Structural neuroimaging differentiates vulnerability from disease manifestation in Colombian families with Huntington's disease

Maria del C. Valdés Hernández, Janna Abu-Hussain, Xinyi Qiu, Josef Priller, Mario Parra Rodríguez, Mariana Pino, Sandra Báez, Agustín Ibáñez

Research output: Contribution to journalArticle

Abstract

Introduction: The volume of the striatal structures has been associated with disease progression in individuals with Huntington's disease (HD) from North America, Europe, and Australia. However, it is not known whether the gray matter (GM) volume in the striatum is also sensitive in differentiating vulnerability from disease manifestation in HD families from a South-American region known to have high incidence of the disease. In addition, the association of enlarged brain perivascular spaces (PVS) with cognitive, behavioral, and motor symptoms of HD is unknown. Materials and Methods: We have analyzed neuroimaging indicators of global atrophy, PVS burden, and GM tissue volume in the basal ganglia and thalami, in relation to behavioral, motor, and cognitive scores, in 15 HD patients with overt disease manifestation and 14 first-degree relatives not genetically tested, which represent a vulnerable group, from the region of Magdalena, Colombia. Results: Poor fluid intelligence as per the Raven's Standard Progressive Matrices was associated with global brain atrophy (p = 0.002) and PVS burden (p ≤ 0.02) in HD patients, where the GM volume in all subcortical structures, with the exception of the right globus pallidus, was associated with motor or cognitive scores. Only the GM volume in the right putamen was associated with envy and MOCA scores (p = 0.008 and 0.015 respectively) in first-degree relatives. Conclusion: Striatal GM volume, global brain atrophy and PVS burden may serve as differential indicators of disease manifestation in HD. The Raven's Standard Progressive Matrices could be a cognitive test worth to consider in the differentiation of vulnerability versus overt disease in HD.

LanguageEnglish
Article numbere01343
Number of pages13
JournalBrain and Behavior
Volume9
Issue number8
Early online date5 Jul 2019
DOIs
Publication statusE-pub ahead of print - 5 Jul 2019

Fingerprint

Huntington Disease
Neuroimaging
Atrophy
Corpus Striatum
Crows
Methylenebis(chloroaniline)
Brain
Behavioral Symptoms
Globus Pallidus
Colombia
Putamen
North America
Basal Ganglia
Thalamus
Intelligence
Disease Progression
Gray Matter
Incidence

Keywords

  • basal ganglia
  • MRI
  • Huntington’s disease
  • cognition
  • perivascular spaces
  • fluid intelligence
  • Raven matrices

Cite this

del C. Valdés Hernández, Maria ; Abu-Hussain, Janna ; Qiu, Xinyi ; Priller, Josef ; Parra Rodríguez, Mario ; Pino, Mariana ; Báez, Sandra ; Ibáñez, Agustín. / Structural neuroimaging differentiates vulnerability from disease manifestation in Colombian families with Huntington's disease. In: Brain and Behavior. 2019 ; Vol. 9, No. 8.
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abstract = "Introduction: The volume of the striatal structures has been associated with disease progression in individuals with Huntington's disease (HD) from North America, Europe, and Australia. However, it is not known whether the gray matter (GM) volume in the striatum is also sensitive in differentiating vulnerability from disease manifestation in HD families from a South-American region known to have high incidence of the disease. In addition, the association of enlarged brain perivascular spaces (PVS) with cognitive, behavioral, and motor symptoms of HD is unknown. Materials and Methods: We have analyzed neuroimaging indicators of global atrophy, PVS burden, and GM tissue volume in the basal ganglia and thalami, in relation to behavioral, motor, and cognitive scores, in 15 HD patients with overt disease manifestation and 14 first-degree relatives not genetically tested, which represent a vulnerable group, from the region of Magdalena, Colombia. Results: Poor fluid intelligence as per the Raven's Standard Progressive Matrices was associated with global brain atrophy (p = 0.002) and PVS burden (p ≤ 0.02) in HD patients, where the GM volume in all subcortical structures, with the exception of the right globus pallidus, was associated with motor or cognitive scores. Only the GM volume in the right putamen was associated with envy and MOCA scores (p = 0.008 and 0.015 respectively) in first-degree relatives. Conclusion: Striatal GM volume, global brain atrophy and PVS burden may serve as differential indicators of disease manifestation in HD. The Raven's Standard Progressive Matrices could be a cognitive test worth to consider in the differentiation of vulnerability versus overt disease in HD.",
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Structural neuroimaging differentiates vulnerability from disease manifestation in Colombian families with Huntington's disease. / del C. Valdés Hernández, Maria; Abu-Hussain, Janna; Qiu, Xinyi; Priller, Josef; Parra Rodríguez, Mario; Pino, Mariana; Báez, Sandra; Ibáñez, Agustín.

In: Brain and Behavior, Vol. 9, No. 8, e01343, 05.07.2019.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Structural neuroimaging differentiates vulnerability from disease manifestation in Colombian families with Huntington's disease

AU - del C. Valdés Hernández, Maria

AU - Abu-Hussain, Janna

AU - Qiu, Xinyi

AU - Priller, Josef

AU - Parra Rodríguez, Mario

AU - Pino, Mariana

AU - Báez, Sandra

AU - Ibáñez, Agustín

PY - 2019/7/5

Y1 - 2019/7/5

N2 - Introduction: The volume of the striatal structures has been associated with disease progression in individuals with Huntington's disease (HD) from North America, Europe, and Australia. However, it is not known whether the gray matter (GM) volume in the striatum is also sensitive in differentiating vulnerability from disease manifestation in HD families from a South-American region known to have high incidence of the disease. In addition, the association of enlarged brain perivascular spaces (PVS) with cognitive, behavioral, and motor symptoms of HD is unknown. Materials and Methods: We have analyzed neuroimaging indicators of global atrophy, PVS burden, and GM tissue volume in the basal ganglia and thalami, in relation to behavioral, motor, and cognitive scores, in 15 HD patients with overt disease manifestation and 14 first-degree relatives not genetically tested, which represent a vulnerable group, from the region of Magdalena, Colombia. Results: Poor fluid intelligence as per the Raven's Standard Progressive Matrices was associated with global brain atrophy (p = 0.002) and PVS burden (p ≤ 0.02) in HD patients, where the GM volume in all subcortical structures, with the exception of the right globus pallidus, was associated with motor or cognitive scores. Only the GM volume in the right putamen was associated with envy and MOCA scores (p = 0.008 and 0.015 respectively) in first-degree relatives. Conclusion: Striatal GM volume, global brain atrophy and PVS burden may serve as differential indicators of disease manifestation in HD. The Raven's Standard Progressive Matrices could be a cognitive test worth to consider in the differentiation of vulnerability versus overt disease in HD.

AB - Introduction: The volume of the striatal structures has been associated with disease progression in individuals with Huntington's disease (HD) from North America, Europe, and Australia. However, it is not known whether the gray matter (GM) volume in the striatum is also sensitive in differentiating vulnerability from disease manifestation in HD families from a South-American region known to have high incidence of the disease. In addition, the association of enlarged brain perivascular spaces (PVS) with cognitive, behavioral, and motor symptoms of HD is unknown. Materials and Methods: We have analyzed neuroimaging indicators of global atrophy, PVS burden, and GM tissue volume in the basal ganglia and thalami, in relation to behavioral, motor, and cognitive scores, in 15 HD patients with overt disease manifestation and 14 first-degree relatives not genetically tested, which represent a vulnerable group, from the region of Magdalena, Colombia. Results: Poor fluid intelligence as per the Raven's Standard Progressive Matrices was associated with global brain atrophy (p = 0.002) and PVS burden (p ≤ 0.02) in HD patients, where the GM volume in all subcortical structures, with the exception of the right globus pallidus, was associated with motor or cognitive scores. Only the GM volume in the right putamen was associated with envy and MOCA scores (p = 0.008 and 0.015 respectively) in first-degree relatives. Conclusion: Striatal GM volume, global brain atrophy and PVS burden may serve as differential indicators of disease manifestation in HD. The Raven's Standard Progressive Matrices could be a cognitive test worth to consider in the differentiation of vulnerability versus overt disease in HD.

KW - basal ganglia

KW - MRI

KW - Huntington’s disease

KW - cognition

KW - perivascular spaces

KW - fluid intelligence

KW - Raven matrices

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