Survival profiles of patients with frontotemporal dementia and motor neuron disease.
BACKGROUND
Frontotemporal dementia and amyotrophic lateral sclerosis are neurodegenerative diseases associated with TAR DNA-binding protein 43- and ubiquitin-immunoreactive pathologic lesions.


OBJECTIVE
To determine whether survival is influenced by symptom of onset in patients with frontotemporal dementia and amyotrophic lateral sclerosis.


METHODS
Retrospective review of patients with both cognitive impairment and motor neuron disease consecutively evaluated at 4 academic medical centers in 2 countries.


METHODS
Clinical phenotypes and survival patterns of patients.


RESULTS
A total of 87 patients were identified, including 60 who developed cognitive symptoms first, 19 who developed motor symptoms first, and 8 who had simultaneous onset of cognitive and motor symptoms. Among the 59 deceased patients, we identified 2 distinct subgroups of patients according to survival. Long-term survivors had cognitive onset and delayed emergence of motor symptoms after a long monosymptomatic phase and had significantly longer survival than the typical survivors (mean, 67.5 months vs 28.2 months, respectively; P < .001). Typical survivors can have simultaneous or discrete onset of cognitive and motor symptoms, and the simultaneous-onset patients had shorter survival (mean, 19.2 months) than those with distinct cognitive or motor onset (mean, 28.6 months) (P = .005).


CONCLUSIONS
Distinct patterns of survival profiles exist in patients with frontotemporal dementia and motor neuron disease, and overall survival may depend on the relative timing of the emergence of secondary symptoms.
Hu
William T
WT
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA. huw@uphs.upenn.edu
Seelaar
Harro
H
Josephs
Keith A
KA
Knopman
David S
DS
Boeve
Bradley F
BF
Sorenson
Eric J
EJ
McCluskey
Leo
L
Elman
Lauren
L
Schelhaas
Helenius J
HJ
Parisi
Joseph E
JE
Kuesters
Benno
B
Lee
Virginia M-Y
VM
Trojanowski
John Q
JQ
Petersen
Ronald C
RC
van Swieten
John C
JC
Grossman
Murray
M
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000473:pathology; D005260:Female; D057174:Frontotemporal Lobar Degeneration / Q000150:complications* / Q000401:mortality* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000401:mortality* / Q000503:physiopathology; D012189:Retrospective Studies