Frontotemporal lobar degeneration
... (FTLD) is a pathological process that occurs in frontotemporal dementia. It is characterized ... The three clinical subtypes of frontotemporal lobar degeneration, frontotemporal dementia, semantic dementia and progressive ... Consortium for Frontotemporal Lobar Degeneration (2007). "Neuropathologic diagnostic and nosologic criteria for frontotemporal ... lobar degeneration: Consensus of the Consortium for Frontotemporal Lobar Degeneration". Acta Neuropathologica. 114 (1): 5-22. ...
Granulin
Ahmed Z, Mackenzie IR, Hutton ML, Dickson DW (February 2007). "Progranulin in frontotemporal lobar degeneration and ... Pickering-Brown SM (July 2007). "Progranulin and frontotemporal lobar degeneration". Acta Neuropathologica. 114 (1): 39-47. doi ... May 2017). "Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations". ... The 2006 discovery of the GRN mutation in a population of patients with frontotemporal dementia has spurred much research in ...
Grandiose delusions
"Delusions in frontotemporal lobar degeneration". Journal of Neurology. 256 (4): 600-607. doi:10.1007/s00415-009-0128-7. ISSN ... while frontal and frontotemporal involvement have been described in patients with grandiose delusions, Cotard's syndrome, and ... while frontal and frontotemporal involvement have described [sic] in patients with grandiose delusions, Cotard's syndrome, and ...
List of OMIM disorder codes
ALX3 Frontotemporal lobar degeneration with ubiquitin-positive inclusions; 607485; GRN Frontotemporal lobar degeneration, ... CHST6 Macular degeneration, age-related, 11; 611953; CST3 Macular degeneration, age-related, 2; 153800; ABCA4 Macular ... frontotemporal; 600274; PSEN1 Dementia, frontotemporal, with or without parkinsonism; 600274; MAPT Dementia, Lewy body; 127750 ... EPB72 Striatal degeneration, autosomal dominant; 609161; PDE8B Striatonigral degeneration, infantile; 271930; NUP62 Stuve- ...
Ian Black (journalist)
Black had frontotemporal lobar degeneration (FTLD). He and his wife, Helen Harris, who lived in north London, had two daughters ... Deaths from frontotemporal dementia, All stub articles, British journalist stubs). ...
Dementia
... frontotemporal lobar degeneration for frontotemporal dementia; or Lewy body disease for dementia with Lewy bodies, and prion ... and the same underlying pathology may cause the clinical syndromes of frontotemporal lobar degeneration). Although the acute ... A frontotemporal dementia associated with amyotrophic lateral sclerosis (ALS) known as (FTD-ALS) includes the symptoms of FTD ( ... Frontotemporal dementias (FTDs) are characterized by drastic personality changes and language difficulties. In all FTDs, the ...
Frontotemporal dementia
... s are mostly early-onset syndromes that are linked to frontotemporal lobar degeneration (FTLD), which is ... Frontotemporal dementia (FTD), or frontotemporal degeneration disease, or frontotemporal neurocognitive disorder, encompasses ... Schroeter ML, Raczka KK, Neumann J, von Cramon DY (2007). "Towards a nosology for frontotemporal lobar degenerations - A meta- ... Sleegers K, Cruts M, Van Broeckhoven C (2010). "Molecular Pathways of Frontotemporal Lobar Degeneration". Annual Review of ...
C9orf72
September 2012). "The genetics and neuropathology of frontotemporal lobar degeneration". Acta Neuropathologica. 124 (3): 353-72 ... "Behavioral variant frontotemporal lobar degeneration with amyotrophic lateral sclerosis with a chromosome 9p21 hexanucleotide ... Frontotemporal dementia is the second most common form of early-onset dementia after Alzheimer's disease in people under the ... March 2012). "Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and ...
Self-conscious emotions
Frontotemporal lobar degeneration (FTLD) is a neurodegenerative disease that attacks the brain selectively in the frontal lobe ... Sturm, V., E. & Rosen, H., J. (2006). Self-conscious emotion deficits in frontotemporal lobar degeneration. Retrieve from the ...
Hippocampal sclerosis
Hippocampal sclerosis occurs in about 66% of those with frontotemporal lobar degeneration arising from TDP-43 or FUS ... 2009). "A new subtype of frontotemporal lobar degeneration with FUS pathology". Brain. 132 (11): 2922-2931. doi:10.1093/brain/ ... Josephs, Keith A.; Dickson, Dennis W. (2007). "Hippocampal sclerosis in tau-negative frontotemporal lobar degeneration". ... 2006). "Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis". Science. 314 (5796): 130- ...
TMEM106B
van der Zee, Julie; Van Broeckhoven, Christine (2011). "TMEM106B a novel risk factor for frontotemporal lobar degeneration". ... March 2010). "Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions". Nature ... Frontotemporal dementia (FTLD) is the third most common neurodegenerative disease after AD and Parkinson disease. Many patients ... Lessons from frontotemporal dementia and amyotrophic lateral sclerosis". Neurobiology of Disease. 154: 105360. doi:10.1016/j. ...
Stereotypy
Among people with frontotemporal lobar degeneration, more than half (60%) had stereotypies. The time to onset of stereotypies ... "The clinical spectrum of stereotypies in frontotemporal lobar degeneration". Movement Disorders. 24 (8): 1237-40. doi:10.1002/ ... in people with frontotemporal lobar degeneration may be years (average 2.1 years). Like tics, stereotypies are patterned and ... Frontotemporal dementia is also a common neurological cause of repetitive behaviors and stereotypies. Several causes have been ...
Proteinopathy
Causes of Parkinson's disease
April 2011). "Genetic and clinical features of progranulin-associated frontotemporal lobar degeneration". Archives of Neurology ... Of related interest are mutations in the progranulin gene that have been found to cause corticobasal degeneration seen in ... "Molecular targets for modulating the protein translation vital to proteostasis and neuron degeneration in Parkinson's disease ...
RNA-binding protein FUS
Frontotemporal lobar degeneration FTLD) is the pathological term for the clinical syndrome of frontotemporal dementia (FTD). ... "A new subtype of frontotemporal lobar degeneration with FUS pathology". Brain. 132 (Pt 11): 2922-31. doi:10.1093/brain/awp214. ... The disease entities which are now considered subtypes of FTLD-FUS are atypical frontotemporal lobar degeneration with ... Behavioral variant frontotemporal dementia (bvFTD), progressive non-fluent aphasia (PNFA) and semantic dementia (SD) are the ...
Applause sign
"The applause sign in frontotemporal lobar degeneration and related conditions". Journal of Neurology. 266 (2): 330-338. doi: ... Progressive supranuclear palsy Parkinson's Disease Cortico-Basal Degeneration and Syndrome Alzheimer's Disease Frontotemporal ... Frontotemporal dementia and Alzheimer's disease. The applause sign can indicate frontal lobe dysfunction because deliberate ... with occasional appearances in individuals with Corticobasal degeneration, Parkinson's disease, Amyotrophic Lateral Sclerosis, ...
Limbic-predominant age-related TDP-43 encephalopathy
"Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis". Science. 314 (5796): 130-133. ...
Virginia Man-Yee Lee
... frontotemporal lobar degeneration, and amyotrophic lateral sclerosis. Together with her late husband John Q. Trojanowski, Lee's ... Lee and Trojanowski also studied frontotemporal lobar degeneration (FTLD), amyotrophic lateral sclerosis (ALS), and multiple ... "Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis". Science. 314 (5796): 130-133. ...
Neuroanatomy of memory
CHMP2B
2006). "CHMP2B mutations are not a common cause of frontotemporal lobar degeneration". Neurosci. Lett. 398 (1-2): 83-4. doi: ... "CHMP2B mutations are rare in French families with frontotemporal lobar degeneration". J Neurol. 257 (12): 2032-6. doi:10.1007/ ... Mutations of this gene cause chromosome 3-linked frontotemporal dementia (FTD3), which has been described in several members of ... GeneReviews/NCBI/NIH/UW entry on CHMP2B-Related Frontotemporal Dementia Human CHMP2B genome location and CHMP2B gene details ...
Multiple system atrophy
"Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration: frontotemporal lobar degeneration ... There have also been occasional instances of frontotemporal lobar degeneration associated with MSA. The most common first sign ... This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior ... These include striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and Shy-Drager syndrome. A table ...
Hyperreligiosity
... frontotemporal lobar degeneration, Anti-NMDA receptor encephalitis, hallucinogen related psychosis and psychotic disorder. In ... "Geschwind Syndrome in frontotemporal lobar degeneration: Neuroanatomical and neuropsychological features over 9 years". Cortex ... psychotic disorders and frontotemporal lobar degeneration. Hyperreligiosity is a symptom of Geschwind syndrome, which is ...
Auditosensory cortex
It is secondary to neurodegenerative diseases, for instance, Alzheimer's disease and frontotemporal lobar degeneration. The ...
Corticobasal degeneration
Frontotemporal lobar degeneration "Corticobasalganglionic Degeneration Information Page: National Institute of Neurological ... CBS patients with greater temporoparietal degeneration are more likely to have AD pathology as opposed to frontotemporal lobar ... "corticonigral degeneration with nuclear achromasia" and "cortical basal ganglionic degeneration". Although the underlying cause ... Although corticobasal degeneration has a plethora of symptoms, some are more prevalent than others. In a study of 147 patients ...
CCL8
2009). "CCL8/MCP-2 association analysis in patients with Alzheimer's disease and frontotemporal lobar degeneration". J. Neurol ...
Progressive nonfluent aphasia
Schroeter ML, Raczka K, Neumann J, Yves von Cramon D (July 2007). "Towards a nosology for frontotemporal lobar degenerations-a ... Progressive nonfluent aphasia (PNFA) is one of three clinical syndromes associated with frontotemporal lobar degeneration. PNFA ... progressive nonfluent aphasia can be regionally dissociated from the other subtypes of frontotemporal lobar degeneration, ... The specific degeneration of the frontal and temporal lobes in PNFA creates hallmark language deficits differentiating this ...
Bernhard Landwehrmeyer
... frontotemporal lobar degeneration (FTD), and amyotrophic lateral sclerosis (ALS). Other topics on which he published include ... "Quantifying change in individual subjects affected by frontotemporal lobar degeneration using automated longitudinal MRI ... "Specific serum and CSF microRNA profiles distinguish sporadic behavioural variant of frontotemporal dementia compared with ...
Temporal lobe
"Clinicopathological characterization of Pick's disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive ... Pick's disease, also known as frontotemporal amnesia, is caused by atrophy of the frontotemporal lobe. Emotional symptoms ... Language symptoms include loss of speech, inability to read or write, loss of vocabulary and overall degeneration of motor ...
Valosin-containing protein
... and frontotemporal lobar degeneration phenotypes". Neurobiology of Aging. 35 (4): 934.e5-6. doi:10.1016/j.neurobiolaging. ... a unique subtype of frontotemporal lobar degeneration with tau inclusions. Mutations in VCP are an example of pleiotropy, where ... GeneReviews/NIH/NCBI/UW entry on Inclusion Body Myopathy with Paget Disease of Bone and/or Frontotemporal Dementia Overview of ... Mutations in VCP were first reported to cause a syndrome characterized by frontotemporal dementia, inclusion body myopathy, and ...
Semantic dementia
... semantic dementia can be regionally dissociated from the other subtypes of frontotemporal lobar degeneration, frontotemporal ... Schroeter, M.L.; Raczka, K.K.; Neumann, J.; von Cramon, D.Y. (2007). "Towards a nosology for frontotemporal lobar degenerations ... SD is one of the three canonical clinical syndromes associated with frontotemporal lobar degeneration (FTLD), with the other ... "Alzheimer Europe - Dementia - Other forms of dementia - Neurodegenerative diseases - Fronto-Temporal Degeneration - Semantic ...