Utility of the Parkinson’s disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington’s disease

Martínez‐Horta, Saül; Horta‐Barba, Andrea; Pérez‐Pérez, Jesús; Sampedro, Frederic; Lucia, Natascia De; Michele, Giuseppe De; Kehrer, Stefanie; Priller, Josef; Migliore, Simone; Squitieri, Ferdinando; Castaldo, Anna; Mariotti, Caterina; Mañanes, Veronica; López‐Sendón, José; Rodriguez, Noelia; Martinez-Descals, Asunción; Ruiz, Pedro; Júlio, Filipa; Januário, Cristina; Delussi, Marianna; Tommaso, Marina de; Noguera, Sandra; Ruiz-Idiago, Jesús; Sitek, Emilia J.; Nuzzi, Angela; Pagonabarraga, Javier; Kulisevsky, Jaime

doi:10.1007/s00415-020-09730-6

Cited by 14 publications

(24 citation statements)

References 32 publications

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“…These cutoff scores were determined in HD population in a previous study using as gold standard the presence of major cognitive and functional impairment. Thus, in accordance with this previous study we used the PD-CRS total score ≤ 64 to classify patients as with major cognitive impairment in the range of dementia( Martinez-Horta et al, 2020 ). This instrument assesses nine subtests: immediate and delayed verbal memory, confrontation naming, attention, working memory, unprompted draw of a clock, copy of a clock, alternating verbal fluency, and action verbal fluency.…”

Section: Methodsmentioning

confidence: 92%

“…The main limitation of the present study is the lack of a gold-standard to classify patients according to cognitive status, but the method we used to classify patients has recently been shown to be reliable in this population( Martinez-Horta et al, 2020 ). Despite this limitation, it is reasonable to assume that the measure we used for classification differentiates patients with normal cognitive function or mild cognitive defects from those with severe cognitive alterations.…”

Section: Discussionmentioning

confidence: 98%

“…HD patients were classified as with severe or without severe cognitive impairment using the HD-specific PD-CRS cutoff scores( Martinez-Horta et al, 2020 ). These cutoff scores were determined in HD population in a previous study using as gold standard the presence of major cognitive and functional impairment.…”

Section: Methodsmentioning

confidence: 99%

“…The specific patterns of brain alterations underlying major cognitive impairment in HD thus remain elusive, partly because of the difficulty in appropriately grouping patients according to cognitive status. To overcome this limitation, we recently validated the Parkinson’s Disease – Cognitive Rating Scale (PD-CRS) as a screening tool for global cognition in HD( Martinez-Horta et al, 2020 ). We showed that the HD-specific PD-CRS cutoff scores have a good capacity to discriminate between categories of cognitive status in patients with HD.…”

Section: Introductionmentioning

confidence: 99%

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Structural brain correlates of dementia in Huntington’s disease

Martínez‐Horta

Sampedro

Horta‐Barba

et al. 2020

NeuroImage: Clinical

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Highlights Dementia may occur in the early stages of HD and with independence of disease burden. More severe posterior-cortical atrophy is associated with dementia in HD. Neuropsychological alterations of dementia in HD extends beyond executive dysfunction. CAG-independent neuropathological mechanisms may contribute to dementia in HD.

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Section: Methodsmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 98%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Structural brain correlates of dementia in Huntington’s disease

Martínez‐Horta

Sampedro

Horta‐Barba

et al. 2020

NeuroImage: Clinical

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“…We did not find a correlation with motor impairment, rather increased synchronization corresponded to worse cognitive performance in both mHD and pHD, in well localized BA areas in prefrontal regions. SDMT and SWRT are both relevant clinical markers in HD research and disease progression (Martinez-Horta et al, 2020). Considering that HD is characterized by an accumulation of subcortical and cortical dysfunctions with a disruption of cortico-subcortical circuits, the current neuroimaging findings are not able to detect in which exact area of the cerebral cortex meaningful neuronal loss firstly occurs.…”

Section: Discussionmentioning

confidence: 99%

EEG Functional Connectivity and Cognitive Variables in Premanifest and Manifest Huntington’s Disease: EEG Low-Resolution Brain Electromagnetic Tomography (LORETA) Study

et al. 2020

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BackgroundScientific literature does not offer sufficient data on electroencephalography (EEG) functional connectivity and its correlations with clinical and cognitive features in premanifest and manifest HD.AimThis study tries to identify abnormal EEG patterns of functional connectivity, in conditions of “brain resting state” and correlations with motor decline and cognitive variable in Huntington’s disease (HD), in premanifest and manifest phase, looking for a reliable marker measuring disease progression.MethodThis was an observational cross-sectional study; 105 subjects with age ≥18 years submitted to HD genetic test. Each subject underwent a neurological, psychiatric, and cognitive assessment, EEG recording and genetic investigation for detecting the expansion of the CAG trait. EEG connectivity analysis was performed by means of exact Low Resolution Electric Tomography (eLORETA) in 18 premanifest HD (pHD), 49 manifest HD (mHD), and 38 control (C) subjects.ResultsHD patients showed a Power Spectral Density reduced in the alpha range and increased in delta band compared to controls; no difference was detectable between pHD and mHD; the Global Connectivity in pHD revealed no significant differences if compared to mHD. The Current Source Density was similar among groups. No statistically significant results when comparing pHD with C group, even in comparison of mHD with Controls, and pHD with mHD. mHD compared to Controls showed a significant increase in delta, alpha1, alpha2, beta2, and beta3. Lagged Phase Synchronization in delta, alpha1, alpha2, beta2, and beta3 bands was increased in HD compared to controls (t = −3.921, p < 0.05). A significant correlation was found in Regression Analysis: statistically significant results in pHD for the “Symbol Digit Modality Test and lagged phase synchronization” in the Beta1 (r = −0.806, p < 0.05) in the prefrontal regions. The same correlation was found in mHD for the Stroop Word Reading Test (SWRT) in the Alpha2 band (r = −0.759, p < 0.05).ConclusionIncreased phase synchronization in main bands characterized EEG in HD patients, as compared to controls. pHD were not dissimilar from mHD as regard to this EEG pattern. Increased phase synchronization correlated to cognitive decline in HD patients, with a similar trend in pHD, suggesting that it would be a potential biomarker of early phenotypical expression.

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Cognitive phenotype and neurodegeneration associated with Tau in Huntington's disease

Martinez‐Horta,

Perez‐Perez,

Perez‐Gonzalez

et al. 2024

Ann Clin Transl Neurol

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ObjectiveThe clinical phenotype of Huntington's disease (HD) can be very heterogeneous between patients, even when they share equivalent CAG repeat length, age, or disease burden. This heterogeneity is especially evident in terms of the cognitive profile and related brain changes. To shed light on the mechanisms participating in this heterogeneity, the present study delves into the association between Tau pathology and more severe cognitive phenotypes and brain damage in HD.MethodsWe used a comprehensive neuropsychological examination to characterize the cognitive phenotype of a sample of 30 participants with early‐to‐middle HD for which we also obtained 3 T structural magnetic resonance image (MRI) and cerebrospinal fluid (CSF). We quantified CSF levels of neurofilament light chain (NfL), total Tau (tTau), and phosphorylated Tau‐231 (pTau‐231). Thanks to the cognitive characterization carried out, we subsequently explored the relationship between different levels of biomarkers, the cognitive phenotype, and brain integrity.ResultsThe results confirmed that more severe forms of cognitive deterioration in HD extend beyond executive dysfunction and affect processes with clear posterior‐cortical dependence. This phenotype was in turn associated with higher CSF levels of tTau and pTau‐231 and to a more pronounced pattern of posterior‐cortical atrophy in specific brain regions closely linked to the cognitive processes affected by Tau.InterpretationOur findings reinforce the association between Tau pathology, cognition, and neurodegeneration in HD, emphasizing the need to explore the role of Tau in the cognitive heterogeneity of the disease.

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Utility of the Parkinson’s disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington’s disease

Cited by 14 publications

References 32 publications

Structural brain correlates of dementia in Huntington’s disease

Structural brain correlates of dementia in Huntington’s disease

EEG Functional Connectivity and Cognitive Variables in Premanifest and Manifest Huntington’s Disease: EEG Low-Resolution Brain Electromagnetic Tomography (LORETA) Study

Cognitive phenotype and neurodegeneration associated with Tau in Huntington's disease

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