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La maladie de Huntington est une affection dégénérative du cerveau d’origine I -Présentation de la chorée de Huntington et description de ses troubles. La clozapine, antipsychotique atypique, semble efficace sur les symptômes psychotiques liés à la chorée de Huntington. Nous rapportons le cas d’une patiente. PDF | On, R. de Diego Balaguer and others published ASPECTS CLINIQUES ET NEUROPSYCHOLOGIQUES DE LA MALADIE DE HUNTINGTON.

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Retrieved 7 May Dissociative identity disorder Psychogenic amnesia Fugue state Depersonalization disorder. Although Huntington’s has been recognized as a disorder since at least the Middle Agesthe cause has been unknown until fairly recently.

Huntington’s disease

There was controversy when Charles Davenport proposed in that compulsory sterilization and immigration control be used for people with certain diseases, including HD, as part of the eugenics movement. Compounds that have failed to prevent or slow progression of Huntington’s disease in human trials include remacemidecoenzyme Q10riluzolecreatineminocyclineethyl-EPAphenylbutyrate and dimebon.

There are multiple cellular changes through which the toxic function of mHTT may manifest and produce the HD pathology. Although glutamine is not found in excessively high amounts, it has been postulated that because of the increased vulnerability, even normal amounts glutamine can cause excitotoxins to be expressed. International journal of palliative nursing.

Retrieved 12 March Stem cell therapy is the replacement of damaged neurons by transplantation of stem cells into affected regions of the brain.

Retrieved 18 December Brain Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic. Cognitive or behavioral symptoms are rarely the first symptoms diagnosed; they are usually only recognized in hindsight or when they develop further.

Seizures are also a common symptom of this form of HD. The first likely description of the disease was in by Charles Oscar Waters. Archived from the original on 27 July These implications include the impact on an individual’s psychology, career, family planning decisions, relatives and relationships. Disorders typically diagnosed in childhood.


Retrieved 14 April Genetic Disorders Sourcebook 3rd ed. Chorew modifications in Huntington’s disease”. Among the approaches aimed at improving cell survival in the presence of mutant huntingtin are correction of transcriptional regulation using histone deacetylase inhibitorsmodulating aggregation of huntingtin, improving metabolism and mitochondrial function and restoring function of synapses. American Journal of Insanity.

Huntington’s disease – Wikipedia

These advances have led to increasingly extensive research into the proteins involved with the disease, potential drug treatments, care methods, and the gene itself. Early symptoms are attributable to functions of the striatum and its cortical connections—namely control over movement, mood and higher cognitive function. Research is being chorre on many different approaches to prevent Huntington’s disease or slow its progression.

Some forms of preimplantation genetic diagnosis—non-disclosure or exclusion testing—allow at-risk people to have HD-free offspring without revealing their own parental genotype, giving no information about whether they themselves are destined to develop HD.

Journal of Neurology, Neurosurgery, and Psychiatry.

As the ability to synchronize movements deteriorates, difficulty clearing the lungs and an increased risk of aspirating food or drink both increase the risk of contracting pneumonia.

This probability is sex-independent.

Archived from the original on 26 November A lower daily dose than those classicaly used in schizophrenia was sufficient to obtain efficient treatment. A epidemiological study of the prevalence of Huntington’s disease in the UK between and found that the average prevalence for the UK was Caspasean enzyme which plays a role in catalyzing apoptosis, is thought to be activated by the mutated gene through damaging the ubiquitin-protease system. Regions of the brain have differing amounts and reliance on these types of neurons, and are affected accordingly.

This technique, where one or two cells are extracted from a typically 4- to 8-cell embryo and then tested for the genetic abnormality, can then be used to ensure embryos affected with HD genes are not implanted, and therefore any offspring will not inherit the disease. Adult personality and behavior. Archived from the original on 29 September Archived from the original on 16 June Journal of the History of the Neurosciences.


Sexual maturation disorder Ego-dystonic sexual orientation Sexual relationship disorder Paraphilia Voyeurism Fetishism. Inclusion bodies have been found in both the cell nucleus and cytoplasm. Seminars in Pediatric Neurology. Retrieved 17 March The second greatest risk is heart diseasewhich causes almost a quarter of fatalities of those with HD.

American Journal of Human Genetics.

It is also possible to obtain a prenatal diagnosis for an embryo or fetus in the womb, using fetal genetic material acquired through chorionic villus sampling.

These cases can now be included in statistics; and, as the test becomes more widely available, estimates of the prevalence and incidence chored the disorder are likely to dd. Experiments have yielded mixed results using this technique in animal models and preliminary human clinical trials. Association of British Insurers.

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Reported impairments range from short-term memory deficits to long-term memory difficulties, including deficits in episodic memory of one’s lifehuuntington memory of the body of how to perform an activity and working memory. Psychoactive substances, substance abuse and substance-related disorders. Archived from the original on 12 May This procedure looks at the amniotic fluid surrounding the baby for indicators of the HD mutation.