• 생물정신의학
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• 제15권1호
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• pp.46-53
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• 2008

Posterior cortical atrophy(PCA) is a presenile dementia that presents primarily with signs and symptoms of cortical visual dysfunction, while memory is relatively preserved until the late stage of the disease. We report a patient with PCA, confirmed by brain magnetic resonance imaging (MRI) and $F^{18}$-fluorodeoxyglucose positron emission tomography(FDG PET). A 58-year-old right-handed woman presented initially with visual dimness and difficulty finding things around her. She had partial Balint's syndrome, partial Gerstmann syndrome, and idiomotor apraxia. She also had a mild memory disturbance, but preserved insight of her disease. Neuropsychological evaluation showed decreased parietal and left temporal functions bilaterally. Brain MRI and $F^{18}$-FDG PET revealed typical bilateral occipitoparietal atrophy and hypometabolism, which were slightly worse on the right side. Cholinesterase inhibitor administration for 6 months improved the memory impairment slightly, but not the cortical visual dysfunction. This is a typical case of PCA, confirmed by neurologic signs and imaging findings.

• Annals of Clinical Neurophysiology
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• 제20권2호
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• pp.85-88
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• 2018

Septo-optic dysplasia is a congenital anomaly with diverse phenotypes from normal to mixtures of visual abnormality, endocrine dysfunction, psychomotor retardations and epileptic seizures. It is characterized by optic atrophy, pituitary dysfunction and midline structure abnormalities in corpus callosum or septum pellucidum. Diagnosis of septo-optic dysplasia plus is made when cortical malformations accompanied. Here we report a middle-aged woman with septo-optic dysplasia plus having unilateral optic atrophy, agenesis of septum pellucidum and cortical malformations.

• 생물정신의학
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• 제22권3호
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• pp.140-148
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• 2015

Objectives The aim of this study is to investigate the correlation between degenerative changes in brain [i.e., global cortical atrophy (GCA), medial temporal atrophy (MTA), white matter hyperintensities (WMH)] and neurocognitive dysfunction in Korean patients with Alzheimer's disease. Methods A total of 62 elderly subjects diagnosed with Alzheimer's disease were included in this study. The degenerative changes in brain MRI were rated with standardized visual rating scales (GCA or global cortical atrophy, MTA or medial temporal atrophy, and Fazekas scales) and the subjects were divided into two groups according to the degree of degeneration for each scale. Cognitive function was evaluated with Korean version of the Consortium to Establish a Registry for Alzheimer's Disease (CERAD-K) and several clinical features, including apolipoprotein E ${\varepsilon}4$ status, lipid profile and thyroid hormones, were also examined. Chi-square test and Fisher's exact test were performed to analyze the relationship between the degree of cerebral degeneration and neurocognitive functions. Results Demographic and clinical features, except for the age, did not show any significant difference between the two groups divided according to the degree of cerebral degenerative changes. However, higher degree of GCA was shown to be associated with poorer performance in verbal fluency test, word list recall test, and word list recognition test. Higher degree of MTA was shown to be associated with poorer performance in Mini-Mental State Examination in the Korean Version of CERAD Assessment Packet (MMSE-KC), word list recognition test and construction praxis recall test. Higher degree of white matter hyperintensities was shown to be associated with poorer performance in MMSE-KC. Conclusions Our results suggest that severe brain degeneration shown in MRI is associated with significantly poorer performance in neurocognitive tests in patients with Alzheimer's disease. Moreover, the degree of GCA, MTA and white matter hyperintensities, represented by scores from different visual rating scales, seems to affect certain neurocognitive domains each, which would provide useful information in clinical settings.

• Journal of Korean Neurosurgical Society
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• 제30권8호
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• pp.985-991
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• 2001

목 적 : 두개인두종은 적어도 일부분이 상부 터어키안에 존재하는 양성종양이다. 그러나 이 종양으로 인한 증상과 징후는 종양의 위치뿐만 아니라 그 크기와 환자의 나이등에 영 향을 받는다고 알려져 있다. 이번 연구에서 본 저자들은 성인과 소아에서 증상과 연관된 두개인두종의 임상적 특징을 후향적으로 분석하였다. 방법 및 대상 : 1990년부터 1999년까지 두개인두종으로 치료를 받은 23명(성인 : 16명, 소아 : 7명)을 대상으로 종양의 크기, 성장양상과 주변조직으로의 침윤정도와 증상과의 연관성을 연구하였다. 분석을 위해 16명의 성인(남자 : 8명, 여자 : 8명, 평균연령 : 43.7세)과 7명의 소아(남자 : 5명, 여자 : 2명, 평균연령 : 10.1세)에게 MRI 및 CT를 시행하였으며 3차원적으로 종양의 부피를 측정하였다. 결 과 : 두개인두종에서 3대 주증상은 두개강 내압상승과 연관된 증상, 내분비 기능 이상, 시력이상이다. 종양의 크기는 소아군에서 성인군보다 컸으며 시력이상, 두개강 내압 상승과 연관된 증상, 뇌수두증도 소아군에서 더 많았다. 그러나 내분비 기능 이상과 시상하부와 시상, 뇌하수체, 전두엽과 다른 피질부위와 연결의 이상으로 인한 신경정신적과적인 증상은 성인군에서 더 많았다. 결 론 : 이번 연구에서 두개인두종은 종양의 크기와 침윤성은 두개강 내압 항진과 연관된 증상 및 시력 증상과 연관이 많으며 소아군에서 더 흔하게 나타났다. 하지만 종양의 성장 양상은 소아군과 성인군에서 주된 차이를 발견하지 못했다.