The frontotemporal dementia-motor neuron disease continuum.

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Title: The frontotemporal dementia-motor neuron disease continuum.
Authors: Burrell, James R., Halliday, Glenda M., Kril, Jillian J., Ittner, Lars M., Götz, Jürgen, Kiernan, Matthew C., Hodges, John R.
Source: Lancet. 8/27/2016, Vol. 388 Issue 10047, p919-931. 13p.
Subjects: Dementia, Motor neuron diseases, Carrier proteins, Cognition disorders, Neurobehavioral disorders, Protein metabolism, Brain metabolism, Neuroprotective agents, Riluzole, Brain, Cognition, DNA, Health care teams, Digital image processing, Neuropsychological tests, Genetic mutation, Neuroradiology, Prognosis, Proteins, DNA-binding proteins, Activities of daily living, Executive function, DNA methylation, Frontotemporal dementia, Psychology, Therapeutics
Abstract: Early reports of cognitive and behavioural deficits in motor neuron disease might have been overlooked initially, but the concept of a frontotemporal dementia-motor neuron disease continuum has emerged during the past decade. Frontotemporal dementia-motor neuron disease is now recognised as an important dementia syndrome, which presents substantial challenges for diagnosis and management. Frontotemporal dementia, motor neuron disease, and frontotemporal dementia-motor neuron disease are characterised by overlapping patterns of TAR DNA binding protein (TDP-43) pathology, while the chromosome 9 open reading frame 72 (C9orf72) repeat expansion is common across the disease spectrum. Indeed, the C9orf72 repeat expansion provides important clues to disease pathogenesis and suggests potential therapeutic targets. Variable diagnostic criteria identify motor, cognitive, and behavioural deficits, but further refinement is needed to define the clinical syndromes encountered in frontotemporal dementia-motor neuron disease. [ABSTRACT FROM AUTHOR]
Copyright of Lancet is the property of Lancet and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
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  Data: The frontotemporal dementia-motor neuron disease continuum.
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  Data: <searchLink fieldCode="AR" term="%22Burrell%2C+James+R%2E%22">Burrell, James R.</searchLink><br /><searchLink fieldCode="AR" term="%22Halliday%2C+Glenda+M%2E%22">Halliday, Glenda M.</searchLink><br /><searchLink fieldCode="AR" term="%22Kril%2C+Jillian+J%2E%22">Kril, Jillian J.</searchLink><br /><searchLink fieldCode="AR" term="%22Ittner%2C+Lars+M%2E%22">Ittner, Lars M.</searchLink><br /><searchLink fieldCode="AR" term="%22Götz%2C+Jürgen%22">Götz, Jürgen</searchLink><br /><searchLink fieldCode="AR" term="%22Kiernan%2C+Matthew+C%2E%22">Kiernan, Matthew C.</searchLink><br /><searchLink fieldCode="AR" term="%22Hodges%2C+John+R%2E%22">Hodges, John R.</searchLink>
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  Data: <searchLink fieldCode="JN" term="%22Lancet%22">Lancet</searchLink>. 8/27/2016, Vol. 388 Issue 10047, p919-931. 13p.
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  Data: <searchLink fieldCode="DE" term="%22Dementia%22">Dementia</searchLink><br /><searchLink fieldCode="DE" term="%22Motor+neuron+diseases%22">Motor neuron diseases</searchLink><br /><searchLink fieldCode="DE" term="%22Carrier+proteins%22">Carrier proteins</searchLink><br /><searchLink fieldCode="DE" term="%22Cognition+disorders%22">Cognition disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Neurobehavioral+disorders%22">Neurobehavioral disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Protein+metabolism%22">Protein metabolism</searchLink><br /><searchLink fieldCode="DE" term="%22Brain+metabolism%22">Brain metabolism</searchLink><br /><searchLink fieldCode="DE" term="%22Neuroprotective+agents%22">Neuroprotective agents</searchLink><br /><searchLink fieldCode="DE" term="%22Riluzole%22">Riluzole</searchLink><br /><searchLink fieldCode="DE" term="%22Brain%22">Brain</searchLink><br /><searchLink fieldCode="DE" term="%22Cognition%22">Cognition</searchLink><br /><searchLink fieldCode="DE" term="%22DNA%22">DNA</searchLink><br /><searchLink fieldCode="DE" term="%22Health+care+teams%22">Health care teams</searchLink><br /><searchLink fieldCode="DE" term="%22Digital+image+processing%22">Digital image processing</searchLink><br /><searchLink fieldCode="DE" term="%22Neuropsychological+tests%22">Neuropsychological tests</searchLink><br /><searchLink fieldCode="DE" term="%22Genetic+mutation%22">Genetic mutation</searchLink><br /><searchLink fieldCode="DE" term="%22Neuroradiology%22">Neuroradiology</searchLink><br /><searchLink fieldCode="DE" term="%22Prognosis%22">Prognosis</searchLink><br /><searchLink fieldCode="DE" term="%22Proteins%22">Proteins</searchLink><br /><searchLink fieldCode="DE" term="%22DNA-binding+proteins%22">DNA-binding proteins</searchLink><br /><searchLink fieldCode="DE" term="%22Activities+of+daily+living%22">Activities of daily living</searchLink><br /><searchLink fieldCode="DE" term="%22Executive+function%22">Executive function</searchLink><br /><searchLink fieldCode="DE" term="%22DNA+methylation%22">DNA methylation</searchLink><br /><searchLink fieldCode="DE" term="%22Frontotemporal+dementia%22">Frontotemporal dementia</searchLink><br /><searchLink fieldCode="DE" term="%22Psychology%22">Psychology</searchLink><br /><searchLink fieldCode="DE" term="%22Therapeutics%22">Therapeutics</searchLink>
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  Data: Early reports of cognitive and behavioural deficits in motor neuron disease might have been overlooked initially, but the concept of a frontotemporal dementia-motor neuron disease continuum has emerged during the past decade. Frontotemporal dementia-motor neuron disease is now recognised as an important dementia syndrome, which presents substantial challenges for diagnosis and management. Frontotemporal dementia, motor neuron disease, and frontotemporal dementia-motor neuron disease are characterised by overlapping patterns of TAR DNA binding protein (TDP-43) pathology, while the chromosome 9 open reading frame 72 (C9orf72) repeat expansion is common across the disease spectrum. Indeed, the C9orf72 repeat expansion provides important clues to disease pathogenesis and suggests potential therapeutic targets. Variable diagnostic criteria identify motor, cognitive, and behavioural deficits, but further refinement is needed to define the clinical syndromes encountered in frontotemporal dementia-motor neuron disease. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Lancet is the property of Lancet and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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        Value: 10.1016/S0140-6736(16)00737-6
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        Text: English
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      – SubjectFull: Motor neuron diseases
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      – SubjectFull: Carrier proteins
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              Text: 8/27/2016
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