Format

Send to

Choose Destination
AJNR Am J Neuroradiol. 2019 Sep;40(9):1464-1468. doi: 10.3174/ajnr.A6168.

Widespread Increased Diffusivity Reveals Early Cortical Degeneration in Huntington Disease.

Author information

1
From the Movement Disorders Unit (F.S., S.M.-H., J.P.-P., A.H.-B., J.M.-L., J.K.), Neurology Department.
2
Biomedical Research Institute (F.S., S.M.-H., J.P.-P., A.H.-B., J.M.-L., A.A.-S., I.C., J.K.), Barcelona, Spain.
3
Centro de Investigación en Red-Enfermedades Neurodegenerativas (F.S., S.M.-H., J.P.-P., A.H.-B., J.M.-L., J.K.), Madrid, Spain.
4
Universitat Autónoma de Barcelona (S.M.-H., J.P.-P., J.M.-L., B.G.-A., J.K.), Barcelona, Spain.
5
Psychiatry Department (I.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
6
Centro de Investigación Biomédica en Red-Salud Mental (I.C.), Madrid, Spain.
7
Neuroradiology, Radiology Department (B.G.-A.).
8
European Huntington's Disease Network (S.M.-H., J.P.-P., A.H.-B., J.K.), Ulm, Germany.
9
From the Movement Disorders Unit (F.S., S.M.-H., J.P.-P., A.H.-B., J.M.-L., J.K.), Neurology Department jkulisevsky@santpau.cat.

Abstract

BACKGROUND AND PURPOSE:

Huntington disease is a devastating genetic neurodegenerative disorder for which no effective treatment is yet available. Although progressive striatal atrophy is its pathologic hallmark, concomitant cortical deterioration is assumed to occur, but it is poorly characterized. Our objective was to study the loss of cortical integrity and its association with clinical indicators throughout the course of the disease.

MATERIALS AND METHODS:

Using a cohort of 39 patients with Huntington disease and 25 controls with available MR imaging (T1WI and DTI), we compared cortical atrophy and intracortical diffusivity across disease stages. Intracortical diffusivity is a DTI-derived metric that has recently been suggested to detect incipient neuronal death because water can diffuse more freely in cortical regions with reduced neural density.

RESULTS:

We observed progressive thinning and increasing diffusivity within the cerebral cortex of patients with Huntington disease (P < .05, corrected for multiple comparisons). Most important, in the absence of pronounced atrophy, widespread increased diffusivity was already present in individuals with premanifest Huntington disease, correlating, in turn, with clinical and disease-specific progression markers.

CONCLUSIONS:

Intracortical diffusivity may be more sensitive than cortical thinning for tracking early neurodegeneration in Huntington disease. Moreover, our findings provide further evidence of an early cortical compromise in Huntington disease, which contributes to our understanding of its clinical phenotype and could have important therapeutic implications.

PMID:
31467235
DOI:
10.3174/ajnr.A6168

Supplemental Content

Full text links

Icon for HighWire
Loading ...
Support Center