Criticisms about amyloid cascade hypothesis of Alzheimer's disease(AD) are based on the findings, first, that the degree of dementia does not correlate with the number of plaques, and second, that the neurofibrillary tangle formation seems to predate plaque formation. In addition, neurofibrillary tangle counts correlate well with the degree of cognitive impairment. These findings suggest the independent importance of tau abnormality in AD research which is involved in the neurofibrillary tangle formation. Recently, tau pathology without amyloid deposits and mutations in tau protein gene were reported to be the major pathogenic mechanism in Pick's disease, progressive supranuclear palsy, corticobasal degeneration and FTDP-17(frontotemporal dementia and parkinsonism linked with chromosome 17). These data suggest that understanding the causes and consequences of tau dysfunction might give new clinical and therapeutic solutions to many known tauopathies.
Amyloid ; Dementia ; Frontotemporal Dementia ; Frontotemporal Lobar Degeneration ; Molecular Biology* ; Neurofibrillary Tangles ; Parkinsonian Disorders ; Pathology ; Pick Disease of the Brain ; Plaque, Amyloid ; Prednisolone ; Supranuclear Palsy, Progressive ; tau Proteins* ; Tauopathies

Frontotemporal dementia (FTD), formerly called Pick's disease, is a progressive dementia that is associated with focal atrophy of the frontal and/or temporal lobes. FTD has three major clinical subtypes ; 1) a frontal variant of frontotemporal dementia (fvFTD), 2) semantic dementia (SD), and 3) progressive nonfluent aphasia (PNFA). These different variants differ in their clinical symptoms, cognitive deficits, and affected brain regions. The insidious onset of personality changes and behavioral abnormalities is the most prominent feature of fvFTD. Poor insight, loss of personal and social awareness, and blunting of affect are common behavioral changes in fvFTD. The most common presenting complaint in SD involves language, and is often described as a loss of memory for words or a loss of word meaning. Patients with PNFA present with changes in fluency, pronunciation, or word finding difficulty. An accumulating body of evidence suggests that FTD overlaps with three other neurodegenerative diseases: motor neuron disease (MND), corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP). Treatment for FTD consists of behavioral and pharmacological approaches. Medications such as selective serotonin reuptake inhibitors, antipsychotics have used in FTD. Cholinesterase inhibitors do not consistently improve cognitive and behavioral symptoms of FTD. Further research should be directed at developing new therapeutic methods to improve the patients' symptoms.
Antipsychotic Agents ; Atrophy ; Behavioral Symptoms ; Brain ; Cholinesterase Inhibitors ; Dementia ; Frontotemporal Dementia ; Frontotemporal Lobar Degeneration ; Humans ; Memory ; Motor Neuron Disease ; Neurobehavioral Manifestations ; Pick Disease of the Brain ; Primary Progressive Nonfluent Aphasia ; Serotonin Uptake Inhibitors ; Supranuclear Palsy, Progressive ; Temporal Lobe

Frontotemporal lobe dementia have been underevaluated because of various clinical features, changing diagnostic criteria, and indifference of clinicians. It is important that frontotemporal lobe dementia patient showing behavioral and lingual problems should be early diagnosed and treated. Because frontotemporal lobe dementia patients often confused with Alzheimer's disease, senile depression, schizophrenia, drug abuse. We have presented a case of frontotemporal lobe dementia. He had typical clinical history and symptoms which deserve to be considered frontotemporal lobe dementia. He showed appropriate findings of frontotemporal lobe dementia in the neuropsychological tests and brain magnetic resonance imaging and single photon emission computed tomography. This case is thought to be helpful for clinicians to give attention to early diagnosis and appropriate treatment of frontotemporal lobe dementia.
Alzheimer Disease ; Brain ; Depression ; Early Diagnosis ; Frontotemporal Dementia* ; Humans ; Magnetic Resonance Imaging ; Neuropsychological Tests ; Pick Disease of the Brain ; Schizophrenia ; Substance-Related Disorders ; Tomography, Emission-Computed, Single-Photon

Frontotemporal dementia (FTD) is a clinical syndrome characterized by profound changes in personality and behavior. It is associated with degeneration of the prefrontal and anterior temporal cortex. There have been reports of patients with FTD who developed a new interest or increased creativity in the visual arts during their illness. To our knowledge, this is the first case report of a patient with a newfound interest in drawing after the onset of FTD in Korea.
Creativity ; Frontotemporal Dementia ; Humans ; Korea

Dementias can be calssified into cortical, subcortical, cortical-subcortical and multifocal ones based on the major pathological distribution within the brain. The literatures of recent knowledge about clinical features of other dementias than Alzheimer's and vascular ones, which were most frequently experienced by many clinicians were reviewed. That is, cortical dementias such as Pick's disease, frontal lobe type dementia and non-Alzheimer's type lobar atrophy including fronto-temporal dementia, progressive dysphasia, fronto-temporal dementia with motor neuron disease, and alcohol-related dementia were reviewed. Subcortical dementias such as dementias accompanying Parkinson's disease, Huntington's disease and progressive supranuclear palsy, and cortical-subcortical dementias such as Lewy body dementiaq and cortical-basal degeneration were also reviewed. As multifocal dementias, prion dementias including KUru, Creutzfeldt-Jakob disease, fatal familial insomnia and Gerstmann-Strussler-Sheinker syndrone, and AIDS dementia were also reviewed.
Aphasia ; Atrophy ; Brain ; Creutzfeldt-Jakob Syndrome ; Dementia* ; Frontal Lobe ; Frontotemporal Dementia ; Huntington Disease ; Insomnia, Fatal Familial ; Kuru ; Lewy Bodies ; Lewy Body Disease ; Motor Neuron Disease ; Parkinson Disease ; Pick Disease of the Brain ; Supranuclear Palsy, Progressive

No abstract available.
Frontotemporal Dementia ; Serotonin ; Supranuclear Palsy, Progressive

BACKGROUND AND SIGNIFICANCE: Frontotemporal dementia is a behavioral disorder arising from nonAlzheimer's disease atrophy of frontal and anterior temporal lobe. Clinical manifestations include frontal lobe dysfunction. Kluver-Bucy syndrome or progressive language impairments. Two types of histological change underline, the atrophy. The commoner pathology is nerve cell loss and spongiform change with astrocytic gliosis. The second one is typical Pick-type histology characterized by intraneuronal inclusion body and astrocytic gliosis. We report a case with biopsy proved Pick's disease presenting with progressive nonfluent speech. CASE: A 41-years, old right-handed woman developed progressive language impairment over a period of 6 months. Brain MRI revealed asymmetric frontotemporal cortical atrophy more severe on the left side and 18F-FDG-brain, PET showed hypometabolism in the same area. Neuropsychological test including Korean version-Western Aphasia Battery revealed non-fluent speech as well as frontal lobe, dysfunction. A biopsy from left frontal lobe, demonstrate neuronal loss and diffuse astrogliosis. In the cytoplasm of remaining neurons are eosinophilic inclusion bodies which are neurofilament-positive with immunostaining. Senile plaque, neurofibrillary tangle and cortical Lewy body were absent. COMMENT: We report a case presenting with progressive nonfluent speech whose imaging and pathological findings are compatible with Pick's disease, which may be the first biopsy proven case in Korea.
Aphasia ; Atrophy ; Biopsy ; Brain ; Cytoplasm ; Eosinophils ; Female ; Frontal Lobe ; Frontotemporal Dementia ; Gliosis ; Humans ; Inclusion Bodies ; Kluver-Bucy Syndrome ; Korea ; Lewy Bodies ; Magnetic Resonance Imaging ; Neurofibrillary Tangles ; Neurons ; Neuropsychological Tests ; Pathology ; Pick Disease of the Brain* ; Plaque, Amyloid ; Temporal Lobe

Approximately 15% of patients with frontotemporal dementia (FTD) have co-occurring motor neuron disease (MND). FTD-MND cases have frontotemporal lobar degeneration (FTLD)-transactive response DNA-binding protein (TDP) pathology, which is divided into four subtypes (types A, B, C, and D) based on the morphological appearance, cellular location, and distribution of the abnormal TDP inclusions and dystrophic neurites. We report a patient with FTD-MND whose pathological diagnosis was FTLD-TDP type B. This is the first documented autopsy-confirmed case of FTD-MND in Korea.
Autopsy* ; Diagnosis ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Korea ; Motor Neuron Disease* ; Motor Neurons* ; Neurites ; Pathology

Approximately 15% of patients with frontotemporal dementia (FTD) have co-occurring motor neuron disease (MND). FTD-MND cases have frontotemporal lobar degeneration (FTLD)-transactive response DNA-binding protein (TDP) pathology, which is divided into four subtypes (types A, B, C, and D) based on the morphological appearance, cellular location, and distribution of the abnormal TDP inclusions and dystrophic neurites. We report a patient with FTD-MND whose pathological diagnosis was FTLD-TDP type B. This is the first documented autopsy-confirmed case of FTD-MND in Korea.
Autopsy* ; Diagnosis ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Korea ; Motor Neuron Disease* ; Motor Neurons* ; Neurites ; Pathology

No abstract available.
Frontotemporal Dementia ; Postural Orthostatic Tachycardia Syndrome* ; Supranuclear Palsy, Progressive