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J Neurol. 2016 Dec;263(12):2476-2483. Epub 2016 Sep 26.

The frontotemporal syndrome of ALS is associated with poor survival.

Author information

1
Department of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. r.a.govaarts@amc.uva.nl.
2
Department of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
3
Center for Home Mechanical Ventilation, University Medical Center Utrecht, Utrecht, The Netherlands.
4
Neuroimaging Center, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
5
Department of Neurology, Brain Center Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, Utrecht, The Netherlands.
6
Department of Home Mechanical Ventilation/Pulmonary Diseases, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
7
Department of Intensive Care/Center for Home Mechanical Ventilation, University Medical Center, Erasmus MC, Rotterdam, The Netherlands.
8
Department of Respiratory Medicine/Center of Home Mechanical Ventilation Maastricht, Maastricht University Medical Center, Maastricht, The Netherlands.
9
Department of Medical Psychology, University of Amsterdam, Amsterdam, The Netherlands.
10
Clinical Research Unit, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
11
Department of Neurology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.

Abstract

Thirty percent of ALS patients have a frontotemporal syndrome (FS), defined as behavioral changes or cognitive impairment. Despite previous studies, there are no firm conclusions on the effect of the FS on survival and the use of non-invasive ventilation (NIV) in ALS. We examined the effect of the FS on survival and the start and duration of NIV in ALS. Behavioral changes were defined as >22 points on the ALS-Frontotemporal-Dementia-Questionnaire or ≥3 points on ≥2 items of the Neuropsychiatric Inventory. Cognitive impairment was defined as below the fifth percentile on ≥2 tests of executive function, memory or language. Classic ALS was defined as ALS without the frontotemporal syndrome. We performed survival analyses from symptom onset and time from NIV initiation, respectively, to death. The impact of the explanatory variables on survival and NIV initiation were examined using Cox proportional hazards models. We included 110 ALS patients (76 men) with a mean age of 62 years. Median survival time was 4.3 years (95 % CI 3.53-5.13). Forty-seven patients (43 %) had an FS. Factors associated with shorter survival were FS, bulbar onset, older age at onset, short time to diagnosis and a C9orf72 repeat expansion. The adjusted hazard ratio (HR) for the FS was 2.29 (95 % CI 1.44-3.65, p < 0.001) in a multivariate model. Patients with an FS had a shorter survival after NIV initiation (adjusted HR 2.70, 95 % CI 1.04-4.67, p = 0.04). In conclusion, there is an association between the frontotemporal syndrome and poor survival in ALS, which remains present after initiation of NIV.

KEYWORDS:

Amyotrophic lateral sclerosis; Behavioral changes; Cognitive impairment; Frontotemporal syndrome; Non-invasive ventilation; Survival

PMID:
27671483
PMCID:
PMC5110703
DOI:
10.1007/s00415-016-8290-1
[Indexed for MEDLINE]
Free PMC Article

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