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

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

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

No abstract available.
Aphasia, Primary Progressive ; Frontotemporal Dementia ; Supranuclear Palsy, Progressive

Frontotemporal lobar degeneration (FTLD) is a progressive dementia with prominent neuropsychiatric features, aphasia or both. FTLD predominantly affects the frontal and anterior part of temporal cortex. FTLD is classified into frontotemporal dementia (FTD), progressive nonfluent aphasia (PA), and semantic dementia (SD). FTLD is estimated to account for 20% of cases of degenerative dementia with presenile onset. This disease typically has onset in the mid- or early fifties. FTD is characterized by behavioral change and executive dysfunction, PA features a progressive nonfluent aphasia. SD is characterized by a progressive semantic aphasia and associative agnosia. Structural imaging shows atrophy of the frontal lobe and the anterior portion of the temporal lobe, bilaterally symmetric or asymmetric. Pathologically, FTLD can be classified into tau-positive pathology, tau-negative, ubiquitin positive pathology, dementia lacking distinctive histology. At present, there are no specific pharmacological therapies approved for use in any of the FTLD syndrome.
Agnosia ; Aphasia ; Atrophy ; Dementia ; Frontal Lobe ; Frontotemporal Dementia ; Frontotemporal Lobar Degeneration* ; Pathology ; Primary Progressive Nonfluent Aphasia ; Temporal Lobe ; Ubiquitin

Semantic dementia is a rare, distinct form of frontotemporal lobar degeneration, characterized by a deficit in semantic memory with relative preservation of attention and executive functions. We report a case of semantic dementia that pre-sented with a 3-year history of progressive word-finding difficulty and prosopagnosia. Brain MRI showed prominent atrophic changes in the left temporal region and neuropsychological tests demonstrated semantic memory deficits.
Brain ; Executive Function ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Magnetic Resonance Imaging ; Memory ; Memory Disorders ; Neuropsychological Tests ; Prosopagnosia ; Semantics*

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

OBJECTIVES: The aim of this study was to draw attention toward so called ‘behavioral variant frontotemporal dementia(bvFTD) phenocopy syndrome’, which is difficult to discriminate with the primary psychiatric disorders, showing poor response to conventional therapeutic drugs, leading to higher risk to misdiagnoses and legal problems. Furthermore, the author insisted that our interest and study on them must be continued. METHODS: English articles published during 2000 thru 2016 had been searched by internet with the combination of words such as ‘frontotemporal’, ‘phenocopy’ and ‘behavioral’, and reviewed. Besides, two clinical vignettes were described. RESULTS: Precise diagnosis is important because patients' behavioral symptoms can influence on their families and community. However, disease-modifying treatment for bvFTD are not developed until now, and recent therapeutic drugs are only good for specific symptoms, while deterioration progresses in spite of proper psychiatric management. The possible bvFTD patients are not progressed into probable bvFTD clinically, showing no decline of cogntive and social function, no decrease of activity function, longer survival time, and normal neuroimaging for several years. CONCLUSIONS: Rather than expected, there are much more patients having clinical symptoms, course and diagnostic findings including neuroimaging, which are atypical to classical frontotemporal dementia and primary psychiatric disorders. If our knowledge and discriminating ability is improved, discovery rate of that cases will be increased. However, the identity of these atypical features are not clarified until now, it must be further actively investigated.
Behavioral Symptoms ; Cognition ; Diagnosis ; Diagnostic Errors ; Frontotemporal Dementia* ; Humans ; Internet ; Neuroimaging