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

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*

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

Aged ; Agraphia ; etiology ; Amyotrophic Lateral Sclerosis ; complications ; pathology ; Female ; Frontotemporal Lobar Degeneration ; complications ; pathology ; Humans ; Magnetic Resonance Imaging

BACKGROUND AND PURPOSE: Only a few studies have investigated the relationship between different subtypes and disease progression or prognosis in patients with behavioral variant frontotemporal dementia (bvFTD). Since a localized injury often produces more focal signs than a diffuse injury, we hypothesized that the clinical characteristics differ between patients with bvFTD who show diffuse frontal lobe atrophy (D-type) on axial magnetic resonance imaging (MRI) scans versus those with focal or circumscribed frontal lobe atrophy (F-type). METHODS: In total, 94 MRI scans (74 scans from bvFTD and 20 scans from age-matched normal controls) were classified into 35 D- and 39 F-type bvFTD cases based on an axial MRI visual rating scale. We compared baseline clinical characteristics, progression in motor and cognitive symptoms, and survival times between D- and F-types. Survival analyses were performed for 62 of the 74 patients. RESULTS: While D-type performed better on neuropsychological tests than F-type at baseline, D-type had higher baseline scores on the Unified Parkinson's Disease Rating Scale (UPDRS) Part III. Evaluations of motor progression showed that the disease duration with motor symptoms was shorter in D-type than F-type. Moreover, the survival time was shorter in D-type (6.9 years) than F-type (9.4 years). Cox regression analyses revealed that a high UPDRS Part III score at baseline contributed to an increased risk of mortality, regardless of the pattern of atrophy. CONCLUSIONS: The prognosis is worse for D-type than for those with F-type. Shorter survival in D-type may be associated with the earlier appearance of motor symptoms.
Atrophy* ; Disease Progression ; Frontal Lobe ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Magnetic Resonance Imaging ; Mortality ; Neurobehavioral Manifestations ; Neuropsychological Tests ; Parkinson Disease ; Prognosis*

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

It is not uncommon for idiopathic parkinson's disease (IPD) to occur concurrently with other degenerative dementing disorders such as Alzheimer's disease. However, there has been no report about the comorbidity of IPD and frontotemporal lobar degeneration. We report a 70-year-old man diagnosed with IPD accompanied by progressive non-fluent aphasia (PA). Brain MRI showed left frontal opercular atrophy, and an 18F-FDG PET scan revealed predominant left frontotemporal hypometabolism. It remains unknown whether or not the co-occurrence of IPD and PA was coincidental.
Aged ; Alzheimer Disease ; Aphasia ; Atrophy ; Brain ; Comorbidity ; Dementia ; Fluorodeoxyglucose F18 ; Frontotemporal Lobar Degeneration ; Humans ; Magnetic Resonance Imaging ; Parkinson Disease* ; Positron-Emission Tomography ; Primary Progressive Nonfluent Aphasia*

Primary progressive aphasia (PPA) is a clinical syndrome diagnosed when three core criteria are met. First, there should be a language impairment (i.e., aphasia) that interferes with the usage or comprehension of words. Second, the neurological work-up should determine that the disease is neurodegenerative, and therefore progressive. Third, the aphasia should arise in relative isolation, without equivalent deficits of comportment or episodic memory. The language impairment can be fluent or non-fluent and may or may not interfere with word comprehension. Memory for recent events is preserved although memory scores obtained in verbally mediated tests may be abnormal. This distinctive clinical pattern is most conspicuous in the initial stages of the disease, and reflects a relatively selective atrophy of the language network, usually located in the left hemisphere. There are different clinical variants of PPA, each with a characteristic pattern of atrophy. Clinicoanatomical correlations in patient with these variants have led to new insights on the organization of the large-scale language network in the human brain. For example, the left anterior temporal lobe, which was not part of the classic language network, has been shown to play a critical role in word comprehension and object naming. Furthermore, patients with PPA have shown that fluency can be dissociated from grammaticality. The underlying neuropathological diseases are heterogeneous and can include Alzheimer's disease as well as frontotemporal lobar degeneration. The clinician's task is to recognize PPA and differentiate it from other neurodegenerative phenotypes, use biomarkers to surmise the nature of the underlying neuropathology, and institute the most fitting multimodal interventions.
Alzheimer Disease ; Aphasia ; Aphasia, Primary Progressive* ; Atrophy ; Biomarkers ; Brain ; Comprehension ; Dementia ; Frontotemporal Lobar Degeneration ; Humans ; Memory ; Memory, Episodic ; Neuropathology ; Phenotype ; Temporal Lobe

BACKGROUND: Semantic dementia (SD) is a temporal variant of frontotemporal lobar degeneration (FTLD), which is characterized by naming difficulty, decreased comprehension of words, prosopagnosia and object visual agnosia. We report clinical features, neuropsychological and neuroimaging findings of 4 patients with SD. METHODS: Of 55 patients diagnosed as having FTLD between Jan 1995 and May 2001 at Samsung Medical Center, four patients fulfilled the diagnostic criteria of SD proposed by consensus on FTLD diagnostic criteria. We investigated their clinical features such as presenting symptoms and abnormal behaviors, neuropsychological and neuroimaging findings. Neuropsychological tests included the Seoul Neuropsychological Screening Battery, the Korean-version of Western Aphasia Battery and Hanja reading and writing. All patients underwent brain MRI and FDG-PET. RESULTS: All of the patients showed naming difficulty as a presenting symptom. Language assessments showed severe naming and compre-hension difficulties with preserved fluency and repetition, which were compatible with transcortical sensory aphasia. Whereas Hangul reading aloud and writing were intact, three patients were impaired at Hanja reading and writing. Other neuropsychological tests were remarkable for prosopagnosia, object visual agnosia and memory loss. Brain MRI showed asymmetric temporal atrophies, mainly left antero-inferior temporal cortices. FDG-PET also showed hypome-tabolism in bilateral anterior temporal lobes, more severe on the left. CONCLUSIONS: Our SD patients had characteristic neuropsychological and neuroimaging findings, which can be differentiated from other neurodegenerative diseases. We also found Hanja alexia and agraphia in SD patients, which has not been reported yet.
Agnosia ; Agraphia ; Aphasia ; Aphasia, Wernicke ; Atrophy ; Brain ; Comprehension ; Consensus ; Dyslexia ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Magnetic Resonance Imaging ; Mass Screening ; Memory Disorders ; Neurodegenerative Diseases ; Neuroimaging* ; Neuropsychological Tests ; Prosopagnosia ; Semantics* ; Seoul ; Temporal Lobe ; Writing