Michael Tierney
A5081934540- Dementia and Cognitive Impairment Research
- Alzheimer's disease research and treatments
- Amyotrophic Lateral Sclerosis Research
- Parkinson's Disease Mechanisms and Treatments
- Memory and Neural Mechanisms
- Neural and Behavioral Psychology Studies
- Neurological diseases and metabolism
- Neurobiology of Language and Bilingualism
- Health, Environment, Cognitive Aging
- Spatial Neglect and Hemispheric Dysfunction
- Epigenetics and DNA Methylation
- Neurogenetic and Muscular Disorders Research
- Botulinum Toxin and Related Neurological Disorders
- Prion Diseases and Protein Misfolding
- Traumatic Brain Injury Research
- Genomics and Rare Diseases
- Bioinformatics and Genomic Networks
- Pharmacological Effects and Toxicity Studies
- Neurological Disease Mechanisms and Treatments
- Genetic Neurodegenerative Diseases
- Functional Brain Connectivity Studies
- Advanced Neuroimaging Techniques and Applications
- Genetics and Neurodevelopmental Disorders
- Neurological and metabolic disorders
- Language Development and Disorders
National Institute of Neurological Disorders and Stroke
2012-2025
National Institutes of Health
2008-2025
Connecticut Children's Medical Center
2025
Wollongong Hospital
2024
University of Wollongong
2024
National Institute of Mental Health
2021
Wright-Patterson Air Force Base
2021
Columbia University
2020
Columbia University Irving Medical Center
2014
Indiana University – Purdue University Indianapolis
2014
Abstract Neuropsychological assessment of older individuals with dementing illnesses has suffered from a lack appropriately designed test instruments. The Repeatable Battery for the Assessment Status (RBANS) was developed dual purposes identifying and characterizing abnormal cognitive decline in adult as neuropsychological screening battery younger patients. entire takes less than 30 minutes to administer, yields scaled scores five domains. current study reports preliminary clinical validity...
Poly(GP) proteins are a promising pharmacodynamic marker for developing and testing therapeutics treating C9ORF72 -associated amyotrophic lateral sclerosis.
Inappropriate social behaviours are early and distinctive symptoms of the temporal frontal variants frontotemporal lobar degeneration (FTLD). Knowledge behaviour is essential for appropriate conduct. It unknown, however, in what way this knowledge degraded FTLD. In a recent functional MRI study, we have identified right-lateralized superior anterior lobe (aTL) region showing selective activation 'social concepts' (i.e. concepts describing behaviour: e.g. 'polite', 'stingy') as compared with...
Background A major question in understanding the functional organization of brain is to delineate divisions prefrontal cortex. Of particular importance cognitive capacities that are uniquely human fronto-polar cortex (Brodmann's area 10), which disproportionally larger humans relative rest than it ape's brain. The specific function this region remains poorly understood, but recent neuroimaging studies have proposed may hold goals mind while exploring and processing secondary goals. Principal...
To determine the pattern of executive dysfunction in frontotemporal dementia (FTD) and corticobasal syndrome (CBS) to brain areas associated with these illnesses.We administered Delis-Kaplan Executive Function System (D-KEFS), a collection standardized function tests, 51 patients behavioral-variant FTD 50 CBS. We also performed discriminant analysis on D-KEFS which tests best distinguished clinical diagnoses Finally, we used voxel-based morphometry (VBM) regional gray matter volume loss...
Abstract Neurodegenerative diseases likely share common underlying pathobiology. Although prior work has identified susceptibility loci associated with various dementias, few, if any, studies have systematically evaluated shared genetic risk across several neurodegenerative diseases. Using genome-wide association data from large (total n = 82,337 cases and controls), we utilized a previously validated approach to identify overlap reveal pathways between progressive supranuclear palsy (PSP),...
Uncertainty is a fact of life that must be accommodated in real-world decision making. Although it has been suggested the right prefrontal cortex (PFC) special role to play making under uncertainty, there very little hard data support this hypothesis. To better understand roles left and PFCs reasoning situations with complete incomplete information, we administered simple inference problems 18 patients lateralized focal lesions PFC (9 hemisphere, 9 hemisphere) 22 age- education-matched...
Abstract Objective Mutations in the Progranulin gene ( PGRN ) recently have been discovered to be associated with frontotemporal dementia (FTD) linked 17q21 without identified MAPT mutations. The range of mutations that can result FTD phenotype and clinical presentation patients yet determined. Methods In this study, we examined 84 from families not known previously illness chromosome 17 for sequenced coding exons flanking intronic regions . We compared prevalence, characteristics, magnetic...
Acute motor and sensory axonal neuropathy (AMSAN) is a rare severe form of acute injury caused by immune damage to the membrane. AMSAN an variant GBS. GBS occurs from myelin sheath, variants (AMSAN AMAN) differ in that insult seldom seen, especially pediatric adolescent patients. Unlike AMAN (acute neuropathy), which has been well described literature be secondary C. Jejuni infection, there no known etiology AMSAN. Here we present case otherwise healthy 15-year-old female who presented with...
Non-Alzheimer's disease dementias, including frontotemporal dementia (FTD) can be difficult to characterize due the predominance of distinct behavioral and neuropsychiatric symptoms. Widely used measurement tools lack structure objectivity. The purpose this study was use systematic direct observation symptoms, via Neurobehavioral Rating Scale (NBRS), clusters symptoms in FTD examine how selected symptom correlate with structural neuroimaging. We performed a factor analysis on NBRS data from...
Abstract Frontotemporal dementia (FTD) is a neurodegenerative disorder predominantly affecting the frontal and temporal lobes. Genome-wide association studies (GWAS) on FTD identified only few risk loci. One of possible explanations that clinically, pathologically, genetically heterogeneous. An important open question to what extent epigenetic factors contribute whether these vary between clinical subgroup. We compared DNA-methylation levels cases (n = 128), with Amyotrophic Lateral...
To assess changes in 3 clinical measures, the Revised ALS Functional Rating Scale (ALSFRS-R), letter fluency, and Frontal Behavioral Inventory (FBI), over time C9orf72 mutation carriers (C9+) with varied phenotypes.Thirty-four unrelated participants mutations were enrolled a prospective natural history study. Participants classified as asymptomatic, amyotrophic lateral sclerosis (ALS), ALS-familial frontotemporal dementia (FTD), or behavioral-variant FTD by diagnostic criteria. Diagnostic...
A nonsense/protein chain-terminating mutation in the CHMP2B gene has recently been reported as a cause of frontotemporal dementia (FTD) single large family known to show linkage chromosome 3. Screening for mutations this series FTD families and individual patients led identification protein-truncating 2 unaffected members an Afrikaner with FTD, but not their affected relatives. The putative pathogenicity is discussed.
The phenotypes of the behavioral variant frontotemporal dementia and corticobasal syndrome present considerable clinical anatomical overlap. respective patterns white matter damage in these syndromes have not been directly contrasted. Beyond cortical involvement, to pathways may critically contribute both common specific symptoms conditions. Here we assessed patients with whole-brain diffusion tensor imaging identify networks underlying pathologies. Twenty dementia, 19 syndrome, 15 healthy...
Summary Degeneration of cortical glutamatergic projections may contribute to the cognitive decline in Alzheimer disease (AD). To evaluate whether 1glutamate system stimulation might confer symptomatic benefit, we administered D-cycloserine, a putative partial indirect agonist at certain N-methyl- D-aspartate (NMDA) glutamate receptors, 12 patients with probable AD. The (seven men, five women) had mean age 65 ± 8.4 years; Mini Mental State Examination scores ranged from 15 25. A dose...
<h3>Objective:</h3> To use the Visual Object and Space Perception Battery (VOSP) to distinguish Alzheimer disease (AD) from non-AD pathology in corticobasal syndrome (CBS). <h3>Methods:</h3> This clinicopathologic study assessed 36 patients with CBS on VOSP. All were autopsied. The primary dependent variable was a binary pathologic outcome: who had diagnosis of AD (CBS-AD, n = 10) vs without (CBS-nonAD, 26). We also determined sensitivity specificity individual VOSP subtests....
BackgroundThe etiology of frontotemporal dementia (FTD) is poorly understood. To identify genes with predicted expression levels associated FTD, we integrated summary statistics external reference gene data using a transcriptome-wide association study approach.MethodsFUSION software was used to leverage FTD (all FTD: n = 2154 cases, 4308 controls; behavioral variant 1337 2754 semantic dementia: 308 616 progressive nonfluent aphasia: 269 538 motor neuron disease: 200 400 controls) from the...
To determine areas of atrophy in patients that are associated with caregiver burden.We measured burden, dementia and neuropsychiatric scores 22 corticobasal syndrome (CBS) 25 frontotemporal (FTD), 14 healthy controls. We used voxel-based morphometry to correlate burden gray matter loss.Increased behavioral disturbances contributed higher CBS patients, while alone significantly affected frontal-variant FTD (FTD-fv) patients. In correlated left inferior middle temporal gyri.Caregivers FTD-fv...