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Multicenter Study
. 2009 Nov;66(11):1359-64.
doi: 10.1001/archneurol.2009.253.

Survival profiles of patients with frontotemporal dementia and motor neuron disease

William T Hu  1 , Harro Seelaar, Keith A Josephs, David S Knopman, Bradley F Boeve, Eric J Sorenson, Leo McCluskey, Lauren Elman, Helenius J Schelhaas, Joseph E Parisi, Benno Kuesters, Virginia M-Y Lee, John Q Trojanowski, Ronald C Petersen, John C van Swieten, Murray Grossman
Affiliations
Multicenter Study

Survival profiles of patients with frontotemporal dementia and motor neuron disease

William T Hu et al. Arch Neurol. 2009 Nov.

Abstract

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.

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

Main outcome measures: 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.

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Figures

Figure 1
Figure 1
Relationship between normalized time of cognitive symptom onset and overall disease survival among deceased patients. Normalized time of cognitive symptom onset was derived by subtracting the date of cognitive symptom onset from the date of motor symptom onset. A negative value indicates cognitive onset; a positive value, motor onset.
Figure 2
Figure 2
Kaplan-Meier survival analysis of patients with frontotemporal dementia and motor neuron disease according to survival grouping.
See this image and copyright information in PMC

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