Juvenile myoclonic epilepsy(JME) of Janz is a syndrome of generalized age related epilepsy with increasing incidence and probable autosomal recessive trait. We have experienced 2 female patients who had awakening myoclonus in both arms, which had started in their early 10th. The patterns of seirures and electroencephalographic findings were consistent with those of JME which had been first described as impulsive petit mal by Janz. We report these cases with brief review of literatures.
Arm ; Epilepsy ; Female ; Humans ; Incidence ; Myoclonic Epilepsy, Juvenile* ; Myoclonus

BACKGROUND: Frontotemporal dementia (FTD) with motor neuron disease (MND) is a syndrome of progressive changes in behavior, language, muscle weakness and atrophy due to loss of function of neurons in the frontal and temporal lobes and in motor neurons. Etiology and pathogenesis of FTD with MND are still uncertain. CASE REPORT: A 71-year-old man presented with a 2-year history of progressive muscle weakness and cognitive deficits. We diagnosed this patient as FTD with MND by neurological examination, electromyography, brain imaging and neuro-psychological evaluation. We also confirmed antiphospholipid syndrome (APS) in this patient as a way to rule out secondary causes of MND. CONCLUSIONS: This was a very rare case of FTD with MND in APS. We should focus study on the possible role of autoimmune pathogenesis in FTD with MND.
Aged ; Antiphospholipid Syndrome* ; Atrophy ; Cognition Disorders ; Electromyography ; Frontotemporal Dementia* ; Humans ; Motor Neuron Disease* ; Motor Neurons* ; Muscle Weakness ; Neuroimaging ; Neurologic Examination ; Neurons ; Temporal Lobe

Twenty patient's capsular lesions were detected by high resolution brain computed tomograhpy (CT). Eighteen of them had systemic hypertension. Two patients had prior stroke episodes that were of the same nature. All of the patients had sudden onset of neurologic deficits. Their neurologic syndromes were pure motor stoke (10 cases), ataxic hemiparesis (3 cases), pure motor stroke with dysphasia (1 cases), pure sensory-motor stroke (3 cases), pure motor stroke with tremor, locked-in syndrome, and hemiparesis with latered consciousness, each 1 case respectively. The CT showed capsular infarcts of ovolid or round hypodense lesion that was smaller than 20mm in diameter and that was seen on only two scan sections. The sites of capsular infarcts were genu(5 cases), posterior limb (9 cases), genu-posterior limb (3 cases), genu-anterior limb (3 cases), and anterior limb (4 cases) respectively. Large capsulo-putamino-caudate infarcts, or giants lacunes (type 1 of Rascol et al ) are seen in 3 cases only in pure motor stroke ; Posterior limb capsular Iacunes (type 2 of Rascol et al), 6 cases in pure motor stroke, 3 cases in ataxic hemiparesis, 2 cases in pure sensory-motor stroke, 1 case in modified pure motor stroke with tremor : Anterior capsular lacunes (type 3 of Rascol et al), 1 case in pure motor stroke, 1 case in modified pure motor stroke with dysphasia, 1 case in modified pure motor stroke with memory and cognitive dysfunction. None of the capsular infarct caused mass effect. In five cases there was a relatively complete neurologic improvement, in 14 cases did the patients show mild residual neurologic deficit, and one patient expired. With the use of CT, the diagnosis of capsular infarcts has moderately increased.
Aphasia ; Brain ; Consciousness ; Diagnosis ; Extremities ; Humans ; Hypertension ; Memory ; Neurologic Manifestations ; Paresis ; Quadriplegia ; Stroke ; Tremor

Stroke in young adults are not common and it is often hard to find their causes. We reviewed the medical records of 154 young adult patients aged 15-45 years who were admitted to the Seoul National Urliversity Hospital with a diagnosis of stroke between March 1989 and February 1991. These cases comprised 13.8% of 1115 patients of all ages admitted for stroke. The number of young adult patients with intracerebral hemorrhage was 47 (30.5%, N&154); the main causes were hypertension, arteriovenous malformation, and moyamoya disease. Subarachnoid hemorrhage was found in 25 young patients (l6.2%, N=154); the majority were due to aneurysms. The number of intraventricular hemorrhage cases was 17 (11.0%, N&154). The causes confirmed by angiography, were moyamoya disease in 7 cases and arteriovenous malformahon in 5 cases. The remaining 73 patients (47.4%, N&154) had cerebral infarction;the major causes were young-aged atherosclerosis (75.3%) and cardiogenic emboli (24.7%). Hypertension, history of transient ischemic attack, and hyperlipidemia were major risk factors of atherosclerotic cerebral infarction. Among the cerebral infarction patients whose risk factors had not been found by conventional diagnostic methods (l6 patients), there were 5 patients in whom the presence of cardiac embolic sources could be demonstrated only by transesophageal echocardiography (left atrial thrombus in two patients; patent foramen ovale in two; and atrial septal aneurvsm in one patient). The total number of all tvpes of young adult stroke patients whose causes (or risk factors) were unable to be classified in detail was 24 (15.6%, N&154). The transesophageal echocardiography and the angiography, in many occasions, were helpful in detecting the causes of young adult stroke of which risk factors had been undetermined.
Aneurysm ; Angiography ; Arteriovenous Malformations ; Atherosclerosis ; Cerebral Hemorrhage ; Cerebral Infarction ; Diagnosis ; Echocardiography, Transesophageal ; Foramen Ovale, Patent ; Hemorrhage ; Humans ; Hyperlipidemias ; Hypertension ; Ischemic Attack, Transient ; Medical Records ; Moyamoya Disease ; Risk Factors ; Seoul ; Stroke* ; Subarachnoid Hemorrhage ; Thrombosis ; Young Adult*

BACKGROUND: The survival rate of grafted fat is difficult to predict, and repeated procedures are frequently required. In this study, the effects of the freezing period of harvested adipose tissue and the addition of human adipose tissue-derived stem cells (ASCs) on the process of fat absorption were studied. METHODS: Adipose tissue was obtained from patients who underwent a lipoaspirated fat graft. The fat tissue was cryopreserved at -20degrees C in a domestic refrigerator. A total of 40 nude mice were used. The mice in the experimental group received three different subcutaneous injections in the back: an injection of fresh fat and ASCs, an injection of fat that had been frozen for one month and ASCs, and an injection of fat that had been frozen for two months and ASCs. The control mice received fat grafts without ASCs. The mice were sacrificed at four or eight weeks after the procedure, and the grafted fat tissues were harvested. The extracted fat was evaluated using photographic analysis, volume measurements, and histological examination. RESULTS: In the control group, the fat resorption rates four weeks after transplantation in the grafts of fresh fat, fat that had been frozen for one month, and fat that had been frozen for two months were 21.14%, 22.46%, and 42.56%, respectively. In the experimental group, the corresponding resorption rates were 6.68%, 13.0%, and 33.9%, respectively. CONCLUSIONS: ASCs can increase the fat graft survival rate. The use of ASCs in fat grafting can reduce the need for repeated fat grafts and provide good long term results.
Absorption ; Adipose Tissue ; Animals ; Cryopreservation ; Freezing ; Graft Survival ; Humans* ; Injections, Subcutaneous ; Mice ; Mice, Nude ; Stem Cells* ; Survival Rate* ; Transplants

Objectives: The purpose was to identify the ingredients that are usually surveyed for assessing real prices and to present the demand for such surveys by nutrition teachers and dietitians for ingredients used by school foodservice. Methods: A survey was conducted online from December 2019 to January 2020. The survey questionnaire was distributed to 1,158 nutrition teachers and dietitians from elementary, middle, and high schools nationwide, and 439 (37.9% return rate) of the 1,158 were collected and used for data analysis. Results: The ingredients which were investigated for price realities directly by schools were industrial products in 228 schools (51.8%), fruits in 169 schools (38.4%), and specialty crops in 166 schools (37.7%). Moreover, nutrition teachers and dietitians in elementary, middle, and high schools searched in different ways for the real prices of ingredients. In elementary schools, there was a high demand for price information about grains, vegetables or root and tuber crops, special crops, fruits, eggs, fishes, and organic and locally grown ingredients by the School Foodservice Support Centers. Real price information about meats, industrial products, and pickled processed products were sought from the external specialized institutions. In addition, nutrition teachers and dietitians in middle and high schools wanted to obtain prices of all of the ingredients from the Offices of Education or the District Office of Education. Conclusions Schools want to efficiently use the time or money spent on research for the real prices of ingredients through reputable organizations or to co-work with other nutrition teachers and dietitians. The results of this study will be useful in understanding the current status of the surveys carried out to determine the real price information for ingredients used by the school foodservice.

Objectives: The purpose was to identify the ingredients that are usually surveyed for assessing real prices and to present the demand for such surveys by nutrition teachers and dietitians for ingredients used by school foodservice. Methods: A survey was conducted online from December 2019 to January 2020. The survey questionnaire was distributed to 1,158 nutrition teachers and dietitians from elementary, middle, and high schools nationwide, and 439 (37.9% return rate) of the 1,158 were collected and used for data analysis. Results: The ingredients which were investigated for price realities directly by schools were industrial products in 228 schools (51.8%), fruits in 169 schools (38.4%), and specialty crops in 166 schools (37.7%). Moreover, nutrition teachers and dietitians in elementary, middle, and high schools searched in different ways for the real prices of ingredients. In elementary schools, there was a high demand for price information about grains, vegetables or root and tuber crops, special crops, fruits, eggs, fishes, and organic and locally grown ingredients by the School Foodservice Support Centers. Real price information about meats, industrial products, and pickled processed products were sought from the external specialized institutions. In addition, nutrition teachers and dietitians in middle and high schools wanted to obtain prices of all of the ingredients from the Offices of Education or the District Office of Education. Conclusions Schools want to efficiently use the time or money spent on research for the real prices of ingredients through reputable organizations or to co-work with other nutrition teachers and dietitians. The results of this study will be useful in understanding the current status of the surveys carried out to determine the real price information for ingredients used by the school foodservice.

To evaluate the current status of cerebrovascular disease in Korea, we performed a retrospective study on 1260 cases of stroke who had been admitted to or had visited Seoul National University Hospital, Choongnam National University Hospital, Hanyang University Hosipital, and Chung Ang University Hospital in the period from Jul 1, 1986 to Jun 30, 1987. The incidence was highest in sixth and seventh decades. Ischemic stroke (51.1%) was more common thar, hemorrhagic stroke (47.6%), and thrombotic infarction was the leading type (37.7%) of all kinds of strokes. Middle cerebral arterial territory was the most commonly involved site in thrombotic and embolic stroke. Among intracerebral hemorrhage, basal ganglia hemorrhage was found in 47.7%, and was followed by thalamic(23.8%), lobar (15.3%) and pontine hemorrhage (9.4%). Primary intraventricular hemorrhage (IVH) was relatively rare (8 cases, 7.5% of all IVH), and hemorrhages in basal ganglia or thalamus frequently induced secondary IVH. 16 cases (10.7%) of subarachnoid hamorrhage were accompanied by secondary IVH. This study suggests changing trends of stroke in Korea, and multicenter prospective study using stroke registry is requird for confirmation.
Basal Ganglia ; Basal Ganglia Hemorrhage ; Cerebral Hemorrhage ; Hemorrhage ; Incidence ; Infarction ; Korea* ; Retrospective Studies ; Seoul ; Stroke ; Thalamus

BACKGROUND: Carotid intima media thickness (CIMT) and the presence of carotid plaque have been used for risk stratification of cardiovascular disease (CVD). To date, however, the association between multi-directional functional properties of carotid artery and CVD has not been fully elucidated. We sought to explore the multi-directional mechanics of the carotid artery in relation to cardiovascular risk. METHODS: Four hundred one patients who underwent carotid ultrasound were enrolled between January 2010 and April 2013. A high risk of CVD was defined as more than 20% of 10-year risk based on the Framingham risk score. Using a speckle-tracking technique, the longitudinal and radial movements were analyzed in the B-mode images. Peak longitudinal and radial displacements, strain and strain rate were also measured. Beta stiffness and elastic modulus index were calculated from the radial measurements. RESULTS: Of the overall sample, 13% (52) of patients comprised the high-risk group. In multivariate logistic regression, CIMT and elastic modulus index were independently associated with a high-risk of CVD {odds ratio (OR): 1.810 [95% confidence interval (CI) 1.249–2.622] and OR: 1.767 (95% CI: 1.177–2.652); p = 0.002, 0.006, respectively}. The combination of CIMT and elastic modulus index correlated with a high-risk of CVD more so than CIMT alone. CONCLUSION: The elastic modulus index of the carotid artery might serve as a novel surrogate marker of high-risk CVD. Measurement of the multi-directional mechanics of the carotid artery using the speckle tracking technique has potential for providing further information over conventional B-mode ultrasound for stratification of CVD risk.
Biomarkers* ; Cardiovascular Diseases ; Carotid Arteries ; Carotid Intima-Media Thickness ; Elastic Modulus* ; Humans ; Logistic Models ; Mechanics ; Ultrasonography*