Literature DB >> 9886451

Differences in duration of Huntington's disease based on age at onset.

T Foroud1, J Gray, J Ivashina, P M Conneally.   

Abstract

OBJECTIVES: Data from a sample of 2494 patients affected with Huntington's disease (HD), collected as part of the National Research Roster for Huntington Disease Patients and Families, were examined to determine if there was a relation between age at onset and duration of illness.
METHODS: Sufficient data for inclusion in analysis was available from 2068 patients, of whom 828 were deceased and 1240 were living. The median duration of disease was 21.4 years with a range of 1.2 to 40.8 years. Patients were categorised into one of four groups based on their age at onset.
RESULTS: Significant differences in duration based on the age at onset were found (p<0.025), with juvenile and late onset patients with HD having shorter duration of illness compared with those with an onset between 20-49 years.
CONCLUSIONS: Duration of disease is influenced by the age at symptom onset with juvenile and late onset patients having the shortest duration.

Entities:  

Mesh:

Year:  1999        PMID: 9886451      PMCID: PMC1736160          DOI: 10.1136/jnnp.66.1.52

Source DB:  PubMed          Journal:  J Neurol Neurosurg Psychiatry        ISSN: 0022-3050            Impact factor:   10.154


  20 in total

1.  Clinical and neuropathologic assessment of severity in Huntington's disease.

Authors:  R H Myers; J P Vonsattel; T J Stevens; L A Cupples; E P Richardson; J B Martin; E D Bird
Journal:  Neurology       Date:  1988-03       Impact factor: 9.910

2.  Trinucleotide repeat length and progression of illness in Huntington's disease.

Authors:  K Kieburtz; M MacDonald; C Shih; A Feigin; K Steinberg; K Bordwell; C Zimmerman; J Srinidhi; J Sotack; J Gusella
Journal:  J Med Genet       Date:  1994-11       Impact factor: 6.318

3.  Factors influencing age at onset and duration of survival in Huntington's chorea.

Authors:  R G Newcombe; D A Walker; P S Harper
Journal:  Ann Hum Genet       Date:  1981-10       Impact factor: 1.670

4.  A huntingtin-associated protein enriched in brain with implications for pathology.

Authors:  X J Li; S H Li; A H Sharp; F C Nucifora; G Schilling; A Lanahan; P Worley; S H Snyder; C A Ross
Journal:  Nature       Date:  1995-11-23       Impact factor: 49.962

5.  Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form.

Authors:  Y Trottier; D Devys; G Imbert; F Saudou; I An; Y Lutz; C Weber; Y Agid; E C Hirsch; J L Mandel
Journal:  Nat Genet       Date:  1995-05       Impact factor: 38.330

6.  Functional decline in Huntington's disease.

Authors:  A Feigin; K Kieburtz; K Bordwell; P Como; K Steinberg; J Sotack; C Zimmerman; C Hickey; C Orme; I Shoulson
Journal:  Mov Disord       Date:  1995-03       Impact factor: 10.338

7.  Expression of the Huntington's disease (IT15) protein product in HD patients.

Authors:  G Schilling; A H Sharp; S J Loev; M V Wagster; S H Li; O C Stine; C A Ross
Journal:  Hum Mol Genet       Date:  1995-08       Impact factor: 6.150

8.  Factors related to onset age of Huntington disease.

Authors:  R H Myers; J J Madden; J L Teague; A Falek
Journal:  Am J Hum Genet       Date:  1982-05       Impact factor: 11.025

9.  Progression rate and age at onset are related in autosomal dominant neurologic diseases.

Authors:  R D Currier; J F Jackson; E F Meydrech
Journal:  Neurology       Date:  1982-08       Impact factor: 9.910

10.  Factors associated with slow progression in Huntington's disease.

Authors:  R H Myers; D S Sax; W J Koroshetz; C Mastromauro; L A Cupples; D K Kiely; F K Pettengill; E D Bird
Journal:  Arch Neurol       Date:  1991-08
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  68 in total

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2.  Nonparametric modeling and analysis of association between Huntington's disease onset and CAG repeats.

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Journal:  Stat Med       Date:  2013-09-12       Impact factor: 2.373

3.  Polysomnographic Findings and Clinical Correlates in Huntington Disease: A Cross-Sectional Cohort Study.

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Journal:  Sleep       Date:  2015-09-01       Impact factor: 5.849

4.  Potential trade-offs in treatment of premanifest Huntington's disease.

Authors:  Roger L Albin; James F Burke
Journal:  Mov Disord       Date:  2015-07-14       Impact factor: 10.338

5.  Sleep in Huntington's disease: a systematic review and meta-analysis of polysomongraphic findings.

Authors:  Ye Zhang; Rong Ren; Linghui Yang; Junying Zhou; Yun Li; Jie Shi; Lin Lu; Larry D Sanford; Xiangdong Tang
Journal:  Sleep       Date:  2019-10-09       Impact factor: 5.849

6.  The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease.

Authors:  Daniel Ryskamp; Jun Wu; Michal Geva; Rebecca Kusko; Iris Grossman; Michael Hayden; Ilya Bezprozvanny
Journal:  Neurobiol Dis       Date:  2016-11-03       Impact factor: 5.996

7.  22 Years of predictive testing for Huntington's disease: the experience of the UK Huntington's Prediction Consortium.

Authors:  Sheharyar S Baig; Mark Strong; Elisabeth Rosser; Nicola V Taverner; Ruth Glew; Zosia Miedzybrodzka; Angus Clarke; David Craufurd; Oliver W Quarrell
Journal:  Eur J Hum Genet       Date:  2016-05-11       Impact factor: 4.246

Review 8.  The ubiquitin-proteasome pathway in Huntington's disease.

Authors:  Steven Finkbeiner; Siddhartha Mitra
Journal:  ScientificWorldJournal       Date:  2008-04-20

9.  Problems assessing uptake of Huntington disease predictive testing and a proposed solution.

Authors:  Roslyn J Tassicker; Betty Teltscher; M Kaye Trembath; Veronica Collins; Leslie J Sheffield; Edmond Chiu; Lyle Gurrin; Martin B Delatycki
Journal:  Eur J Hum Genet       Date:  2008-07-30       Impact factor: 4.246

10.  Predicting Disease Onset from Mutation Status Using Proband and Relative Data with Applications to Huntington's Disease.

Authors:  Tianle Chen; Yuanjia Wang; Yanyuan Ma; Karen Marder; Douglas R Langbehn
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