Literature DB >> 25794860

Progress in Huntington's disease: the search for markers of disease onset and progression.

Sarah Mason1, Roger A Barker.   

Abstract

Unlike most neurodegenerative disorders, individuals at risk from Huntington's disease can be identified prior to the onset of clinical signs of the disease by virtue of it being an autosomal dominant condition. This provides the hypothetical opportunity to delay disease onset and/or slow down the progression of the disease in the very early stages ahead of overt features of disease. To help prepare for therapeutic trials of disease-modifying compounds, extensive work has gone into (1) finding ways of better predicting the onset of disease in pre-manifest HD gene carriers (PMGC), (2) defining the extent of non-motor features of HD and (3) identifying robust and reliable tests by which to measure disease progression. In this short review, we summarise some of the major findings in this area of clinical research.

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Year:  2015        PMID: 25794860     DOI: 10.1007/s00415-015-7700-0

Source DB:  PubMed          Journal:  J Neurol        ISSN: 0340-5354            Impact factor:   4.849


  43 in total

1.  The distribution of structural neuropathology in pre-clinical Huntington's disease.

Authors:  M J Thieben; A J Duggins; C D Good; L Gomes; N Mahant; F Richards; E McCusker; R S J Frackowiak
Journal:  Brain       Date:  2002-08       Impact factor: 13.501

2.  Substance abuse may be a risk factor for earlier onset of Huntington disease.

Authors:  Joanne A Byars; Leigh J Beglinger; David J Moser; Pedro Gonzalez-Alegre; Peg Nopoulos
Journal:  J Neurol       Date:  2012-01-25       Impact factor: 4.849

3.  Neurocognitive signs in prodromal Huntington disease.

Authors:  Julie C Stout; Jane S Paulsen; Sarah Queller; Andrea C Solomon; Kathryn B Whitlock; J Colin Campbell; Noelle Carlozzi; Kevin Duff; Leigh J Beglinger; Douglas R Langbehn; Shannon A Johnson; Kevin M Biglan; Elizabeth H Aylward
Journal:  Neuropsychology       Date:  2011-01       Impact factor: 3.295

4.  Longitudinal pilot-study of Sustained Attention to Response Task and P300 in manifest and pre-manifest Huntington's disease.

Authors:  Ellen P Hart; Eve M Dumas; Erik W van Zwet; Karin van der Hiele; Caroline K Jurgens; Huub A M Middelkoop; J Gert van Dijk; Raymund A C Roos
Journal:  J Neuropsychol       Date:  2013-10-24       Impact factor: 2.864

Review 5.  Glucocorticoids and hippocampal atrophy in neuropsychiatric disorders.

Authors:  R M Sapolsky
Journal:  Arch Gen Psychiatry       Date:  2000-10

6.  Reduced basal ganglia blood flow and volume in pre-symptomatic, gene-tested persons at-risk for Huntington's disease.

Authors:  G J Harris; A M Codori; R F Lewis; E Schmidt; A Bedi; J Brandt
Journal:  Brain       Date:  1999-09       Impact factor: 13.501

7.  Autonomic nervous system function in Huntington's disease.

Authors:  J Andrich; T Schmitz; C Saft; T Postert; P Kraus; J T Epplen; H Przuntek; M W Agelink
Journal:  J Neurol Neurosurg Psychiatry       Date:  2002-06       Impact factor: 10.154

8.  Association between caffeine intake and age at onset in Huntington's disease.

Authors:  Clémence Simonin; Cécile Duru; Julia Salleron; Pascale Hincker; Perrine Charles; Arnaud Delval; Katia Youssov; Sylvie Burnouf; Jean-Philippe Azulay; Christophe Verny; Clarisse Scherer; Christine Tranchant; Cyril Goizet; Sabrina Debruxelles; Luc Defebvre; Bernard Sablonnière; Monique Romon-Rousseaux; Luc Buée; Alain Destée; Olivier Godefroy; Alexandra Dürr; Bernhard Landwehrmeyer; Anne-Catherine Bachoud-Levi; Florence Richard; David Blum; Pierre Krystkowiak
Journal:  Neurobiol Dis       Date:  2013-05-31       Impact factor: 5.996

9.  Cognitive and autonomic dysfunction in presymptomatic and early Huntington's disease.

Authors:  Jan Kobal; Ziva Melik; Ksenija Cankar; Martin Strucl
Journal:  J Neurol       Date:  2014-04-02       Impact factor: 4.849

Review 10.  A review of cognition in Huntington's disease.

Authors:  Eve M Dumas; Simon J A van den Bogaard; Huub A M Middelkoop; Raymund A C Roos
Journal:  Front Biosci (Schol Ed)       Date:  2013-01-01
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