Background: and Purpose: We investigated the correlation between the deep distribution of white matter hyperintensity (WMH) (dWMH: WMH in deep and corticomedullary areas, with minimal periventricular WMH) and a positive agitated saline contrast echocardiography result. Methods: We retrospectively recruited participants with comprehensive dementia evaluations, an agitated saline study, and brain imaging. The participants were classified into two groups according to WMH-distributions: dWMH and dpWMH (mainly periventricular WMH with or without deep WMH.) We hypothesized that dWMH is more likely associated with embolism, whereas dpWMH is associated with small-vessel diseases. We compared the clinical characteristics, WMH-distributions, and positive rate of agitated saline studies between the two groups. Results: Among 90 participants, 27 and 12 met the dWMH and dpWMH criteria, respectively. The dWMH-group was younger (62.2±7.5 vs. 78.9±7.3, p<0.001) and had a lower prevalence of hypertension (29.6% vs. 75%, p=0.008), diabetes mellitus (3.7% vs.25%, p=0.043), and hyperlipidemia (33.3% vs. 83.3%, p=0.043) than the dpWMH-group. Regarding deep white matter lesions, the number of small lesions (<3 mm) was higher in the dWMH-group(10.9±9.7) than in the dpWMH-group (3.1±6.4) (p=0.008), and WMH was predominantly distributed in the border-zones and corticomedullary areas. Most importantly, the positive agitated saline study rate was higher in the dWMH-group than in the dpWMH-group (81.5% vs. 33.3%, p=0.003). Conclusions The dWMH-group with younger participants had fewer cardiovascular risk factors, showed more border-zone-distributions, and had a higher agitated saline test positivity rate than the dpWMH-group, indicating that corticomedullary or deep WMHdistribution with minimal periventricular WMH suggests embolic etiologies.

Purpose: Carcinoma arising from Crohn disease (CD) is rare, and there is no clear guidance on how to properly screen for at-risk patients and choose appropriate care. This study aimed to evaluate the clinicopathological characteristics, treatment, and oncologic outcomes of CD patients diagnosed with colorectal cancer (CRC). Methods: Using medical records, we retrospectively enrolled a single-center cohort of 823 patients who underwent abdominal surgery for CD between January 2006 and December 2015. CD-associated CRC patients included those with adenocarcinoma, lymphoma, or neuroendocrine tumors of the colon and rectum. Results: Nineteen patients (2.3%) underwent abdominal surgery to treat CD-associated CRC. The mean duration of CD in the CD-associated CRC group was significantly longer than that in the benign CD group (124.7 ± 77.7 months vs. 68.9 ± 60.2 months, P = 0.006). The CD-associated CRC group included a higher proportion of patients with a history of perianal disease (73.7% vs. 50.2%, P = 0.035) and colonic location (47.4% vs. 6.5%, P = 0.001). Among 19 CD-associated CRC patients, 17 (89.5%) were diagnosed with adenocarcinoma, and of the 17 cases, 15 (88.2%) were rectal adenocarcinoma. On multivariable analyses for developing CRC, only colonic location was a risk factor (relative risk, 7.735; 95% confidence interval, 2.862–20.903; P = 0.001). Conclusion Colorectal malignancy is rare among CD patients, even among patients who undergo abdominal surgery. Rectal adenocarcinoma accounted for most of the CRC, and colonic location was a risk factor for developing CRC.

BACKGROUND AND PURPOSE: The positive effects of galantamine on cognition and activities of daily living (ADL) in Alzheimer's disease (AD) are thought to be mediated via improvements in attention. The purpose of this study was to determine the effect of galantamine on attention in AD patients using a computerized attention test and to elucidate the relationship between improvements in attention and change in cognition and ADL. METHODS: In this multicenter, open-label, prospective study, patients with mild to moderate AD received galantamine and then submitted to computerized attention tests, the Alzheimer's Disease Assessment Scale-cognitive subscale, and instrumental ADL (IADL) at baseline, 4 weeks, and 12 weeks. The differences in reaction time on computerized tests were explored relative to the changes in cognition and IADL. RESULTS: After 12 weeks of taking the trial medication there was a significant reduction from baseline levels in the choice reaction time (baseline, 5,216+/-3,650 sec; 12 weeks, 4,139+/-2,920 sec; p<0.01) and the simple reaction time (baseline, 1,089+/-782 sec; 12 weeks, 908+/-606 sec; p<0.01). Correlation analyses of changes in choice or simple reaction times relative to cognition and ADL measures yielded no significant associations. The improvement in attention observed at 4 weeks of galantamine treatment was not associated with any significant changes in outcome measures at the end of trial. CONCLUSIONS: This study found no significant association between the improvement in attention after treatment with galantamine and changes in cognition and ADL in patients with mild to moderate AD, despite the significant improvement in attention over the course of the treatment.
Activities of Daily Living* ; Alzheimer Disease* ; Cognition* ; Galantamine* ; Humans ; Outcome Assessment (Health Care) ; Prospective Studies ; Reaction Time

OBJECTIVES: This study aimed to compare the prevalence and severity of clinically relevant neuropsychiatric symptoms between subjects with Alzheimer's disease (AD) and subcortical vascular dementia (SVD). METHODS: The CREDOS (Clinical Research for Dementia Of South Korea) is a multicenter longitudinal cohort study organized to evaluate the long-term outcome of dementia patients. Out of a total 3,080 subjects, we selected 1,392 AD subjects and 247 SVD subjects with mild to moderate levels of dementia. The Neuropsychiatric Inventory (NPI) and Clinical Dementia Rating Scale (CDR), and CDR sum of box scores were used for evaluation. RESULTS: After controlling for severity of dementia and duration of education, SVD subjects had relatively more symptoms of apathy compared to AD subjects (OR : 1.397, p=0.025). SVD subjects also had relatively higher NPI composite scores in the apathy domain compared to AD subjects, after controlling for severity of dementia, age and duration of education (F=7.88, p=0.01). Subjects with moderate levels of dementia had more frequent and severe neuropsychiatric symptoms compared to those with mild dementia. CONCLUSION: Symptoms of apathy were more prevalent and severe in subjects with SVD, compared to subjects with AD.
Alzheimer Disease ; Apathy ; Cohort Studies ; Dementia ; Dementia, Vascular ; Deoxycytidine ; Humans ; Prevalence

Although mutations in three genes, amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2), have been identified as genetic causes of earlyonset Alzheimer s disease (EOAD), there has been a single report on a PSEN1 mutation in Koreans. In the present study, we performed a genetic analysis of six Korean patients with EOAD. Direct sequencing analysis of the APP, PSEN1 and PSEN2 genes revealed two different mutations of the PSEN1 gene (G206S and M233T) and one mutation of the APP gene (V715M) in three patients with age-atonset of 34, 35, and 42 yr, respectively. In addition, two patients with age-at-onset of 55 and 62 yr, respectively, were homozygous for APOE epsilon 4 allele. One woman had no genetic alterations. These findings suggest that PSEN1 and APP gene mutations may not be uncommon in Korean patients with EOAD and that genetic analysis should be provided to EOAD patients not only for the identification of their genetic causes but also for the appropriate genetic counseling.
Adult ; Alleles ; Alzheimer Disease/*genetics ; Amyloid/*genetics ; Apolipoproteins E/*genetics ; Female ; Humans ; Korea ; Male ; Middle Aged ; Models, Genetic ; *Mutation ; Pedigree ; Presenilin-1/*genetics ; Sequence Analysis, DNA

BACKGROUND: Patients with Creutzfeldt-Jakob disease (CJD) show periodic sharp wave complexes (PSWCs) on electroencephalography (EEG) during the course of their illness. However, the source location of PSWCs and the pathophysiological mechanism remains unclear. METHODS: Ten patients with sporadic CJD who showed typical PSWCs on EEG underwent brain magnetic resonance imaging (MRI) and positron emission tomography (PET, n=8) or single-photon emission computed tomography (SPECT, n=2) scans. A 30-second epoch from the EEG was selected for analysis. The recording was separated blindly using independent component analysis. The separate independent components were subjected to dipole source localization using a single dipole model. The source locations were compared with neuroimaging findings in each patient. RESULTS: Two to three independent components responsible for the PSWCs seen in CJD were identified. The EEG recording reconstructed from the selected independent components accounted for about 70% of the variance in the original recording. All but one patient had dipole sources localized in both cortical and subcortical areas. One patient had only subcortical dipole sources in both caudate nuclei. The cortical locations included the cingulate, insula, frontal, temporal, and occipital areas. The subcortical sources were located in the striate, thalamic, and subthalamic nuclei. All the dipole sources were localized within lesions seen as neuroimaging abnormalities. CONCLUSIONS: These results suggest that a subcortical mechanism, in addition to cortical structures, is involved in producing the generalized periodic discharges in CJD.
Brain ; Creutzfeldt-Jakob Syndrome* ; Electroencephalography ; Humans ; Magnetic Resonance Imaging ; Neuroimaging ; Positron-Emission Tomography ; Tomography, Emission-Computed

No abstract available.
Dementia* ; Humans ; Urinary Incontinence*

BACKGROUND: Palilalia is characterized mainly by compulsive repetitions of words and phrases, which are attenuated in loudness and increased in rate. Up to date, approximately 29 cases of palilalia secondary to various neurological insults were reported mostly as a single case study. To our knowledge, there has been no reported case of palilalia in Korea until now. In this report, we present two patients with palilalia. CASES: The first patient KH, a 52-year-old man, with vascular parkinsonism, exhibited the repetition after an episode of stroke in the bilateral basal ganglia area. The second patient SH, a 60-year-old man with idiopathic Parkinson's disease, also manifested the repetition of words and phrases. The speech performance of both patients varied with the type of speech tasks, for instance, propositional language as opposed to automatic language. COMMENT: Palilalia should be differentlly diagnosed from neurogenic stuttering, echolalia, or aphasic perseveration. A pathophysiological explanation of patient KH's palilalia would be an widespread extrapyramidal impairment that resulted in destruction of the inhibitory motor circuit which might lead to the abnormal, uncontrolled speech-related neuronal firing in the frontal lobe. On the other hand, patient SH's palilalic speech may result from hyperexcitation of downstream of dopaminergic system related to peak-dose dyskinesia.
Basal Ganglia ; Dyskinesias ; Echolalia ; Fires ; Frontal Lobe ; Hand ; Humans ; Korea ; Middle Aged ; Neurons ; Parkinson Disease ; Parkinsonian Disorders ; Stroke ; Stuttering

BACKGROUND: The Samsung Dementia Questionnaire (SDQ) has recently been developed for screening dementia. Objectives of this study are to develop a short form of SDQ (S-SDQ) and to cross-validate S-SDQ in a new sample. METHODS: Factorial analysis of SDQ items was used to develop the S-SDQ. The S-SDQ was administered to informants of 100 dementia patients (61 Alzheimer's disease, 39 vascular dementia) and to those of 100 hospital control subjects. Two groups were matched in terms of age and sex. The retest of S-SDQ was conducted in 75 informants to determine the reliability of tests and retests which were spanned average 23.9 days. RESULTS: The S-SDQ was not influenced by education, age or sex. S-SDQ scores negatively correlated with the K-MMSE (r=-0.84). The S-SDQ showed stepwise scaling with dementia severity. The area under the ROC curve for the S-SDQ was 0.974 (S.E.=0.084). With respect to a diagnosis of dementia, the SDQ (cut-off point 8) had a sensitivity of 94% and a specificity of 90%. The S-SDQ was found to have a high test-retest reliability (r=0.97). CONCLUSIONS: An abbreviated version of SDQ (S-SDQ) had high sensitivity, specificity and test-retest reliability which were comparable to those of original SDQ. Thus, the S-SDQ can be a useful, brief dementia screening questionnaire.
Alzheimer Disease ; Dementia* ; Diagnosis ; Education ; Humans ; Mass Screening ; Surveys and Questionnaires* ; ROC Curve ; Sensitivity and Specificity

No abstract available.
Gait*