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Article: Abnormal diffusion tensor in nonsymptomatic familial amyotrophic lateral sclerosis with a causative superoxide dismutase 1 mutation
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TitleAbnormal diffusion tensor in nonsymptomatic familial amyotrophic lateral sclerosis with a causative superoxide dismutase 1 mutation
 
AuthorsNg, MC1 1
Ho, JT1
Ho, SL1 1 1
Lee, R1
Li, G1
Cheng, TS1
Song, YQ1
Ho, PWL1
Fong, GCY1
Mak, W1
Chan, KH1
Li, LSW1
Luk, KDK1
Hu, Y1
Ramsden, DB2
Leong, LLY1
 
KeywordsALS
DTI
Familial
Nonsymptomatic
SOD1 mutation
 
Issue Date2008
 
PublisherJohn Wiley & Sons, Inc. The Journal's web site is located at http://www.interscience.wiley.com/jpages/1053-1807/
 
CitationJournal Of Magnetic Resonance Imaging, 2008, v. 27 n. 1, p. 8-13 [How to Cite?]
DOI: http://dx.doi.org/10.1002/jmri.21217
 
AbstractPurpose: To determine whether diffusion abnormalities can be observed in nonsymptomatic family members with a known causative Cu/Zn superoxide dismutase mutation (asymptomatic familial amyotrophic lateral sclerosis; AFALS +SOD1) in a family with autosomal dominant familial amyotrophic lateral sclerosis (ALS) using diffusion tensor imaging (DTI). Materials and Methods: A total of eight AFALS +SOD1 subjects (aged 17-43 years) were age-matched with 13 healthy controls (aged 19-45 years) without SOD1 mutations. DTI was carried out on a 1.5T scanner. The diffusion index maps derived were then normalized spatially for voxel-based analysis, region of interest (ROI)-based analysis was also carried out. Results: Our voxel-based and ROI-based analysis showed that AFALS +SOD1 subjects have decreased fractional anisotropy (FA) (0.5401 vs. 0.5168, P < 0.05) and increased tensor trace (TT) (2.5854 × 10 -3 mm 2/second vs. 2.6226 × 10 -3 mm 2/second, P < 0.04) at the posterior limb of the internal capsule compared to the control subjects. Increased radial diffusivity (E (2,3)/2) was detected on both sides (right = 0.5710 × 10 -3 mm 2/second vs. 0.5943 × 10 -3 mm 2/second, P < 0.05; left = 0.5666 × 10 -3 mm 2/second vs. 0.5872 × 10 -3 mm 2/second, P < 0.05). No significant change in axial diffusivity (E 1) was detected. Conclusion: Abnormal diffusivity was found at the posterior limb of the internal capsule in AFALS +SOD1 subjects, hitherto unreported. Our results suggest that DTI may detect diffusion abnormalities in AFALS +SOD1 subjects before symptoms develop. © 2007 Wiley-Liss, Inc.
 
ISSN1053-1807
2012 Impact Factor: 2.566
2012 SCImago Journal Rankings: 1.330
 
DOIhttp://dx.doi.org/10.1002/jmri.21217
 
ISI Accession Number IDWOS:000252104100002
 
ReferencesReferences in Scopus
 
DC FieldValue
dc.contributor.authorNg, MC
 
dc.contributor.authorHo, JT
 
dc.contributor.authorHo, SL
 
dc.contributor.authorLee, R
 
dc.contributor.authorLi, G
 
dc.contributor.authorCheng, TS
 
dc.contributor.authorSong, YQ
 
dc.contributor.authorHo, PWL
 
dc.contributor.authorFong, GCY
 
dc.contributor.authorMak, W
 
dc.contributor.authorChan, KH
 
dc.contributor.authorLi, LSW
 
dc.contributor.authorLuk, KDK
 
dc.contributor.authorHu, Y
 
dc.contributor.authorRamsden, DB
 
dc.contributor.authorLeong, LLY
 
dc.date.accessioned2010-09-06T06:00:35Z
 
dc.date.available2010-09-06T06:00:35Z
 
dc.date.issued2008
 
dc.description.abstractPurpose: To determine whether diffusion abnormalities can be observed in nonsymptomatic family members with a known causative Cu/Zn superoxide dismutase mutation (asymptomatic familial amyotrophic lateral sclerosis; AFALS +SOD1) in a family with autosomal dominant familial amyotrophic lateral sclerosis (ALS) using diffusion tensor imaging (DTI). Materials and Methods: A total of eight AFALS +SOD1 subjects (aged 17-43 years) were age-matched with 13 healthy controls (aged 19-45 years) without SOD1 mutations. DTI was carried out on a 1.5T scanner. The diffusion index maps derived were then normalized spatially for voxel-based analysis, region of interest (ROI)-based analysis was also carried out. Results: Our voxel-based and ROI-based analysis showed that AFALS +SOD1 subjects have decreased fractional anisotropy (FA) (0.5401 vs. 0.5168, P < 0.05) and increased tensor trace (TT) (2.5854 × 10 -3 mm 2/second vs. 2.6226 × 10 -3 mm 2/second, P < 0.04) at the posterior limb of the internal capsule compared to the control subjects. Increased radial diffusivity (E (2,3)/2) was detected on both sides (right = 0.5710 × 10 -3 mm 2/second vs. 0.5943 × 10 -3 mm 2/second, P < 0.05; left = 0.5666 × 10 -3 mm 2/second vs. 0.5872 × 10 -3 mm 2/second, P < 0.05). No significant change in axial diffusivity (E 1) was detected. Conclusion: Abnormal diffusivity was found at the posterior limb of the internal capsule in AFALS +SOD1 subjects, hitherto unreported. Our results suggest that DTI may detect diffusion abnormalities in AFALS +SOD1 subjects before symptoms develop. © 2007 Wiley-Liss, Inc.
 
dc.description.natureLink_to_subscribed_fulltext
 
dc.identifier.citationJournal Of Magnetic Resonance Imaging, 2008, v. 27 n. 1, p. 8-13 [How to Cite?]
DOI: http://dx.doi.org/10.1002/jmri.21217
 
dc.identifier.doihttp://dx.doi.org/10.1002/jmri.21217
 
dc.identifier.epage13
 
dc.identifier.hkuros143686
 
dc.identifier.isiWOS:000252104100002
 
dc.identifier.issn1053-1807
2012 Impact Factor: 2.566
2012 SCImago Journal Rankings: 1.330
 
dc.identifier.issue1
 
dc.identifier.openurl
 
dc.identifier.pmid18022844
 
dc.identifier.scopuseid_2-s2.0-37849027014
 
dc.identifier.spage8
 
dc.identifier.urihttp://hdl.handle.net/10722/68021
 
dc.identifier.volume27
 
dc.languageeng
 
dc.publisherJohn Wiley & Sons, Inc. The Journal's web site is located at http://www.interscience.wiley.com/jpages/1053-1807/
 
dc.publisher.placeUnited States
 
dc.relation.ispartofJournal of Magnetic Resonance Imaging
 
dc.relation.referencesReferences in Scopus
 
dc.rightsJournal of Magnetic Resonance Imaging. Copyright © John Wiley & Sons, Inc.
 
dc.subject.meshAdolescent
 
dc.subject.meshAdult
 
dc.subject.meshAmyotrophic Lateral Sclerosis - enzymology - genetics - pathology
 
dc.subject.meshAnisotropy
 
dc.subject.meshDiffusion Magnetic Resonance Imaging - methods
 
dc.subject.meshFemale
 
dc.subject.meshHumans
 
dc.subject.meshImage Processing, Computer-Assisted
 
dc.subject.meshMale
 
dc.subject.meshMutation
 
dc.subject.meshStatistics, Nonparametric
 
dc.subject.meshSuperoxide Dismutase - genetics
 
dc.subjectALS
 
dc.subjectDTI
 
dc.subjectFamilial
 
dc.subjectNonsymptomatic
 
dc.subjectSOD1 mutation
 
dc.titleAbnormal diffusion tensor in nonsymptomatic familial amyotrophic lateral sclerosis with a causative superoxide dismutase 1 mutation
 
dc.typeArticle
 
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<description.abstract>Purpose: To determine whether diffusion abnormalities can be observed in nonsymptomatic family members with a known causative Cu/Zn superoxide dismutase mutation (asymptomatic familial amyotrophic lateral sclerosis; AFALS +SOD1) in a family with autosomal dominant familial amyotrophic lateral sclerosis (ALS) using diffusion tensor imaging (DTI). Materials and Methods: A total of eight AFALS +SOD1 subjects (aged 17-43 years) were age-matched with 13 healthy controls (aged 19-45 years) without SOD1 mutations. DTI was carried out on a 1.5T scanner. The diffusion index maps derived were then normalized spatially for voxel-based analysis, region of interest (ROI)-based analysis was also carried out. Results: Our voxel-based and ROI-based analysis showed that AFALS +SOD1 subjects have decreased fractional anisotropy (FA) (0.5401 vs. 0.5168, P &lt; 0.05) and increased tensor trace (TT) (2.5854 &#215; 10 -3 mm 2/second vs. 2.6226 &#215; 10 -3 mm 2/second, P &lt; 0.04) at the posterior limb of the internal capsule compared to the control subjects. Increased radial diffusivity (E (2,3)/2) was detected on both sides (right = 0.5710 &#215; 10 -3 mm 2/second vs. 0.5943 &#215; 10 -3 mm 2/second, P &lt; 0.05; left = 0.5666 &#215; 10 -3 mm 2/second vs. 0.5872 &#215; 10 -3 mm 2/second, P &lt; 0.05). No significant change in axial diffusivity (E 1) was detected. Conclusion: Abnormal diffusivity was found at the posterior limb of the internal capsule in AFALS +SOD1 subjects, hitherto unreported. Our results suggest that DTI may detect diffusion abnormalities in AFALS +SOD1 subjects before symptoms develop. &#169; 2007 Wiley-Liss, Inc.</description.abstract>
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Author Affiliations
  1. The University of Hong Kong
  2. University of Birmingham