• Korean Journal of Biological Psychiatry
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• v.15 no.1
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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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• v.20 no.2
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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.

• Korean Journal of Biological Psychiatry
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• v.22 no.3
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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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• v.30 no.8
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• pp.985-991
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• 2001

Objectives : The craniopharyngioma is a benign tumor located at least in part in the suprasellar cistern. However, the symptoms and signs from this tumor may be determined not only by the location of the tumor but also by its size and the age of the patient. The objective of our study is to analyze retrospectively the clinical manifestations of craniopharyngiomas with regards to tumor characteristics in children and adults. Material and Methods: Twenty-three patients(16 adults, 7 children) treated for craniopharyngioma between 1990 and 1999 were studied to demonstrate the relationship of tumor size, growth pattern, and its invasiveness with clinical symptoms. As part of the assessment, 16 adults(M : F=8 : 8, mean age : 43.7 years) and 7 children(M : F=5 : 2, mean age : 10.1 years) underwent magnetic resonance(MR) imaging and computerized tomography(CT) scanning with a three-dimensional volume acquisition sequence. Results : The three major cardinal signs were defined to increased intracranial pressure, endocrine dysfunction, and visual problems. The tumor size in child group was larger than that in adult group. Also, visual problems, symptoms of increased intracranial pressure and hydrocephalus were more frequently observed in child group. However, endocrine dysfunction and neuropsychological symptoms related with hypothalamic connections to the thalamus, pituitary, frontal lobe, and other cortical areas were more frequent in adult group. Conclusions: In our series, the tumor size and invasiveness of craniopharyngioma revealed to be relevent with initial symptoms of increased intracranial pressure and visual symptoms which were more frequent in child group. As for the growth pattern, we did not find major difference between adults and children.