Monozygotic twins suffering from Huntington's disease show different cognitive and behavioural symptoms

European Neurology

Volumn 57, Issue 1, 2007, Pages 26-30

  Gomez Esteban J C ^a,b   Lezcano, E ^a   Zarranz, J J ^a   Velasco, F ^a   Garamendi, Iñigo ^a   Pérez, Tomás ^a   Tijero, Beatriz ^a  

^a UNIVERSITY OF THE BASQUE COUNTRY UPV EHU   (Spain)

^b HOSPITAL DE CRUCES   (Spain)

Author keywords

Huntington's disease; Huntington's disease, cognition, behaviour; Twins, homozygotic

Indexed keywords

ADULT; ARTICLE; CASE REPORT; CLINICAL FEATURE; COGNITION; DISEASE EXACERBATION; EVALUATION; HUMAN; HUNTINGTON CHOREA; LOCOMOTION; MALE; MOLECULAR EVOLUTION; MONOZYGOTIC TWINS; PHENOTYPE; PRIORITY JOURNAL; TRINUCLEOTIDE REPEAT;

ADULT; ANXIETY; BEHAVIORAL SYMPTOMS; BRAIN; CHOREA; COGNITION DISORDERS; HUMANS; HUNTINGTON DISEASE; MALE; MOTOR ACTIVITY; NEUROPROTECTIVE AGENTS; NEUROPSYCHOLOGICAL TESTS; PARANOID DISORDERS; PEDIGREE; POLYMERASE CHAIN REACTION; PSYCHOMOTOR PERFORMANCE; RANDOMIZED CONTROLLED TRIALS; RILUZOLE; TRINUCLEOTIDE REPEAT EXPANSION;

EID: 33846409843     PISSN: 00143022     EISSN: None     Source Type: Journal    
DOI: 10.1159/000097006     Document Type: Article

References (19)

1. Quinn N, Schrag A: Huntington's disease and other choreas. J Neurol 1998;245:709-716.
2. Hogarth P: Huntington's disease: a decade beyond gene discovery. Curr Neurol Neurosci Rep 2003;3:279-284.
3. The Huntington's Disease Collaborative Research Group: A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993;72:971-983.
4. MacDonald ME, Gusella JF: Huntington's disease: translating a CAG repeat into a pathogenic mechanism. Curr Opin Neurobiol 1996;6:638-643.
5. Ross CA: Huntington's disease: new paths to pathogenesis. Cell 2004;118:4-7.
6. Illarioshkin SN, Igarashi S, Onodera O, Markova ED, Nikolskaya NN, Tanaka H, Chabrashwili TZ, Insarova NG, Endo K, Ivanova-Smolenskaya IA, et al: Trinucleotide repeat length and rate of progression of Huntington's disease. Ann Neurol 1994;36:630-635.
7. Rubinsztein DC, Leggo J, Chiano M, Dodge A, Norbury G, Rosser E, Craufurd D: Genotypes at the GluR6 kainate receptor locus are associated with variation in the age of onset of Huntington disease. Proc Natl Acad Sci USA 1997;94:3872-3876.
8. Warner JP, Barron LH, Brock DJ: A new polymerase chain reaction (PCR) assay for the trinucleotide repeat that is unstable and expanded on Huntington's disease chromosomes. Mol Cell Probes 1993;7:235-239.
9. EHID/EHDI: RIL.DE1.201 Trial. Huntington Disease World Congress, Manchester, 2005.
10. Duyao M, Ambrose C, Myers R, Novelletto A, Persichetti F, Frontali M, Folstein S, Ross C, Franz M, Abbott M, et al: Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet 1993;4:387-392.
11. Van Vugt JP, van Hilten BJ, Roos RA: Hypokinesia in Huntington's disease. Mov Disord 1996;11:384-388.
12. Anca MH, Gazit E, Loewenthal R, Ostrovsky O, Frydman M, Giladi N: Different phenotypic expression in monozygotic twins with Huntington disease. Am J Med Genet 2004;124A:89-91.
13. Georgiou N, Bradshaw JL, Chiu E, Tudor A, O'Gorman L, Phillips JG: Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease. Mov Disord 1999;14:320-325.
14. Kieburtz K, MacDonald M, Shih C, Feigin A, Steinberg K, Bordwell K, Zimmerman C, Srinidhi J, Sotack J, Gusella J, et al: Trinucleotide repeat length and progression of illness in Huntington's disease. J Med Genet 1994;31:872-874.
15. Van Dellen A, Hannan AJ: Genetic and environmental factors in the pathogenesis of Huntington's disease. Neurogenetics 2004;5:9-17.
16. Sudarsky L, Myers RH, Walshe TM: Huntington's disease in monozygotic twins reared apart. J Med Genet 1983;20:408-411.
17. Bird TD, Omenn GS: Monozygotic twins with Huntington's disease in a family expressing the rigid variant. Neurology 1975;25:1126-1129.
18. Huntington Study Group: Unified Huntington's Disease Rating Scale: reliability and consistency. Mov Disord 1996;11:136-142.
19. Mayeux R, Stern Y, Herman A, Greenbaum L, Fahn S: Correlates of early disability in Huntington's disease. Ann Neurol 1986;20:727-731.