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==Epidemiology==
==Epidemiology==


There seem to be an increased prevalence of Huntington's disease among white people as compared to Africans and Asians. European populations exhibit a comparatively high prevalence with 4-8 per 100,000 individuals suffering from HD.<ref><pubmed>1535611</pubmed></ref>  
There seem to be an increased prevalence of Huntington's disease among white people as compared to Africans and Asians. European populations exhibit a comparatively high prevalence with 4-8 per 100,000 individuals suffering from HD.<ref><pubmed>1535611</pubmed></ref> Two of the most well-known populations in which high prevalence of HD was notably in the state of Zulia, Venezuela <ref><pubmed>2139171</pubmed></ref> and Northern Ireland with a prevalence of 6.4 individuals per 100,000.<ref><pubmed>7562964</pubmed></ref> A fairly recent study in Slovenia showed that the prevalence of HD in the country was estimated to be 5.16 individuals per 100,000.<ref>Peterlin B, Kobal J, Teran N, Flisar D, Lovrecić L.'''Epidemiology of Huntington’s disease in Slovenia.''' Acta Neurol Scand.: 2009 PMID:18976322 [http://www.ncbi.nlm.nih.gov/pubmed/18976322]</ref> The overall prevalence of HD in Mexico was also expected to be comparable or even higher to that of European populations.<ref><pubmed>19672992</pubmed></ref> An epidemiologic study in Taiwan showed that the prevalence was 0.42 individuals per 100,000 in 2007.<ref><pubmed>20881427</pubmed></ref>  
 
This is significantly lower than that of European populations mentioned above.  
Two of the most well-known populations in which high prevalence of HD was notably in the state of Zulia, Venezuela <ref><pubmed>2139171</pubmed></ref> and Northern Ireland with a prevalence of 6.4 individuals per 100,000.<ref><pubmed>7562964</pubmed></ref> A fairly recent study in Slovenia showed that the prevalence of HD in the country was estimated to be 5.16 individuals per 100,000.<ref>Peterlin B, Kobal J, Teran N, Flisar D, Lovrecić L.'''Epidemiology of Huntington’s disease in Slovenia.''' Acta Neurol Scand.: 2009 PMID:18976322 [http://www.ncbi.nlm.nih.gov/pubmed/18976322]</ref> The overall prevalence of HD in Mexico was also expected to be comparable or even higher to that of European populations.<ref><pubmed>19672992</pubmed></ref> An epidemiologic study in Taiwan showed that the prevalence was 0.42 individuals per 100,000 in 2007.<ref><pubmed>20881427</pubmed></ref> This is significantly lower than that of European populations mentioned above.  





Revision as of 10:27, 5 September 2011

History

Huntington's disease has existed since at least the seventeenth century and several physicians provided earlier descriptions of hereditary chorea but without much detail. In 1872, Huntington’s disease was first documented with great details by George Huntington in “On Chorea”.(link) Huntington’s disease was initially known as chorea, derived from the Greek word “khoreia” which means dancing in unison. (link: http://www.memidex.com/choreography+notational-system#etymology). George Huntington described the disease as “an heirloom from generations away back in the dim past” as he realized that HD was hereditary. This conclusion was reached when he observed that if one of the parents had the disease, the offspring will inevitably have the disease too. In his paper, “On Chorea”, he described:

"Of its hereditary nature. When either or both the parents have shown manifestations of the disease ..., one or more of the offspring almost invariably suffer from the disease ... But if by any chance these children go through life without it, the thread is broken and the grandchildren and great-grandchildren of the original shakers may rest assured that they are free from the disease.".[1]

Huntington thus was able to explain the precise pattern of inheritance of autosomal dominant disease years before the rediscovery by scientists of Mendelian inheritance.

Epidemiology

There seem to be an increased prevalence of Huntington's disease among white people as compared to Africans and Asians. European populations exhibit a comparatively high prevalence with 4-8 per 100,000 individuals suffering from HD.[2] Two of the most well-known populations in which high prevalence of HD was notably in the state of Zulia, Venezuela [3] and Northern Ireland with a prevalence of 6.4 individuals per 100,000.[4] A fairly recent study in Slovenia showed that the prevalence of HD in the country was estimated to be 5.16 individuals per 100,000.[5] The overall prevalence of HD in Mexico was also expected to be comparable or even higher to that of European populations.[6] An epidemiologic study in Taiwan showed that the prevalence was 0.42 individuals per 100,000 in 2007.[7] This is significantly lower than that of European populations mentioned above.


The difference in prevalence of HD between European and East Asian populations is due to HTT haplotypes. It was hypothesised that different HD haplotypes have different mutation rates. This is supported by the findings that higher risk A1 and A2 HD halotypes composed the majority of HD chromosomes in Europe whereas it is absent in China and Japan.[8]


For those areas where there are intermarriages with white people, there is a higher occurrence of the disease. This is related to the higher frequency of huntingtin alleles with 28–35 CAG repeats in white individuals and the fact the disease is autosomal dominant. [9]

Treatment

There is no cure for Huntington's disease. Similar to AIDS, only the symptoms can be treated to slow down the progression of the disease.

Medications

  • Movement disorders
  • Psychiatric disorders

Therapies

  • Psychotherapy
  • Speech Therapy
  • Physical Therapy
  • Occupational Therapy

Quite recently, there are a couple of breakthroughs for the treatment of Huntington's.

  • Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. [10]
  • Using adult neurotrophic factor-secreting stem cells. [11]

<a href="http://www.disabled-world.com/health/neurology/breakthrough-cure.php">Huntington's Disease Breakthrough May Provide Cure</a>

References

  1. <pubmed>11232352</pubmed>
  2. <pubmed>1535611</pubmed>
  3. <pubmed>2139171</pubmed>
  4. <pubmed>7562964</pubmed>
  5. Peterlin B, Kobal J, Teran N, Flisar D, Lovrecić L.Epidemiology of Huntington’s disease in Slovenia. Acta Neurol Scand.: 2009 PMID:18976322 [1]
  6. <pubmed>19672992</pubmed>
  7. <pubmed>20881427</pubmed>
  8. <pubmed>21248742</pubmed>
  9. DC Rubinsztein, Molecular biology of Huntington's disease (HD) and HD-like disorders. In: S Pulst, Editor, Genetics of movement disorders, Academic Press, California (2003), pp. 365–377.
  10. <pubmed>19361997</pubmed>
  11. <pubmed>19603590</pubmed>