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Objectives. The proposed clinical study has two goals: First, to assess the efficacy of a central nervous system stimulant and an atypical antipsychotic in treating the behavioral symptoms of FTD and second, to further characterize the biological markers, including genetic, imaging, and CSF proteins, of FTD in relation to our existing group of Alzheimer's patients.
Rationale. Frontotemporal dementia (FTD) is increasingly recognized as an important neuropsychiatric disorder. Symptoms of FTD include disinhibition, impulsivity, apathy, affective lability, and language dysfunction. The clinical syndrome is associated with frontal and/or anterior temporal atrophy on imaging and autopsy. Levels of the CSF proteins tau and (Beta)-amyloid 1-42, shown to have diagnostic utility in patients with Alzheimer's Disease (AD), have also been found to be abnormal in FTD. FTD is less associated with APOE genotype than AD, however some familial cases of FTD are associated with specific mutations in the gene encoding the tau protein. Currently, no treatments have been proven to be effective for altering the course or clinical symptoms of FTD.
Design. Study subjects will include 50 male and female patients with mild-moderate frontotemporal dementia recruited from participants in NINDS protocol 02-N-0001. In a double-blinded crossover 11-week study without a placebo control, patients will be treated with a stimulant (dextroamphetamine) and an atypical antipsychotic (quetiapine). The primary outcome measures will be the Neuropsychiatric Inventory and the Clinical Global Impression of Change. Cerebrospinal fluid, cognitive and genetic measures, brain MRIs, and side effects scales will also be collected.
Objectives. The goal of the proposed clinical study is to assess the efficacy of a central nervous system stimulant and an atypical antipsychotic in treating the behavioral symptoms of FTD.
Rationale. Frontotemporal dementia (FTD) is increasingly recognized as an important neuropsychiatric disorder. Symptoms of FTD include disinhibition, impulsivity, apathy, affective lability, and language dysfunction. The clinical syndrome is associated with frontal and/or anterior temporal atrophy on imaging and autopsy. Currently, no treatments have been proven to be effective for altering the course or clinical symptoms of FTD.
Design. Study subjects will include 20 male and female patients with mild-moderate frontotemporal dementia recruited from participants in NINDS protocols 02-N-0001 and 81-N-0010. In a double-blinded crossover 11-week study without a placebo control, patients will be treated with a stimulant (dextroamphetamine) and an atypical antipsychotic (quetiapine). The primary outcome measures will be the Neuropsychiatric Inventory and the Clinical Global Impression of Change. Cognitive measures and side effects scales will also be collected.
Frontotemporal Lobar Degeneration
National Institutes of Health Clinical Center, 9000 Rockville Pike
National Institutes of Health Clinical Center (CC)
Published on BioPortfolio: 2014-08-27T03:54:10-0400
Frontotemporal Lobar Degeneration (FTLD) is the neuropathological term for a collection of rare neurodegenerative diseases that correspond to four main overlapping clinical syndromes: fro...
This is a 52-week, multicenter, open label trial of memantine (Namenda) for frontotemporal lobar degeneration (FTLD). The goal is to determine the safety and tolerability of this FDA-appr...
Psycho-behavioral disorders assessment is crucial for the diagnosis and follow-up of patients with frontotemporal degeneration (FTD). DAPHNE scientific staff have therefore developed a qua...
Fronto-Temporal Lobar Degeneration (FTLD) is neurodegenerative disease which comes in three clinical categories: Fronto-Temporal Dementia behavioral variant (FTD), Primary Progressive Apha...
This is an observational study that aims to better understand the genetic causes of frontotemporal degeneration (FTD), Multiple Systems Atrophy (MSA), and Progressive Supranuclear Palsy (P...
Cerebrospinal fluid (CSF) core Alzheimer disease (AD) biomarkers have shown an excellent capacity for the in vivo detection of AD. Previous studies have shown that CSF levels of phosphorylated tau (p-...
The behavioural variant of frontotemporal dementia (bvFTD) is the most common phenotype of frontotemporal lobar degeneration (FTLD). FTLD is divided into three main pathological subtypes: tau-positive...
Recent studies have suggested a role for immune dysregulation behind the etiology of frontotemporal lobar degeneration (FTLD). Here, we have investigated the prevalence of immunological diseases in FT...
Frontotemporal lobar degeneration (FTLD) represents a group of neurodegenerative brain diseases with highly heterogeneous clinical, neuropathological and genetic characteristics. This high degree of h...
Molecular alterations compromising key metabolic pathways are poorly understood in sporadic frontotemporal lobar degeneration with TDP-43 pathology (sFTLD-TDP). Whole-transcriptome array, RT-qPCR vali...
The most common clinical form of FRONTOTEMPORAL LOBAR DEGENERATION, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight.
Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of TAU PROTEIN inclusions. FTLD includes three clinical syndromes: FRONTOTEMPORAL DEMENTIA, semantic dementia, and PRIMARY PROGRESSIVE NONFLUENT APHASIA.
A form of frontotemporal lobar degeneration and a progressive form of dementia characterized by motor speech impairment and AGRAMMATISM, with relative sparing of single word comprehension and semantic memory.
Diseases characterized by the presence of abnormally phosphorylated, ubiquitinated, and cleaved DNA-binding protein TDP-43 in affected brain and spinal cord. Inclusions of the pathologic protein in neurons and glia, without the presence of AMYLOID, is the major feature of these conditions, thus making these proteinopathies distinct from most other neurogenerative disorders in which protein misfolding leads to brain amyloidosis. Both frontotemporal lobar degeneration and AMYOTROPHIC LATERAL SCLEROSIS exhibit this common method of pathogenesis and thus they may represent two extremes of a continuous clinicopathological spectrum of one disease.
A retrogressive pathological change in the retina, focal or generalized, caused by genetic defects, inflammation, trauma, vascular disease, or aging. Degeneration affecting predominantly the macula lutea of the retina is MACULAR DEGENERATION. (Newell, Ophthalmology: Principles and Concepts, 7th ed, p304)
Biological therapy involves the use of living organisms, substances derived from living organisms, or laboratory-produced versions of such substances to treat disease. Some biological therapies for cancer use vaccines or bacteria to stimulate the body&rs...
Psychiatry is the study of mental disorders and their diagnosis, management and prevention. Conditions include schizophrenia, severe depression and panic disorders among others. There are pharmaceutical treatments as well as other therapies to help...