• Clinical and Experimental Pediatrics
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• v.53 no.12
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• pp.994-999
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• 2010

Purpose: Mitochondrial dysfunction can present with various symptoms depending on the organ it has affected. This research tried to analyze the ophthalmologic symptoms and ophthalmologic examination (OE) results in patients with mitochondrial disease (MD). Methods: Seventy-four patients diagnosed with mitochondrial respiratory chain complex defect with biochemical enzyme assay were included in the study. They were divided into 2 groups based on the OE results by funduscopy and were analyzed on the basis of their clinical features, biochemical test results, morphological analysis, and neuroimaging findings. Results: Thirty-seven (50%) of the 74 MD patients developed ophthalmologic symptoms. Abnormal findings were observed in 36 (48.6%) patients during an OE, and 16 (21.6%) of them had no ocular symptoms. Significantly higher rates of prematurity, clinical history of epilepsy or frequent apnea events, abnormal light microscopic findings in muscle pathology, diffuse cerebral atrophy in magnetic resonance imaging, and brainstem hyperintensity and lactate peaks in magnetic resonance spectroscopy were noted in the group with abnormal OE results. Conclusion: Although the ophthalmologic symptoms are not very remarkable in MD patients, an OE is required. When the risk factors mentioned above are observed, a more active approach should be taken in the OE because a higher frequency of ocular involvement can be expected.

• Journal of Veterinary Clinics
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• v.33 no.1
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• pp.10-15
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• 2016

The purpose of this study was to establish reproducible ischemic infarction model using allogenic blood clot in beagle dogs and identify induced ischemic lesion after middle cerebral artery occlusion using magnetic resonance imaging (MRI) and histopathologic findings. Twenty eight male beagle dogs with no evidence of neurologic disease were experimented. Allogenic embolus was made using a healthy beagle dog. After internal carotid artery (ICA) was exposure, 16G catheter was introduced through the ICA. The dog was administered 0.3 ml blood clot for 15 seconds followed by 3 ml of saline for 15 seconds. MRI scans were performed with 1.5T to evaluate ischemic lesion at 7 days after middle cerebral artery occlusion procedure. Evaluation parameters of MRI include location, distribution, infarction type, margin, shape, mass effect and intensity of T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), fluid attenuated inversion recovery (FLAIR) sequence, diffusion weighted imaging (DWI) and apparent diffusion coefficient (ADC). On MRI, all dogs (28/28) showed focal or multifocal lesion including telencephalon and thalamus lesions, especially caudate nucleus (24/28). These lesions had well-defined margin from adjacent brain parenchyma, none or mild mass effect and various shape. Most of dogs appeared hyperintensity on T1WI, T2WI, FLAIR, and DWI/ADC, corresponding to chronic infarction. These lesions were histopathologically confirmed atrophic changes and unstained lesion. In conclusion, MRI is the useful method to provide information about ischemic infarction in dogs and the best reproducible ischemic infarction model was developed by using allogenic blood clot.

• Clinics in Shoulder and Elbow
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• v.12 no.1
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• pp.89-93
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• 2009

Purpose: Kimura's disease is an uncommon benign lymphoproliferative inflammatory disorder with an unknown etiology. The recurrence rate after surgical excision is relatively high and renal involvement is its only systemic manifestation. The condition mainly involves the head and neck, and peripheral involvement is extremely rare. Materials and Methods: We encountered the case of a 28-year-old man who had a non-tender mass with mild brownish skin color changes and pruritus around the medial side of the distal arm and elbow. Results: The peripheral blood investigation revealed peripheral eosinophilia and elevated serum IgE levels. Magnetic resonance imaging showed an isointensity signal relative to the muscle on the T1-weighted images and hyperintensity signal relative to the muscle on the T2-weighted images. Conclusion: A marginal resection of the lesion was performed and there was no recurrence at 2 years postoperatively.

• Journal of Korean Neurosurgical Society
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• v.39 no.3
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• pp.210-214
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• 2006

Objective : We investigate risk factors of cerebral microbleeds[MBs] and their relation to concomitant magnetic resonance[MR] findings in intracerebral hemorrhages[ICHs] patients. Methods : We studied 100 consecutive patients with primary ICH over a 1-year period. These patients underwent brain MR images using 3.0-T scanners within the first week of the hemorrhage. MBs and old hematomas were located and counted by using $T2^*-weighted$ gradient-echo MR imaging. We also counted lacunes and graded white matter and periventricular hyperintensity on T1- and T2-weighted spin-echo sequences. The association between MBs and vascular risk factors and MR abnormalities were analyzed. Results : MBs were seen in 77 of ICH patients, and their number ranged from 1 to 65 lesions [mean 11, median 6]. The locations of MBs were subcortex-cortex [40.6%], basal ganglia [26.7%], thalamus [14.1 %], brain stem [12.5%], and cerebellum [9.1 %]. Analysis of clinical data revealed that age, hypertension, history of stroke, and duration of hypertension were frequently associated with MBs. The incidence of lacunes, old hematomas, and advanced leukoaraiosis was significantly higher in the MBs group, compared with the patients without MBs. Conclusion : MBs are frequently observed in ICH patients with advancing age, chronic hypertension, and previous hemorrhagic stroke, and are also closely related with morphological signs of occlusive type microangiopathy, such as lacunar infarct and severe leukoaraiosis.

• Psychiatry investigation
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• v.15 no.12
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• pp.1162-1167
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• 2018

Objective Motor, perceptual, and cognitive functions are known to affect driving competence. Subcortical ischemic changes on brain magnetic resonance imaging (MRI) can reflect reduction in cognitive and motor performance. However, few studies have reported the relationship between subcortical ischemic changes and driving competence of the elderly. Thus, the objective of this study was to investigate the association between subcortical ischemic changes on MRI and driving abilities of the elderly. Methods Participants (n=540) were drawn from a nationwide, multicenter, hospital-based, longitudinal cohort. Each participant underwent MRI scan and interview for driving capacity categorized into 'now driving' and 'driving cessation (driven before, not driving now)'. Participants were divided into three groups (mild, n=389; moderate, n=116; and severe, n=35) depending on the degree of white matter hyperintensity (WMH) on MRI at baseline. Driving status was evaluated at follow-up. Statistical analyses were conducted using ${\chi}^2$ test, analysis of variance (ANOVA), structured equation model (SEM), and generalized estimating equation (GEE). Results In SEM, greater baseline degree of WMH was directly associated with driving cessation regardless of cognitive or motor dysfunction (${\beta}=-0.110$, p<0.001). In GEE models after controlling for age, sex, education, cognitive, and motor dysfunction, more severe change in the degree of WMH was associated with faster change from 'now driving' state to 'driving cessation' state over time in the elderly (${\beta}=-0.508$, p<0.001). Conclusion In both cross-sectional and longitudinal results, the degree of subcortical ischemic change on MRI might predict driving cessation in the elderly.

• Journal of the Korean neurological association
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• v.36 no.4
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• pp.302-309
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• 2018

Background: Although orthostatic hypotension (OH) and cognitive impairment (CI) are common non- motor symptoms of Parkinson's disease (PD), the relationship between OH and CI remains to be clarified. This study was aimed to investigate the relationship between OH and CI in PD. Methods: We recruited 192 patients who were diagnosed as PD based on the UK Brain Bank diagnostic criteria. The Hoehn & Yahr stages were ranged I to III and patients underwent extensive clinical evaluation, including brain magnetic resonance imaging (MRI) for cerebral white matter hyperintensity (WMH), tilt table test, the Korean version of Montreal Cognitive Assessments and the Korean version of Mini-Mental Status Examination in one month from the first clinic visit. The participants were divided into two groups according to the presence of OH (OH+ vs. OH-) and cognitive function (cognitive normal, CN vs. CI), respectively. Results: Significant relationship between OH and cognitive function (p=0.04) was found in our patients. The patients with OH+ had higher risk of CI by 2.6 times than that of OH- patients. Maximum heart rate change during tilt table test was correlated with cognitive function and white matter changes, whereas blood pressure change during tilt table test showed no correlation with those parameters. Conclusions: There was significant relationship between OH and CI in PD. Therefore, PD patients with either symptom may need periodic evaluation and proper management for OH and cognitive functions.

• 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.

• Investigative Magnetic Resonance Imaging
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• v.25 no.2
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• pp.101-108
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• 2021

Purpose: To identify characteristic magnetic resonance imaging (MRI) features to differentiate between Krabbe disease and metachromatic leukodystrophy (MLD) in young children. Materials and Methods: We collected all confirmed cases of Krabbe disease and MLD between October 2004 and September 2020 at Seoul National University Children's Hospital. Patients with initial MRI available were included. Their initial MRIs were retrospectively reviewed for the following: 1) presence of white matter signal abnormality involving the periventricular and deep white matter, subcortical white matter, internal capsule, brainstem, and cerebellum; 2) presence of volume decrease and signal alteration in the corpus callosum and thalamus; 3) presence of the tigroid sign; 4) presence of optic nerve hypertrophy; and 5) presence of enhancement or diffusion restriction. Results: Eleven children with Krabbe disease and 12 children with MLD were included in this study. There was no significant difference in age or symptoms at onset. Periventricular and deep white matter signal alterations sparing the subcortical white matter were present in almost all patients of the two groups. More patients with Krabbe disease had T2 hyperintensities in the internal capsule and brainstem than patients with MLDs. In contrast, more patients with MLD had T2 hyperintensities in the splenium and genu of the corpus callosum. No patient with Krabbe disease showed T2 hyperintensity in the corpus callosal genu. A decrease in volume in the corpus callosum and thalamus was more frequently observed in patients with Krabbe disease than in those with MLD. Other MRI findings including the tigroid sign and optic nerve hypertrophy were not significantly different between the two groups. Conclusion: Signal abnormalities in the internal capsule and brainstem, decreased thalamic volume, decreased splenial volume accompanied by signal changes, and absence of signal changes in the callosal genu portion were MRI findings suggestive of Krabbe disease rather than MLD based on initial MRI. Other MRI findings such as the tigroid sign could not help differentiate between these two diseases.

• Journal of Yeungnam Medical Science
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• v.40 no.2
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• pp.136-145
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• 2023

Hepatic encephalopathy (HE) is a severe neuropsychiatric abnormality in patients with either acute or chronic liver failure. Typical brain magnetic resonance imaging findings of HE are bilateral basal ganglia high signal intensities due to manganese deposition in chronic liver disease and hyperintensity in T2, fluid-attenuated inversion recovery, or diffusion-weighted imaging (DWI) with hemispheric white matter changes including the corticospinal tract. Low values on apparent diffusion coefficient mapping of the affected area on DWI, indicating cytotoxic edema, can be observed in acute HE. However, neuropsychological impairment in HE ranges from mild deficits in psychomotor abilities affecting quality of life to stupor or coma with higher grades of hepatic dysfunction. In particular, the long-lasting compensatory mechanisms for the altered metabolism in chronic liver disease make HE imaging results variable. Therefore, the clinical relevance of imaging findings is uncertain and differentiating HE from other metabolic diseases can be difficult. The recent introduction of concepts such as "acute-on-chronic liver failure (ACLF)," a new clinical entity, has led to a change in the clinical view of HE. Accordingly, there is a need to establish a corresponding concept in the field of neuroimaging diagnosis. Herein, we review HE from a historical and etiological perspective to increase understanding of brain imaging and help establish an imaging approach for advanced new concepts such as ACLF. The purpose of this manuscript is to provide an understanding of HE by reviewing neuroimaging findings based on pathological and clinical concepts of HE, thereby assisting in neuroimaging interpretation.

• Journal of the Korean Society of Radiology
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• v.81 no.4
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• pp.985-989
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• 2020

Chlorfenapyr is widely used as an insecticide, despite it being fatal to humans. However, chlorfenapyr-induced central nervous system toxicity has rarely been reported. We report the magnetic resonance imaging (MRI) findings in a rare case of chlorfenapyr-induced toxic leukoencephalopathy. A 71-year-old man who had ingested chlorfenapyr approximately two weeks prior visited our hospital and presented with bilateral lower motor weakness and voiding dysfunction that had developed two days before admission. Brain MRI revealed extensive bilateral white matter abnormalities involving the corpus callosum, internal capsule, brain stem, and bilateral middle cerebellar peduncle. Furthermore, spine MRI revealed diffuse swelling and hyperintensity on the T2-weighted images.