Access the full text.
Sign up today, get DeepDyve free for 14 days.
N. Wexler, Laura Collett, A. Wexler, M. Rawlins, S. Tabrizi, I. Douglas, L. Smeeth, S. Evans (2016)Incidence of adult Huntington's disease in the UK: a UK-based primary care study and a systematic review
BMJ Open, 6
J. Ritton, J. Ahlskog, R. Robinson, B. Kremer, M. Hayden (2019)Hereditary late-onset chorea without significant dementia: Genetic evidence for substantial phenotypic variation in Huntington’s disease
N. Wexler, J. Lorimer, J. Porter, F. Gomez, C. Moskowitz, Edith Shackell, K. Marder, G. Penchaszadeh, S. Roberts, J. Gayán, D. Brocklebank, S. Cherny, L. Cardon, J. Gray, S. Dlouhy, Sandra Wiktorski, M. Hodes, P. Conneally, J. Penney, J. Gusella, J. Cha, M. Irizarry, D. Rosas, S. Hersch, Z. Hollingsworth, M. MacDonald, A. Young, J. Andresen, D. Housman, M. Young, E. Bonilla, Theresa Stillings, A. Negrette, S. Snodgrass, Maria Martinez-Jaurrieta, M. Ramos-Arroyo, J. Bickham, J. Ramos, F. Marshall, I. Shoulson, G. Rey, A. Feigin, N. Arnheim, A. Acevedo-Cruz, L. Acosta, J. Alvir, K. Fischbeck, L. Thompson, A. Young, L. Dure, C. O’Brien, J. Paulsen, A. Brickman, D. Krch, S. Peery, P. Hogarth, D. Higgins, B. Landwehrmeyer (2004)Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset.
Proceedings of the National Academy of Sciences of the United States of America, 101 10
M. Nance, B. Westphal, S. Nugent (1996)Diagnosis of patients presenting to a Huntington disease (HD) clinic without a family history of HD
J. Groen, R. Bie, E. Foncke, C. Roos, K. Leenders, J. Tijssen, R. Bie, E. Foncke, R. Roos, M. Tijssen, Dr. Koning-Tijssen (2009)Late-onset Huntington’s disease with intermediate CAG-repeats: true or false?
Jong-Min Lee, V. Wheeler, M. Chao, J. Vonsattel, R. Pinto, D. Lucente, K. Abu-Elneel, E. Ramos, J. Mysore, T. Gillis, M. MacDonald, J. Gusella, D. Harold, T. Stone, V. Escott-Price, Jun Han, Alexey Vedernikov, P. Holmans, L. Jones, S. Kwak, Mithra Mahmoudi, M. Orth, G. Landwehrmeyer, Jane Paulsen, E. Dorsey, I. Shoulson, R. Myers (2015)Identification of Genetic Factors that Modify Clinical Onset of Huntington’s Disease
A. Rosenblatt, B. Kumar, R. Margolis, Claire Welsh, C. Ross (2011)Factors contributing to institutionalization in patients with Huntington's disease
Movement Disorders, 26
J. Andrich, L. Arning, S. Wieczorek, P. Kraus, R. Gold, C. Saft (2008)Huntington's disease as caused by 34 CAG repeats
Movement Disorders, 23
Jason Warren, F. Firgaira, Elizabeth Thompson, C. Kneebone, Peter Blumbergs, Philip Thompson (1998)The causes of sporadic and 'senile' chorea.
Australian and New Zealand journal of medicine, 28 4
Donata Gallucci, G. Frisoni, M. Trabucchi, I. Appollonio (1996)THE RELEVANCE OF GENETIC TESTING IN LATE‐ONSET HUNTINGTON'S DISEASE
Journal of the American Geriatrics Society, 44
Phillip Adams, Arthur Falek, Jonathan ArnoldII (1988)Huntington disease in Georgia: age at onset.
American journal of human genetics, 43 5
A. Semaka, S. Creighton, S. Warby, Michael Hayden (2006)Predictive testing for Huntington disease: interpretation and significance of intermediate alleles
Clinical Genetics, 70
P. Morrison, W. Johnston, N. Nevin (1995)The epidemiology of Huntington's disease in Northern Ireland.
Journal of Medical Genetics, 32
EW Almqvist, D. Elterman, PM Macleod, M. Hayden (2001)High incidence rate and absent family histories in one quarter of patients newly diagnosed with Huntington disease in British Columbia
Clinical Genetics, 60
Joseph Martin (1984)Huntington's disease
A. Semaka, S. Warby, B. Leavitt, M. Hayden (2008)Re: Autopsy‐proven Huntington's disease with 29 trinucleotide repeats
Movement Disorders, 23
A. Ha, C. Beck, J. Jankovic (2012)Intermediate CAG Repeats in Huntington's Disease: Analysis of COHORT
Tremor and Other Hyperkinetic Movements, 2
M. Ramos-Arroyo, S. Moreno, A. Valiente (2005)Incidence and mutation rates of Huntington’s disease in Spain: experience of 9 years of direct genetic testing
Journal of Neurology, Neurosurgery & Psychiatry, 76
D. Langbehn, R. Brinkman, D. Falush, Jane Paulsen, M. Hayden (2004)A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length
Clinical Genetics, 65
P. Garcia-Ruiz, J. García‐Caldentey, C. Feliz, J. Val, A. Herranz, J. Martínez-Castrillo (2016)Late onset Huntington's disease with 29 CAG repeat expansion
Journal of the Neurological Sciences, 363
E. Cubo, M. Ramos-Arroyo, S. Martínez-Horta, Asunción Martínez-Descalls, S. Calvo, Cecilia Gil-Polo (2016)Clinical manifestations of intermediate allele carriers in Huntington disease
A. Killoran, K. Biglan, J. Jankovic, S. Eberly, E. Kayson, D. Oakes, A. Young, I. Shoulson (2013)Characterization of the Huntington intermediate CAG repeat expansion phenotype in PHAROS
S. Evans, I. Douglas, M. Rawlins, N. Wexler, S. Tabrizi, L. Smeeth (2013)Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records
Journal of Neurology, Neurosurgery, and Psychiatry, 84
Iris Reuter, Michele Hu, Thomasin Andrews, David Brooks, Chris Clough, K. Chaudhuri (2000)Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome
Journal of Neurology, Neurosurgery & Psychiatry, 68
B. Kremer, F. Squitieri, H. Telenius, S. Andrew, J. Theilmann, N. Spence, Y. Goldberg, M. Hayden (1993)Molecular analysis of late onset Huntington's disease.
Journal of Medical Genetics, 30
A. Rosenblatt, B. Kumar, Alisa Mo, Claire Welsh, R. Margolis, C. Ross (2012)Age, CAG repeat length, and clinical progression in Huntington's disease
Movement Disorders, 27
A. Rosenblatt, K. Liang, H. Zhou, M. Abbott, L. Gourley, R. Margolis, Jason Brandt, Christopher Ross (2006)The association of CAG repeat length with clinical progression in Huntington disease
E. Faught, J. Falgout, D. Leli (1983)Late‐Onset Variant of Huntington's Chorea
Southern Medical Journal, 76
R. Roos, J. Hermans, M. Vlis, G. Ommen, G. Bruyn (1993)Duration of illness in Huntington's disease is not related to age at onset.
Journal of Neurology, Neurosurgery & Psychiatry, 56
M. Alonso, A. Ochoa, M. Boll, A. Sosa, P. Yescas, Marisol López, R. Macías, I. Familiar, A. Rasmussen (2009)Clinical and genetic characteristics of Mexican Huntington's disease patients
Movement Disorders, 24
G. Koutsis, G. Karadima, A. Kladi, M. Panas (2014)Late-onset Huntington's disease: diagnostic and prognostic considerations.
Parkinsonism & related disorders, 20 7
B. Ravina, Megan Romer, R. Constantinescu, K. Biglan, A. Brocht, K. Kieburtz, I. Shoulson, M. Mcdermott (2008)The relationship between CAG repeat length and clinical progression in Huntington's disease
Movement Disorders, 23
M. Oosterloo, M. Belzen, E. Bijlsma, R. Roos (2016)Reply: Late onset Huntington's disease with 29 CAG repeat expansion
Journal of the Neurological Sciences, 368
Mario Cornejo-Olivas, Miguel Inca-Martinez, Keren Espinoza-Huertas, D. Véliz-Otani, Mario Velit-Salazar, V. Marca, O. Ortega, Iván Cornejo-Herrera, Saúl Lindo-Samanamud, Pamela Mora-Alférez, P. Mazzetti (2015)Clinical and Molecular Features of Late Onset Huntington Disease in a Peruvian Cohort.
Journal of Huntington's disease, 4 1
T. Bird, H. Lipe, E. Steinbart (2008)Geriatric neurogenetics: oxymoron or reality?
Archives of neurology, 65 4
G. Warren, P. Yellowlees (1990)The prevalence of Huntington's disease
Medical Journal of Australia, 153
H. Lipe, T. Bird (2009)Late onset Huntington Disease: Clinical and genetic characteristics of 34 cases
Journal of the Neurological Sciences, 276
N. Reynolds (2008)Autopsy‐proven Huntington's disease with 29 trinucleotide repeats
Movement Disorders, 23
Jae Keum, Aram Shin, T. Gillis, J. Mysore, Kawther Elneel, D. Lucente, Tiffany Hadzi, P. Holmans, L. Jones, M. Orth, S. Kwak, M. MacDonald, J. Gusella, Jong-Min Lee (2016)The HTT CAG-Expansion Mutation Determines Age at Death but Not Disease Duration in Huntington Disease.
American journal of human genetics, 98 2
A. Ha, J. Jankovic (2011)Exploring the Correlates of Intermediate CAG Repeats in Huntington Disease
Postgraduate Medicine, 123
D. Falush, E. Almqvist, R. Brinkmann, Y. Iwasa, M. Hayden (2001)Measurement of mutational flow implies both a high new-mutation rate for Huntington disease and substantial underascertainment of late-onset cases.
American journal of human genetics, 68 2
C. James, G. Houlihan, R. Snell, J. Cheadle, P. Harper (1994)Late-onset Huntington's disease: a clinical and molecular study.
Age and ageing, 23 6
I. Appollonio, G. Frisoni, N. Curtò, M. Trabucchi, L. Frattola (1997)Which Diagnostic Procedures in the Elderly? The Case of Late-Onset Huntington's Disease
Journal of Geriatric Psychiatry and Neurology, 10
T. Foroud, J. Gray, Julia Ivashina, P. Conneally (1999)Differences in duration of Huntington’s disease based on age at onset
Journal of Neurology, Neurosurgery & Psychiatry, 66
M. Oosterloo, M. Belzen, E. Bijlsma, R. Roos (2015)Is There Convincing Evidence that Intermediate Repeats in the HTT Gene Cause Huntington's Disease?
Journal of Huntington's disease, 4 2
P. Ruiz, E. Gómez-Tortosa, A. Barrio, J. Benítez, B. Morales, L. Vela, Ayoze Castro, I. Requena (1997)Senile chorea: a multicenter prospective study
Acta Neurologica Scandinavica, 95
(2003)Huntington’s disease Clinical correlates of disability and progression
Melody Caramins, G. Halliday, Elizabeth McCusker, Ronald Trent (2003)Genetically confirmed clinical Huntington’s disease with no observable cell loss
Journal of Neurology, Neurosurgery & Psychiatry, 74
S. Frank (2009)Tetrabenazine as anti-chorea therapy in Huntington Disease: an open-label continuation study. Huntington Study Group/TETRA-HD Investigators
BMC Neurology, 9
J. MacMillan, P. Davies, P. Harper (1995)Molecular diagnostic analysis for Huntington's disease: a prospective evaluation.
Journal of Neurology, Neurosurgery & Psychiatry, 58
R. Myers, §. Daniel, S. Sax, M. Schoenfeld, E. Bird, P. Wolf, II VONSATTEL, Roberta White, Joseph Martin, D. Myers (1985)Late onset of Huntington's disease.
Journal of Neurology, Neurosurgery & Psychiatry, 48
C. Ross, A. Pantelyat, J. Kogan, J. Brandt (2014)Determinants of functional disability in Huntington's disease: Role of cognitive and motor dysfunction
Movement Disorders, 29
(1993)The Huntington’s Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes
(1996)Diagnosis of patients presenting to a Huntington’s disease clinic without a family history of Huntington’s disease
P. Panegyres, J. Goh (2011)The neurology and natural history of patients with indeterminate CAG repeat length mutations of the Huntington disease gene
Journal of the Neurological Sciences, 301
E. Gómez-Tortosa, A. Barrio, P. Ruiz, R. Pernaute, J. Benítez, A. Barroso, F. Jiménez, J. Yébenes (1998)Severity of cognitive impairment in juvenile and late-onset Huntington disease.
Archives of neurology, 55 6
N. Hindley, G. Norbury, K. Jobst, E. Rosser, S. Huson, M. Pearce, Elizabeth King (1996)LATE ONSET HUNTINGTON'S DISEASE AS A CAUSE OF DEMENTIA: WHERE SHOULD THE CLINICIAN'S INDEX OF SUSPICION LIE?
International Journal of Geriatric Psychiatry, 11
M. Davis, D. Bateman, N. Quinn, C. Marsden, A. Harding (1994)Mutation analysis in patients with possible but apparently sporadic Huntington's disease
The Lancet, 344
Kunihiro Yoshida, S. Yanagawa, A. Tsuchiya, T. Nakajima, Y. Fukushima, S. Ikeda (2007)Huntington’s disease with onset ages greater than 60 years
Geriatrics & Gerontology International, 7
M. MacDonald, C. Ambrose, M. Duyao, R. Myers, Carol Lin, L. Srinidhi, G. Barnes, Sherryl Taylor, M. James, Nicolet Groot, Heather Macfarlane, B. Jenkins, M. Anderson, N. Wexler, J. Gusella, G. Bates, S. Baxendale, H. Hummerich, S. Kirby, M. North, S. Youngman, R. Mott, G. Zehetner, Z. Sedlacek, A. Poustka, A. Frischauf, H. Lehrach, A. Buckler, D. Church, L. Doucette-Stamm, M. O’Donovan, Laura Riba-Ramírez, Manish Shah, V. Stanton, S. Strobel, K. Draths, Jennifer Wales, P. Dervan, D. Housman, M. Altherr, R. Shiang, L. Thompson, T. Fielder, J. Wasmuth, D. Tagle, J. Valdes, Lon Elmer, M. Allard, L. Castilla, M. Swaroop, K. Blanchard, F. Collins, R. Snell, T. Holloway, Kathleen Gillespie, N. Datson, D. Shaw, P. Harper (1993)A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
Background: Although the typical age of onset for Huntington’s disease (HD) is in the fourth decade, between 4.4–11.5% of individuals with HD have a late onset (over 60 years of age). Diagnosis of Late onset HD (LoHD) can be missed, due to the perceived low likelihood of HD in the over 60-year-olds.Objective: To review the epidemiology, genotype and phenotype of LoHD.Methods: We systematically searched MEDLINE, EMBASE and Web of Science (inception-November 2016). Web of Science was then used to search for papers citing identified studies. Content experts were consulted for any additional studies. We included all studies reporting the clinical phenotype of LoHD for more than one participant.Results: 20 studies were identified from a potential list of 1243. Among Caucasian HD cohorts, 4.4–11.5% of individuals have LoHD, and this proportion may be increasing. Proportion of LoHD without a positive family history ranges from 3–68%. 94.4% of reported cases of LoHD had CAG repeat lengths of ≤44. Motor manifestations are the commonest initial presentation, although 29.2% presented with non-motor manifestations as the first clinical feature in one case series. Individuals with LoHD may have slower progression of illness. Cognitive impairment rather than chorea may be the major source of disability in this group.Conclusions: LoHD represents a substantial proportion of new diagnoses of HD and has some unique features. Further characterization of this population will aid clinicians in diagnosis.
Journal of Huntington's Disease – IOS Press
Published: Jan 1, 2017
Access the full text.
Sign up today, get DeepDyve free for 14 days.