Survival profiles of patients with frontotemporal dementia and motor neuron disease

William T. Hu, Harro Seelaar, Keith Anthony Josephs, David S Knopman, Bradley F Boeve, Eric James Sorenson, Leo McCluskey, Lauren Elman, Helenius J. Schelhaas, Joseph E Parisi, Benno Kuesters, Virginia M Y Lee, John Q. Trojanowski, Ronald Carl Petersen, John C. Van Swieten, Murray Grossman

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)1359-1364
Number of pages6
JournalArchives of Neurology
Volume66
Issue number11
DOIs
StatePublished - Nov 2009

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Survival
Neurobehavioral Manifestations
Survivors
Frontotemporal Dementia With Motor Neuron Disease
Neuron
Frontotemporal Dementia
Motor Neuron Disease
DNA-Binding Proteins
Ubiquitin
Neurodegenerative Diseases
Onset
Outcome Assessment (Health Care)
Phenotype
Amyotrophic Lateral Sclerosis

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Survival profiles of patients with frontotemporal dementia and motor neuron disease. / Hu, William T.; Seelaar, Harro; Josephs, Keith Anthony; Knopman, David S; Boeve, Bradley F; Sorenson, Eric James; McCluskey, Leo; Elman, Lauren; Schelhaas, Helenius J.; Parisi, Joseph E; Kuesters, Benno; Lee, Virginia M Y; Trojanowski, John Q.; Petersen, Ronald Carl; Van Swieten, John C.; Grossman, Murray.

In: Archives of Neurology, Vol. 66, No. 11, 11.2009, p. 1359-1364.

Research output: Contribution to journalArticle

Hu, WT, Seelaar, H, Josephs, KA, Knopman, DS, Boeve, BF, Sorenson, EJ, McCluskey, L, Elman, L, Schelhaas, HJ, Parisi, JE, Kuesters, B, Lee, VMY, Trojanowski, JQ, Petersen, RC, Van Swieten, JC & Grossman, M 2009, 'Survival profiles of patients with frontotemporal dementia and motor neuron disease', Archives of Neurology, vol. 66, no. 11, pp. 1359-1364. https://doi.org/10.1001/archneurol.2009.253
Hu, William T. ; Seelaar, Harro ; Josephs, Keith Anthony ; Knopman, David S ; Boeve, Bradley F ; Sorenson, Eric James ; McCluskey, Leo ; Elman, Lauren ; Schelhaas, Helenius J. ; Parisi, Joseph E ; Kuesters, Benno ; Lee, Virginia M Y ; Trojanowski, John Q. ; Petersen, Ronald Carl ; Van Swieten, John C. ; Grossman, Murray. / Survival profiles of patients with frontotemporal dementia and motor neuron disease. In: Archives of Neurology. 2009 ; Vol. 66, No. 11. pp. 1359-1364.
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AU - Josephs, Keith Anthony

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AU - Boeve, Bradley F

AU - Sorenson, Eric James

AU - McCluskey, Leo

AU - Elman, Lauren

AU - Schelhaas, Helenius J.

AU - Parisi, Joseph E

AU - Kuesters, Benno

AU - Lee, Virginia M Y

AU - Trojanowski, John Q.

AU - Petersen, Ronald Carl

AU - Van Swieten, John C.

AU - Grossman, Murray

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