fronto temporal dementia review article

Frontotemporal dementias (FTD) are one of the prevalent forms of early neurodegenerative diseases. FTD are characterized by heterogeneous clinical manifestations and syndromes. The current methods of FTD treatment and the clinical trials of new methods of FTD treatment are considered in the article. Biomarkers and their relationships with the results of recently completed clinical trials, as well as future therapeutic perspectives, are reviewed.

Maintenance of protein homeostasis is vitally important in post-mitotic cells, particularly neurons. Neurodegenerative diseases such as polyglutamine expansion disorders-like Huntington's disease or spinocerebellar ataxia (SCA), Alzheimer's disease, fronto-temporal dementia (FTD), amyotrophic lateral sclerosis (ALS) and Parkinson's disease-are often characterized by the presence of inclusions of aggregated protein. Neurons contain complex protein networks dedicated to protein quality control and maintaining protein homeostasis, or proteostasis...

Ribonucleoprotein (RNP) granules transport, store, or degrade messenger RNAs, thereby indirectly regulating protein synthesis. Normally, RNP granules are highly dynamic compartments. However, because of aging or severe environmental stress, RNP granules, in particular stress granules (SGs), convert into solid, aggregate-like inclusions. There is increasing evidence that such RNA-protein inclusions are associated with several age-related neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), fronto-temporal dementia (FTD) and Alzheimer's disease (AD)...

This study aimed at describing uncommon or bizarre symptoms observed in patients suffering from dementia. Medline and Google scholar searches were conducted for relevant articles, chapters, and books published since 1967. Search terms used included uncommon presentation, behavioural and psychological symptoms, dementia, Alzheimer's disease, and fronto-temporal dementia. Publications found through this indexed search were reviewed for further relevant references. The uncommon symptoms are described as case-reports and there are no systematic investigations...

The structural and functional neuroimaging of dementia have substantially evolved over the last few years. The most common forms of dementia, Alzheimer disease (AD), Lewy body dementia (LBD) and fronto-temporal lobar degeneration (FTLD), have distinct patterns of cortical atrophy and hypometabolism that evolve over time, as reviewed in the first part of this article. The second part discusses unspecific white matter alterations on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images as well as cerebral microbleeds, which often occur during normal aging and may affect cognition...

The purpose of this article is to review several studies on extramotor feature in Amyotrophic Lateral Sclerosis (ALS), its association with cognitive impairment, dementia, sensory and autonomic abnormalities suggesting that ALS is a multisystem disorder rather than a pure motor neuron disease. Evidence from neuroimaging, neuropsychological and histologic studies suggests that other brain regions can be involved in the pathologic process. However, the extent of the extramotor involvement remains unclear. A recent finding on the deposition of pathological TDP-43 (43 kDA transactivating responsive sequence DNA binding protein) in the Central Nervous System of Amyotrophic Lateral Sclerosis patients implies that ALS may be situated of clinicopathological spectrum of multisystem degeneration including Fronto-Temporal Lobar degeneration, that is related to the same protein...

OBJECTIVE: To review the rapidly expanding literature of amyloid PET imaging with particular attention to Pittsburgh compound-B (PIB) in Alzheimer's disease (AD), dementia with Lewy bodies (DLB), fronto-temporal dementia (FTD), mild cognitive impairment (MCI) and cognitively normal volunteers. DESIGN: Literature searches were performed using Medline up to February 2010. Individual articles were then examined for additional references not revealed by automated searches...

In this review, we consider recent work using zebrafish to validate and study the functional consequences of mutations of human genes implicated in a broad range of degenerative and developmental disorders of the brain and spinal cord. Also we present technical considerations for those wishing to study their own genes of interest by taking advantage of this easily manipulated and clinically relevant model organism. Zebrafish permit mutational analyses of genetic function (gain or loss of function) and the rapid validation of human variants as pathological mutations...

In this review article the significance of molecular imaging techniques, single photon emission tomography and positron emission tomography (SPET and PET), in the diagnosis and differential diagnosis of various types of dementia is described. A major part of this article is focused on molecular imaging in Alzheimer's disease where parietal, temporal and posterior cingulate cortex hypoperfusion and hypometabolism are the predominant findings in molecular imaging modalities. In cases of mild amnestic cognitive impairment, molecular SPET imaging can differentiate patients converting to Alzheimer's disease from non converters...

BACKGROUND: Dementia is frequently associated with behavioral disturbances, some of which have a significant impact on patient quality of life and the likelihood of institutionalization. Cholinergic systems, among other neurotransmitters in the brain, appear to be involved with different behaviors, such as psychosis, depression, agitation, and personality changes. SCOPE: This paper reviews the clinical data on the effectiveness of rivastigmine, a dual inhibitor of acetylcholinesterase and butyrylcholinesterase, in ameliorating behavioral disturbances in different patient populations...

The development of abnormal protein aggregates in the form of extracellular plaques and intracellular inclusions is a characteristic feature of many neurodegenerative diseases such as Alzheimer's disease (AD), Creutzfeldt-Jakob disease (CJD) and the fronto-temporal dementias (FTD). An important aspect of a pathological protein aggregate is its spatial topography in the tissue. Lesions may not be randomly distributed within a histological section but exhibit spatial pattern, a departure from randomness either towards regularity or clustering...

OBJECTIVE: The authors sought to determine the diagnostic accuracy of 99mTc-HMPAO-SPECT in discriminating between Alzheimer disease (AD) and other dementias. METHODS: Articles published between 1985 and 2002 were retrieved systematically from MEDLINE and EMBASE, cross-referencing with personal collections and 13 narrative reviews. Of 301 studies identified, 48 survived exclusion criteria and contained extractable data. Two authors independently assessed and graded the methodology of all included studies...