• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.95-99
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• 2003

Krabbe disease is a rare autosomal recessive disorder clinically characterized by retardation in motor development, prominent spasticity, seizures, and optic atrophy. Pathologically, there are many globoid cells in the white matter, in addition to the lack of myelin and the presence of severe gliosis. Hence Krabbe disease is known as globoid cell leukodystrophy. Biochemically, the primary enzymatic deficiency in Krabbe disease is galactocerebroside beta-galactosidase. Patients with Krabbe disease can be subdivided into the early-onset type and late-onset type, according to the onset of clinical manifestations. Most patients with early-onset type die before their second birthday. We describe a girl with Krabbe disease associated with uncontrolled seizures, which was confirmed with biochemical study and MRI. The clinical findings of this patient included hyperirritability, scissoring of the legs, flexion of arm, and clenching of the fists, and generalized tonic seizures. EEG showed hypsarrhythmia, and MRI demonstrated degenerative white matter changes in bilateral periventricular white matter, posterior rim of internal capsule, basal ganglia and brain stem on T2W1 and FLAIR image. The diagnosis was based on clinical features of progressive neurologic deterioration in conjunction with low galactocerebroside beta-galactosidase activity.

• Journal of the Institute of Electronics and Information Engineers
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• v.53 no.12
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• pp.131-138
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• 2016

In this study, we tried to analyze the influence of ICM(Iodinated Contrast Media) in MR imaging compare to GBCA(Gadolinium Based Contrast Agent), and as this result we discussed whether resonable or not the protocol which is MRI scan after enhanced CT scan without proper time interval in clinical field. For this research, we assembled two phantoms. which one was iodine and another one was gadolinium. We did test two phantoms in conventional MRI scan which is T1, T2, T2 FLAIR and 3D angio. After that, quantitative analysis was progressed. The results of study were as follow : SSI(Saline's Signal Intensity) was shown as each sequences 175, 1231, 333, 37 [a.u] at iodine. and 1297, 123, 757, 232 [a.u] was recorded at gadolinium. BDEPS(the Biggest Difference of EPS) was shown as each sequences 1297, 123, 757, 232 [a.u] at iodine and 793, 6, 1495, 365 [a.u] was recorded at gadolinium. At this time, EPS(Enhancement Percentage to Saline) was shown 641.1, -90.0, 127.3, 527% at iodine and 685.1, 99.4, 365.7, 1077.4% was recorded at gadolinium. BP(BDEPS's point) was shown 900, 900, 477, 900 mmol at iodine and 4, 0.2, 0.2, 40 mmol was recorded at gadolinium. CPSS(Change Point of SI to SSI) was shown 63, 423, 63, 29 mmol at iodine and each [50, 30], [4, 0.2], [4, 1], 0.2 mmol was recorded at gadolinium. According to this research, we could not only discover the fact that was iodine could effect on MR signal, but also the pattern is different as various sequences compare to gadolinium. Therefore, we expect useful diagnostic MR image in clinical field with this quantitative data for deciding protocol regarding MRI and CT scan order.

• Investigative Magnetic Resonance Imaging
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• v.20 no.2
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• pp.105-113
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• 2016

Purpose: Susceptibility vessel sign (SVS) on gradient echo image, which is caused by MR signal loss due to arterial thrombosis, has been reported in acute middle cerebral artery (MCA) infarction. However, the reported sensitivity and diagnostic accuracy of SVS have been variable. Susceptibility-weighted imaging (SWI) is a newly developed MR sequence. Recent studies have found that SWI may be useful in the field of cerebrovascular diseases, especially for detecting the presence of prominent veins, microbleeds and the SVS. The purpose of this study was to evaluate the diagnostic values of SWI for the detection of hyperacute MCA occlusion. Materials and Methods: Sixty-nine patients (37 males, 32 females; 46-89 years old [mean, 69.1]) with acute stroke involving the MCA territory underwent MR imaging within 6 hours after the symptom onset. MR examination included T2, FLAIR (fluid-attenuated inversion recovery), DWI, SWI, PWI (perfusion-weighted imaging), contrast-enhanced MR angiography (MRA) and contrast-enhanced T1. Of these patients, 28 patients also underwent digital subtraction angiography (DSA) within 2 hours after MR examination. Presence or absence of SVS on SWI was assessed without knowledge of clinical, DSA and other MR imaging findings. Results: On MRA or DSA, 34 patients (49.3%) showed MCA occlusion. Of these patients, SVS was detected in 30 (88.2%) on SWI. The sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy of SWI were 88.2%, 97.1%, 96.8%, 89.5% and 92.8%, respectively. Conclusion: SWI was sensitive, specific and accurate for the detection of hyperacute MCA occlusion.