Retest effects and cognitive decline in longitudinal follow-up of patients  with early HD

Bachoud‐Lévi, Anne‐Catherine; Maison, Patrick; Bartolomeo, Paolo; Boissé, MF; Barba, G. Dalla; Ergis, Anne-Marie; Baudic, Sophie; Degos, JD; Césaro, P.; Peschanski, Marc

doi:10.1212/wnl.56.8.1052

Cited by 84 publications

(94 citation statements)

References 44 publications

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“…A quantitative analysis of the total number of correctly produced words showed that the patients score deteriorated over time on both letter and category fluency tasks and they also produced more repetitions. This finding is consistent with the role of the frontal lobe in tests of verbal fluency [2,9,10,17] and provides further evidence that longitudinal deterioration within HD individuals occurs, even though this is not evident from studies utilising cross-sectional paradigms [5,6].…”

Section: Discussionsupporting

confidence: 81%

“…The effect sizes for the mean of patients' regression scores for each measure was then tested for significance with α at 0.05 (two-tailed). As patients' successive performances over time was of interest, a healthy control group was not used [2,3].…”

Section: Discussionmentioning

confidence: 99%

“…The measure of performance most commonly used is the number of correctly generated words within 60 s. This measure is believed to tap frontal [9,10] and temporal [8,11,24] lobe function and is generally decreased in Huntington's disease (HD) patients relative to controls [5][6][7]36]. Indeed, there is some evidence for a decline in performance over time for letter [17] and category fluency in HD [2], despite the lack of cross-sectional difference for both letter and category fluency when comparing early versus late HD [5,6].…”

Section: Introductionmentioning

confidence: 99%

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Verbal fluency in Huntington’s disease: a longitudinal analysis of phonemic and semantic clustering and switching

Sahakian

Robbins

et al. 2002

Neuropsychologia

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Two underlying components of verbal fluency tasks have been identified as clustering (the ability to generate successive words within a sub-category) and switching (the ability to shift from one sub-category to another). Selective impairment of switching ability occurs in patients with frontostriatal pathology, whilst clustering ability is compromised with temporal lobe dysfunction. Letter fluency tasks have been shown to be especially sensitive to frontostriatal deficits, whereas, category fluency tasks tend to be compromised by temporal lobe pathology. This study examined two types of verbal fluency task (letter fluency and category fluency) using two levels of analysis (phonemic and semantic) for clustering and switching measures. The performance of 21 frontostriatally compromised Huntington's disease (HD) patients was followed over an average of 3.5 annual follow-up visits. HD patients showed a significant reduction of correctly generated words over time, together with a significant increase in word repetitions. Phonemic switching decreased significantly over time for both letter and category fluency. Semantic switching, however, remained stable over time for both verbal fluency tasks. Clustering (both semantic and phonemic) likewise remained stable and did not vary longitudinally for either letter or category fluency. Hence, phonemic switching alone drove verbal fluency performance and this selective impairment can be explained by the progressive involvement of frontostriatal circuitry in the natural progression of HD.

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Section: Discussionsupporting

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Verbal fluency in Huntington’s disease: a longitudinal analysis of phonemic and semantic clustering and switching

Sahakian

Robbins

et al. 2002

Neuropsychologia

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“…The cognitive tests were part of a comprehensive battery which has been detailed previously [16] . Cognitive tests were selected with respect to their reliability.…”

Section: Neuropsychological and Motor Assessmentmentioning

confidence: 99%

Cognitive Impairment Related to Apathy in Early Huntington’s Disease

Baudic¹,

Maison²,

Dolbeau³

et al. 2006

Dement Geriatr Cogn Disord

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Objective: To investigate the relationships between cognitive impairment and apathy in patients with early Huntington’s disease (HD) and to further explore the influence of depression on the outcome of cognitive changes associated with apathy. Methods: We included 36 early HD patients, among them 20 were apathetic (HDA) and 16 were not (HDnA). The two groups were matched by age, education and severity of disease. Cognitive functions were evaluated by a comprehensive neuropsychological battery that measures memory, attention, executive function, language and visuospatial abilities. Results: The HDA patients had significantly lower scores on memory, attention and executive function tests when compared with the HDnA patients (p values <0.05). We compared the performance of patients with (50%) and without depression on cognitive tasks and showed that depression per se did not influence performance. Finally, the results demonstrate that interactions between apathy and motor disturbance have a significant effect on cognitive impairment in HD. Discussion: The presence of apathy is associated with more severe deficits of attention, executive function and episodic memory in early HD patients. Furthermore, the findings suggest that depression has little or no effect on cognitive deficits. Finally, apathy increased in parallel with both motor and cognitive dysfunction.

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“…Although there is a rich cross-sectional literature of the functional pathology in Huntington's disease 62,63 and the unified Huntington's disease rating scale (UHDRS) provides a useful index of the stage of the disease, 64 until recently there has been very little careful analysis of the consistency of disease progression or determination of the most reliable tests in cognitive, motor, or psychiatric domains that can be used repeatedly to characterize this. As a result of the need for better assessment tools for transplantation studies, there have now been a number of detailed longitudinal studies published, 65,66 alongside the development of a core assessment protocol to allow comparison of small numbers of patients in multiple centers under standardized conditions. 67 In addition, both experimental and clinical studies have been employed to determine the efficacy and sensitivity of alternative imaging modalities to evaluate not only the extent of neurodegeneration in the disease, but also the survival of functional graft tissues in the host striatum.…”

Section: Preclinical Studiesmentioning

confidence: 99%

Cell therapy in Huntington’s disease

Dunnett

Rosser

2004

Neurotherapeutics

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Summary: Huntington's disease is an autosomal dominant genetic disease, which results in progressive neuronal degeneration in the neostriatum and neocortex, and associated functional impairments in motor, cognitive, and psychiatric domains. Although the genetic mutation is identified, involving an abnormal CAG expansion within the htt gene on chromosome 4, the mechanism by which this leads to neuronal cell death and the question of why striatal neurones are targeted both remain unknown. Thus, in addition to the search for molecular and genetic strategies to inhibit development of the disease, we still need to identify effective strategies for cellular repair in affected individuals. Aspects of the human neuropathology can be well modeled by excitotoxic or metabolic lesions in experimental animals, and in transgenic mice carrying the htt mutation, providing the basis for testing alternative therapeutic strategies. The rationale and efficacy of alternative cell therapies are reviewed, including transplantation repair with embryonic striatal tissues, expansion and differentiation of striatal-like cells from stem cells, and in vivo and ex vivo gene therapy for delivery of neuroprotective growth factor molecules. Pilot and experimental clinical trials of several approaches are now also underway, and the alternative strategies are compared.

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Retest effects and cognitive decline in longitudinal follow-up of patients with early HD

Cited by 84 publications

References 44 publications

Verbal fluency in Huntington’s disease: a longitudinal analysis of phonemic and semantic clustering and switching

Verbal fluency in Huntington’s disease: a longitudinal analysis of phonemic and semantic clustering and switching

Cognitive Impairment Related to Apathy in Early Huntington’s Disease

Cell therapy in Huntington’s disease

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