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A longitudinal study of self-reported Quality of Life in ALS patients
Uppsala University, Medicinska vetenskapsområdet, Faculty of Medicine, Department of Neuroscience.
Article in journal (Refereed) Submitted
Identifiers
URN: urn:nbn:se:uu:diva-92659OAI: oai:DiVA.org:uu-92659DiVA: diva2:165821
Available from: 2005-03-17 Created: 2005-03-17Bibliographically approved
In thesis
1. ALS – a Clinical Thesis
Open this publication in new window or tab >>ALS – a Clinical Thesis
2005 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Amyotrophic lateral sclerosis (ALS) is characterized by a progressive loss of upper and lower motor neurons, resulting in muscle weakness and death from respiratory failure within 3-5 years after onset. The incidence is 1.5-2.7/100,000 inhabitants. 5-10% of all cases are hereditary. The aetiology of sporadic ALS is still unknown.

The only neuroprotective drug approved for the treatment of ALS is riluzole, a glutamate-antagonist, which has shown to improve survival. We evaluated if riluzole sales statistics can be used as a method for estimating the prevalence of ALS/motor neuron disease in Sweden. We found that this method, which is less time consuming than conventional methods, could be used as a crude marker for the prevalence.

In a longitudinal study of overall Quality of Life (QoL) in ALS we found that QoL changes only slightly over time despite disease progression. ALS does not necessarily result in a low QoL.

Growth factors are important for the survival of neurons. In ALS we found increased or normal levels of GDNF mRNA and BDNF mRNA in muscle biopsies, VEGF in serum and spinal cord and FGF-2 in serum and cerebrospinal fluid. There is thus no deficit of these growth factors although there may be a relative lack because of high demands of the motor neurons. Polyamines are small aliphatic molecules that are important for the function of cells. The level of the polyamines spermidine and spermine were increased in red blood cells in both patients with ALS and patients with Parkinson’s disease, suggesting that polyamines may have a role for the neurodegenerative process. Polyamines in spinal cord were of the same level in the patients with ALS and in controls, indicating a maintained regulation of polyamines at the end-stage of the disease.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2005. 61 p.
Series
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 12
Keyword
Neurosciences, ALS, epidemiology, drug sales statistics, QoL, growth factors, polyamines, Neurovetenskap
National Category
Neurology
Identifiers
urn:nbn:se:uu:diva-4804 (URN)91-554-6157-3 (ISBN)
Public defence
2005-04-08, Hedstrandsalen, Akademiska sjukhuset, Uppsala, 13:15
Opponent
Supervisors
Available from: 2005-03-17 Created: 2005-03-17Bibliographically approved

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