Survival profiles of patients with frontotemporal dementia and motor neuron disease
| Autor: | Hu, W.T., Seelaar, H., Josephs, K.A., Knopman, D.S., Boeve, B.F., Sorenson, E.J., McCluskey, L., Elman, L., Schelhaas, H.J., Parisi, J.E., Kuesters, B., Lee, V.M., Trojanowski, J.Q., Petersen, R.C., Swieten, J.C. van, Grossman, M. |
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| Rok vydání: | 2009 |
| Předmět: | |
| Zdroj: | Archives of Neurology, 66, 1359-64 Archives of Neurology, 66, 11, pp. 1359-64 |
| ISSN: | 0003-9942 |
| Popis: | Contains fulltext : 81426.pdf (Publisher’s version ) (Closed access) 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. |
| Databáze: | OpenAIRE |
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