1,460 results match your criteria Dementia Frontotemporal Lobe


F-Flortaucipir in TDP-43 associated frontotemporal dementia.

Sci Rep 2019 Apr 15;9(1):6082. Epub 2019 Apr 15.

Clinical Memory Research Unit, Department of Clinical Sciences, Malmö, Lund University, Lund, Sweden.

Retention of F-Flortaucipir is reportedly increased in the semantic variant of primary progressive aphasia (svPPA), which is dominated by TDP-43 pathology. However, it is unclear if F-Flortaucipir is also increased in other TDP-43 diseases, such as bvFTD caused by a C9orf72 gene mutation. We therefore recruited six C9orf72 expansion carriers, six svPPA patients, and 54 healthy controls. Read More

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http://dx.doi.org/10.1038/s41598-019-42625-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6465310PMC
April 2019
2 Reads

Machine learning based hierarchical classification of frontotemporal dementia and Alzheimer's disease.

Neuroimage Clin 2019 Apr 3;23:101811. Epub 2019 Apr 3.

Department of Bio-convergence Engineering, Korea University, Seoul, Republic of Korea; School of Biomedical Engineering, Korea University, Seoul, Republic of Korea. Electronic address:

Background: In a clinical setting, an individual subject classification model rather than a group analysis would be more informative. Specifically, the subtlety of cortical atrophy in some frontotemporal dementia (FTD) patients and overlapping patterns of atrophy among three FTD clinical syndromes including behavioral variant FTD (bvFTD), non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), and semantic variant PPA (svPPA) give rise to the need for classification models at the individual level. In this study, we aimed to classify each individual subject into one of the diagnostic categories in a hierarchical manner by employing a machine learning-based classification method. Read More

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http://dx.doi.org/10.1016/j.nicl.2019.101811DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458431PMC
April 2019
2 Reads

Alzheimer's Disease Including Focal Presentations.

Semin Neurol 2019 Apr 29;39(2):213-226. Epub 2019 Mar 29.

Department of Neurology, Institute of Memory and Alzheimer's Disease, Assistance Publique - Hopitaux de Paris, Pitié-Salpêtrière Hospital, Paris, France.

Alzheimer's disease (AD) is the commonest neurodegenerative disease and the most frequent cause of dementia. It affects 30 million people worldwide. Current research criteria focus on biomarkers' status for amyloid and tau using positron emission tomography and cerebrospinal fluid analysis, independent of clinical status. Read More

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http://dx.doi.org/10.1055/s-0039-1681041DOI Listing
April 2019
2 Reads

Serum insulin-like growth factor-1 levels in neurodegenerative diseases.

Acta Neurol Scand 2019 Mar 23. Epub 2019 Mar 23.

Department of Neurology, Dokkyo Medical University, Mibu, Japan.

Background: We investigated serum insulin-like growth factor (IGF)-1 levels in patients with neurodegenerative diseases and correlated these levels with clinical parameters.

Methods: One hundred and fifty-six patients with neurodegenerative diseases were included in this study, and serum IGF-1 levels were determined.

Results: Serum IGF-1 levels (mean ± standard error) were not significantly different among the patients with different neurodegenerative diseases: Parkinson's disease (PD; n = 73), 112. Read More

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http://dx.doi.org/10.1111/ane.13091DOI Listing
March 2019
1 Read

Variably protease-sensitive prionopathy mimicking frontotemporal dementia.

Neuropathology 2019 Apr 7;39(2):135-140. Epub 2019 Mar 7.

Clinical Neuropathology, King's College Hospital, NHS Foundation Trust, London, UK.

Sporadic prion diseases are fatal neurodegenerative disorders characterized clinically by rapidly progressive dementia and myoclonus. Variably protease-sensitive prionopathy (VPSPr) is a recently identified sporadic human prion disorder that may present with a lengthy atypical clinical history. Here, we describe a case of VPSPr in a patient with a long history of suspected frontotemporal dementia (FTD). Read More

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http://dx.doi.org/10.1111/neup.12538DOI Listing
April 2019
4 Reads

Hereditary primary lateral sclerosis and progressive nonfluent aphasia.

J Neurol 2019 May 5;266(5):1079-1090. Epub 2019 Mar 5.

Department of Neurology, Hospital Universitario Marqués de Valdecilla (IDIVAL), University of Cantabria, CIBERNED, Santander, Spain.

Objective: To report a kindred with an association between hereditary primary lateral sclerosis (PLS) and progressive nonfluent aphasia.

Patients And Methods: Six members from a kindred with 15 affected individuals spanning three generations, suffered from spasticity without muscle atrophy or fasciculation, starting in the lower limbs and spreading to the upper limbs and bulbar musculature, followed by effortful speech, nonfluent language and dementia, in 5 deceased members. Disease onset was during the sixth decade of life, or later. Read More

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http://link.springer.com/10.1007/s00415-019-09235-x
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http://dx.doi.org/10.1007/s00415-019-09235-xDOI Listing
May 2019
9 Reads

Automatically computed rating scales from MRI for patients with cognitive disorders.

Eur Radiol 2019 Feb 22. Epub 2019 Feb 22.

Combinostics Ltd., Hatanpään valtatie 24, 33100, Tampere, Finland.

Objectives: The aims of this study were to examine whether visual MRI rating scales used in diagnostics of cognitive disorders can be estimated computationally and to compare the visual rating scales with their computed counterparts in differential diagnostics.

Methods: A set of volumetry and voxel-based morphometry imaging biomarkers was extracted from T1-weighted and FLAIR images. A regression model was developed for estimating visual rating scale values from a combination of imaging biomarkers. Read More

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http://dx.doi.org/10.1007/s00330-019-06067-1DOI Listing
February 2019
2 Reads

Smoking and Obesity as Risk Factors in Frontotemporal Dementia and Alzheimer's Disease: The HUNT Study.

Dement Geriatr Cogn Dis Extra 2019 Jan-Apr;9(1):1-10. Epub 2019 Jan 9.

Clinic for Mental Health and Substance Abuse, Namsos Hospital, North-Trøndelag Hospital Trust, Namsos, Norway.

Background: Few studies have assessed smoking and obesity together as risk factors for frontotemporal dementia (FTD) and Alzheimer's disease (AD).

Objective: To study smoking and obesity as risk factors for FTD and AD.

Methods: Ninety patients with FTD and 654 patients with AD were compared with 116 cognitively healthy elderly individuals in a longitudinal design with 15-31 years between measurements of risk factors before the dementia diagnosis. Read More

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http://dx.doi.org/10.1159/000495607DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6381906PMC
January 2019
1 Read

Twin CHCH Proteins, CHCHD2, and CHCHD10: Key Molecules of Parkinson's Disease, Amyotrophic Lateral Sclerosis, and Frontotemporal Dementia.

Int J Mol Sci 2019 Feb 20;20(4). Epub 2019 Feb 20.

Department of Research for Parkinson's Disease, Juntendo University Graduate School of Medicine, Tokyo 113-8421, Japan.

Mutations of () and () have been found to be linked to Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and/or frontotemporal lobe dementia (FTD). CHCHD2 and CHCHD10 proteins, which are homologous proteins with 54% identity in amino acid sequence, belong to the mitochondrial coiled-coil-helix-coiled-coil-helix (CHCH) domain protein family. A series of studies reveals that these twin proteins form a multimodal complex, producing a variety of pathophysiology by the disease-causing variants of these proteins. Read More

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http://dx.doi.org/10.3390/ijms20040908DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6412816PMC
February 2019
8 Reads

Longitudinal Changes in Semantic Concreteness in Semantic Variant Primary Progressive Aphasia (svPPA).

eNeuro 2018 Nov-Dec;5(6). Epub 2018 Jan 10.

Department of Neurology and Penn Frontotemporal Degeneration Center.

This study examines longitudinal changes in the concreteness of nouns produced by human patients with semantic variant primary progressive aphasia (svPPA). Cross-sectional studies show that patients with svPPA demonstrate severe loss of concrete noun knowledge linked to atrophy of the left ventral temporal lobe. It is unknown how disease spread and duration affect the magnitude of the concreteness impairment in svPPA. Read More

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http://dx.doi.org/10.1523/ENEURO.0197-18.2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6377408PMC
March 2019
1 Read

Dementia spectrum disorders: lessons learnt from decades with PET research.

J Neural Transm (Vienna) 2019 Mar 14;126(3):233-251. Epub 2019 Feb 14.

Neurodegeneration Imaging Group, Maurice Wohl Clinical Neuroscience Institute, 125 Coldharbour Lane, Camberwell, London, SE5 9NU, UK.

The dementia spectrum encompasses a range of disorders with complex diagnosis, pathophysiology and limited treatment options. Positron emission tomography (PET) imaging provides insights into specific neurodegenerative processes underlying dementia disorders in vivo. Here we focus on some of the most common dementias: Alzheimer's disease, Parkinsonism dementias including Parkinson's disease with dementia, dementia with Lewy bodies, progressive supranuclear palsy and corticobasal syndrome, and frontotemporal lobe degeneration. Read More

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http://dx.doi.org/10.1007/s00702-019-01975-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6449308PMC
March 2019
4 Reads

Music Therapy and Physical Activity to Ease Anxiety, Restlessness, Irritability, and Aggression in Individuals With Dementia With Signs of Frontotemporal Lobe Degeneration.

J Psychosoc Nurs Ment Health Serv 2019 Feb 8:1-9. Epub 2019 Feb 8.

The purpose of the current study was to evaluate whether a combined intervention of physical activity and music therapy could reduce anxiety, restlessness, irritability, and aggression among individuals with severe dementia. An exploratory design was used to evaluate a combined intervention of physical activity, music therapy, and daily walking. Interventions were systematically implemented for 8 weeks. Read More

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http://dx.doi.org/10.3928/02793695-20190124-02DOI Listing
February 2019
5 Reads

Atypical globular glial tauopathy with a combination of types I and II pathology.

Neuropathology 2019 Apr 5;39(2):127-134. Epub 2019 Feb 5.

Department of Neuropathology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.

Globular glial tauopathy (GGT) is a group of 4-repeat tauopathies characterized by widespread globular glial inclusions (GGIs). GGT is now classified into three subtypes based on the distribution and morphology of the GGIs. We report an autopsy case of GGT in an 85-year-old woman who presented with semantic dementia, a rare phenotype in GGT. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/neup.12536
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http://dx.doi.org/10.1111/neup.12536DOI Listing
April 2019
14 Reads

Gray and white matter changes in presymptomatic genetic frontotemporal dementia: a longitudinal MRI study.

Neurobiol Aging 2019 Apr 7;76:115-124. Epub 2019 Jan 7.

Department of Neurology, Erasmus Medical Center, Rotterdam, the Netherlands. Electronic address:

In genetic frontotemporal dementia, cross-sectional studies have identified profiles of presymptomatic neuroanatomical loss for C9orf72 repeat expansion, MAPT, and GRN mutations. In this study, we characterize longitudinal gray matter (GM) and white matter (WM) brain changes in presymptomatic frontotemporal dementia. We included healthy carriers of C9orf72 repeat expansion (n = 12), MAPT (n = 15), GRN (n = 33) mutations, and related noncarriers (n = 53), that underwent magnetic resonance imaging at baseline and 2-year follow-up. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2018.12.017DOI Listing
April 2019
1 Read

TDP43 pathology in the brain, spinal cord, and dorsal root ganglia of a patient with FOSMN.

Neurology 2019 Feb 30;92(9):e951-e956. Epub 2019 Jan 30.

From the MRC Centre for Neuromuscular Diseases (A.M.R., M.M.R.), Department of Neurodegenerative Disease (Z.J.), Queen Square Brain Bank (Z.J., G.H.), and Dementia Research Centre (M.N.R.), UCL Institute of Neurology; National Hospital for Neurology and Neurosurgery (A.M.R., M.M.R., M.N.R.); and Division of Neuropathology (Z.J.), National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, UK.

Objective: To describe the histopathologic features of a case of facial-onset sensory and motor neuronopathy (FOSMN).

Methods: We describe a postmortem examination performed on a 54-year-old man with FOSMN associated with personality change.

Results: Postmortem examination revealed TAR DNA-binding protein (TDP) 43 proteinopathy with widespread distribution. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6404468PMC
February 2019
13 Reads

Imaging of tau deposits in adults with Niemann-Pick type C disease: a case-control study.

Eur J Nucl Med Mol Imaging 2019 May 28;46(5):1132-1138. Epub 2019 Jan 28.

The Florey Institute of Neuroscience and Mental Health, The University of Melbourne, Melbourne, VIC, Australia.

Purpose: Niemann-Pick type C (NPC) is a cholesterol storage disease characterized by disruption in the endosomal-lysosomal transport system that leads to the accumulation of cholesterol and glycolipids in lysosomes. Developmental cognitive delay and progressive motor and cognitive impairment are characteristic of the disease. Tau accumulation has been reported in some NPC patients. Read More

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http://dx.doi.org/10.1007/s00259-019-4273-7DOI Listing
May 2019
3 Reads

Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 22:1-13. Epub 2019 Jan 22.

a Department of Neurology, Memory and Aging Center , University of California , San Francisco , CA , USA.

Objective: Behavioral variant frontotemporal dementia (bvFTD), is commonly considered the cognitive presentation of the frontotemporal dementia-motor neuron disease (FTD-MND) spectrum disorder. We evaluated the prevalence of primary progressive aphasia in a series of pathologically confirmed cases of FTD-MND spectrum.

Methods: Pathologically confirmed cases of frontotemporal lobar degeneration-motor neuron disease (FTLD-MND) were obtained from the UCSF brain bank. Read More

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http://dx.doi.org/10.1080/21678421.2018.1556695DOI Listing
January 2019
24 Reads

Expression and Function of Zinc-α2-Glycoprotein.

Neurosci Bull 2019 Jan 4. Epub 2019 Jan 4.

Department of Neurology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, 400010, China.

Zinc-α2-glycoprotein (ZAG), encoded by the AZGP1 gene, is a major histocompatibility complex I molecule and a lipid-mobilizing factor. ZAG has been demonstrated to promote lipid metabolism and glucose utilization, and to regulate insulin sensitivity. Apart from adipose tissue, skeletal muscle, liver, and kidney, ZAG also occurs in brain tissue, but its distribution in brain is debatable. Read More

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http://dx.doi.org/10.1007/s12264-018-00332-xDOI Listing
January 2019
3 Reads

The Frontotemporal Dementia versus Primary Psychiatric Disorder (FTD versus PPD) Checklist: A Bedside Clinical Tool to Identify Behavioral Variant FTD in Patients with Late-Onset Behavioral Changes.

J Alzheimers Dis 2019 ;67(1):113-124

Department of Neurology, Alzheimer Center Amsterdam, Amsterdam Neuroscience Campus, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, The Netherlands.

Background: Differentiating early behavioral variant frontotemporal dementia (bvFTD) and primary psychiatric disorders (PPD) is complex and biomarkers have limited accuracy, leading to inaccurate diagnoses.

Objectives: Develop a simple bedside clinical tool to differentiate bvFTD from PPD.

Methods: A checklist of clinical features differentiating bvFTD from PPD was developed based on literature and clinical experience. Read More

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http://dx.doi.org/10.3233/JAD-180839DOI Listing
January 2019
4 Reads

Dual-phase [18F]florbetapir in frontotemporal dementia.

Eur J Nucl Med Mol Imaging 2019 02 19;46(2):304-311. Epub 2018 Dec 19.

Wolfson Molecular Imaging Centre, Faculty of Medicine, Biology and Health, University of Manchester, 27 Palatine Road, Manchester, M20 3LJ, UK.

Purpose: The PET tracer [18F]florbetapir is a specific fibrillar amyloid-beta (Aβ) biomarker. During the late scan phase (> 40 min), it provides pathological information about Aβ status. Early scan phase (0-10 min) can provide FDG-'like' information. Read More

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http://dx.doi.org/10.1007/s00259-018-4238-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333719PMC
February 2019
8 Reads

Cognitive and Pathological Influences of Tau Pathology in Lewy Body Disorders.

Ann Neurol 2019 Feb 7;85(2):259-271. Epub 2019 Jan 7.

Department of Neurology, Perelman School of Medicine at the University of Pennsylvania.

Objective: To use digital histology in a large autopsy cohort of Lewy body disorder (LBD) patients with dementia to test the hypotheses that co-occurring Alzheimer disease (AD) pathology impacts the anatomic distribution of α-synuclein (SYN) pathology and that co-occurring neocortical tau pathology in LBDs associates with worse cognitive performance and occurs in a pattern differing from AD.

Methods: Fifty-five autopsy-confirmed LBD (Parkinson disease with dementia, n = 36; dementia with Lewy bodies, n = 19) patients and 25 AD patients were studied. LBD patients were categorized as having moderate/severe AD copathology (SYN + AD = 20) or little/no AD copathology (SYN-AD = 35). Read More

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http://dx.doi.org/10.1002/ana.25392DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375484PMC
February 2019
3 Reads

Presence of tau astrogliopathy in frontotemporal dementia caused by a novel Grn nonsense (Trp2*) mutation.

Neurobiol Aging 2019 Apr 20;76:214.e11-214.e15. Epub 2018 Nov 20.

Brain Tissue Bank, Fundación CIEN, Instituto de Salud Carlos III, Madrid, Spain.

Frontotemporal lobar degeneration caused by GRN mutations is mainly associated with a TDP-43 type A proteinopathy. We present a family with autosomal dominant frontotemporal lobar degeneration caused by a novel GRN nonsense mutation (c.5G>A: p. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2018.11.010DOI Listing

Structural and metabolic cerebral alterations between elderly bipolar disorder and behavioural variant frontotemporal dementia: A combined MRI-PET study.

Aust N Z J Psychiatry 2018 Dec 13:4867418815976. Epub 2018 Dec 13.

1 Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.

Background:: Elderly bipolar disorder (BD) and behavioural variant of frontotemporal dementia (bvFTD) may exhibit similar symptoms and both disorders are characterized by selective abnormalities in cortical and subcortical regions that are associated with cognitive and emotional impairments. We aimed to investigate common and distinct neural substrates of BD and bvFTD by coupling, for the first time, magnetic resonance imaging (MRI) and positron emission tomography (PET) techniques.

Methods:: 3-Tesla MRI and 18 fluorodeoxyglucose-PET scans were acquired for 16 elderly BD patients, 23 bvFTD patients with mild cognitive impairments and 68 healthy controls (48 for PET and 20 for MRI analyses). Read More

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http://dx.doi.org/10.1177/0004867418815976DOI Listing
December 2018
2 Reads

Spatiotemporal analysis for detection of pre-symptomatic shape changes in neurodegenerative diseases: Initial application to the GENFI cohort.

Neuroimage 2019 Mar 6;188:282-290. Epub 2018 Dec 6.

Department of Medical Physics and Biomedical Engineering, University College London, United Kingdom; Dementia Research Centre, UCL Queen Square Institute of Neurology, University College of London, WC1N 3BG, London, United Kingdom; School of Biomedical Engineering and Imaging Sciences, King's College London, United Kingdom.

Brain atrophy as measured from structural MR images, is one of the primary imaging biomarkers used to track neurodegenerative disease progression. In diseases such as frontotemporal dementia or Alzheimer's disease, atrophy can be observed in key brain structures years before any clinical symptoms are present. Atrophy is most commonly captured as volume change of key structures and the shape changes of these structures are typically not analysed despite being potentially more sensitive than summary volume statistics over the entire structure. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10538119183214
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http://dx.doi.org/10.1016/j.neuroimage.2018.11.063DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414401PMC
March 2019
14 Reads

Review: Fluid biomarkers for frontotemporal dementias.

Neuropathol Appl Neurobiol 2019 Feb 3;45(1):81-87. Epub 2018 Dec 3.

Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, Queen Square, London, UK.

Frontotemporal dementias (FTDs) are clinically, genetically and pathologically heterogeneous neurodegenerative disorders that affect the frontal and anterior temporal lobes of the brain. They are relatively common causes of young-onset dementia and usually present with behavioural disturbance (behavioural variant FTD) or language impairment (primary progressive aphasia), but there is also overlap with motor neurone disease and the atypical parkinsonian disorders, corticobasal syndrome and progressive supranuclear palsy. At post mortem, neuronal inclusions containing tau, TDP-43 or infrequently FUS protein are seen in most cases. Read More

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http://doi.wiley.com/10.1111/nan.12530
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http://dx.doi.org/10.1111/nan.12530DOI Listing
February 2019
17 Reads

The Edinburgh Cognitive and Behavioral ALS screen: relationship to age, education, IQ and the Addenbrooke's Cognitive Examination-III.

Amyotroph Lateral Scler Frontotemporal Degener 2018 Nov 29;19(7-8):585-590. Epub 2018 Oct 29.

a Human Cognitive Neuroscience - Department of Psychology , The University of Edinburgh , Edinburgh , UK.

The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) was developed to assess cognitive and behavioral changes common in Amyotrophic Lateral Sclerosis and other diseases affecting motor functions. It focuses on domains typically affected by the frontotemporal syndrome (executive and language functions, fluency and behavior), but assesses also memory and visuospatial functions.

Objectives: (A) To investigate the relationship between the ECAS and the Addenbrooke's Cognitive Examination (ACE-III). Read More

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http://dx.doi.org/10.1080/21678421.2018.1491601DOI Listing
November 2018
14 Reads

Predicting progression in the late onset frontal lobe syndrome.

Int Psychogeriatr 2018 Oct 26:1-6. Epub 2018 Oct 26.

Department of Old Age Psychiatry,GGZinGeest/VU University Medical Center,Amsterdam,the Netherlands.

ABSTRACTA late onset frontal lobe syndrome (LOF) refers to a clinical syndrome with apathy, disinhibition, or stereotypical behavior arising in middle or late adulthood. Diagnostics are challenging, and both clinicians and patients need reliable predictors of progression to improve clinical guidance. In this longitudinal multicenter and genetically screened prospective study, 137 LOF patients with frontal behavior (FBI score≥11) and/or stereotypical behavior (SRI≥10) were included. Read More

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http://dx.doi.org/10.1017/S1041610218001242DOI Listing
October 2018
20 Reads

Serum neurofilament light chain in behavioral variant frontotemporal dementia.

Neurology 2018 Oct 12;91(15):e1390-e1401. Epub 2018 Sep 12.

From the Department of Neurology (P.S., S.A.-S., E.S., I.U., C.A.F.v.A., J. Kassubek, B.L., P.O., A.C.L., M.O.) and Institute of Epidemiology and Medical Biometry (B.M.), University of Ulm; Department of Psychiatry and Psychotherapy (J.D.-S., H.F., T.G.), Klinikum Rechts der Isar, Technical University of Munich; Department of Nuclear Medicine (H.B.), Leipzig University Hospital; Department of Neurology (A.D.), Ludwig-Maximilians-University, Munich; Department of Neurology (K.F.), Saarland University, Homburg; Department of Psychiatry and Psychotherapy (K.F.), University of Bonn, Germany; Swiss Epilepsy Center (H.-J.H.), Zurich, Switzerland; Department of Psychiatry and Psychotherapy (H.J.), University Medical Center Hamburg-Eppendorf, Hamburg; AMEOS Klinikum (H.J.), Heiligenhafen; Department of Psychiatry and Psychotherapy (J. Kornhuber, J.M.M.), Friedrich-Alexander-University of Erlangen-Nuremberg, Erlangen; Department of Psychiatry and Psychotherapy (M.L.), University of Würzburg; Department of Neurology (J.P.), University of Rostock; DZNE (J.P.), Rostock; Department of Neurodegenerative Diseases and Geriatric Psychiatry (A.S.), University Hospital Bonn; DZNE (A.S.), Bonn; Institute of Human Genetics (A.E.V.), University Medical Center Hamburg-Eppendorf, Hamburg; Department of Psychiatry and Psychotherapy (J.W.), University Medical Center Göttingen; DZNE (J.W.), Göttingen, Germany; iBiMED (J.W.), Medical Sciences Department, University of Aveiro, Portugal; Clinic for Cognitive Neurology (M.L.S.), University Clinic Leipzig; and Max Planck Institute for Human Cognitive and Brain Sciences (M.L.S.), Leipzig, Germany.

Objective: To determine the association of serum neurofilament light chain (NfL) with functional deterioration and brain atrophy during follow-up of patients with behavioral variant frontotemporal dementia (bvFTD).

Methods: Blood NfL levels from 74 patients with bvFTD, 26 with Alzheimer disease (AD), 17 with mild cognitive impairment (MCI), and 15 healthy controls (Con) at baseline and follow-up were determined and analyzed for the diagnostic potential in relation to functional assessment (Clinical Dementia Rating Scale Sum of Boxes [CDR-SOB], frontotemporal lobar degeneration-related CDR-SOB, Mini-Mental State Examination [MMSE]) and brain volumetry.

Results: At baseline, serum NfL level correlated with CSF NfL (bvFTD = 0. Read More

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http://dx.doi.org/10.1212/WNL.0000000000006318DOI Listing
October 2018
11 Reads
8.290 Impact Factor

Differences in gray and white matter F-THK5351 uptake between behavioral-variant frontotemporal dementia and other dementias.

Eur J Nucl Med Mol Imaging 2019 02 14;46(2):357-366. Epub 2018 Aug 14.

Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.

Purpose: We investigated the regional distribution of F-THK5351 uptake in gray (GM) and white matter (WM) in patients with behavioral-variant frontotemporal dementia (bvFTD) and compared it with that in patients with Alzheimer's disease (AD) or semantic dementia (SD).

Methods: F-THK-5351 positron emission tomography (PET), F-florbetaben PET, magnetic resonance imaging, and neuropsychological testing were performed in 103 subjects including 30, 24, 9, and 8 patients with mild cognitive impairment, AD, bvFTD, and SD, respectively, and 32 normal subjects. Standardized uptake value ratios (SUVRs) of F-THK-5351 PET images were measured from six GM and WM regions using cerebellar GM as reference. Read More

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http://link.springer.com/10.1007/s00259-018-4125-x
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http://dx.doi.org/10.1007/s00259-018-4125-xDOI Listing
February 2019
12 Reads

Disease trajectories in behavioural variant frontotemporal dementia, primary psychiatric and other neurodegenerative disorders presenting with behavioural change.

J Psychiatr Res 2018 Sep 1;104:183-191. Epub 2018 Aug 1.

Alzheimer Center Amsterdam, Department of Neurology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdan UMC, Amsterdam, the Netherlands.

Behavioural variant frontotemporal dementia (bvFTD) is characterized by behavioural and social cognitive disturbances, while various psychiatric and neurodegenerative disorders may have similar clinical symptoms. Since neurodegenerative disorders are eventually progressive, whereas primary psychiatric disorders are not, this study aimed to investigate whether the change in clinical symptoms over time differed between groups and which biomarkers predicted rate of decline. Disease trajectories (median follow-up = 3 years) of frontal and stereotyped behaviour, general and frontal cognitive functioning, and social cognition were examined in bvFTD (n = 34), other neurodegenerative (n = 28) and primary psychiatric disorders (n = 43), all presenting with late-onset frontal lobe syndrome (45-75 years), using linear mixed models. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00223956183050
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http://dx.doi.org/10.1016/j.jpsychires.2018.07.014DOI Listing
September 2018
30 Reads

Regional thalamic MRI as a marker of widespread cortical pathology and progressive frontotemporal involvement in amyotrophic lateral sclerosis.

J Neurol Neurosurg Psychiatry 2018 Dec 26;89(12):1250-1258. Epub 2018 Jul 26.

Wellcome Centre for Integrative Neuroimaging, University of Oxford, Oxford, UK

Background: The thalamus is a major neural hub, with selective connections to virtually all cortical regions of the brain. The multisystem neurodegenerative syndrome amyotrophic lateral sclerosis (ALS) has pathogenic overlap with frontotemporal dementia, and objective in vivo markers of extra-motor pathological spread are lacking. To better consider the role of the thalamus in neurodegeneration, the present study assessed the integrity of the thalamus and its connectivity to major cortical regions of the brain in a longitudinal manner. Read More

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http://dx.doi.org/10.1136/jnnp-2018-318625DOI Listing
December 2018
11 Reads

Behavioral Variant Frontotemporal Dementia Performance on a Range of Saccadic Tasks.

J Alzheimers Dis 2018 ;65(1):231-242

Department of Optometry and Vision Sciences, The University of Melbourne, Melbourne, Australia.

Background: Saccadic paradigms display changes across a number of degenerative conditions reflecting changes in the oculomotor pathway which in some conditions have been linked to disease presentation.

Objective: To examine a novel range of saccadic paradigms in behavioral variant frontotemporal dementia (bvFTD).

Methods: Prosaccade, predictive, self-paced, memory-guided, and anti-saccade tasks were examined in bvFTD patients and controls. Read More

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http://dx.doi.org/10.3233/JAD-170797DOI Listing
January 2018
3 Reads

[Inclusion Body Myopathy, Paget's Disease, and Fronto-temporal Dementia: a VCP-related Multi-systemic Proteinopathy].

Fortschr Neurol Psychiatr 2018 07 20;86(7):434-438. Epub 2018 Jul 20.

Institut für Neuropathologie, Universitätsklinikum Erlangen, Erlangen.

Mutations of the human VCP gene, which encodes the V: alosin C: ontaining P: rotein (synonyms: p97, TER ATPase), are associated with various multi-systemic protein aggregation diseases. We report on a patient with progressive myopathy and incipient cognitive deficits. A diagnostic muscle biopsy revealed an inclusion body myopathy with protein aggregates. Read More

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http://dx.doi.org/10.1055/s-0044-101033DOI Listing
July 2018
2 Reads

Cortical markers of cognitive syndromes in amyotrophic lateral sclerosis.

Neuroimage Clin 2018 17;19:675-682. Epub 2018 May 17.

Institute for Advanced Study-IUSS Pavia, Palazzo del Broletto e Piazza Vittoria 15, 27100 Pavia, Italy; IRCCS S. Giovanni di Dio Fatebenefratelli, via Pilastroni 4, 25125 Brescia, Italy.

Amyotrophic lateral sclerosis (ALS) can be associated with a spectrum of cognitive and behavioural symptoms, but the related patterns of focal cortical atrophy in non-demented ALS patients remain largely unknown. We enrolled 48 non-demented ALS patients and 26 healthy controls for a comprehensive neuropsychological assessment and a magnetic resonance exam. Behavioural and cognitive impairment was defined on the basis of a data-driven multi-domain approach in 21 ALS patients. Read More

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http://dx.doi.org/10.1016/j.nicl.2018.05.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6046611PMC
January 2019
35 Reads

Cerebellar atrophy and its contribution to cognition in frontotemporal dementias.

Ann Neurol 2018 Jul 14;84(1):98-109. Epub 2018 Aug 14.

School of Psychology, University of Sydney.

Objective: Increasing evidence suggests that cerebellar damage impacts on cognitive functions. Frontotemporal dementias (FTDs) are neurodegenerative brain conditions, primarily affecting the frontal and/or temporal lobe. Three main phenotypes are recognized, each with a distinct clinical and cognitive profile: behavioral-variant FTD (bvFTD), semantic dementia (SD), and progressive nonfluent aphasia (PNFA). Read More

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http://dx.doi.org/10.1002/ana.25271DOI Listing
July 2018
29 Reads

Distinct expression of the neurotoxic microRNA family let-7 in the cerebrospinal fluid of patients with Alzheimer's disease.

PLoS One 2018 16;13(7):e0200602. Epub 2018 Jul 16.

Institute of Cell Biology and Neurobiology, Center for Anatomy, Charité - Universitaetsmedizin Berlin, Berlin, Germany.

MicroRNAs (miRNAs) are non-coding RNAs originally involved in RNA silencing and post-transcriptional regulation of gene expression. We have shown in previous work that the miRNA let-7b can act as a signalling molecule for Toll-like receptor 7, thereby initiating innate immune pathways and apoptosis in the central nervous system. Here, we investigated whether different members of the miRNA family let-7, abundantly expressed in the brain, are released into the human cerebrospinal fluid (CSF) and whether quantitative differences in let-7 copies exist in neurodegenerative diseases. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0200602PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6047809PMC
January 2019
10 Reads

Distinct Neuroanatomical Correlates of Neuropsychiatric Symptoms in the Three Main Forms of Genetic Frontotemporal Dementia in the GENFI Cohort.

J Alzheimers Dis 2018 ;65(1):147-163

Clinique Interdisciplinaire de Mémoire(CIME), Université Laval, QC, Canada.

Background: The overlap between frontotemporal dementia (FTD) and primary psychiatric disorders has been brought to light by reports of prominent neuropsychiatric symptoms (NPS) in FTD-related genetic mutations, particularly among C9orf72 and GRN carriers. It has been recently demonstrated that early neuroanatomical changes in genetic FTD may be different across the major disease-causing mutations.

Objective: We aimed to identify whether NPS could be driven by distinct structural correlates. Read More

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http://dx.doi.org/10.3233/JAD-180053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6087430PMC
January 2018
13 Reads

The neuropsychological profiles and semantic-critical regions of right semantic dementia.

Neuroimage Clin 2018 29;19:767-774. Epub 2018 May 29.

Department of Neurology, Huashan Hospital, Fudan University, Shanghai, China. Electronic address:

Introduction: Previous literature has revealed that the anterior temporal lobe (ATL) is the semantic hub of left-sided or mixed semantic dementia (SD), whilst the semantic hub of right-sided SD has not been examined.

Methods: Seventeen patients with right-sided SD, 18 patients with left-sided SD and 20 normal controls (NC) underwent neuropsychological assessments and magnetic resonance imaging scans. We investigated the relationship between the degree of cerebral atrophy in the whole brain and the severity of semantic deficits in left and right-sided SD samples, respectively. Read More

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http://dx.doi.org/10.1016/j.nicl.2018.05.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6041419PMC
January 2019
15 Reads

[Resting-state functional MRI studies of amyotrophic lateral sclerosis patients with various levels of cognitive impairment].

Zhonghua Yi Xue Za Zhi 2018 Jul;98(25):2002-2006

Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.

To characterize the brain functional changes of amyotrophic lateral sclerosis (ALS) patients with various levels of cognitive impairment as measured by resting-state functional MRI (RS-fMRI). From September 2013 to March 2017, a total of 55 patients diagnosed with ALS in Peking Union Medical College Hospital and 20 healthy controls (HCs) were included in this study, and all participants underwent neuropsychological assessments and diffusion tensor imaging scans. According to their cognitive performance, ALS patients were further subclassified into ALS with normal cognition (ALS-Cn, =27), those with cognitive impairment (ALS-Ci, =17) and ALS-FTD (=11). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2018.25.007DOI Listing
July 2018
3 Reads

Reorganization of cortical oscillatory dynamics underlying disinhibition in frontotemporal dementia.

Brain 2018 Aug;141(8):2486-2499

Department of Clinical Neurosciences, University of Cambridge, UK.

The distribution of pathology in frontotemporal dementia is anatomically selective, to distinct cortical regions and with differential neurodegeneration across the cortical layers. The cytoarchitecture and connectivity of cortical laminae preferentially supports frequency-specific oscillations and hierarchical information transfer between brain regions. We therefore predicted that in frontotemporal dementia, core functional deficits such as disinhibition would be associated with differences in the frequency spectrum and altered cross-frequency coupling between frontal cortical regions. Read More

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http://dx.doi.org/10.1093/brain/awy176DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6061789PMC
August 2018
5 Reads

Early-stage right temporal lobe variant of frontotemporal dementia: 3 years of follow-up observations.

BMJ Case Rep 2018 Jun 29;2018. Epub 2018 Jun 29.

Center for Health Affairs, Kindai University, Higashiosaka, Osaka, Japan.

The right temporal lobe variant of frontotemporal dementia (FTD) is an uncommon progressive neurodegenerative disorder. We present the case of a 77-year-old right-handed man who presented with altered behaviour and problems with interpersonal relationships. He had no decline in cognitive function but brain perfusion single-photon emission CT demonstrated distinct hypoperfusion in the right temporal pole. Read More

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http://dx.doi.org/10.1136/bcr-2018-224431DOI Listing
June 2018
12 Reads

A mosquito bites and a butterfly flies: A specific response type of frontal patients in a similarity task.

Neuropsychologia 2018 08 26;117:371-378. Epub 2018 Jun 26.

Institut du Cerveau et de la Moelle épinière (ICM), UPMC UMRS 1127, Inserm U 1127, CNRS UMR 7225, Paris, France; Neurology department, Memory and Aging center, Pitié-Salpêtrière Hospital, AP-HP, Paris, France; Neurology department, Behavioral Neuro-psychiatry Unit, Pitié-Salpêtrière Hospital, AP-HP, Paris, France.

Background: Patients with neurodegenerative diseases affecting the frontal lobes have difficulties in categorization tasks, such as the similarity tasks. They give two types of unusual response to the question: "In what way are an orange and a banana alike?", either a differentiation ("one is yellow, the other is orange") or a concrete similarity ("they are sweet").

Objective: To characterize the categorization deficit of frontal patients and develop a short diagnostic tool to assess the nature of these difficulties. Read More

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http://dx.doi.org/10.1016/j.neuropsychologia.2018.06.022DOI Listing
August 2018
6 Reads

Parkinsonism is associated with altered primary motor cortex plasticity in frontotemporal dementia-primary progressive aphasia variant.

Neurobiol Aging 2018 09 29;69:230-238. Epub 2018 May 29.

IRCCS Neuromed Institute, Pozzilli, Province of Isernia, Italy; Department of Human Neuroscience, Sapienza University of Rome, Rome, Italy. Electronic address:

In frontotemporal dementia (FTD), the behavioral variant (bv-FTD) and nonfluent variant of primary progressive aphasia (nfv-PPA) reflect a prominent neurodegenerative involvement of the frontal lobe networks, which may include the premotor and motor areas and thus cause heterogeneous clinical symptoms including parkinsonism. With the technique of transcranial magnetic stimulation, we investigated long-term potentiation- and long-term depression-like plasticity in the primary motor cortex of bv-FTD and nfv-PPA patients, with and without parkinsonism, by using the theta-burst stimulation (TBS) protocol. We applied the intermittent TBS and continuous TBS in 20 FTD patients and 18 age-matched healthy subjects. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2018.05.026DOI Listing
September 2018
5 Reads

Hippocampal Subfield Volumetry: Differential Pattern of Atrophy in Different Forms of Genetic Frontotemporal Dementia.

J Alzheimers Dis 2018 ;64(2):497-504

Dementia Research Centre, Department of Neurodegenerative Disease, Institute of Neurology, University College London, London, UK.

Background: Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder, with a strong genetic component. Previous research has shown that medial temporal lobe atrophy is a common feature of FTD. However, no study has so far investigated the differential vulnerability of the hippocampal subfields in FTD. Read More

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http://dx.doi.org/10.3233/JAD-180195DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6027942PMC
January 2018
16 Reads

Brain Structural and Perfusion Signature of Amyotrophic Lateral Sclerosis With Varying Levels of Cognitive Deficit.

Front Neurol 2018 24;9:364. Epub 2018 May 24.

Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.

Objective: To characterize the patterns of brain atrophy and perfusion as measured by arterial spin labeling (ASL)-MRI, in amyotrophic lateral sclerosis (ALS) patients with varying levels of cognitive deficit, including ALS with frontotemporal dementia (FTD).

Methods: A total of 55 ALS patients and 20 healthy controls (HCs) were included, and all participants underwent neuropsychological assessments and MRI scans. According to their cognitive performance, ALS patients were further subclassified into ALS with normal cognition (ALS-Cn,  = 27), ALS with cognitive impairment (ALS-Ci,  = 17), and ALS-FTD ( = 11). Read More

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https://www.frontiersin.org/article/10.3389/fneur.2018.00364
Publisher Site
http://dx.doi.org/10.3389/fneur.2018.00364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5976730PMC
May 2018
13 Reads

Prefrontal cortical thickness in motor neuron disease.

Neuroimage Clin 2018 2;18:648-655. Epub 2018 Mar 2.

German Center for Neurodegenerative Diseases (DZNE), Site Magdeburg, Leipziger Straße 44, 39120 Magdeburg, Germany.

Objective: To examine whether the distribution of prefrontal cortical thickness in patients with motor neuron disease is normal or bimodal and how it compares to the normal population.

Methods: 158 patients with motor neuron disease (MND) and 86 healthy controls (HC) were enrolled in a prospective, two-center study with a common structural MRI protocol. Cortical thickness measures were extracted for the prefrontal cortex, premotor cortex, motor cortex, and occipital cortex using FreeSurfer, adjusted for age and sex, and tested for normality of distribution. Read More

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http://dx.doi.org/10.1016/j.nicl.2018.03.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5987868PMC
January 2019
23 Reads

Distinct patterns of brain atrophy in Genetic Frontotemporal Dementia Initiative (GENFI) cohort revealed by visual rating scales.

Alzheimers Res Ther 2018 05 24;10(1):46. Epub 2018 May 24.

Department of Pathophysiology and Transplantation, "Dino Ferrari" Center, University of Milan, Milan, Italy.

Background: In patients with frontotemporal dementia, it has been shown that brain atrophy occurs earliest in the anterior cingulate, insula and frontal lobes. We used visual rating scales to investigate whether identifying atrophy in these areas may be helpful in distinguishing symptomatic patients carrying different causal mutations in the microtubule-associated protein tau (MAPT), progranulin (GRN) and chromosome 9 open reading frame (C9ORF72) genes. We also analysed asymptomatic carriers to see whether it was possible to visually identify brain atrophy before the appearance of symptoms. Read More

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http://dx.doi.org/10.1186/s13195-018-0376-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5968621PMC
May 2018
11 Reads

Can visuospatial measures improve the diagnosis of Alzheimer's disease?

Alzheimers Dement (Amst) 2018 6;10:66-74. Epub 2017 Nov 6.

Central Medical School, The University of Sydney, Sydney, New South Wales, Australia.

Introduction: Overlapping and evolving symptoms lead to ambiguity in the diagnosis of dementia. Visuospatial function relies on parietal lobe function, which may be affected in the early stages of Alzheimer's disease (AD). This review evaluates visuospatial dysfunction in patients with AD, frontotemporal dementia, dementia with Lewy bodies, and vascular dementia to determine the diagnostic and prognostic potential of visuospatial tasks in AD. Read More

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http://dx.doi.org/10.1016/j.dadm.2017.10.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5956809PMC
November 2017
5 Reads

Inhibition impairment in frontotemporal dementia, amyotrophic lateral sclerosis, and Alzheimer's disease: clinical assessment and metabolic correlates.

Brain Imaging Behav 2018 May 10. Epub 2018 May 10.

Department of Neurology. Hospital Clínico San Carlos, San Carlos Health Research Institute (IdISSC), Universidad Complutense de Madrid, Profesor Martin Lagos St s/n, 28040, Madrid, Spain.

The ability to reject an automatic tendency, i.e. inhibition, has been linked to the prefrontal cortex, but its neural underpinnings are still controversial. Read More

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http://dx.doi.org/10.1007/s11682-018-9891-3DOI Listing
May 2018
7 Reads
3.380 Impact Factor

Selective impairments in components of affective prosody in neurologically impaired individuals.

Brain Cogn 2018 07 1;124:29-36. Epub 2018 May 1.

Johns Hopkins University School of Medicine, Department of Neurology, USA; Johns Hopkins University School of Medicine, Department of Physical and Medicine & Rehabilitation, USA; Johns Hopkins University, Department of Cognitive Science, USA. Electronic address:

The intent and feelings of the speaker are often conveyed less by what they say than by how they say it, in terms of the affective prosody - modulations in pitch, loudness, rate, and rhythm of the speech to convey emotion. Here we propose a cognitive architecture of the perceptual, cognitive, and motor processes underlying recognition and generation of affective prosody. We developed the architecture on the basis of the computational demands of the task, and obtained evidence for various components by identifying neurologically impaired patients with relatively specific deficits in one component. Read More

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http://dx.doi.org/10.1016/j.bandc.2018.04.001DOI Listing
July 2018
17 Reads