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

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

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*

BACKGROUND AND PURPOSE: To analyze 18F-THK5351 positron emission tomography (PET) scans of patients with clinically diagnosed nonfluent/agrammatic variant primary progressive aphasia (navPPA). METHODS: Thirty-one participants, including those with Alzheimer's disease (AD, n=13), navPPA (n=3), and those with normal control (NC, n=15) who completed 3 Tesla magnetic resonance imaging, 18F-THK5351 PET scans, and detailed neuropsychological tests, were included. Voxel-based and region of interest (ROI)-based analyses were performed to evaluate retention of 18F-THK5351 in navPPA patients. RESULTS: In ROI-based analysis, patients with navPPA had higher levels of THK retention in the Broca's area, bilateral inferior frontal lobes, bilateral precentral gyri, and bilateral basal ganglia. Patients with navPPA showed higher levels of THK retention in bilateral frontal lobes (mainly left side) compared than NC in voxel-wise analysis. CONCLUSIONS: In our study, THK retention in navPPA patients was mainly distributed at the frontal region which was well correlated with functional-radiological distribution of navPPA. Our results suggest that tau PET imaging could be a supportive tool for diagnosis of navPPA in combination with a clinical history.
Alzheimer Disease ; Aphasia, Primary Progressive* ; Basal Ganglia ; Broca Area ; Diagnosis ; Frontal Lobe ; Humans ; Magnetic Resonance Imaging ; Neurofibrillary Tangles ; Neuropsychological Tests ; Positron-Emission Tomography ; Primary Progressive Nonfluent Aphasia ; tau Proteins

Frontotemporal dementia (FTD) is a degenerative disease characterized by the selective frontal and temporal lobe atrophy, and progressive deficits in behavior, executive function, or language. The prevalence and incidence of FTD are 15-22/100000 and 2.7-4.1/100000, respectively, in midlife. Hereditary is an important risk factor for FTD. Although there is some controversy regarding the further syndromatic subdivision of the different types of FTD, FTD is clinically classified into behavioral variant of frontotemporal dementia, semantic dementia and progressive nonfluent aphasia. FTD can be misdiagnosed as many psychiatric disorders because of similarity of the prominent behavioral features. Advances in clinical, imaging, and molecular characterization have increased the accuracy of FTD diagnosis, thus developing for the accurate differentiation of these syndromes from psychiatric disorders. We also discuss about therapeutic strategies for symptom management of FTD. Medications such as serotonin reuptake inhibitors, antipsychotics, and other novel treatments have been used in FTD with various rates of success. Further advanced research should be directed at understanding and developing new diagnostic and therapeutic modalities to improve the FTD patients' prognosis and quality of life.
Antipsychotic Agents ; Atrophy ; Diagnosis ; Drug Therapy ; Executive Function ; Frontotemporal Dementia* ; Genetics ; Incidence ; Prevalence ; Primary Progressive Nonfluent Aphasia ; Prognosis ; Quality of Life ; Risk Factors ; Serotonin Uptake Inhibitors ; Temporal Lobe

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