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
Ho, JT1
Ho, SL1
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
2011 Impact Factor: 2.698
2011 SCImago Journal Rankings: 0.242
DOIhttp://dx.doi.org/10.1002/jmri.21217
ISI Accession Number IDWOS:000252104100002
ReferencesReferences in Scopus
DC Field
Value
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
2011 Impact Factor: 2.698
2011 SCImago Journal Rankings: 0.242
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
Author Affiliations
  1. The University of Hong Kong
  2. University of Birmingham