternotomy for bleeding (5), pericardiostomy for effusion (4), implantation of a permanent pacemaker (1), right lower lobe lobectomy for aspergilloma (1), removal of urinary stone (1), cholecystectomy for gall bladder stone (1), drainage of a perianal abscess (1), paranasal sinus drainage (1), total hip replacement (1), partial gingivectomy due to gingival hypertrophy (1), urethrostomy (1), herniated intervertebral disc operation (1) and total hysterectomy for myoma uteri (1). The locations of the complications were mediastinal in 10 (27%) cases and extramediastinal in 10 (27%) cases. CONCLUSION: The relatively high incidence of extrathoracic complications associated with heart transplantation emphasizes the importance of a multidisciplinary approach to the improve long-term survival when managing those complex patients.
Abscess ; Arthroplasty, Replacement, Hip ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Cardiomyopathy, Hypertrophic, Familial ; Cholecystectomy ; Drainage ; Gingival Hypertrophy ; Gingivectomy ; Heart ; Heart Transplantation ; Hemorrhage ; Humans ; Hysterectomy ; Incidence ; Intervertebral Disc ; Myoma ; Pericardial Window Techniques ; Postoperative Complications ; Rejection (Psychology) ; Transplants ; Urinary Bladder Calculi ; Urinary Calculi ; Uterus