The effects of executive and behavioral dysfunction on the course of ALS
Abstract
Objective: To determine whether patients with ALS–frontotemporal lobar dementia (FTLD) have a shorter survival and are less compliant with recommended treatments than those with ALS who have normal executive and behavioral function (classic ALS).
Methods: Survival analysis from ALS symptom onset to death included 81 of 100 consecutive patients who could be classified definitely as ALS with abnormal executive or behavioral function or as classic ALS. Criteria were defined for compliance with noninvasive positive-pressure ventilation (NPPV) and percutaneous endoscopic gastrostomy (PEG).
Results: Median survival was 2 years 4 months for the 28 patients with FTLD and 3 years 3 months for the 53 patients with classic ALS (relative hazard for death 1.93, CI 1.09 to 3.43; p = 0.024). However, the relative hazard associated with FTLD (1.49) in the multivariate model was diminished by the association of FTLD with bulbar onset and older age and was not significant in this sample size. With bulbar onset, median survival was 2 years 0 months for the 14 with ALS-FTLD and 2 years 10 months for the 10 with classic ALS (relative hazard for death 2.78, CI 1.02 to 7.55; p = 0.045), and older age was not a significant risk. Noncompliance with NPPV and PEG were 75% and 72% in ALS-FTLD, respectively, vs 38% and 31% in classic ALS (relative risks 2.00 and 2.34; p = 0.013 and 0.022).
Conclusions: Survival is significantly shorter among patients with ALS-FTLD than with classic ALS. Furthermore, patients with ALS-FTLD are twice as likely to be noncompliant.
Get full access to this article
View all available purchase options and get full access to this article.
References
1.
Majoor-Krakauer D, Willems PJ, Hofman A. Genetic epidemiology of amyotrophic lateral sclerosis. Clin Genet 2003;63:83–101.
2.
Hudson AJ. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: A review. Brain 1981;104:217–247.
3.
Bak TH, Hodges JR. Cognition, language and behaviour in motor neurone disease: Evidence of frontotemporal dysfunction. Dement Geriatr Cogn Disord 1999;10(suppl 1):29–32.
4.
Strong MJ, Grace GM, Orange JB, Leeper HA, Menon RS, Aere C. A prospective study of cognitive impairment in ALS. Neurology 1999;53:1665–1670.
5.
Lomen-Hoerth C, Murphy J, Langmore S, Kramer JH, Olney RK, Miller B. Are amyotrophic lateral sclerosis patients cognitively normal? Neurology 2003;60:1094–1097.
6.
Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000;1:293–299.
7.
Folstein MF, Folstein SE, McHugh PR. Mini-Mental State: A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189–198.
8.
Cummings JL. The Neuropsychiatric Inventory: Assessing psychopathology in dementia patients. Neurology 1997;48(Suppl 6):S10–S16.
9.
Heaton RK, Chelune FJ, Talley JL, Kay GG, Curtis G. Wisconsin Card Sorting Test (WCST) Manual: Revised and Expanded. Odessa: Psychological Assessment Resources; 1993.
10.
Delis DC, Kaplan E, Kramer JH. Delis–Kaplan Executive Function System. San Antonio: Psychological Corp.; 2001.
11.
Delis DC, Kramer JH, Kaplan E, Ober BA. California Verbal Learning Test. San Antonio: Psychological Corp.; 2000.
12.
Wechsler D. Wechsler Memory Scale. 3rd ed. San Antonio: Psychological Corp.; 1997.
13.
Kaplan K. The Boston Naming Test. Philadelphia: Lea & Febiger; 1982.
14.
Wechsler D. Wechsler Adult Intelligence Scale. 3rd ed. San Antonio: Psychological Corp.; 1997.
15.
Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria. Neurology 1998;51:1546–1554.
16.
Mazzini L, Corra T, Zaccala M, Mora G, Del Piano M, Galante M. Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis. J Neurol 1995;242:695–698.
17.
Aboussouan LS, Khan SU, Meeker DP, Stelmach K, Mitsumoto H. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med 1997;127:450–453.
18.
Kleopa KA, Sherman M, Neal B, Romano GJ, Heiman-Patterson T. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci 1999;164:82–88.
Information & Authors
Information
Published In
Copyright
© 2005.
Publication History
Published online: December 12, 2005
Published in print: December 13, 2005
Authors
Metrics & Citations
Metrics
Citation information is sourced from Crossref Cited-by service.
Citations
Download Citations
If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.
Cited By
- Clinical, Cortical, Subcortical, and White Matter Features of Right Temporal Variant FTD, Brain Sciences, 14, 8, (806), (2024).https://doi.org/10.3390/brainsci14080806
- Limbic Network and Papez Circuit Involvement in ALS: Imaging and Clinical Profiles in GGGGCC Hexanucleotide Carriers in C9orf72 and C9orf72-Negative Patients, Biology, 13, 7, (504), (2024).https://doi.org/10.3390/biology13070504
- Factors associated with adherence to noninvasive positive pressure ventilation in amyotrophic lateral sclerosis, PLOS ONE, 19, 5, (e0302515), (2024).https://doi.org/10.1371/journal.pone.0302515
- Amyotrophic Lateral Sclerosis: Exploring Disability Beyond Motor Weakness, The Palgrave Encyclopedia of Disability, (1-15), (2024).https://doi.org/10.1007/978-3-031-40858-8_218-1
- The Basis of Cognitive and Behavioral Dysfunction in Amyotrophic Lateral Sclerosis, Brain and Behavior, 14, 11, (2024).https://doi.org/10.1002/brb3.70115
- Development and Validation of the Korean Version of the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis Screen (ECAS-K), Journal of Clinical Neurology, 19, 5, (454), (2023).https://doi.org/10.3988/jcn.2022.0403
- Norwegian version of the Edinburgh cognitive and behavioural ALS screen: Construct validity, internal consistency, inter-rater, and test-retest reliability, PLOS ONE, 18, 5, (e0285307), (2023).https://doi.org/10.1371/journal.pone.0285307
- Targeting RACK1 to alleviate TDP-43 and FUS proteinopathy-mediated suppression of protein translation and neurodegeneration, Acta Neuropathologica Communications, 11, 1, (2023).https://doi.org/10.1186/s40478-023-01705-8
- The complexity of multidisciplinary respiratory care in amyotrophic lateral sclerosis, Breathe, 19, 3, (220269), (2023).https://doi.org/10.1183/20734735.0269-2022
- Cognitive impairment is associated with gait variability and fall risk in amyotrophic lateral sclerosis, European Journal of Neurology, 30, 10, (3056-3067), (2023).https://doi.org/10.1111/ene.15936
- See more
Loading...
View Options
Login options
Check if you have access through your login credentials or your institution to get full access on this article.
Personal login Institutional LoginPurchase Options
The neurology.org payment platform is currently offline. Our technical team is working as quickly as possible to restore service.
If you need immediate support or to place an order, please call or email customer service:
- 1-800-638-3030 for U.S. customers - 8:30 - 7 pm ET (M-F)
- 1-301-223-2300 for customers outside the U.S. - 8:30 - 7 pm ET (M-F)
- [email protected]
We appreciate your patience during this time and apologize for any inconvenience.