Literature DB >> 12507683

Future directions in research with presymptomatic individuals carrying the gene for Huntington's disease.

Nellie Georgiou-Karistianis1, Eleanor Smith, John L Bradshaw, Phyllis Chua, John Lloyd, Andrew Churchyard, Edmond Chiu.   

Abstract

Presymptomatic individuals carrying the gene for Huntington's disease (HD) provide researchers with a unique opportunity of learning more about the neuropathophysiology, symptom onset, behavioural functioning, and mediating factors of this fatal disease. In this review, we attempt to demonstrate that research over the last 8 years, since the isolation of the gene, has remained at large controversial. Although we are aware of some of the factors that can influence age at onset and disease progression, we are still unable to determine exactly when an individual will develop HD symptoms, and how fast these symptoms will progress. In an era rapidly advancing with respect to therapeutic intervention that could forestall the onset and progression of HD, systematic research with improved inclusion criteria is paramount. A greater understanding of the time course of the disease would be beneficial not only in monitoring the effectiveness of future treatments, but also in determining the most appropriate time to administer them. Finally, we present various ethical considerations, as well as put forward various recommendations that could assist in better diagnosing preclinical deficits in presymptomatic individuals.

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Year:  2003        PMID: 12507683     DOI: 10.1016/s0361-9230(02)00877-8

Source DB:  PubMed          Journal:  Brain Res Bull        ISSN: 0361-9230            Impact factor:   4.077


  4 in total

1.  Functional connectivity of the prefrontal cortex in Huntington's disease.

Authors:  D R Thiruvady; N Georgiou-Karistianis; G F Egan; S Ray; A Sritharan; M Farrow; A Churchyard; P Chua; J L Bradshaw; T-L Brawn; R Cunnington
Journal:  J Neurol Neurosurg Psychiatry       Date:  2006-10-06       Impact factor: 10.154

2.  Measuring executive dysfunction longitudinally and in relation to genetic burden, brain volumetrics, and depression in prodromal Huntington disease.

Authors:  Kathryn V Papp; Peter J Snyder; James A Mills; Kevin Duff; Holly J Westervelt; Jeffrey D Long; Spencer Lourens; Jane S Paulsen
Journal:  Arch Clin Neuropsychol       Date:  2012-12-16       Impact factor: 2.813

3.  Cognitive changes in patients with Huntington's disease (HD) and asymptomatic carriers of the HD mutation--a longitudinal follow-up study.

Authors:  Jurgen Lemiere; Marleen Decruyenaere; Gery Evers-Kiebooms; Erik Vandenbussche; Rene Dom
Journal:  J Neurol       Date:  2004-08       Impact factor: 4.849

4.  Neuropsychological deficits in Huntington's disease gene carriers and correlates of early "conversion".

Authors:  Jason Brandt; Anjeli B Inscore; Julianna Ward; Barnett Shpritz; Adam Rosenblatt; Russell L Margolis; Christopher A Ross
Journal:  J Neuropsychiatry Clin Neurosci       Date:  2008       Impact factor: 2.198

  4 in total

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