Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis.
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...
| Main Authors: | , , , , , , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
2009
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| _version_ | 1826287001894125568 |
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| author | Stanton, B Shinhmar, D Turner, M Williams, VC Williams, S Blain, C Giampietro, V Catani, M Leigh, P Andersen, P Simmons, A |
| author_facet | Stanton, B Shinhmar, D Turner, M Williams, VC Williams, S Blain, C Giampietro, V Catani, M Leigh, P Andersen, P Simmons, A |
| author_sort | Stanton, B |
| collection | OXFORD |
| description | 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. |
| first_indexed | 2024-03-07T01:52:04Z |
| format | Journal article |
| id | oxford-uuid:9a74c58c-a5ac-430e-aea6-15c63d9c1f92 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T01:52:04Z |
| publishDate | 2009 |
| record_format | dspace |
| spelling | oxford-uuid:9a74c58c-a5ac-430e-aea6-15c63d9c1f922022-03-27T00:21:22ZDiffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:9a74c58c-a5ac-430e-aea6-15c63d9c1f92EnglishSymplectic Elements at Oxford2009Stanton, BShinhmar, DTurner, MWilliams, VCWilliams, SBlain, CGiampietro, VCatani, MLeigh, PAndersen, PSimmons, ABACKGROUND: 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. |
| spellingShingle | Stanton, B Shinhmar, D Turner, M Williams, VC Williams, S Blain, C Giampietro, V Catani, M Leigh, P Andersen, P Simmons, A Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. |
| title | Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. |
| title_full | Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. |
| title_fullStr | Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. |
| title_full_unstemmed | Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. |
| title_short | Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. |
| title_sort | diffusion tensor imaging in sporadic and familial d90a sod1 forms of amyotrophic lateral sclerosis |
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