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Arch Neurol. 2009 Jan;66(1):109-15. doi: 10.1001/archneurol.2008.527.

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

Author information

1
Medical Research Council Centre for Neurodegeneration Research and Department of Clinical Neuroscience, Institute of Psychiatry, King's College London, London SE5 8AF, England.

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.

PMID:
19139308
DOI:
10.1001/archneurol.2008.527
[Indexed for MEDLINE]
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