No abstract available.
Alzheimer Disease* ; Aphasia, Primary Progressive*

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

BACKGROUND AND PURPOSE: The occurrence of PWD in neurodegenerative disease is very rare, and this is the first report of it being related to early-onset AD. We describe a patient with early-onset Alzheimer's disease (AD) who presented with pure word deafness (PWD). CASE REPORT: The patient had experienced PWD for 2 years, followed by other cognitive deficits suggestive of parietotemporal dysfunction. Brain imaging including 18FDG-PET and [11C] PIB-PET supported the diagnosis of AD. CONCLUSIONS: Our case highlights the clinical variability that characterizes early-onset AD.
Alzheimer Disease ; Aphasia, Primary Progressive ; Deafness ; Humans ; Neurodegenerative Diseases ; Neuroimaging

Aphasia, Primary Progressive/genetics* ; Humans ; Neuroimaging ; Neuropsychological Tests

BACKGROUND: Tauopathies are a group of diseases caused by the accumulation of hyperphosphorylated tau protein in the central nervous system. Previous studies have revealed that there is considerable overlap in clinical, pathological, and genetic features among different taupathies. CASE REPORT: We report a patient with non-fluent/agrammatic primary progressive aphasia at the initial assessment. Over time, other symptoms belonging to corticobasal degeneration and progressive supranuclear palsy appeared in this patient. CONCLUSIONS: Clinical overlapping features in these disorders may represent different phenotypes of a single disease process.
Aphasia, Primary Progressive* ; Central Nervous System ; Humans ; Phenotype ; Supranuclear Palsy, Progressive ; tau Proteins ; Tauopathies

BACKGROUND: Non-fluent agrammatic primary progressive aphasia (naPPA) is characterized by progressive non-fluent speech disorder and might be associated with taupathy such as corticobasal degeneration (CBD) and progressive supranuclear palsy. We report a case of overlap syndrome presented with language impairment, and diagnosed as naPPA with possible CBD. CASE REPORT: A 58-year-old woman visited a memory and dementia clinic, with a 10-month history of progressive language disturbance. She was diagnosed as naPPA and overlapping CBD, based on the clinical features and neuroimaging findings including florbetaben PET. CONCLUSIONS: naPPA is pathologically caused by taupathy, and might progress to asymmetrical parkinsonism and apraxia, suggestive of CBD. Overlapping clinical features in our case represent various phenotypes of taupathy.
Aphasia, Primary Progressive* ; Apraxias ; Dementia ; Female ; Humans ; Memory ; Middle Aged ; Neuroimaging ; Parkinsonian Disorders ; Phenotype ; Supranuclear Palsy, Progressive

Primary progressive aphasia (PPA) is classified into agrammatic, semantic, and logopenic variants, but differential diagnosis is often challenging. There is accumulating evidence that the neuropathology of logopenic PPA is distinct from that of the other types. We report herein a woman with logopenic PPA who was diagnosed by clinical, neuropsychological, and radiologic data during 2 years of follow-up. Interestingly, her cerebrospinal fluid biomarkers were found to be similar to those found in Alzheimer's disease, with a decreased concentration of Abeta42 and an increased concentration of pTau181 (tTau).
Alzheimer Disease* ; Aphasia* ; Aphasia, Primary Progressive ; Biomarkers* ; Cerebrospinal Fluid* ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; 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

Primary progressive aphasia (PPA) is an uncommon neurodegenerative syndrome characterized by a progressive deterioration of language, while nonverbal cognitive and other neurological functions of PPA are relatively preserved for a longer period. However, it still remains unclear whether PPA represents a distinct diagnostic entity or a precursor of global dementia syndrome. We report PPA cases that presented with a slowly progressive language dysfunction without disturbing other daily living activities for several years. Repeated neuropsychological tests revealed progres-sive deterioration of executive aspects of language and mild memory dysfunction, although their receptive language and nonverbal cognitive functions were relatively preserved. The imaging of the brain showed prominent atrophic changes in the left perisylvian and the adjacent temporal region. In considering the mild cognitive decline accompanied by language deterioration, we conclude that in these cases it is clinically heterogenous and may be parts of a spectrum of focal forms of non-Alzeimer type dementia.
Activities of Daily Living ; Aphasia, Primary Progressive* ; Brain ; Dementia ; Memory ; Neuropsychological Tests

A woman developed a slowly progressive speech disturbance at age 51. Three years latter she showed difficulty in calculation, reading and writing. At age 57, she complained of right shoulder pain. At age 58, neurological examination revealed rigidity, bradykinesia and ideomotor apraxia in the right upper extremity. This case demonstrats a clinical overlap between progressive nonfluent aphasia and corticobasal degeneration.
Apraxia, Ideomotor ; Female ; Humans ; Hypokinesia ; Neurologic Examination ; Primary Progressive Nonfluent Aphasia ; Shoulder Pain ; Upper Extremity ; Writing