Survival profiles of patients with frontotemporal dementia and motor neuron disease.

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.

• Authors: William T Hu, 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
• Journal: Archives of neurology (Arch Neurol) Vol. 66 Issue 11 Pg. 1359-64 (Nov 2009) ISSN: 1538-3687 [Electronic] United States
• PMID: 19901167 (Publication Type: Journal Article, Multicenter Study, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Topics

• Age of Onset
• Aged
• Aged, 80 and over
• Brain (pathology)
• Female
• Frontotemporal Lobar Degeneration (complications, mortality, physiopathology)
• Humans
• Male
• Middle Aged
• Motor Neuron Disease (complications, mortality, physiopathology)
• Retrospective Studies