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Please use this identifier to cite or link to this item: http://hdl.handle.net/1959.14/117593
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- Title
- Corticomotoneuronal function in asymptomatic SOD-1 mutation carriers
- Related
- Clinical neurophysiology, Vol. 121, Issue 10 (2010), p.1781-1785
- DOI
- 10.1016/j.clinph.2010.02.164
- Publisher
- Elsevier
- Date
- 2010
- Author/Creator
- Vucic, Steve
- Author/Creator
- Winhammar, Jennica M. C
- Author/Creator
- Rowe, Dominic B
- Author/Creator
- Kiernan, Matthew C
- Description
- Objective: Diffusion tensor imaging (DTI) recently identified structural abnormalities of corticomotoneurons in asymptomatic copper/zinc superoxide-dismutase-1 (SOD-1) gene mutation carriers. The potential existence of longstanding corticomotoneuronal dysfunction would clearly have consequences for the medical management of asymptomatic SOD-1 mutation carriers. To clarify this unexpected finding, DTI techniques were combined with threshold tracking transcranial magnetic stimulation (TMS) to assess the anatomical and functional integrity of corticomotoneurons in asymptomatic SOD-1 mutation carriers. Methods: TMS studies were undertaken using a 90 mm circular coil on seven asymptomatic SOD-1 mutation carriers and results were compared to 62 healthy controls. DTI studies were carried out using a 3 T magnetic resonance device in the same asymptomatic SOD-1 mutation carriers. Results were compared to age-matched healthy controls. Results: In contrast to previous findings, there were no significant differences in fractional anisotropy (SOD-1 mutation carriers, 0.62 ± 0.01; controls, 0.61 ± 0.02, P = 0.2) and trace apparent diffusion coefficient (SOD-1 mutation carriers, 0.003 ± 0.0001; controls, 0.003 ± 0.0001) in asymptomatic SOD-1 mutation carriers. Of further relevance, there were no significant differences in short-interval intracortical inhibition (SOD-1 mutation carriers, 7.9 ± 3.4%; controls, 8.5 ± 1.1%, P = 0.26), intracortical facilitation (P = 0.5), MEP amplitude (P = 0.44), resting motor threshold (P = 0.36) and cortical silent period duration (P = 0.29). Conclusions: Combined anatomical and functional modalities established normal integrity of corticomotoneurons in asymptomatic SOD-1 mutation carrier subjects. Significance: Additional factors other than simply SOD-1 mutation expression are required to trigger cortical hyperexcitability and neurodegeneration in FALS.
- Description
- 5 page(s)
- Subject Keyword
- 110300 Clinical Sciences
- Subject Keyword
- 170200 Cognitive Sciences
- Subject Keyword
- 110900 Neurosciences
- Subject Keyword
- diffusion tensor tractography
- Subject Keyword
- familial ALS
- Subject Keyword
- SOD-1 mutation carrier
- Resource Type
- journal article
- Organisation
- Macquarie University. Australian School of Advanced Medicine
- Identifier
- http://hdl.handle.net/1959.14/117593
- Identifier
- ISSN:1388-2457
- Identifier
- mq-rm-2010003967
- Language
- eng
- Reviewed
Macquarie University ResearchOnline
