Developmental Trajectories of Attention in Normal Korean Population.

OBJECTIVES: This study aimed to investigate the trajectory of change of diverse attention and working memory in Koreans from 4 to 40 years of age. METHODS: The data of 912 subjects from 4 to 15 years of age obtained from a previous standardization study of the computerized comprehensive attention test were merged with the newly obtained data of 150 subjects aged 16 to 40 years from this study. We evaluated the various kinds of attention, in which each subtest had five indicators. Working memory, with parameters such as number of correct responses and span, was also measured. RESULTS: Our findings indicated that attention developed as age increased, and it decreased or was maintained after a certain age. Selective and sustained attention developed rapidly in children and adolescents, until mid-teens or 20 s when it ceased development. Divided attention, however, developed up to approximately age 20. In addition, working memory developed until mid-teens or 20 s. CONCLUSION: We presented the standardized data on diverse kinds of attention and working memory in children, adolescents, and adults in Korea. We could recognize any patterns of change in attention and working memory with increasing age.

Immunoglobulin E to allergen components of house dust mite in Korean children with allergic disease.

BACKGROUND: House dust mites (HDMs) are important sources of indoor allergens. Seventeen components have been identified from Dermatophagoides pteronyssinus (Der p). OBJECTIVE: Our aim was to define the prevalence of specific IgE to components of Der p in Korea and investigate the clinical features of them in children with allergic disease. METHODS: We performed a prospective evaluation of 80 HDM sensitized patients with history of allergic rhinitis (AR), atopic dermatitis (AD), asthma and urticaria (UC). Patients underwent ImmunoCAP for total IgE, Der p, Der f, Der p 1, Der p 2, and Der p 10. RESULTS: Seventy-nine patients had detectable serum IgE to Der p, 80 patients were sensitized to Der f, 66 patients were sensitized to Der p 1, 63 patients to Der p 2, and 7 patients were sensitized to Der p 10. Der p 1 specific IgE was significantly lower in the UC group compared with the AD and AR group. Total IgE was significantly higher in the Der p 10 sensitized group. Der p 10 serum IgE level was highly correlated with crab and shrimp specific IgE. There was a significant positive correlation between total IgE and specific IgE to Der p and its components and Der f. CONCLUSION: Sensitization to HDM and its components in Korea is similar to previous studies from temperate climate. The determination of Der p 1, Der p 2, and Der p 10 specific IgE helps in obtaining additional information in regards to allergic disease.

Effects of dexmedetomidine on the ratio of T helper 1 to T helper 2 cytokines in patients undergoing laparoscopic cholecystectomy.

STUDY OBJECTIVES: To investigate the effect of dexmedetomidine on T helper 1 (Th1) and T helper 2 (Th2) cytokines and their ratio during and after surgery. DESIGN: Single-blinded, randomized, placebo-controlled clinical comparison study. SETTING: Academic medical center. PATIENTS: 46 adult, ASA physical status 1 and 2 patients scheduled for laparoscopic cholecystectomy. INTERVENTIONS: Patients were randomized to two groups: the dexmedetomidine group (n = 23), in which dexmedetomidine was infused with a 1.0 µg/kg loading dose followed by infusion of 0.5 µg/kg/h; or the saline group (n = 23). MEASUREMENTS: Interferon-gamma (IFN-gamma) and interleukin-4 (IL-4) as Th1 and Th2 cytokines, respectively, were quantified three times: after induction of anesthesia (T0), at the end of peritoneal closure (T1), and 60 minutes after surgery (T2). The IFN-gamma/IL-4 ratio was then calculated. MAIN RESULTS: The dexmedetomidine group displayed higher levels of IFN-gamma at T1 and T2 (42.30 pg/dL vs 6.91 pg/dL at T1 [P = 0.025]; 40.51 pg/dL vs 8.29 pg/dL at T2 [P = 0.030]) than the saline group. The dexmedetomidine group was also associated with higher ratios of IFN-gamma/IL-4 (1.22 vs 0.32, respectively, at T1 [P = 0.012]; 1.53 vs 0.13, respectively, at T2 [P = 0.012]). CONCLUSIONS: Dexmedetomidine plays an immunomodulatory role, shifting the Th1/Th2 cytokine balance toward Th1 in patients with surgical and anesthetic stress.

Zoster-associated segmental paresis in a patient with cervical spinal stenosis.

Segmental zoster paresis is a rare complication of herpes zoster, characterized by focal motor weakness that does not always present simultaneously with skin lesions. Zoster paresis can be easily confused with other neuromuscular or spinal diseases. This case report describes the case of a 72-year-old woman with herpes zoster and cervical spinal stenosis at the same spinal level, where it was difficult to distinguish segmental zoster paresis from cervical radiculopathy combined with motor neuropathy. Although segmental zoster paresis in the upper extremity is rare, it should be included in the differential diagnosis of segmental pain and weakness in the extremities, especially in older or immunocompromised patients. Correct diagnosis is required, to avoid unnecessary surgery and allow timely antiviral treatment.

A randomized, double-blind study to evaluate the efficacy of ramosetron and palonosetron for prevention of postoperative nausea and vomiting after gynecological laparoscopic surgery.

BACKGROUND: Postoperative nausea and vomiting (PONV) is a common complication after anesthesia and surgery; 5-hydroxytryptamine type 3 (5-HT3) receptor antagonists have been considered as a first-line therapy. Ramosetron and palonosetron are more recently developed drugs and have greater receptor affinity and a longer elimination half-life compared with older 5-HT3 receptor antagonists. The purpose of this study was to determine which drug is more effective for preventing PONV between ramosetron and palonosetron. METHODS: We enrolled 100 patients undergoing gynecological laparoscopic surgery into this study. The subjects were divided into ramosetron group and palonosetron group. The medications were provided immediately before the induction of anesthesia. The occurrence of nausea and vomiting, severity of nausea according to a visual analogue scale, and rescue anti-emetic drug use were monitored immediately after the end of surgery and at 0-6 h, 6-24 h, and 24-48 h post-surgery. RESULTS: The incidence of vomiting was significantly lower in the palonosetron group than in the ramosetron group during 0-6 h (6% vs 26%, P = 0.012) and 0-48 h (14% vs 34%, P = 0.034). The incidence of nausea and overall PONV, and the use of rescue antiemetic were not significantly different during all time intervals. The severity of nausea was not different between the two groups. CONCLUSIONS: In conclusion, the incidence of PONV between the ramosetron and the palonosetron group have not shown the difference during 0-48 h, although palonosetron results in a lower incidence of vomiting during 0-6 h post-surgery.

A complex phenotype of peripheral neuropathy, myopathy, hoarseness, and hearing loss is linked to an autosomal dominant mutation in MYH14.

Both peripheral neuropathy and distal myopathy are well-established inherited neuromuscular disorders characterized by progressive weakness and atrophy of the distal limb muscles. A complex phenotype of peripheral neuropathy, myopathy, hoarseness, and hearing loss was diagnosed in a large autosomal dominant Korean family. A high density single nucleotide polymorphism (SNP)-based linkage study mapped the underlying gene to a region on chromosome 19q13.3. The maximum multipoint LOD score was 3.794. Sequencing of 34 positional candidate genes in the segregating haplotype revealed a novel c.2822G>T (p.Arg941Leu) mutation in the gene MYH14, which encodes the nonmuscle myosin heavy chain 14. Clinically we observed a sequential pattern of the onset of muscle weakness starting from the anterior to the posterior leg muscle compartments followed by involvement of intrinsic hand and proximal muscles. The hearing loss and hoarseness followed the onset of distal muscle weakness. Histopathologic and electrodiagnostic studies revealed both chronic neuropathic and myopathic features in the affected patients. Although mutations in MYH14 have been shown to cause nonsyndromic autosomal dominant hearing loss (DFNA4), the peripheral neuropathy, myopathy, and hoarseness have not been associated with MYH14. Therefore, we suggest that the identified mutation in MYH14 significantly expands the phenotypic spectrum of this gene.

Myotonic dystrophy type I combined with X-linked dominant Charcot-Marie-Tooth neuropathy.

Both the myotonic dystrophy type 1 (DM1) and the X-linked dominant Charcot-Marie-Tooth disease (CMTX1) are well-established inherited neuromuscular disorders characterized by progressive weakness and atrophy of the distal limb muscles. The underlying causes of the DM1 and CMTX1 are mutations in the DMPK and GJB1 gene, respectively. A patient with both DM1 and CMTX1 inherited these from his father and mother, respectively. Histopathological and electrodiagnostic studies revealed both chronic neuropathic and myopathic features. Physical disabilities were more severe than seen with either DM1 or CMTX1 alone. In addition, the present case reveals an asymmetric atrophy (22%) of the right calf muscle compared to the left side.

Characterization of a Novel Monoclonal Antibody (27H2) Recognizing Human CD34 Class III Epitope.

BACKGROUND: Monoclonal antibodies (mAbs) recognizing Class III epitope of CD34 are essential for flow cytometric diagnosis of leukemia. METHODS: 27H2 mAb was developed from a mouse alternatively immunized with human acute leukemia cell lines, KG1 and Molm-1. Using flow cytometric analysis of various leukemic cell lines and peripheral blood, immunohistochemical study of frozen tonsil, we characterized 27H2 mAb. Antigen immunoprecipitated with 27H2 mAb immunobloted with anti-CD34 mAb. A case of bone marrow sample of acute lymphoblastic leukemia (ALL) patient was obtained at CBNU Hospital. For epitope identification enzyme treatment with neuraminidase and O-sialoglycoprotein endopeptidase (OSGE) and blocking assay with known classIII mAb (HPCA-2) were done. RESULTS: Only KG1 and Molm-1 revealed positive immunoreactivity. Immunohistochemical staining disclosed strong membranous immunoreactivity on high endothelial venules. Antigen immunoprecipitated by 27H2 mAb showed approximately 100 kDa sized band immunoblotted with anti-CD34 under non-reducing conditions. Epitope recognized by 27H2 mAb disclosed resistancy to both neuraminidase and OSGE treatment and completely blocked with known class III mAb preincubation. CD34 positive leukemic cells in BM of pre B cell ALL patient detected by FITC-conjugated 27H2 and HPCA-2 were identified with similar sensitivity. CONCLUSION: A novel murine mAb recognizing class III epitope of human CD34 with high affinity, which is useful for flow cytometric diagnosis of leukemia, was developed.

NEFL Pro22Arg mutation in Charcot-Marie-Tooth disease type 1.

Charcot-Marie-Tooth disease (CMT) is classified into demyelinating neuropathy (CMT1) and axonal neuropathy (CMT2). Mutations in the neurofilament light chain polypeptide (NEFL) gene are present in CMT2E and CMT1F neuropathies. Two types of Pro22 mutations have been previously reported: Pro22Ser in CMT2E with giant axons, and Pro22Thr in CMT1F. In this study, we identified another Pro22 mutation, Pro22Arg, in a Korean CMT1 family. An investigation to identify the clinical and pathological characteristics of the Pro22Arg revealed that it is associated with demyelinating neuropathy features in CMT1F. Histopathological findings showed onion bulb formations but no giant axons. It appears that the Pro22 mutations may influence not only the Thr-Pro phosphorylation site by proline-directed protein kinases but also other structural alteration of the NEFL protein in a different way.

Distal hereditary motor neuropathy in Korean patients with a small heat shock protein 27 mutation.

Distal hereditary motor neuropathy (dHMN) is a heterogeneous disorder characterized by degeneration of motor nerves in the absence of sensory abnormalities. Recently, mutations in the small heat shock protein 27 (HSP27) gene were found to cause dHMN type II or Charcot-Marie-Tooth disease type 2F (CMT2F). The authors studied 151 Korean axonal CMT or dHMN families, and found a large Korean dHMN type II family with the Ser135Phe mutation in HSP27. This mutation was inherited in an autosomal dominant manner, and was well associated with familial members with the dHMN phenotype. This mutation site is located in the alpha-crystallin domain and is highly conserved between different species. The frequency of this HSP27 mutation in Koreans was 0.6%. Magnetic resonance imaging analysis revealed that fatty infiltrations tended to progressively extend distal to proximal muscles in lower extremities. In addition, fatty infiltrations in thigh muscles progressed to affect posterior and anterior compartments but to lesser extents in medial compartment, which differs from CMT1A patients presenting with severe involvements of posterior and medial compartments but less involvement of anterior compartment. The authors describe the clinical and neuroimaging findings of the first Korean dHMN patients with the HSP27 Ser135Phe mutation. To our knowledge, this is the first report of the neuroimaging findings of dHMN type II.

A novel GDAP1 Q218E mutation in autosomal dominant Charcot-Marie-Tooth disease.

A wide range of phenotypes have been reported in autosomal recessive (AR) Charcot-Marie-Tooth disease (CMT) patients carrying mutations in the ganglioside-induced differentiation-associated protein 1 (GDAP1) gene, such as axonal, demyelinating, and intermediate forms of AR CMT. There have been very few reports of GDAP1 mutations in autosomal dominant (AD) CMT. Here, we report an AD CMT family with a novel Q218E mutation in the GDAP1 gene. The mutation was located within the well-conserved glutathione S-transferase (GST) core region and co-segregated with the affected members in the pedigree. The affected AD CMT individuals had a later disease onset and much milder phenotypes than the AR CMT patients, and the histopathologic examination revealed both axonal degeneration and demyelination.

Left atrial volume measured by real-time 3-dimensional echocardiography predicts clinical outcomes in patients with severe left ventricular dysfunction and in sinus rhythm.

BACKGROUND: The clinical value of real-time 3-dimensional echocardiography assessments of left atrial volume in patients with left ventricular dysfunction has not been determined. METHODS: Real-time 3-dimensional echocardiography and 2-dimensional Doppler echocardiography were performed on the same day in 108 patients with severe left ventricular dysfunction and in sinus rhythm. End-systolic left atrial volumes were measured using real-time 3-dimensional echocardiography images (LAV-3D) and end-systolic left atrial volumes were calculated by the biplane area-length formula using 2-dimensional echocardiography (LAV-2D). Patients were observed clinically over 10 +/- 7 months. RESULTS: LAV-2D showed excellent correlation with LAV-3D (r = 0.88, P < .001), but the former was significantly smaller than the latter (-12 +/- 21 mL, P < .001). During follow-up, 31 patients (29%) showed clinical events, including 3 cardiac deaths and 28 hospitalizations as a result of heart failure. Patients with clinical events had larger initial LAV-3D (P < .05) and LAV-2D (P = .05), higher transmitral E velocity, higher E/E' ratio, more severe mitral and tricuspid regurgitation, and higher maximal velocity of tricuspid regurgitation than the 77 patients without events. LAV-3D (P < .001) and age (P < .05) were independent predictors of cardiac events by Cox proportional hazard model, whereas LAV-2D was negatively involved. Patients with initial LAV-3D less than 100 mL had a significantly higher 1-year event-free survival than those with LAV-3D greater than or equal to 100 mL (80 +/- 7 vs 48 +/- 10%, P < .001). CONCLUSIONS: LAV-3D is a major predictor of clinical events in patients with severe left ventricular dysfunction and in sinus rhythm. The clinical value of LAV-3D seems to be superior to that of LAV-2D.