¹⁸F-THK5351 PET Imaging in Nonfluent-Agrammatic Variant Primary Progressive 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.

Frontotemporal Dementia

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.

Prevalence of frontotemporal dementia in community-based studies in Latin America: a systematic review / Prevalência de demência frontotemporal em estudos de base comunitária na America Latina: uma revisão sistemática

Latin America (LA) is experiencing a rise in the elderly population and a consequent increase in geriatric problemssuch as dementia. There are few epidemiological studies assessing the magnitude of dementia and dementia subtypesin LA. Objective: To identify published community-based studies on the prevalence of FTD in LA countries. Methods: Adatabase search for door-to-door studies on FTD prevalence in LA was performed. The search was carried out on Medline,Embase, and LILACS databases for research conducted between 1994 and 2012. The main inclusion criteria were: use ofany internationally accepted diagnostic criteria and investigation of community samples. Results: Four hundred and ninetytwo articles were found, of which 26 were initially pre-selected by title or abstract review. Five studies from 3 differentcountries were included. The FTD prevalence rates in community-dwelling elderly were 1.2 (Venezuela), 1.3 (Peru) and1.7-1.8 (Brazil) per thousand persons, depending on age group. Conclusion: The FTD prevalence in LA studies showedvalues mid-way between those observed in western and in oriental populations. Despite the magnitude of this problem,epidemiological information on FTD remains scarce in LA.

A América Latina (AL) está experimentando um aumento na população de idosos e um consequente aumento nosproblemas geriátricos, como demência. Existem poucos estudos epidemiológicos avaliando a magnitude de demência edemência subtipos na AL Objetivo: Identificar publicações baseadas em estudos sobre a prevalência da FTD em países daAL. Métodos: A pesquisa realizada foi por estudos de prevalência de FTD em comunidade na AL. A pesquisa foi realizada emMed-line, Embase, e LILACS no período entre 1994 e 2012. Os principais critérios de inclusão foram: utilização de quaisquercritérios internacionalmente aceitos de diagnóstico e investigação de amostras em comunidade. Resultados: Quatrocentose noventa e dois artigos foram encontrados, dos quais 26 foram inicialmente pré-selecionados pelo título ou fiscalização doabstract. Cinco estudos de 3 países diferentes foram incluídos. As taxas de prevalência na comunidade em idosos com FTDeram 1,2 (Venezuela), 1,3 (Peru) e 1,7-1,8 (Brasil) por mil pessoas, dependendo da faixa etária. Conclusão: A prevalênciaFTD em estudos da AL, apresentaram valores intermediários entre os observados em populações ocidentais e orientais. Apesarda magnitude do problema, informações epidemiológicas sobre FTD permanecem escassas em AL.

Frontotemporal dementia and neurocysticercosis: a case report / Demência frontotemporal e neurocisticercose: um relato de caso

We report a case of a 67-year-old woman with frontotemporal dementia (FTD) and a history of neurocysticercosis. After her retirement she showed progressive behavioral changes and neuropsychiatric symptoms with relative preservation ofcognitive functioning. During the next three years, the patient manifested progressive deterioration of verbal communication gradually evolving to mutism, a hall mark of cases of progressive nonfluent aphasia.

Caso de uma mulher de 71 anos com demência frontotemporal e historia de neurocisticercose. Após sua aposentadoria ela apresentou progressivas mudanças comportamentais e sintomas neuropsiquiátricos, com relativa preservação do funcionamento cognitivo. Durante os seguintes três anos, a paciente foi desenvolvendo uma deteriorização progressiva da comunicação verbal evoluindo gradualmente a mutismo, marca que descreve um caso de afasia progressiva não fluente.

Clinical Features and Therapeutic Approaches of Frontotemporal Dementia

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.

Progressive Nonfluent Aphasia With Ideomotor Apraxia and Rigidity in the Right Upper Extremity

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.

Frontotemporal Lobar Degeneration

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.