• Clinical and Experimental Pediatrics
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• v.54 no.6
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• pp.234-240
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• 2011

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.

• The Journal of Korean Medicine
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• v.21 no.1
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• pp.84-90
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• 2000

Background and Purpose: Stroke in young adults is relatively uncommon. Only 3.7-14.4% of all strokes occur in patients aged 15-45 years. Stroke in young adults has more various and diverse possible causes than stroke in the elderly. We studied to gain further insight into both pathogenic and etiologic determinants in young adults with stroke. Methods : We retrospectively reviewed the medical records of 230 young patients aged 15-45 years who were admitted to the 2nd internal medicine department of Kyung Hee Oriental Medical Center with a diagnosis of stroke between May 1995 and May 1999. We analysed clinical features and diagnostic tests, such as brain imaging, cerebral angiography, echocardiography, 24 hours holter monitoring and other laboratory tests. Results : 1. Of 230 young patients with stroke aged 15-45 years(176 males(76.5%) and 54 females(23.5%)), 140 patients(60.9%) showed ischemic stroke and 90 patients(39.1 %) showed hemorragic stroke. 2. The most prevalent age group was from 40 to 45 years with 142 patients(61.7%) 3. The most frequent site of 140 ischemic stroke was MCA territory in 93 cases(66.4%) and Multiple, VA territory, PCA territory, ACA territory in order of frequency. 4. The most frequent site of 90 hemorrhagic stroke was basal galglia hemorrhage 57 cases(63.3%) and subcortical 13 cases(14.5%), pons, thalmus, subarachnoid, cerebellum in order of frequency. 5. The causes of hemorrhagic stroke were hypertension 49 cases(54.5%), arteriovenous malformation 7 cases(7.8%), ruptured aneurysm 4 cases(4.5%), angioma 3 cases(3.3%). 6. The risk factors of ischemic stroke were smoking, alcohol drinking, hyperlipidemia, hypertension, obesity, heart disease, history of CVA, diabetes mellitus, in order of frequency. 7. The comparison of risk factors between ischemic and hemorrhagic stroke: hypertension was prevalent in hemorrhagic stroke, heart disease and history of CVA were prevalent in ischemic stroke. Conclusions: From the above results, we found that stroke in young adults had various possible causes. Young adults with stroke deserve an extensive but tailored evaluation which include angiography and echocardiography for diagnosis.

• Journal of Medicine and Life Science
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• v.17 no.3
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• pp.65-73
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• 2020

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a single-gene disease of the cerebral small blood vessels caused by mutations in the NOTCH3 gene on chromosome 19. Although CADASIL was known as a rare disease, recent research has suggested that the NOTCH variants could be found frequently even in the general population. The main clinical features included recurrent stroke, migraine, psychiatric symptoms, and progressive cognitive decline. On brain magnetic resonance imaging, patients with CADASIL showed multifocal white matter hyperintensity lesions, lacunar infarcts, microbleeds, and brain atrophy. Among them, lacunar infarcts and brain atrophy are important in predicting the clinical outcomes of patients with CADASIL. In the Jeju National University Hospital, we have diagnosed 213 CADASIL patients from 2004 to 2020. Most NOTCH3 mutations were located in exon 11 (94.4%), and p.Arg544Cys was the most common mutation. The mean age at diagnosis was 61.0±12.8 years. The most common presenting symptoms were ischemic stroke (24.4%), followed by cognitive impairment(15.0%), headache (8.9%), and dizziness(8.0%). Although the exact prevalence of CADASIL in Jeju is still unknown, the disease prevalence could be as high as 1% of the population considering the prevalence reported in Taiwan. Therefore, it is necessary to discover efficient biomarkers and genetic tests that can accurately screen and diagnose patients suspected of having CADASIL in this region. Ultimately, it is urgent to explore the exact pathogenesis of the disease to identify leading substances of treatment potential, and for this, multi-disciplinary research through active support from the Jeju provincial government as well as the national government is essential.

• Sleep Medicine and Psychophysiology
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• v.5 no.2
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• pp.119-133
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• 1998

Attentional processes facilitate cognitive and behavioral performance in several ways. Attention serves to reduce the amount of information to receive. Attention enables humans to direct themselves to appropriate aspects of external environmental events and internal operations. Attention facilitates the selection of salient information and the allocation of cognitive processing appropriate to that information. Attention is not a unitary process that can be localized to a single neuroanatomical region. Before the cortical registration of sensory information, activation of important subcortical structures occurs, which is called as an orienting response. Once sensory information reaches the sensory cortex, a large number of perceptual processes occur, which provide various levels of perceptual resolution of the critical features of the stimuli. After this preattentional processing, information is integrated within higher cortical(heteromodal) systems in inferior parietal and temporal lobes. At this stage, the processing characteristics can be modified, and the biases of the system have a direct impact on attentional selection. Information flow has been traced through sensory analysis to a processing stage that enables the new information to be focused and modified in relation to preexisting biases. The limbic and paralimbic system play significant roles in modulating attentional response. It is labeled with affective salience and is integrated according to ongoing pressures from the motivational drive system of the hypothalamus. The salience of information greatly influences the allocation of attention. The frontal lobe operate response selection system with a reciprocal interaction with both the attention system of the parietal lobe and the limbic system. In this attentional process, the search with the spatial field is organized and a sequence of attentional responses is generated. Affective, motivational and appectitive impulses from limbic system and hypothalamus trigger response intention, preparation, planning, initiation and control of frontal lobe on this process. The reticular system, which produces ascending activation, catalyzes the overall system and increases attentional capacity. Also additional energetic pressures are created by the hypothalamus. As psychophysiological measurement, skin conductance, pupil diameter, muscle tension, heart rate, alpha wave of EEG can be used. Event related potentials also provide physiological evidence of attention during information process. NI component appears to be an electrophysiological index of selective attention. P3 response is developed during the attention related to stimulus discrimination, evaluation and response.

• Investigative Magnetic Resonance Imaging
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• v.7 no.1
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• pp.47-55
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• 2003

Purpose : Cortical dysplasia is known to be of variety of MR imaging findings. We attempted to classify MR imaging findings of cortical dysplasia into several types and to correlate those with histopathologic grades and subtypes. Materials and Methods : Preoperative MR images of 97 patients with pathologically-proven cortical dysplasia were retrospectively reviewed with knowledge of the diagnosis and operative sites. The patients were divided into MR-positive and MR-negative groups based on the presence or absence of MR imaging abnormalities. In MR-positive group, MR imaging features were arbitrarily classified into four types (atrophic, cortical-band, inward-rounding, and nonspecific types) on the basis of size of the gyrus and adjacent CSF space, cortical thickness, signal intensity of the subcortical white matter, and blurring of the gray-white matter junction. The pathologic findings were also retrospectively reviewed without knowledge of MR imaging findings and divided into three grades (mild, moderate, and severe) and two subtypes (nonballoon-cell and balloon-cell). Pathologic grades and subtypes we re compared between MR-positive and MR-negative groups. Four MR types of the MR-positive group were correlated with the pathologic grades and subtypes. Results : MR-positive and MR-negative groups consisted of 39 (40%) and 58 (60%) patients, respectively. Of the MR-positive group, atrophic type was seen in 13 patients (33 %), cortical-band type in 9 (23%), inward-rounding type in 9 (23%), and nonspecific type in 8 (21%). There was no significant difference in the pathologic grades between MR-positive and MR-negative groups, although MR-positive group tended to have higher pathologic grades than MR-negative group did. Balloon-cell subtype was found significantly higher in MR-positive group than in MR-negative group (p<0 .05): 21% (8/39) versus 5% (3/58). The inward-rounding type corresponded to the pathologically severe grade and balloon-cell subtype in 78% (7/9) and 56% (5/9) of the patients, respectively, while the atrophic type to the mild grade and nonballoon-cell subtype in 77% (10/13) and 100% (13/13), respectively. Conclusion : A variety of MR imaging abnormalities were found in 40% of the patients with cortical dysplasia and those were classified into four types (atrophic, cortical-band, inward-rounding, and nonspecific types), of which the inward-rounding type correlated well with the pathologically severe grade and balloon-cell subtype, whereas the atrophic type with the mild grade and nonballoon-cell subtype.