Tools
Tools
Wicks, P, Abrahams, S, Papps, B, Al-Chalabi, A, Shaw, C E, Leigh, P N and Goldstein, L H (2009) SOD1 and cognitive dysfunction in familial amyotrophic lateral sclerosis. Journal of neurology, 256 (2). pp. 234-41. ISSN 1432-1459
Full text not available from this repository.Abstract
BACKGROUND
Sporadic Amyotrophic Lateral Sclerosis (sALS) is associated with frontotemporal dementia (ALS-FTD) or milder deficits of cognitive (predominantly executive) dysfunction (ALSCi) in some patients. Some forms of familial ALS (FALS) have a family history of FTD, ALS-FTD, or both, but there have been few reports of ALS-FTD in FALS patients with mutations of the gene superoxide dismutase-1 (SOD1 FALS). The aim of this study was to test the hypothesis that ALSCi may be found in non-SOD1 FALS, but that SOD1 FALS patients would show little or no evidence of cognitive change.
METHODS
A neuropsychological test battery was administered to 41 SALS patients, 35 control participants, 7 FALS patients with a SOD1 mutation (SOD1 FALS) and 10 FALS patients without a SOD1 mutation (non-SOD1 FALS).
RESULTS
Relative to control participants, non-SOD1 FALS patients had impaired performance on written verbal fluency and confrontation naming, and reported higher levels of executive behavioural problems. These deficits were absent in SOD1 FALS patients. SALS patients performed poorer than controls only on the Graded Naming Test. All ALS groups had higher levels of behavioural apathy and emotional lability than were found in control participants. Cognitive domains of memory, receptive language, and visuospatial perception were spared. Groups were matched for age, gender, premorbid full-scale IQ, anxiety and depression.
DISCUSSION
Individuals with SOD1 gene mutations are less likely to have significant cognitive changes compared to non-SOD1 FALS patients. Cognitive abnormalities in ALS are heterogeneous and may reflect underlying genetic variations rather than a simple spectrum of extra-motor involvement
| Item Type: | Article |
|---|---|
| Keywords: | ALS - cognition - SOD1 - neuropsychology - dementias |
| Schools and Departments: | Brighton and Sussex Medical School > Clinical and Experimental Medicine Brighton and Sussex Medical School > Neuroscience |
| Subjects: | R Medicine > RC Internal medicine > RC0321 Neurosciences. Biological psychiatry. Neuropsychiatry > RC0346 Neurology. Diseases of the nervous system Including speech disorders |
| Related URLs: | |
| Depositing User: | Patricia Butler |
| Date Deposited: | 15 Nov 2012 14:12 |
| Last Modified: | 25 Sep 2017 13:53 |
| URI: | http://srodev.sussex.ac.uk/id/eprint/42509 |