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Motor pathway degeneration in young ataxia telangiectasia patients: A diffusion tractography study.

Sahama I, Sinclair K, Fiori S, Doecke J, Pannek K, Reid L, Lavin M, Rose S - Neuroimage Clin (2015)

Bottom Line: White matter fiber tracking and whole tract statistical analyses were used to assess quantitative fractional anisotropy and mean diffusivity differences along the cortico-ponto-cerebellar, cerebellar-thalamo-cortical, somatosensory and lateral corticospinal tract length in patients using a linear mixed effects model.Mean diffusivity was significantly elevated in anterior tract locations but was reduced within cerebellar peduncle regions of all patient tracts (p < 0.001).In addition, reduced apparent fiber density in the left and right corticospinal and right somatosensory tracts (p < 0.006) occurred in patients.

View Article: PubMed Central - PubMed

Affiliation: University of Queensland, School of Medicine, Brisbane, Australia.

ABSTRACT

Background: Our understanding of the effect of ataxia-telangiectasia mutated gene mutations on brain structure and function is limited. In this study, white matter motor pathway integrity was investigated in ataxia telangiectasia patients using diffusion MRI and probabilistic tractography.

Methods: Diffusion MRI were obtained from 12 patients (age range: 7-22 years, mean: 12 years) and 12 typically developing age matched participants (age range 8-23 years, mean: 13 years). White matter fiber tracking and whole tract statistical analyses were used to assess quantitative fractional anisotropy and mean diffusivity differences along the cortico-ponto-cerebellar, cerebellar-thalamo-cortical, somatosensory and lateral corticospinal tract length in patients using a linear mixed effects model. White matter tract streamline number and apparent fiber density in patient and control tracts were also assessed.

Results: Reduced fractional anisotropy along all analyzed patient tracts were observed (p < 0.001). Mean diffusivity was significantly elevated in anterior tract locations but was reduced within cerebellar peduncle regions of all patient tracts (p < 0.001). Reduced tract streamline number and tract volume in the left and right corticospinal and somatosensory tracts were observed in patients (p < 0.006). In addition, reduced apparent fiber density in the left and right corticospinal and right somatosensory tracts (p < 0.006) occurred in patients.

Conclusions: Whole tract analysis of the corticomotor, corticospinal and somatosensory pathways in ataxia telangiectasia showed significant white matter degeneration along the entire length of motor circuits, highlighting that ataxia-telangiectasia gene mutation impacts the cerebellum and multiple other motor circuits in young patients.

No MeSH data available.


Related in: MedlinePlus

Number of white matter (WM) streamlines, tract volume and apparent fiber density (AFD) in control and patient tracts: A) Mean number of streamlines in WM tracts (corticospinal (CST), somatosensory (Somato), cortico-ponto-cerebellar (CPC), cerebellar-thalamo-cortical (CTC)) (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). B) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). C) The mean tract volume (mL) in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). D) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) corrected for partial volume effects is plotted with significance (*, p < 0.006).
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f0025: Number of white matter (WM) streamlines, tract volume and apparent fiber density (AFD) in control and patient tracts: A) Mean number of streamlines in WM tracts (corticospinal (CST), somatosensory (Somato), cortico-ponto-cerebellar (CPC), cerebellar-thalamo-cortical (CTC)) (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). B) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). C) The mean tract volume (mL) in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). D) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) corrected for partial volume effects is plotted with significance (*, p < 0.006).

Mentions: Reduced streamline number in the left and right CST and right somatosensory tracts was observed in A–T patients (p < 0.006, Fig. 5A). Tract volume reductions were observed in the left CTC, left somatosensory, right CPC and right CST A–T tracts (p < 0.006, Fig. 5C).


Motor pathway degeneration in young ataxia telangiectasia patients: A diffusion tractography study.

Sahama I, Sinclair K, Fiori S, Doecke J, Pannek K, Reid L, Lavin M, Rose S - Neuroimage Clin (2015)

Number of white matter (WM) streamlines, tract volume and apparent fiber density (AFD) in control and patient tracts: A) Mean number of streamlines in WM tracts (corticospinal (CST), somatosensory (Somato), cortico-ponto-cerebellar (CPC), cerebellar-thalamo-cortical (CTC)) (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). B) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). C) The mean tract volume (mL) in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). D) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) corrected for partial volume effects is plotted with significance (*, p < 0.006).
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4561673&req=5

f0025: Number of white matter (WM) streamlines, tract volume and apparent fiber density (AFD) in control and patient tracts: A) Mean number of streamlines in WM tracts (corticospinal (CST), somatosensory (Somato), cortico-ponto-cerebellar (CPC), cerebellar-thalamo-cortical (CTC)) (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). B) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). C) The mean tract volume (mL) in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) is plotted with significance (*, p < 0.006). D) The mean AFD in WM tracts (left (L) and right (R) ± pointwise 95% confidence interval) corrected for partial volume effects is plotted with significance (*, p < 0.006).
Mentions: Reduced streamline number in the left and right CST and right somatosensory tracts was observed in A–T patients (p < 0.006, Fig. 5A). Tract volume reductions were observed in the left CTC, left somatosensory, right CPC and right CST A–T tracts (p < 0.006, Fig. 5C).

Bottom Line: White matter fiber tracking and whole tract statistical analyses were used to assess quantitative fractional anisotropy and mean diffusivity differences along the cortico-ponto-cerebellar, cerebellar-thalamo-cortical, somatosensory and lateral corticospinal tract length in patients using a linear mixed effects model.Mean diffusivity was significantly elevated in anterior tract locations but was reduced within cerebellar peduncle regions of all patient tracts (p < 0.001).In addition, reduced apparent fiber density in the left and right corticospinal and right somatosensory tracts (p < 0.006) occurred in patients.

View Article: PubMed Central - PubMed

Affiliation: University of Queensland, School of Medicine, Brisbane, Australia.

ABSTRACT

Background: Our understanding of the effect of ataxia-telangiectasia mutated gene mutations on brain structure and function is limited. In this study, white matter motor pathway integrity was investigated in ataxia telangiectasia patients using diffusion MRI and probabilistic tractography.

Methods: Diffusion MRI were obtained from 12 patients (age range: 7-22 years, mean: 12 years) and 12 typically developing age matched participants (age range 8-23 years, mean: 13 years). White matter fiber tracking and whole tract statistical analyses were used to assess quantitative fractional anisotropy and mean diffusivity differences along the cortico-ponto-cerebellar, cerebellar-thalamo-cortical, somatosensory and lateral corticospinal tract length in patients using a linear mixed effects model. White matter tract streamline number and apparent fiber density in patient and control tracts were also assessed.

Results: Reduced fractional anisotropy along all analyzed patient tracts were observed (p < 0.001). Mean diffusivity was significantly elevated in anterior tract locations but was reduced within cerebellar peduncle regions of all patient tracts (p < 0.001). Reduced tract streamline number and tract volume in the left and right corticospinal and somatosensory tracts were observed in patients (p < 0.006). In addition, reduced apparent fiber density in the left and right corticospinal and right somatosensory tracts (p < 0.006) occurred in patients.

Conclusions: Whole tract analysis of the corticomotor, corticospinal and somatosensory pathways in ataxia telangiectasia showed significant white matter degeneration along the entire length of motor circuits, highlighting that ataxia-telangiectasia gene mutation impacts the cerebellum and multiple other motor circuits in young patients.

No MeSH data available.


Related in: MedlinePlus