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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Archived from the original on 18 February Caspasean enzyme which plays a role in catalyzing apoptosis, is thought to be activated by the mutated gene through damaging the ubiquitin-protease system. The second greatest risk is heart diseasewhich causes almost a quarter of fatalities of those with HD. Retrieved 12 March Delirium Post-concussion syndrome Organic brain syndrome.

Journal of Huntington’s Disease. The rate of occurrence is highest in peoples of Western European descent, huntingtln around 7 perpeople, and is lower in the rest of the world; e. The discovery that mHTT fragments misfold led to the discovery of the nuclear inclusions they cause.

Damage uuntington the basal ganglia can cause the release or reinstatement of the inhibitions to be erratic and choee, which results in an awkward start to motion or motions to be unintentionally initiated, or a motion to be halted before, or beyond, its intended completion.

HTT is expressed in all cells.

Huntington’s disease – Wikipedia

Association of British Insurers. Thanks to the HDF, the ongoing US-Venezuela Huntington’s Disease Collaborative Research Project was started inand reported a major breakthrough in with the discovery of the approximate location of a causal gene. Trends in Biochemical Sciences. The Huntington’s disease mutation is genetically dominant and almost fully penetrant: Neurology, Psychiatry and Brain Research 8: Huntington’s and the Making of a Genetic Disease.


Archived PDF from the original on 4 March HD Society of Canada. The condition was formerly called ‘Huntington’s chorea’ but this term has been replaced by ‘Huntington’s disease’ because not all patients develop chorea and due to the importance of cognitive and behavioral problems.

Chlree 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. As per the Law relating to information storage and huntingtoh integrity, you have the right to oppose art 26 of that lawaccess art 34 of that law and rectify art 36 of that law your personal data. Also covered is information concerning family planning choices, care management, and other considerations.

Adult personality and behavior Gender dysphoria Sexual maturation disorder Ego-dystonic sexual orientation Sexual relationship disorder Paraphilia Voyeurism Fetishism. By using this site, you agree to the Terms of Use and Privacy Policy.

Contact Help Who are we? Brain herniation Reye’s Hepatic encephalopathy Toxic encephalopathy Hashimoto’s encephalopathy. Their functions are not fully understood, but current theories propose that they are part of the cognitive executive system [17] and the motor circuit.

It involved over 18, people—mostly from a single extended family. Retrieved 7 May HTT also facilitates vesicular transport and synaptic transmission and controls neuronal gene transcription. There are ethical concerns related to prenatal genetic testing or preimplantation genetic diagnosis to ensure a child is not born with a given disease.

Top of the page – Article Outline. Although Huntington’s has been recognized as a disorder since at least the Middle Agesthe cause has been unknown until fairly recently. Personality disorder Impulse control disorder Kleptomania Trichotillomania Pyromania Dermatillomania Factitious disorder Munchausen syndrome.

Huntington’s disease

In exclusion testing, the embryos’ DNA is compared with that of the parents and grandparents to avoid inheritance of the chromosomal region containing the HD gene from the affected grandparent. European Journal of Human Genetics. It is unclear to what extent suicidal thoughts are influenced by behavioral symptoms, as they signify sufferers’ desires to avoid the later stages of the disease. Archived from the original on 20 September Mutant Huntingtin protein has been found to play a key role in mitochondrial dysfunction.


Huntington’s disease, particularly the application of the genetic test for the disease, has raised several ethical issues. Archived from the original on 21 October HD is one of several trinucleotide repeat disorders which are caused by the length of a repeated section of a gene exceeding a normal range. Retrieved 18 April Over time, the aggregates accumulate to form inclusion bodies within cells, ultimately interfering with neuron function. An Interpretative Phenomenological Analysis”. These cases can now be included in statistics; and, as the test becomes more widely available, estimates of the prevalence and incidence of the disorder are likely to increase.

Schizoaffective disorder Schizophreniform disorder Brief reactive psychosis. Weight loss and eating difficulties due to dysphagia and other muscle discoordination are common, making nutrition management increasingly important as the disease advances. Archived from the original on 8 October Since then, support and research organizations have formed in many countries around the world and have helped to increase public awareness of HD. Testing before the onset of symptoms is a life-changing event and a very personal decision.

Annual Review of Medicine.

Focal Generalised Status epilepticus Myoclonic epilepsy. International Review of Huntinggon. Psychiatric symptoms can be treated with medications similar to those used in the general population.