Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis

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

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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
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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
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