Marchiafava-Bignami disease (MBD) is a rare disorder of demyelination or necrosis of the corpus callosum. Mainly, MBD is associated with alcohol and malnutrition. We report a 60-year-old woman with no history of alcohol consumption or malnutrition who had MBD as a possible complication of normal pressure hydrocephalus (NPH). The patient presented with a 2-month history of progressive gait unsteadiness, urinary incontinence, and forgetfulness, for which the patient underwent ventriculoperitoneal shunt surgery with remarkable improvement. Magnetic resonance imaging (MRI) demonstrated hyperintensity in the body and splenium of corpus callosum when she was brought to the hospital again with rapid deterioration of her mental ststus. It might be postulated that cerebrospinal fluid (CSF) tumor necrosis factor-alpha (TNF-alpha) might have contributed to the development of MBD although not measured in this patient, given that TNF-alpha, as a proinflammatory cytokine mediating demyelinating process have been found in be increased in the CSF of NPH.
Alcohol Drinking ; Cerebrospinal Fluid ; Corpus Callosum ; Demyelinating Diseases ; Female ; Gait ; Humans ; Hydrocephalus, Normal Pressure* ; Magnetic Resonance Imaging ; Malnutrition ; Marchiafava-Bignami Disease* ; Middle Aged ; Necrosis ; Negotiating ; Tumor Necrosis Factor-alpha ; Urinary Incontinence ; Ventriculoperitoneal Shunt

Progressive multifocal leukoencephalopathy (PML) is a very rare and often fatal demyelinating disease of central nervous system (CNS), which mostly occurs in patients with immunosuppression such as acquired immunodeficiency syndrome (AIDS) patients, transplant patients and patients receiving chemotherapy. PML usually manifests with acute or subacute neurologic deficit. and its late diagnosis may lead death or significant permanent disability. We report a 33-year old man diagnosed with PML in AIDS, who initially presented with gradual onset of dementia. Most symptoms of PML were progressed rapidly for several months, and characterized by focal neurological symptoms. On the other hand, we were experienced in patients without focal neurological symptoms and ongoing overall cognitive decline slowly. Patients with immunosuppression can be presented in a variety of neurological symptoms, detailed examinations for cognitive functions were needed in early stage of the disease.
Acquired Immunodeficiency Syndrome* ; Adult ; Central Nervous System ; Delayed Diagnosis ; Dementia* ; Demyelinating Diseases ; Drug Therapy ; Hand ; Humans ; Immunosuppression ; Leukoencephalopathy, Progressive Multifocal* ; Neurologic Manifestations

No abstract available.
Amnesia, Transient Global* ; Infarction*

BACKGROUND: Alzheimer's disease is a chronic neurodegenerative condition, mostly affecting the medial temporal lobe and associated neocortical structures. In this report, we present a rare clinical manifestation of this disease. CASE REPORT: A 61-year-old female with word finding difficulty and memory disturbances was diagnosed with Alzheimer's disease. Two years later, she complained of right homonymous hemianopia without optic ataxia, ocular apraxia, and simultagnosia. No findings other than parenchymal disease were apparent in magnetic resonance imaging and laboratory tests. CONCLUSIONS: In this case, in a patient initially diagnosed with Alzheimer's dementia with progressive disease, we found only homonymous hemianopia, without signs of Balint's syndrome or Gerstmann's syndrome. After careful investigation showing that Alzheimer's dementia with visual symptom was not associated with parenchymal disease, we concluded a case of atypical variant of Alzheimer's disease.
Alzheimer Disease ; Apraxias ; Ataxia ; Dementia* ; Female ; Gerstmann Syndrome ; Hemianopsia* ; Humans ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Temporal Lobe

BACKGROUND AND PURPOSE: Amyloid beta (Aβ) is the main component of amyloid plaques, which are deposited in the brains of patients with Alzheimer's disease (AD). Biochemical and animal studies support the central role of Aβ in AD pathogenesis. Despite several investigations focused on the pathogenic mechanisms of Aβ, it is still unclear how Aβ accumulates in the central nervous system and subsequently initiates the disease at the cellular level. In this study, we investigated the pathogenic mechanisms of Aβ using proteomics and antibody microarrays. METHODS: To evaluate the effect of Aβ on neural stem cells (NSCs), we treated primary cultured cortical NSCs with several doses of Aβ for 48 h. A 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyltetrazolium bromide assay, trypan blue staining, and bromodeoxyuridine cell proliferation assay were performed. We detected several intracellular proteins that may be associated with Aβ by proteomics and Western blotting analysis. RESULTS: Various viability tests showed that Aβ decreased NSCs viability and cell proliferation in a concentration-dependent manner. Aβ treatment significantly decreased lactate dehydrogenase B, high-mobility group box 1, aldolase C, Ezrin, and survival signals including phosphorylated phosphoinositide 3-kinase, Akt, and glycogen synthase kinase-3β. CONCLUSIONS: These results suggest that several factors determined by proteomics and Western blot hold the clue to Aβ pathogenesis. Further studies are required to investigate the role of these factors.
Alzheimer Disease ; Amyloid* ; Animals ; Blotting, Western ; Brain ; Bromodeoxyuridine ; Cell Proliferation ; Central Nervous System ; Fructose-Bisphosphate Aldolase ; Glycogen Synthase ; Humans ; L-Lactate Dehydrogenase ; Neural Stem Cells* ; Plaque, Amyloid ; Proteomics ; Trypan Blue

BACKGROUND AND PURPOSE: The cerebrospinal fluid (CSF) biomarkers play an important supportive role as diagnostic and predictive indicators of Alzheimer's disease (AD). About 30% of controls in old age show abnormal values of CSF biomarkers and display a higher risk for AD compared with those showing normal values. The cut-off values are determined by their diagnostic accuracy. However, the current cut-off values may be less accurate, because controls include high-risk groups of AD. We sought to develop models of patients with AD, who are homogenous for CSF biomarkers. METHODS: We included participants who had CSF biomarker data in the Alzheimer's Disease Neuroimaging Initiative database. We investigated the factors related to CSF biomarkers in patients with AD using linear mixed models. Using the factors, we developed models corresponding to CSF biomarkers to classify patients with mild cognitive impairment (MCI) into high risk and low risk and analyzed the conversion from MCI to AD using the Cox proportional hazards model. RESULTS: APOE ε4 status and age were significantly related to CSF Aβ1-42. CSF t-tau, APOE ε2 status and sex were significant factors. The CSF p-tau181 was associated with age and frequency of diagnosis. Accordingly, we modeled the three CSF biomarkers of AD. In MCI without APOE ε4, our models were better predictors of conversion. CONCLUSIONS: We can interpret CSF biomarkers based on the models derived from the data obtained from patients with AD.
Alzheimer Disease* ; Apolipoproteins E ; Biomarkers ; Cerebrospinal Fluid ; Diagnosis ; Humans ; Methods* ; Mild Cognitive Impairment* ; Neuroimaging ; Proportional Hazards Models ; Reference Values

BACKGROUND AND PURPOSE: Loneliness is a significant concern among the elderly, particularly in societies with rapidly growing aging populations. While loneliness may influence neuropsychological function, the exact nature of the association between loneliness and neuropsychological function is poorly understood. METHODS: We evaluated 50 elderly patients with mild cognitive impairment (MCI) and 33 without cognitive dysfunction with respect to demographics, clinical characteristics, cognitive and functional performance, depression scale, and loneliness scale. The associations between loneliness and neuropsychological assessments were evaluated. RESULTS: Although loneliness was not associated with cognitive or functional performance, it was correlated with depression in elderly patients. For elderly patients with MCI, depressive symptoms were reported more frequently in individuals with a high degree of loneliness (p < 0.05). CONCLUSIONS: Neither cognitive performance nor functional performance is associated with loneliness; however, loneliness is associated with depressive symptoms in elderly patients with MCI.
Aged* ; Aging ; Demography ; Depression ; Humans ; Loneliness* ; Mild Cognitive Impairment* ; Pilot Projects*

BACKGROUND AND PURPOSE: Nicergoline is an ergoline derivative that is used to treat cognitive deficits in cerebrovascular disease and various forms of dementia. Although therapeutic effects of nicergoline have been established, little is known about its effects on cerebral perfusion in Alzheimer's disease (AD). The aim of this study was to examine the role of nicergoline in regional cerebral blood flow (rCBF) of AD patients using technetium-99m hexa-methyl-propylene-amine-oxime single photon emission computed tomography (SPECT). METHODS: Sixteen patients with early AD underwent a comprehensive clinical assessment including cognitive testing and SPECT scans before and after nicergoline treatment. Nicergoline (30 mg twice daily) was administered for an average duration of 1.5 years. Clinical and cognitive functioning was assessed using the Mini-Mental State Examination, Clinical Dementia Rating (CDR), CDR-Sum of Boxes, Global Deterioration Scale, Barthel Activities of Daily Living Index, Instrumental Activities of Daily Living, and Geriatric Depression Scale. RESULTS: Nicergoline treatment induced changes in the severity of dementia, cognitive function, activities of daily living, and depressive symptoms, which were not statistically significant. During the follow-up, the patients showed significant increases in their relative rCBF in the superior frontal gyrus, precentral gyrus, and postcentral gyrus. CONCLUSIONS: Nicergoline treatment improves perfusion of the frontal and parietal regions in early AD patients. It is possible that the increased perfusion in the superior frontal gyrus may be related to the mechanisms that delay or prevent progressive deterioration of cognitive functions in AD.
Activities of Daily Living ; Alzheimer Disease* ; Cerebrovascular Circulation ; Cerebrovascular Disorders ; Cognition ; Cognition Disorders ; Dementia ; Depression ; Ergolines ; Follow-Up Studies ; Frontal Lobe ; Humans ; Nicergoline* ; Parietal Lobe ; Perfusion* ; Pilot Projects* ; Prefrontal Cortex ; Somatosensory Cortex ; Therapeutic Uses ; Tomography, Emission-Computed, Single-Photon

BACKGROUND AND PURPOSE: This study was performed to newly develop the Way-Finding Ability Scale (WFAS) for middle-aged and older adults and validate its clinical utility. METHODS: The participants for verifying construct validity included 324 adults aged from 45 to 79 years, and 22 normal old adults without way-finding deficit (WFD), 41 amnestic mild cognitive impairment (aMCI), and 35 patients with Parkinson's disease (PD-MCI) for verifying discriminant validity. All participants were administered the newly constructed 28-item WFAS. RESULTS: Exploratory factor analysis of the WFAS revealed a four-factor solution (sense of direction and inattention, spatial learning and memory, strategic ability, and cardinal direction). This four-factor structure was confirmed by confirmatory factor analysis. The discriminant validity was examined by administering the WFAS to normal older adults and two patient groups (aMCI & PD-MCI). The results showed that the total scores of two patient groups were lower than that of normal older adults. The patients with WFD had significantly lower total scores than those without WFD. Interestingly, the total scores of patients without WFD were significantly lower than those of normal older adults suggesting that the cognitive functions associated with way-finding ability (WFA) were partially impaired in aMCI and PD-MCI patients without apparent WFD. The patients with WFD had consistently lower scores in every four-factor than those without WFD. CONCLUSIONS: These results indicated that the WFAS assesses the WFD reliably as well as estimates the degree of decline in WFA.
Adult* ; Cognition ; Humans ; Memory ; Mild Cognitive Impairment ; Parkinson Disease ; Spatial Learning

No abstract available.
Cerebrospinal Fluid* ; Limbic Encephalitis*