Huntington’s Disease

Authors: doc. Mudr Jan Roth, CSc.
Authors‘ workplace: Univerzita Karlova, Neurologická klinika 1. LF UK a VFN Praha
Published in: Cesk Slov Neurol N 2010; 73/106(2): 107-123
Category: Minimonography


Huntington’s disease (HD) is an autosomal dominantly inherited neuropsychiatric degenerative condition with a fatal prognosis. It is caused by mutation – the expansion of CAG (containing cytosine-adenine-guanine) triplet repeats 40 and more at the short arm of 4th chromosome. The main clinical features are motor impairment (especially choreatic dyskinesias and the impairment of voluntary movements), progressive cognitive deterioration and personality changes. The product of the mutation is an aberrant protein known as huntingtin with an enlarged polyglutamine stretch. The prevalence of HD is approximately 1 : 10–15,000. The typical onset of HD is in the 4th decade with a minority of cases starting in childhood or adolescence (juvenile HD) or in patients over 65 years of age (late-onset of HD). The initial symptoms are usually non-specific: behavioural changes, personality and affective disorders. As HD progresses, cognitive disturbance appears, leading to severe dementia. Typical motor symptoms are choreatic and/or dystonic dyskinesias, the impairment of voluntary movements, gait disorder, dysfagia and dysarthria. The progression of HD leads inevitably to a marantic, cachectic state and loss of the ability to make voluntary movements, to ingest food, control of urination and defecation, etc. Therapeutic interventions can only target symptoms. The degree of choreatic dyskinesias may be alleviated by antipsychotics, risperidone or tiapride for preference, or by GABAergics (clonazepam) or depletors of dopamine (tetrabenazine). Marked improvement of dystonia may occur with amantadine. In juvenile forms of HD with akinetic syndrome it is possible cautiously to use L-DOPA or amantadine. Irritability and agresivity respond to antipsychotics, sertraline or valproate. Psychotic symptoms may be treated in accord with routine psychiatric protocoles. Adequate (hypercaloric) food intake is extremely important. Sipping or even percutaneous gastrotomy is often indicated in the advanced stages of HD. A multidisciplinary approach (neurology, genetics, psychiatry, speech therapy, physiotherapy and ergotherapy, dietetics, social support etc.) in HD is essential. Genetic testing confirms the clinical diagnosis. Adult and healthy people at risk may be able to request predictive genetical testing, but specific protocolary procedure is realized. Children and adolescents are not tested. Prenatal testing is also available. Pre-implantation genetic diagnosis allows to give the birth of a healthy child without knowing of the genetical state of whichever parent at HD risk.

Key words:
Huntington’s disease – CAG triplet – genetic testing – chorea – dementia


1. Huntington G. On chorea. Med Surg Rep 1872; 26: 317–321.

2. Harper PS. Huntington’s disease: a historical back­ground. In: Bates G, Harper PS, Jones L (eds). Huntington’s Disease. 3rd ed. Oxford: Oxford University Press 2002: 3–27.

3. Harper PS. The epidemiology of Huntington’s disease. Hum Genet 1992; 89(4): 365–376.

4. Kremer B, Goldberg P, Andrew SE, Theilmann J, Telenius H, Zeisler Jutta et al. A worldwide study of the Huntington’s disease mutation. N Engl J Med 1994; 330(20): 1401–1406.

5. Al‑Jader LN, Harper PS, Krawczak M, Palmer SR. The frequency of inherited disorders database: prevalence of Huntington disease. Community Genet 2001; 4(3): 148–157.

6. Walker DA, Harper PS, Wells CEC, Tyler A, Davies K, Newcombe RG. Huntington’s chorea in South Wales: a genetic and epidemiological study. Clin Genet 1981; 19(4): 213–221.

7. Quarrell OW, Tyler A, Jones MP, Nordin M, Harper PS. Population studies of Huntington disease in Wales. Clin Genet 1988; 33(3): 189–195.

8. Folstein SE, Chase GA, Wahl WE, McDonnell AM, Folstein MF. Huntington’s disease in Maryland: clinical aspects of racial variation. Am J Hum Genet 1987; 41(2): 168–179.

9. Penney JB jr, Young AB, Shoulson I, Starosta‑Rubenstein S, Snodgrass SR, Sanchez‑Ramos J et al. Huntington’s disease in Venezuela: 7 years of follow‑up on symptomatic and asymptomatic individuals. Mov Disord 1990; 5(2): 93–99.

10. Perry TL, Hansen S, Kloster M. Huntington’s chorea. Deficiency of gamma‑aminobutyric acid in brain. New Engl J Med 1973; 288(7): 337–342.

11. Spokes EG. Neurochemical alterations in Huntington’s chorea: a study of post‑mortem brain tissue. Brain 1980; 103(1): 179–210.

12. Vonsattel JP, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP jr. Neuropathological classification of Huntington’s disease. J Neuropathol Exp Neurol 1985; 44(6): 559–577.

13. Roth J, Klempir J, Jech R, Zidovská J, Uhrová T, Doubek P et al. Caudate nucleus atrophy in Huntington’s disease and its relationship with clinical and genetic parameters. Funct Neurol 2005; 20(3): 127–130.

14. Aylward EH, Codori AM, Rosenblatt A, Sherr M, Brandt J, Stine OC et al. Rate of caudate atrophy in presymptomatic and symptomatic stages of Huntington’s disease. Mov Disord 2000; 15(3): 552–560.

15. Jech R, Klempír J, Vymazal J, Zidovská J, Klempírová O, Růzicka E et al. Variation of selective gray and white matter atrophy in Huntington’s disease. Mov Disord 2007; 22(2): 1783–1789.

16. de la Monte SM, Vonsattel JP, Richardson EP jr. Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington’s disease. J Neuropathol Exp Neurol 1988; 47(5): 516–525.

17. Myers RH, Vonsattel JP, Stevens TJ, Cupples LA, Richardson EP, Martin JB et al. Clinical and neuropathologic assessment of severity in Huntington’s disease. Neurology 1988; 38(3): 341–347.

18. Rosas HD, Salat DH, Lee SY, Zaleta AK, Pappu V, Fischl B et al. Cerebral cortex and the clinical expression of Huntington’s disease: complexity and heterogeneity. Brain 2008; 131(4): 1057–1068.

19. The Huntington’s Disease Colaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell 1993; 72(6): 971–983.

20. DiFiglia M, Sapp E, Chase K, Schwarz C, Meloni A,Young C et al. Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 1995; 14(5): 1075–1081.

21. Sapp E, Schwarz C, Chase K, Bhide PG, Young AB, Penney J et al. Huntingtin localization in brains of normal and Huntington’s disease patients. Ann Neurol 1997; 42(4): 604–612.

22. Borrell‑Pagès M, Zala D, Humbert S, Saudou F. Huntington’s disease: from huntingtin function and dysfunction to therapeutic strategies. Cell Mol Life Sci 2006; 63(22): 2642–2660.

23. Zuccato C, Cattaneo E. Brain‑derived neurotrophic factor in neurodegenerative diseases. Nat Rev Neurol 2009; 5(6): 311–322.

24. Zeitlin S, Liu JP, Chapman DL, Papaioannou VE, Efstratiadis A. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington’s disease gene homologue. Nat Genet 1995; 11(2): 155–163.

25. Harjes P, Wanker EE. The hunt for huntingtin function: interaction partners tell many different stories. Trends Biochem Sci 2003; 28(8): 425–433.

26. Roth J. Neurodegenerace: jeden mechanismus pro mnoho chorob? Lékařské listy 2009; 7: 17–18.

27. Gil JM, Rego AC. Mechanisms of neurodegeneration in Huntington’s disease. Eur J Neurosci 2008; 27(11): 2803–2820.

28. Petrucelli L, Dawson TM. Mechanism of neurodegenerative disease: role of the ubiquitin proteasome system. Ann Med 2004; 36(4): 315–320.

29. Lehman NL. The ubiquitin proteasome system in neuropathology. Acta Neuropathol 2009; 118(3): 329–347.

30. Ravikumar B, Duden R, Rubinsztein DC. Aggregate‑prone proteins with polyglutamine and polyalanine expansions are dehraded by autophagy. Hum Mol Genet 2002; 11(9): 1107–1117.

31. Saudou F, Finkbeiner S, Devys D, Greenberg ME. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 1998; 95(1): 55–66.

32. Andrew SE, Goldberg YP, Kremer B, Telenius H, Theilmann J, Adam S et al. The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington’s disease. Nat Genet 1993; 4(4): 398–403.

33. Snell RG, MacMillan JC, Cheadle JP, Fenton I, Lazarou LP, Davies P et al. Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington’s disease. Nat Genet 1993; 4(4): 393–397.

34. Rubinsztein DC, Leggo J, Coles R, Almquist E, Biancalana V, Cassiman JJ et al. Phenotypic characterization of individuals with 30–40 CAG repeats in the Huntington’s disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36–39 repeats. Am J Hum Genet 1996; 59(1): 16–22.

35. Bates GP, Benn C. The polyglutamine diseases. In: Huntington’s Disease. Bates G, Harper PS, Jones L (eds). Oxford: Oxford University Press 2002: 430–472.

36. Semaka A, Collins JA, Hayden MR. Unstable familial transmissions of Huntington disease alleles with 27–35 CAG repeats (intermediate alleles). Am J Med Genet B Neuropsychiatr Genet 2010; 153B(1): 314–320.

37. Kovtun IV, Welch G, Guthrie HD, Hafner KL, McMurray CT. CAG repeat lengths in X‑ and Y‑bearing sperm indicate that gender bias during transmission of Huntington’s disease gene is determined in the embryo. J Biol Chem 2004; 279(10): 9389–9391.

38. Telenius H, Kremer B, Goldberg YP, Theilmann J,Andrew SE, Zeisler J et al. Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm. Nat Genet 1994; 6(4): 409–414.

39. Leeflang EP, Zhang L, Tavaré S, Hubert R, Srinidhi J, MacDonald ME et al. Single sperm analysis of the trinucleotide repeats in the Huntington’s disease gene: quantification of the mutation frequency spectrum. Hum Mol Genet 1995; 4(9): 1519–1526.

40. MacMillan JC, Snell RG, Tyler A, Houlihan GD, Fenton I, Cheadle JP et al. Molecular analysis and clinical correlations of the Huntington’sdisease mutation. Lancet 1993; 342(8877): 954–958.

41. Duyao M, Ambrose C, Myers R, Novelletto A, Persichetti F, Frontali M et al. Trinucleotide repeat length instability and age of onset in Huntington’s disease. Nat Genet 1993; 4(4): 387–392.

42. Wexler NS, Lorimer J, Porter J, Gomez F, Moskowitz C, Shackell E et al. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington’sdisease age of onset. Proc Natl Acad Sci U S A 2004; 101(10): 3498–3503.

43. Metzger S, Bauer P, Tomiuk J, Laccone F, Didonato S, Gellera C et al. Genetic analysis of candidate genes modifying the age‑at‑onset in Huntington’s disease. Hum Genet 2006; 120(2): 285–292.

44. Wexler NS, Young AB, Tanzi RE, Travers H, Starosta‑Rubinstein S, Penney JB et al. Homozygotes for Huntington’s disease. Nature 1987; 326(6109): 194–197.

45. Squitieri F, Gellera C, Cannella M, Mariotti C, Cislaghi G, Rubinsztein DC et al. Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course. Brain 2003; 126(4): 946–955.

46. Trottier Y, Biancalana V, Mandel JL. Instability of CAG repeats in Huntington’sdisease: relation to parental transmission and age of onset. J Med Genet 1994; 31(5): 377–382.

47. Kremer B, Almqvist E, Theilmann J, Spence N, Telenius H, Goldberg YP et al. Sex‑dependent mechanisms for expansions and contractions of the CAG repeat on affected Huntington disease chromosomes. Am J Hum Genet 1995; 57(2): 343–350.

48. Wheeler VC, Persichetti F, McNeil SM, Mysore JS, Mysore SS, MacDonald ME et al. Factors associated with HD CAG repeat instability in Huntington disease. J Med Genet 2007; 44(11): 695–701.

49. Gonitel R, Moffitt H, Sathasivam K, Woodman B, Detloff PJ, Faull RL et al. DNA instability in postmitotic neurons. Proc Natl Acad Sci U S A 2008; 105(9): 3467–3472.

50. Ishiguro H, Yamada K, Sawada H, Nishii K, Ichino N,Sawada M et al. Age‑dependent and tissue‑specific CAG repeat instability occurs in mouse knock‑in for a mutant Huntington’s disease gene. J Neurosci Res 2001; 65(4): 289–297.

51. Shelbourne PF, Keller‑McGandy C, Bi WL, Yoon SR, Dubeau L, Veitch NJ et al. Triplet repeat mutation length gains correlate with cell‑type specific vulnerability in Huntington disease brain. Hum Mol Genet 2007; 16(10): 1133–1142.

52. Alexander GE, DeLong MR, Strick PL. Parallel organization of functionally segregated circuits linking basal ganglia and cortex. Annu Rev Neurosci 1986; 9: 357–381.

53. Flaherty AW, Graybiel AM. Anatomy of the basal ganglia. In: Marsden CD, Fahn S (eds). Movement Disorders 3. Oxford: Butterworth‑Heinemann 1994: 3–27.

54. Albin RL, Young AB, Penney JB. The functional anatomy of basal ganglia. Trends Neurosci 1989; 12(10): 366–375.

55. Alexander GE, Crutcher MD. Functional architecture of basal ganglia circuits: neuronal substrates of parallel processing. Trend Neurosci 1990; 13(7): 266–271.

56. Cummings JL. Frontal‑subcortical circuits and human behavior. Arch Neurol 1993; 50(8): 873–880.

57. Bonelli RM, Cummings JL. Frontal‑subcortical dementias. Neurologist 2008; 14(2): 100–107.

58. Tekin S, Cummings JL. Frontal‑subcortical neuronal circuits and clinical neuropsychiatry: an update. J Psychosom Res 2002; 53(2): 647–654.

59. Graybiel AM. Neurotransmitters and neuromodulators in the basal ganglia. Trends Neurosci 1990; 13(7): 244–254.

60. Tibben A. Predictive testing for Huntington’s disease. Brain Res Bull 2007; 72(2–3): 165–171.

61. International Huntington Association (IHA) and the World Federation of Neurology (WFN) Research Group on Huntington’s Chorea. Guidelines for the molecular genetics predictive test in Huntington’s disease. Neurology 1994; 44(8): 1533–1536.

62. Timman R, Roos R, Maat‑Kievit A, Tibben A. Adverse effects of predictive testing for Huntington disease underestimated: long‑term effects 7–10 years after the test. Health Psychol 2004; 23(2): 189–197.

63. Langbehn DR, Hayden MR, Paulsen JS; PREDICT‑HD Investigators of the Huntington Study Group. CAG‑repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches. Am J Med Genet B Neuropsychiatr Genet 2010; 153B(2): 397–408.

64. Židovská J, Růžičková Š, Vojtíšková M, Havrdová E, Roth J, Kapras J. DNA analýza genu Huntingtonovy choroby v české populaci. Psychiatrie 1998; 2: 70–73.

65. Židovská J, Klempíř J, Kebrdlová V, Uhrová T, Koblihová J, Anders M et al. Huntingtonova nemoc: zkušenosti s genetickým testováním v letech 1994–2005. Cesk Slov Neurol N 2007; 70/103(1): 72–77.

66. Wild EJ, Tabrizi SJ. Huntington’s disease phenocopy syndromes. Curr Opin Neurol 2007; 20(6): 681–687.

67. Kucharík M, Klempíř J, Roth J. Huntingtonské fenokopie dospělého věku. Neurologie pro praxi 2009; 10(3): 180–183.

68. Tsuji S. Dentatorubral‑pallidoluysian atrophy: clinical features and molecular genetics. Adv Neurol 1999; 79: 399–409.

69. Burke JR, Ikeuchi T, Koide R, Tsuji S, Yamada M, Pericak‑Vance MA et al. Dentatorubral‑pallidoluysian atrophy and Haw River Syndrome. Lancet 1994; 344(8938): 1711–1712.

70. Mariotti C, Alpini D, Fancellu R, Soliveri P, Grisoli M, Ravaglia S et al. Spinocerebellar ataxia type 17 (SCA17): oculomotor phenotype and clinical characterization of 15 Italian patients. J Neurol 2007; 254(11): 1538–1546.

71. Schneider SA, van de Warrenburg BP, Hughes TD, Davis M, Sweeney M, Wood N, Quinn NP et al. Phenotypic homogeneity of the Huntington disease‑like presentation in a SCA17 family. Neurology 2006; 67(9): 1701–1703.

72. Rubio­ JP, Danek A, Stone C, Chalmers R, Wood N, Verellen C et al. Chorea‑acanthocytosis: genetic linkage to chromosome 9q21. Am J Hum Genet 1997; 61(4): 899–908.

73. Klempíř J, Mikulenková D, Písačka M, Klempířová O.Diferenciální diagnostika neuroakantocytóz. Cesk Slov Neurol N 2009; 72/105(1): 24–29.

74. Danek A, Rubio­ JP, Rampoldi L, Ho M, Dobson‑Stone C, Tison F et al. McLeod neuroacanthocytosis: genotype and phenotype. Ann Neurol 2001; 50(6): 755–764.

75. Curtis AR, Fey C, Morris CM, Bindoff LA, Ince PG, Chinnery PF et al. Mutation in the gene encoding ferritin light polypeptide causes dominant adult‑onset basal ganglia disease. Nat Genet 2001; 28(4): 350–354.

76. Crompton DE, Chinnery PF, Fey C, Curtis AR, Morris CM, Kierstan J et al. Neuroferritinopathy: a window on the role of iron in neurodegeneration. Blood Cells Mol Dis 2002; 29(3): 522–531.

77. Filla A, De Michele G, Coppola G, Federico A, Vita G,Toscano A et al. Accuracy of clinical diagnostic criteria for Friedreich’s ataxia. Mov Disord 2000; 15(6): 1255–1258.

78. Hanna MG, Davis MB, Sweeney MG, Noursadeghi M,Ellis CJ, Elliot P et al. Generalized chorea in two patients harboring the Friedreich’s ataxia gene trinucleotide repeat expansion. Mov Disord 1998; 13(2): 339–340.

79. Bhidayasiri R, Perlman SL, Pulst SM, Geschwind DH. Late‑onset Friedreich ataxia: phenotypic analysis, magnetic resonance imaging findings, and review of the literature. Arch Neurol 2005; 62(12): 1865–1869.

80. Lawrence AD, Sahakian BJ, Hodges JR, Rosser AE, Lange KW, Robbins TW. Executive and mnemonic functions in early Huntington’s disease. Brain 1996; 119(5): 1633–1645.

80. Kirkwood SC, Siemers E, Hodes ME, Conneally PM, Christian JC, Foroud T. Subtle c1anges among presymptomatic carriers of the Huntington’s disease gene. J Neurol Neurosurg Psychiatry 2000; 69(6): 773–779.

82. Sprengelmeyer R. Young AW, Calder AJ, Karnat A,Lange H, Hömberg V et al. Loss of disgust. Perception of faces and emotions in Huntington’s disease. Brain 1996; 119(5): 1647–1665.

83. Gray JM, Young AW, Barker WA, Curtis A, Gibson D.Impaired recognition of disgust in Huntington’s disease gene carriers. Brain 1997; 120(11): 2029–2038.

84. Johnson SA, Stout JC, Solomon AC, Langbehn DR, Aylward EH, Cruce CB et al. Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington’s disease. Brain 2007; 130(7): 1732–1744.

85. Julien CL, Thompson JC, Wild S, Yardumian P, Snowden JS, Turner G et al. Psychiatric disorders in preclinical Huntington’s disease. J Neurol Neurosurg Psychiatry 2007; 78(9): 939–943.

86. Marshall J, White K, Weaver M, Flury Wetherill L, Hui S, Stout JC et al. Specific psychiatric manifestations among preclinical Huntington disease mutation carriers. Arch Neurol 2007; 64(1): 116–121.

87. Duff K, Paulsen JS, Beglinger LJ, Langbehn DR, Stout JC; Predict‑HD Investigators of the Huntington Study Group. Psychiatric symptoms in Huntington’s disease before diagnosis: the predict‑HD study. Biol Psychiatry 2007; 62(12): 1341–1346.

88. Zimbelman JL, Paulsen JS, Mikos A, Reynolds NC, Hoffmann RG, Rao SM. fMRI detection of early neural dysfunction in preclinical Huntington’sdisease. J Int Neuropsychol Soc 2007; 13(5): 758–769.

89. Roth J, Židovská J. Chorea. In: Růžička E, Roth J,Kaňovský P (eds). Extrapyramidová onemocnění II. Dyskinetické syndromy. Praha: Galén 2002: 71–99.

90. Di Maio L, Squitieri F, Napolitano G, Campanella G,Trofatter JA, Conneally PM. Suicide risk in Huntington’s disease. J Med Genet 1993; 30(4): 293–295.

91. Craufurd D, Thompson JC, Snowden JS. Behavioral changes in Huntington’s disease. Neuropsychiatry Neuropsychol Behav Neurol 2001; 14(4): 219–226.

92. Thompson JC, Snowden J, Craufurd D, Neary D. Behavior in Huntington’s disease: dissociating cognition‑based and mood‑based changes. J Neuropsychiatry Clin Neurosci 2002; 14(1): 37–44.

93. Paulsen JS, Ready RE, Hamilton JM, Mega MS, Cummings JL. Neuropsychiatric aspects of Huntington’s disease. J Neurol Neurosurg Psychiatry 2001; 71(3): 310–314.

94. Mendez MF. Huntington’s disease: update and review of neuropsychiatric aspects. Int J Psychiatry Med 1994; 24(3): 189–208.

95. Farrer LA. Suicide and attempted suicide in Huntington’s disease: implications for preclinical testing of persons at risk. Am J Med Genet 1986; 24(2): 305–311.

96. Marder K, Zhao H, Myers RH, Cudkowicz M, Kayson E, Kieburtz K et al. Rate of functional decline in Huntington’s disease. Huntington Study Group. Neurology 2000; 54(2): 452–458.

97. Beglinger LJ, Langbehn DR, Duff K, Stierman L, Black DW, Nehl C et al. Probability of obsessive and compulsive symptoms in Huntington’s disease. Biol Psychiatry 2007; 61(3): 415–418.

98. Jensen P, Fenger K, Bolwig T, Sørensen SA. Crime in Huntington’s disease: a study of registered offences among patients, relatives, and controls. J Neurol Neurosurg Psychiatry 1998; 65(4): 467–471.

99. King M. Alcohol abuse in Huntington’s disease. Psychol Med 1985; 15(4): 815–819.

100. Verny C, Allain P, Prudean A, Malinge MC, Gohier B, Scherer C et al. Cognitive changes in asymptomatic carriers of the Huntington disease mutation gene. Eur J Neurol 2007; 14(12): 1344–1350.

101. Folstein SE, Brandt J, Folstein MF. Huntington’s disease. In: Cummings JL (ed). Subcortical dementia. New York: Oxford University Press 1990: 87–107.

102. Roth J, Klempíř J, Špačková N. Neuropsychologie Huntingtonovy nemoci. In: Preiss M, Kučerová H(eds). Klinická neuropsychologie. Praha: Grada Publish­ing 2006: 271–302.

103. Klempír J, Klempírová 0, Stochl J, Spacková N, Roth J. Relationship between impairment of voluntary movements and cognitive impairment in Huntington’s disease. J Neurol 2009, 256(10): 1629–1633.

104. Ribaï P, Nguyen K, Hahn‑Barma V, Gourfinkel‑An I, Vidailhet M, Legout A et al. Psychiatric and cognitive difficulties as indicators of juvenile huntington disease onset in 29 patients. Arch Neurol 2007; 64(6): 813–819.

105. Bonelli RM, Wenning GK. Pharmacological management of Huntington’s disease: an evidence‑based review. Curr Pharm 2006; 12(21): 2701–2720.

106. Roth J, Klempíř J, Uhrová T. Současné terapeutické možnosti u Huntingtonovy nemoci. Psychiatrie pro praxi 2009; 10(5): 205–208.

107. Bilney B, Morris ME, Perry A. Effectiveness of physiotherapy, occupational therapy, and speech pathology for people with Huntington’s disease: a systematic review. Neurorehabil Neural Repair 2003; 17(1): 12–24.

108. Zinzi P, Salmaso D, De Grandis R, Graziani G, Maceroni S, Bentivoglio A et al. Effects of an intensive rehabilitation programme on patients with Huntington’s disease: a pilot study. Clin Rehabil 2007; 21(7): 603–613.

109. Cubo E, Shannon KM, Tracy D, Jaglin JA, Bernard BA, Wuu J et al. Effect of donepezil on motor and cognitive function in Huntington disease. Neurology 2006; 67(7): 1268–1271.

110. Moskowitz CB, Marder K. Palliative care for people with late‑stage Huntington’s disease. Neurol Clin 2001; 19(4): 849–865.

111. Ross CA, Shoulson I. Huntington disease: pathogenesis, bio­markers, and approaches to experimental therapeutics. Parkinsonism Relat Disord 2009; 15 (Suppl 3): S135–S138.

112. Wright BL, Barker RA. Established and emerging therapies for Huntington’s disease. Curr Mol Med 2007; 7(6): 579–587.

113. Dunnett SB, Rosser AE. Cell transplantation for Huntington’s disease. Should we continue? Brain Res Bull 2007; 72(2–3): 132–147.

114. Roth J, Uhrová T, Doubek P, Preiss M, Židovská J,Havrdová E et al. The impact of Huntington’s disease on caregivers. The Czech Experience. Revista Neuro‑psiquiatría 2002; 65(3–4): 168–173.

Paediatric neurology Neurosurgery Neurology
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account