TY - CHAP
T1 - Frontotemporal Dementia
AU - Wider, Christian W.
AU - Dickson, Dennis W.
AU - Rademakers, Rosa
AU - Wszolek, Zbigniew K.
N1 - Publisher Copyright:
© 2010 Elsevier Inc.
PY - 2010/1
Y1 - 2010/1
N2 - This chapter discusses the most relevant and recent advances in clinical diagnosis, imaging, pathology, genetics, and treatment strategies in frontotemporal lobar degeneration (FTLD). Special emphasis is on the genetically determined forms of FTLD. FTLD refers to a group of neurodegenerative diseases comprising the clinical and pathological entities frontotemporal dementia (FTD), progressive nonfluent aphasia (PNFA), and semantic dementia (SD). Although the diagnostic criteria for FLTD are mainly clinical, a relationship between the symptoms and the predominantly affected areas exists with FTD mainly affecting the frontal lobes, PNFA affecting the left frontal lobe, and SD affecting the left temporal lobe. The frontotemporal dementia associated with motor neuron disease (FTD-MND) is also discussed. Early symptoms in FTD patients are dominated by impairment in social behavior and character changes. Additional symptoms include a wide range of frontal-type behaviors, mostly of the disinhibited form, along with speech alterations without true aphasia. Dietary changes, including binge eating, are common among FTD patients. The familial forms of FTLD discussed are FTDP-17 tau-positive families and FTDP-17 tau-negative, ubiquitin-positive families.
AB - This chapter discusses the most relevant and recent advances in clinical diagnosis, imaging, pathology, genetics, and treatment strategies in frontotemporal lobar degeneration (FTLD). Special emphasis is on the genetically determined forms of FTLD. FTLD refers to a group of neurodegenerative diseases comprising the clinical and pathological entities frontotemporal dementia (FTD), progressive nonfluent aphasia (PNFA), and semantic dementia (SD). Although the diagnostic criteria for FLTD are mainly clinical, a relationship between the symptoms and the predominantly affected areas exists with FTD mainly affecting the frontal lobes, PNFA affecting the left frontal lobe, and SD affecting the left temporal lobe. The frontotemporal dementia associated with motor neuron disease (FTD-MND) is also discussed. Early symptoms in FTD patients are dominated by impairment in social behavior and character changes. Additional symptoms include a wide range of frontal-type behaviors, mostly of the disinhibited form, along with speech alterations without true aphasia. Dietary changes, including binge eating, are common among FTD patients. The familial forms of FTLD discussed are FTDP-17 tau-positive families and FTDP-17 tau-negative, ubiquitin-positive families.
UR - http://www.scopus.com/inward/record.url?scp=85097041255&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85097041255&partnerID=8YFLogxK
U2 - 10.1016/B978-1-4160-6641-5.00023-4
DO - 10.1016/B978-1-4160-6641-5.00023-4
M3 - Chapter
AN - SCOPUS:85097041255
SN - 9781416066415
T3 - Blue Books of Neurology
SP - 397
EP - 416
BT - MOVEMENT DISORDERS 4
A2 - Schapira, Anthony H.V.
A2 - Lang, Anthony E.T.
A2 - Lang, Anthony E.T.
A2 - Lang, Anthony E.T.
A2 - Fahn, Stanley
PB - Elsevier Inc.
ER -