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Stanton, Biba R, Shinhmar, Daisy, Turner, Martin R, Williams, Victoria C, Williams, Steven C R, Blain, Camilla R V, Giampietro, Vincent P, Catani, Marco, Leigh, P Nigel, Andersen, Peter M and Simmons, Andrew (2009) Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. Archives of Neurology, 66 (1). pp. 109-15. ISSN 0003-9942
Full text not available from this repository.Abstract
BACKGROUND
The basis of heterogeneity in the clinical presentation and rate of progression of amyotrophic lateral sclerosis (ALS) is poorly understood.
OBJECTIVES
To use diffusion tensor imaging as a measure of axonal pathologic features in vivo in ALS and to compare a homogeneous form of familial ALS (homozygous D90A SOD1 [superoxide dismutase 1]) with sporadic ALS.
DESIGN
Cross-sectional diffusion tensor imaging study.
SETTING
Tertiary referral neurology clinic.
PATIENTS
Twenty patients with sporadic ALS, 6 patients with homozygous D90A SOD1 ALS, and 21 healthy control subjects.
MAIN OUTCOME MEASURE
Fractional anisotropy in cerebral white matter.
RESULTS
Patients with homozygous D90A SOD1 ALS showed less extensive pathologic white matter in motor and extramotor pathways compared with patients with sporadic ALS, despite similar disease severity assessed clinically using a standard functional rating scale. Fractional anisotropy correlated with clinical measures of severity and upper motor neuron involvement.
CONCLUSION
In vivo diffusion tensor imaging measures demonstrate differences in white matter degeneration between sporadic ALS and a unique familial form of the disease, indicating that genotype influences the distribution of cerebral pathologic features in ALS
| Item Type: | Article |
|---|---|
| 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 12:48 |
| Last Modified: | 25 Sep 2017 13:53 |
| URI: | http://sro.sussex.ac.uk/id/eprint/42513 |