• 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, and Murray Grossman.
• Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA. huw@uphs.upenn.edu
• Arch. Neurol. 2009 Nov 1; 66 (11): 1359-64.

BackgroundFrontotemporal dementia and amyotrophic lateral sclerosis are neurodegenerative diseases associated with TAR DNA-binding protein 43- and ubiquitin-immunoreactive pathologic lesions.ObjectiveTo determine whether survival is influenced by symptom of onset in patients with frontotemporal dementia and amyotrophic lateral sclerosis.Design, Setting, And PatientsRetrospective review of patients with both cognitive impairment and motor neuron disease consecutively evaluated at 4 academic medical centers in 2 countries.Main Outcome MeasuresClinical phenotypes and survival patterns of patients.ResultsA 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).ConclusionsDistinct 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.

18 keywords...

hide…