Structural brain correlates of dementia in Huntington’s disease

Martínez‐Horta, Saül; Sampedro, Frederic; Horta‐Barba, Andrea; Pérez‐Pérez, Jesús; Pagonabarraga, Javier; Gómez‐Anson, Beatriz; Kulisevsky, Jaime

doi:10.1016/j.nicl.2020.102415

Cited by 23 publications

(30 citation statements)

References 45 publications

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“…MoCA global performance correlations analysis demonstrated extensive cortical areas associated with cognitive performance in HD patients. In this sense, our findings are consistent with previous reports in HD that identified specific cognitive deficits –attention, working memory, and executive functions– correlating with cortical changes including cortices of the superior fronto-parietal motor circuits, frontal cognitive control centers, temporal auditory and semantic processing hubs, and occipital visual centers ( Rosas et al, 2005 , 2008 ; Harrington et al, 2014 ; Coppen et al, 2018 ; Martinez-Horta et al, 2020 ). It is important to mention that the MoCA test involves the assessment of several cognitive functions associated with different neurological substrates; therefore, it is understandable that the atrophy of discreet areas within the whole cerebral cortex contributed to the deterioration of MoCA global performance of the HD patients.…”

Section: Discussionsupporting

confidence: 92%

Montreal Cognitive Assessment (MoCA) performance in Huntington’s disease patients correlates with cortical and caudate atrophy

Ramirez-García

Gálvez

Dı́az

et al. 2022

PeerJ

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Huntington’s Disease (HD) is an autosomal neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms. Cognitive impairment develops gradually in HD patients, progressing later into a severe cognitive dysfunction. The Montreal Cognitive Assessment (MoCA) is a brief screening test commonly employed to detect mild cognitive impairment, which has also been useful to assess cognitive decline in HD patients. However, the relationship between MoCA performance and brain structural integrity in HD patients remains unclear. Therefore, to explore this relationship we analyzed if cortical thinning and subcortical nuclei volume differences correlated with HD patients’ MoCA performance. Twenty-two HD patients and twenty-two healthy subjects participated in this study. T1-weighted images were acquired to analyze cortical thickness and subcortical nuclei volumes. Group comparison analysis showed a significantly lower score in the MoCA global performance of HD patients. Also, the MoCA total score correlated with cortical thinning of fronto-parietal and temporo-occipital cortices, as well as with bilateral caudate volume differences in HD patients. These results provide new insights into the effectiveness of using the MoCA test to detect cognitive impairment and the brain atrophy pattern associated with the cognitive status of prodromal/early HD patients.

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Section: Discussionsupporting

confidence: 92%

Montreal Cognitive Assessment (MoCA) performance in Huntington’s disease patients correlates with cortical and caudate atrophy

Ramirez-García

Gálvez

Dı́az

et al. 2022

PeerJ

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“…In patients with HD, widespread cortical thinning was observed compared with healthy controls. This pattern of extensive atrophy has been previously reported by our group 7,32 and others 2,4 and shows that the loss of cortical thickness occurs in frontal, temporo-parietal, and occipital cortices in patients with HD. Patients with pre-HD showed no differences compared with healthy controls using stringent statistical thresholds, a fact that has been shown in previous studies in patients with early HD, 33 although others have shown cortical thinning in the early stages.…”

Section: Discussionsupporting

confidence: 88%

“…However, recent works have shown that progressive atrophy of a wide range of cortical regions is linked to the clinical profile of the disease from the early stages. This cortical atrophy involves several regions that participate in cognitive functions such as the precuneus, 5 the anterior cingulate cortex, 6 and the supramarginal, fusiform, and lateral occipital gyrii 7 . The majority of these regions are integral to the functioning of large‐scale cognitive networks, 8 such the salience network (SN) or default‐mode network (DMN).…”

mentioning

confidence: 99%

Structure and Dynamics of Large‐Scale Cognitive Networks in Huntington's Disease

et al. 2021

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A BS TRACT: Background: Huntington's disease is a neurodegenerative disorder characterized by clinical alterations in the motor, behavioral, and cognitive domains. However, the structure and disruptions to large-scale brain cognitive networks have not yet been established. Objective: We aimed to profile changes in large-scale cognitive networks in premanifest and symptomatic patients with Huntington's disease. Methods: We prospectively recruited premanifest and symptomatic Huntington's disease mutation carriers as well as healthy controls. Clinical and sociodemographic data were obtained from all participants, and resting-state functional connectivity data, using both time-averaged and dynamic functional connectivity, was acquired from whole-brain and cognitively oriented brain parcellations. Results: A total of 64 gene mutation carriers and 23 healthy controls were included; 21 patients with Huntington's disease were classified as premanifest and 43 as symptomatic Huntington's disease. Compared with healthy controls, patients with Huntington's disease showed decreased network connectivity within the posterior hubs of the default-mode network and the medial prefrontal cortex, changes that correlated with cognitive (t = 2.25, P = 0.01) and disease burden scores (t = À2.42, P = 0.009). The salience network showed decreased functional connectivity between insular and supramarginal cortices and also correlated with cognitive (t = 2.11, P = 0.02) and disease burden scores (t = À2.35, P = 0.01). Dynamic analyses showed that network variability was decreased for default-central executive networks, a feature already present in premanifest mutation carriers (dynamic factor 8, P = 0.02). Conclusions: Huntington's disease shows an early and widespread disruption of large-scale cognitive networks. Importantly, these changes are related to cognitive and disease burden scores, and novel dynamic functional analyses uncovered subtler network changes even in the premanifest stages.

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“…Dementia, associated with several neurodegenerative diseases such as Huntington’s disease (HD), causes genetic defects that decline a patient’s memory, thinking, and emotional state. 6 Tau protein accumulation around brain cells causes the formation of neurofibrillary tangles via amyloid-β protein. Plaques may cause Alzheimer’s disease, or they may be a byproduct of the disease process and related to dementia.…”

Section: Introductionmentioning

confidence: 99%

Potential Roles of Glucagon-Like Peptide-1 and Its Analogues in Dementia Targeting Impaired Insulin Secretion and Neurodegeneration

Mehan¹,

Bhalla²,

Siddiqui³

et al. 2022

DNND

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Dementia is a chronic, irreversible condition marked by memory loss, cognitive decline, and mental instability. It is clinically related to various progressive neurological diseases, including Parkinson’s disease, Alzheimer’s disease, and Huntington’s. The primary cause of neurological disorders is insulin desensitization, demyelination, oxidative stress, and neuroinflammation accompanied by various aberrant proteins such as amyloid-β deposits, Lewy bodies accumulation, tau formation leading to neurofibrillary tangles. Impaired insulin signaling is directly associated with amyloid-β and α-synuclein deposition, as well as specific signaling cascades involved in neurodegenerative diseases. Insulin dysfunction may initiate various intracellular signaling cascades, including phosphoinositide 3-kinase (PI3K), c-Jun N-terminal kinases (JNK), and mitogen-activated protein kinase (MAPK). Neuronal death, inflammation, neuronal excitation, mitochondrial malfunction, and protein deposition are all influenced by insulin. Recent research has focused on GLP-1 receptor agonists as a potential therapeutic target. They increase glucose-dependent insulin secretion and are beneficial in neurodegenerative diseases by reducing oxidative stress and cytokine production. They reduce the deposition of abnormal proteins by crossing the blood-brain barrier. The purpose of this article is to discuss the role of insulin dysfunction in the pathogenesis of neurological diseases, specifically dementia. Additionally, we reviewed the therapeutic target (GLP-1) and its receptor activators as a possible treatment of dementia.

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Structural brain correlates of dementia in Huntington’s disease

Cited by 23 publications

References 45 publications

Montreal Cognitive Assessment (MoCA) performance in Huntington’s disease patients correlates with cortical and caudate atrophy

Montreal Cognitive Assessment (MoCA) performance in Huntington’s disease patients correlates with cortical and caudate atrophy

Structure and Dynamics of Large‐Scale Cognitive Networks in Huntington's Disease

Potential Roles of Glucagon-Like Peptide-1 and Its Analogues in Dementia Targeting Impaired Insulin Secretion and Neurodegeneration

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