• Clinical and Experimental Pediatrics
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• 제50권2호
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• pp.182-189
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• 2007

Purpose : The primary purpose of this study was to evaluate the growth and neuropsychologic function following treatments for pediatric hematologic and oncologic diseases. Healthy monozygotic twins served as ideal controls for comparison to exclude possible confounding factors. Methods : Seven children treated with various hematologic and oncologic diseases were included in the study: acute lymphoblastic leukemia (ALL; n=2), Diamond-Blackfan anemia twins (n=2), and aplastic anemia (n=3). The median age at the diagnosis was 5.2 (0.3-15) years. The median duration of follow-up was 7.2 (4.9-10) years. Controls were healthy monozygotic twins. Growth was measured and the percentile channels were evaluated sequentially for patients. The K-WISC III was applied and compared in 5 pairs of patients and controls. Results : Similar growth profiles were noted for the twins. The percentiles at diagnosis was 3-10 in 3, 25-50 in 2, and 50-75 in 2 cases. All patients stayed in their growth percentiles through follow-up, except for 1 patient who became obese. For IQ tests, the mean behavioral, verbal and full scale IQ scores of patients were 88.0, 93.8, and 89.8, respectively, and those from their corresponding controls were 92.2, 97.0, and 91.7 (P>0.05). However, 2 children who were treated for ALL had lower IQ scores. Conclusion : Similar growth profiles were observed in the monozygotic twins in terms of height and weight. The IQ scores of patients were similar to those of monozygotic twins. However, prophylactic CNS-directed therapy for leukemia might adversely affect the IQ scores. A further prospective study on larger number of twins is warranted.

• Korean Journal of Psychosomatic Medicine
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• 제24권2호
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• pp.200-207
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• 2016

Objectives : Alzheimer's disease(AD) is characterized by progressive decline of cognitive function and also by various behavioral psychological symptoms of dementia(BPSD) which causes distress to their caregivers. The purpose of this study was to investigate association between each AD patients' behavioral psychological symptoms and their caregivers' burden. Methods : Participants were 80 AD patients and their caregivers. We used Korean neuropsychiatric inventory (K-NPI) to assess the symptoms of patients and Korean version of Zarit Burden Interview(ZBI) to evaluate caregivers' burden. Results : The results showed ZBI score, which is the index for caregivers' burden, had a statistically significant positive correlation with the frequency of delusion, hallucination, agitation/aggression, depression, anxiety, disinhibition and irritability, the severity of hallucination, agitation/aggression, anxiety, disinhibition, aberrant motor, and sleep, and the global score(frequency${\times}$severity) for delusion, hallucination, agitation/aggression, depression, anxiety, disinhibition, aberrant motor, and sleep. There were significant correlations between each scale for cognitive function(i.e. MMSE-KC, CDR, GDS) and ZBI scale. Correlations between each scale for activity of daily living(i.e. Barthel -ADL, K-ADL) and ZBI scale were also significant. Conclusions : There were a significant correlation between BPSD and caregiver burden. Caregiver burden was also correlated with cognitive function and activity of daily living. Early detection and preventive treatment of these symptoms in BPSD might make improvement of caregivers' quality of life as well as AD patients'.

• The Korean Journal of Nuclear Medicine
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• 제34권6호
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• pp.445-455
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• 2000

Purpose: We evaluated the distribution of hypoperfusion in patients with traumatic brain injury (TBI) and the relationship of thalamic hypoperfusion to severity of cognitive and behavioral sequelae. Materials and Methods: Tc-99m ECD SPECT and MRI were performed in 103 patients (M/F=81/22, mean age $34.7{\pm}15.4$ yrs) from 0.5 to 55 months (mean 10.3 months) after TBI. The patients were divided into three groups showing no abnormalities (G1), focal (G2) and diffuse injury (G3) on MRI. Psychometric tests assessed 11 cognitive or behavioral items. In all patients, we evaluated the distribution of hypoperfused areas in SPECT, and in 57/103 patients, neuropsychological (NP) abnormalities in patients with thalamic hypoperfusion were compared with those of patients without thalamic hypoperfusion. Results: The perfusion deficits were most frequently located in the frontal lobe (G1, 42.3%: G2 34.5%: G3 33.3%), temporal lobe ($24{\sim}26%$) thalami ($21{\sim}22.4%$), parietal and occipital lobe (${\leq}10%$). Numbers of NP abnormalities in the cases of cortical hypoperfusion with or without concomitant thalamic hypoperfusion were following: the former $4.7{\pm}1.5$ and the latter $3.2{\pm}1.4\;in\;G1,\;5.0{\pm}1.1\;and\;4.8{\pm}1.2\;in\;G2,\;6.8{\pm}1.8\;and\;6.3{\pm}1.1\;in\;G3$, respectively. This difference according to thalamic hypoperfusion was significant in G1 (p=0.002), but was not significant in G2 or G3. Conclusion: SPECT in patients with TBI had demonstrated hypoperfusion mostly involving the frontal, temporal and thalami. In normal group on MRI, frontal hypoperfusion was more prominent than that of any other group, Furthermore in this group, SPECT could predict severity of NP outcome by concomitant thalamic hypoperfusion with cerebral cortical abnormalities.

• Journal of the Korea Academia-Industrial cooperation Society
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• 제12권1호
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• pp.294-300
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• 2011

This study was performed to examine the presence of dysphagia and analyze characteristics of the symptoms in cerebrovascular accident(CVA) patients without awareness of dysphagia. A questionnaire for this study was given to CVA patients who had visited P rehabilitation medical center in Busan. Eleven patients (4 males and 7 females) who answered no awareness of dysphasia were given to VFSS, functional dysphasia scale, and NCSE. Descriptive statistics and Pearson correlation analysis were performed by SPSS 12.0. All of subjects without awareness of dysphasia showed characteristics of dysphasia symptoms. Prominent dysfunctions were problems in oral phase and delay of swallowing reflex in pharyngeal phase. For the aspect of cognition, they showed lower score in construction, memory, and similarity than other NCSE items. There was highly significant correlation between orientation, judgment and delay of swallowing reflex. Verbal comprehension and residual materials in oral cavity showed closed correlation. CVA patients without awareness had dysphagia with high probability. The early evaluation of dysphagia should be performed in CVA patients in order to prevent complications due to CVA, so it is necessary to increase the effectiveness of rehabilitation therapy.

• Korean Journal of Biological Psychiatry
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• 제19권3호
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• pp.115-120
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• 2012

Objectives : Event-related potentials have been suggested as an objective marker for brain functions in psychiatric disorders. This study was aimed to investigate the relationships between P300, the mismatch negativity (MMN), the loudness dependence of the auditory evoked potential (LDAEP), demographic and clinical variables including neurocognitive abilities in patients with schizophrenia. Methods : P300, the MMN, and the LDAEP were measured and the Korean Stroop color-word test (K-stroop test) and the Wisconsin Card Sorting Test (WCST) were performed in 43 patients with schizophrenia. The relationship of the latency and amplitude of P300 and the MMN as well as regression slope of the LDAEP with demographic and clinical variables were analyzed by t-test and correlation analyses for categorical and continuous variables, respectively. Results : After controlling for age, the latency of central (Cz) and parietal (Pz) P300 posivitively correlated with GAF at admission (Cz ; ${\gamma}$ = 0.385, p = 0.047, Pz ; ${\gamma}$ = 0.421, p = 0.029). The amplitude of parietal P300 correlated with the correction rate of the K-stroop test (${\gamma}$ = 0.575, p = 0.002). In addition, the frontal (Fz) P300 latency tended to negatively correlated with the correction rate of the WCST (${\gamma}$ = -0.371, p = 0.057). Conclusions : Our findings suggest that the values of P300 latency and amplitude might be correlated with GAF at admission and working memory measured by the K-Stroop test and the WCST. Meanwhile, the MMN and the LDAEP did not correlate with demographic and clinical variables. These results support the results of previous studies showing associations with P300 and impaired cognitive ability.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제1권1호
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• pp.55-64
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• 1990

The purpose of the present study is to investigate cognitive characteristics of ADHD children by comparing their performances on KEDI-WISC according to age and EEG variables. Subjects were 56 ADHD children who visited Seoul National University Children's Hospital during the period from January, 1988 to March, 1989. Group differences on age and EEG variables were tested by ANOVA, and Hierarchical Cluster Analysis was performed to investigate how ADHD children were classified based on their performances on KEDI-WISC. The results Indicated that ADHD children showed low scores on Coding, Digit Span, and Comprehension subtests, suggesting their attention deficits and impulsivity. ADHD children were clustered Into three groups based on only FSIQ. In post-hoc tests three groups showed different cognitive strengths and weaknesses on KEDI-WISC. Group differences on age were not significant, and abnormal EEG group showed lower PIQ than normal EEG group, suggesting the possibility that their attention deficits were related to neurological factors.

• Clinical and Experimental Pediatrics
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• 제53권4호
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• pp.603-606
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• 2010

Paraneoplastic limbic encephalitis, a remote effect of cancer without nervous system metastasis, is rare, especially in childhood. Here, we report a case of paraneoplastic limbic encephalitis associated with an ovarian mature teratoma in an adolescent girl. The 15-year-old girl developed neuropsychiatric symptoms, memory loss, seizures, and unconsciousness. Cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI) findings were normal, while single photon emission computed tomography imaging showed hypoperfusion in both temporal lobes. Ultrasound and MRI of the abdomen revealed a left ovarian cystic mass. The patient experienced a significant recovery of cognitive function after surgical resection of the tumor, which was pathologically identified as a mature ovarian teratoma, and treatment with intravenous immunoglobulin.

• Korean Journal of Clinical Laboratory Science
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• 제54권4호
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• pp.249-255
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• 2022

Alzheimer's disease (AD) represents a major public health concern and has been identified as a research priority. Clinical research evidence supports that the core cerebrospinal fluid (CSF) biomarkers for AD, including amyloid-β (Aβ42), total tau (T-tau), and phosphorylated tau (P-tau), reflect key elements of AD pathophysiology. Nevertheless, advances in the clinical identification of new indicators will be critical not only for the discovery of sensitive, specific, and reliable biomarkers of preclinical AD pathology, but also for the development of tests that facilitate the early detection and differential diagnosis of dementia and disease progression monitoring. The early detection of AD in its presymptomatic stages would represent a great opportunity for earlier therapeutic intervention. The chance of successful treatment would be increased since interventions would be performed before extensive synaptic damage and neuronal loss would have occurred. In this study, the importance of developing an early diagnostic method using cognitive decline biomarkers that can discriminate between normal, mild cognitive impairment (MCI), and AD preclinical stages has been emphasized.

• Journal of The Korean Society of Inherited Metabolic disease
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• 제23권2호
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• pp.39-44
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• 2023

Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.

• Journal of the Korea Academia-Industrial cooperation Society
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• 제16권9호
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• pp.6201-6213
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• 2015

The purpose of this study was to investigate the effect of Reality Orientation Therapy and Spaced Retrieval Therapy of the applied of CERAD-K type cognitive rehabilitation program for mild Alzheimer's patients. The tool used to this study was CERAD-K neuropsychological test consists of Boston Naming, a simple mental status, the word list recall, and configure behavior. A non-equivalent control group pretest-posttest design was used. The participants were 85 mild Alzheimer's patients (experimental group: 32, control group: 53) who registered G Health Center, in Gyeonggi province. Those Data were collected from 10:00 to 11:00, 14:00 to 15:00, twice a week on Tuesday and Friday, 8 session, from September 1 to September 30, 2014. Control group was participated daily routine program only in the G public health center.The data were analyzed using SPSS WIN 21.0 program. The results of this study were as follows;Boston Naming(F=3.22, p<.001), simple mental status(F=5.92, p<.001), and configure behavior(F=4.67, p<.001) were accepted, but word list recall(F=.75 p>.05) was not accepted. The study findings indicate that the Reality Orientation Therapy and Spaced Retrieval Therapy of the applied of CERAD-K type cognitive rehabilitation program is effective and can be recommended as intervention for mild Alzheimer's patients.