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Research ArticleADULT BRAIN
Open Access

Ataxia Severity Correlates with White Matter Degeneration in Spinocerebellar Ataxia Type 7

C.R. Hernandez-Castillo, I. Vaca-Palomares, F. Barrios, L. Martinez, M.-C. Boll and J. Fernandez-Ruiz
American Journal of Neuroradiology November 2016, 37 (11) 2050-2054; DOI: https://doi.org/10.3174/ajnr.A4903
C.R. Hernandez-Castillo
aFrom the Consejo Nacional de Ciencia y Tecnologia–Instituto de Neuroetologia (C.R.H.-C.), Universidad Veracruzana, Veracruz, Mexico
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  • ORCID record for C.R. Hernandez-Castillo
I. Vaca-Palomares
bDepartamento de Fisiologia (I.V.-P., J.F.-R.), Facultad de Medicina
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  • ORCID record for I. Vaca-Palomares
F. Barrios
cInstituto de Neurobiologia (F.B.), Universidad Nacional Autonoma de Mexico, Mexico City, Mexico
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L. Martinez
dDepartamento de Neurogenética y Biologıa Molecular (L.M.)
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M.-C. Boll
eLaboratorio de Investigacion Clinica (M.-C.B.), Instituto Nacional de Neurología y Neurocirugía, “Manuel Velasco Suárez,” Mexico City, México.
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J. Fernandez-Ruiz
bDepartamento de Fisiologia (I.V.-P., J.F.-R.), Facultad de Medicina
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    Fig 1.

    TBSS significant differences in diffusion measurements between patients with SCA7 and healthy controls. A, Fractional anisotropy. B, Mean diffusivity. Warm and cold colors indicate an increase and decrease in these measures in patients with SCA7, respectively.

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    Fig 2.

    White matter regions where MD correlates with the SARA score in the SCA7 group. Warm colors indicate significant correlations between MD values and SARA scores.

Tables

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    Table 1:

    Demographic information of the SCA7 group

    IDSexAge (yr)Years from OnsetMMSEMoCASARA
    P01F21126NC4
    P02M20129NC4
    P03F68124NC6
    P04F18427NC7
    P05F35626NC8.5
    P06F44626169
    P07F641328129.5
    P08M444241811
    P09F22226NC11
    P10M527261612
    P11F457262112
    P12M233242712.5
    P13M476261413
    P14M254261314
    P15M6410261514.5
    P16M3813271814.5
    P17F432126NC15
    P18M351426916
    P19M606261316
    P20M306251216
    P21M341028NC17
    P22M30526NC17
    P23F32627NC19
    P24M188261619.5
    P25F401325NC23
    P26M54625NC24
    P27M61722NC25
    P28M297211725
    P29F4021221827
    P30F3923251827
    P31F4217231529.5
    P32M197221029.5
    P33F518261830
    • Note:—MMSE indicates Mini-Mental State Examination; MoCA, Montreal Cognitive Assessment; NC, not completed; ID, identification.

    • View popup
    Table 2:

    Significant group differences in fractional anisotropy between patients with SCA7 and healthy controlsa

    Anatomic RegionVoxelsT-ValueXYZ
    Right superior cerebellar peduncle179718.97−47−28
    Left corticospinal tract100911−13−56−28
    Left anterior corona radiata7336.86−263111
    Body of corpus callosum3274.04−61322
    Right corticospinal tract2621113−50−43
    Left uncinate fasciculus2536.27−2118−12
    Left superior longitudinal fasciculus1805.08−102749
    Left inferior longitudinal fasciculus1775.75−40−47−13
    Right superior longitudinal fasciculus1624.5336−1334
    Left anterior limb of the internal capsule1345.07−20175
    Left cingulate WM1316.36−6334
    Left inferior fronto-occipital fasciculus1214.89−163533
    Left superior longitudinal fasciculus1144.75−44−4113
    Right superior longitudinal fasciculus1124.2251−723
    • ↵a Coordinates are in millimeters in Montreal Neurological Institute space. Labels are from the Johns Hopkins University white matter atlas.

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    Table 3:

    Significant group differences in mean diffusivity between patients with SCA7 and healthy controlsa

    Anatomic RegionVoxelsT-ValueXYZ
    Right middle cerebellar peduncle271211.126−51−37
    Left middle cerebellar peduncle204712.6−26−48−38
    Left anterior thalamic radiation9115.12−3−1111
    Right inferior fronto-occipital fasciculus8555.1919−8316
    Left inferior longitudinal fasciculus6284.21−14−812
    Left posterior thalamic radiation3503.23−35−448
    Right inferior longitudinal fasciculus1824.3937−742
    Right cingulate WM1413.5927−45−2
    Right posterior corona radiata1125.2229−3419
    Right anterior thalamic radiation1084.161−20−10
    Splenium of corpus callosum1044.9119−5022
    Left cingulate WM1013.58−28−50−1
    Left inferior longitudinal fasciculus1003.38−17−7715
    • ↵a Coordinates are in millimeters in Montreal Neurological Institute space. Labels are from the Johns Hopkins University white matter atlas.

    • View popup
    Table 4:

    White matter regions showing significant correlation between MD and the SARA score in the SCA7 groupa

    Anatomic RegionVoxelsT-ValueXYZ
    Right inferior longitudinal fasciculus64286.7440−746
    Right superior cerebellar peduncle14209.167−43−23
    Left cerebellum anterior lobule10147.03−14−53−28
    Left inferior fronto-occipital fasciculus6847.89−19−86−9
    Right anterior thalamic radiation2395.9214−6049
    Right superior longitudinal fasciculus1585.5444−3434
    Left anterior thalamic radiation14810.1−3−912
    • ↵a Coordinates are in millimeters in Montreal Neurological Institute space. Labels are from the Johns Hopkins University white matter atlas.

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C.R. Hernandez-Castillo, I. Vaca-Palomares, F. Barrios, L. Martinez, M.-C. Boll, J. Fernandez-Ruiz
Ataxia Severity Correlates with White Matter Degeneration in Spinocerebellar Ataxia Type 7
American Journal of Neuroradiology Nov 2016, 37 (11) 2050-2054; DOI: 10.3174/ajnr.A4903

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Ataxia Severity Correlates with White Matter Degeneration in Spinocerebellar Ataxia Type 7
C.R. Hernandez-Castillo, I. Vaca-Palomares, F. Barrios, L. Martinez, M.-C. Boll, J. Fernandez-Ruiz
American Journal of Neuroradiology Nov 2016, 37 (11) 2050-2054; DOI: 10.3174/ajnr.A4903
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