• 생물정신의학
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• 제4권1호
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• pp.60-66
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• 1997

서울대학교병원 신경정신과에 입원하여 뇌자기공명영상촬영을 시행한 32명(남자 18명, 여자 12명)의 양극성장애환자를 대상으로 피질하 $T_2$ 강조-고신호광도의 임상적 변수에 관한 연구를 시행하여 다음과 같은 결과를 얻었다. 1) 32명의 환자중 피질하 고신호광도를 보인 환자의 수는 7명(21.8%)이었고, 8명의 40세 이상의 환자중에서는 5명(62%)이 고신호광도를 보였다. 2) 피질하 고신호광도를 보인 환자군이 그렇지 않은 군에 비해 통계적으로 유의한 수준으로 연령이 높았고(p<0.01), 연령과 정량화된 피질하 고신호광도 점수와의 상관분석에서도 유의한 양의 상관관계를 보였다(r=0.51, p<0.01). 3) 피질하 고신호광도를 보인 환자군에서 정신병적 증상의 빈도는 증가되고(p=0.06), 정동장애 가족력의 빈도는 감소한 경향을 보였다(p=0.01). 이상의 결과는 발병연령이 늦은 양극성장애 환자의 상당수에서 발견되는 피질하 고신호광도가, 유전적요인에 주로 영향을 받는 조기 발병군과는 다른 경과나 임상양상 등을 보이는 만발성 양극성장애 환자의 원인적 요소일 수 있다는 가설을 뒷받침해주는 소견이다.

• Investigative Magnetic Resonance Imaging
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• 제25권2호
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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.

• Annals of Clinical Neurophysiology
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• 제20권2호
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• pp.79-84
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• 2018

Background: Pulsatility of cerebral arteries and aortic stiffness have been associated with white matter hyperintensities (WMH). We explored which is better correlated with the severity of WMH in a population with acute lacunar infarct. Methods: We included patients with acute small subcortical infarcts who underwent transcranial Doppler (TCD) and brachial ankle pulse wave velocity (baPWV). Exclusion criteria were any stenosis or occlusion on major cerebral arteries on magnetic resonance angiography; poor temporal insonation windows; ankle brachial index < 0.9; and atrial fibrillation. We assessed the performance of the pulsatility index of bilateral middle cerebral arteries (PI-MCA) and baPWV for predicting moderate-to-severe WMH, defined as an Age Related White Matter Changes score > 5, and then sought to find independent predictors using binary logistic regression analysis. Results: Eighty-three patients (56 males, mean age $61.5{\pm}11.4$) participated in the study. Uni-variate analysis showed old age and high PI-MCA were significantly correlated with moderate-to-severe WMH. However, baPWV was not associated with the severity of WMH. Multivariate analysis revealed old age (odds ratio per 1-year increase, 1.068; p = 0.044) and upper tertile of PI-MCA (odds ratio, 5.138; p = 0.049) were independently associated with moderate-to-severe WMH. Receiver-operating characteristics showed PI-MCA differentiated those with and without moderate-to-severe WMH with an area under the curve of 0.719. Conclusions: PI-MCA derived from TCD was better correlated with the severity of WMH than baPWV in a population with lacunar infarction. Pulsatility of cerebral arteries may better predict cerebral small vessel disease than the aortic stiffness index.