Distinct antemortem profiles in patients with pathologically defined frontotemporal dementia.
BACKGROUND
Clinical-pathologic studies are crucial to understanding brain-behavior relations and improving diagnostic accuracy in neurodegenerative diseases.


OBJECTIVE
To establish clinical, neuropsychological, and imaging features of clinically diagnosed patients with frontotemporal dementia (FTD) that help discriminate between pathologically determined tau-positive FTD, tau-negative FTD, and frontal-variant Alzheimer disease.


METHODS
Retrospective clinical-pathologic survey.


METHODS
Academic medical center. Patients Sixty-one participants with the clinical diagnosis of a frontotemporal spectrum disorder who underwent a neuropsychological evaluation and had an autopsy-confirmed disease.


METHODS
Neuropsychological performance and high-resolution structural magnetic resonance imaging (MRI).


RESULTS
Distinguishing features of patients with tau-positive FTD include visual perceptual-spatial difficulty and an extrapyramidal disorder significantly more often than other patients, significant cortical atrophy in the frontal and parietal regions as evidenced on MRI, and the burden of pathology is greatest in the frontal and parietal regions. Patients with tau-negative FTD are distinguished by their greater difficulties with social, language, and verbally mediated executive functions, significant cortical atrophy in the frontal and temporal regions as evidenced on MRI, and significant frontal and temporal pathology. Patients with Alzheimer disease at autopsy have significantly impaired delayed recall during episodic memory testing; atrophy that involves temporal areas, including the hippocampus, as evidenced on MRI; and widely distributed pathology including the medial temporal structures. A discriminant function analysis grouped patients on the basis of clinical and neuropsychological features with 87.5% accuracy.


CONCLUSIONS
Clinical, neuropsychological, and imaging profiles can contribute to accurate antemortem diagnosis in FTD.
Grossman
Murray
M
Department of Neurology, 2 Gibson, University of Pennsylvania School of Medicine, 3400 Spruce St, Philadelphia, PA 19104-4283, USA. mgrossma@mail.med.upenn.edu
Libon
David J
DJ
Forman
Mark S
MS
Massimo
Lauren
L
Wood
Elisabeth
E
Moore
Peachie
P
Anderson
Chivon
C
Farmer
Jennifer
J
Chatterjee
Anjan
A
Clark
Christopher M
CM
Coslett
H Branch
HB
Hurtig
Howard I
HI
Lee
Virginia M-Y
VM
Trojanowski
John Q
JQ
D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D001344:Autopsy / Q000379:methods; D001931:Brain Mapping; D003704:Dementia / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006306:Health Surveys; D006801:Humans; D007297:Inpatients; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D012189:Retrospective Studies; D016875:tau Proteins / Q000378:metabolism*
D016875:tau Proteins