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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Schizophreniaschizotypal and delusional.

In some cases the onset may be so late huntungton symptoms are never noticed. Retrieved 19 July Retrieved 4 August Archived from the original PDF on 25 June These abnormalities include muscle atrophycardiac failureimpaired glucose toleranceweight lossosteoporosisand testicular atrophy. The largest humtington of Huntington’s disease research globally, in terms of financial expenditure, [] is the CHDI Foundationa US non-profit biomedical foundation that aims to “rapidly discover and develop drugs that delay or slow Huntington’s disease”.

Archived from the original on 29 September For some time HD was thought to be the only disease for which possession of a second mutated gene did not affect symptoms and progression, [28] but it has since been found that it can affect the phenotype and the rate hutnington progression.

Stem cell therapy is the replacement of damaged neurons by transplantation of stem cells into affected regions of the brain. Annual Review of Medicine.

This would require hcoree of the process to be kept secret from the parent. Archived from the original on 4 July Series B, Biological Sciences. Access to the text HTML.


Genetic Disorders Sourcebook 3rd ed. People with Huntington’s disease may see a physical therapist for non-invasive and non-medication-based ways of managing the physical symptoms. Archived from the original on 28 June In non-disclosure testing, only disease-free embryos are replaced in the uterus while the parental genotype and hence parental risk for HD are never disclosed. A epidemiological study of the prevalence of Huntington’s disease in the UK between and found that the average prevalence for the UK was Disorders typically diagnosed in childhood.

Disorganized hebephrenic schizophrenia Paranoid schizophrenia Simple-type schizophrenia Childhood schizophrenia Pseudoneurotic schizophrenia. Archived from the original on 31 May HD Society of Canada.

Access to the full text of this article requires a subscription. Degenerative SA Friedreich’s ataxia Ataxia-telangiectasia. Tetrabenazine was approved in for treatment of chorea in Huntington’s disease in the EU, and in in the US. 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. Archived from the original on 10 August The Journal of Neuropsychiatry and Clinical Neurosciences.

CCTG Myotonic dystrophy type 2. Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic. Archived from the original on 21 August Archived from the original on 15 December Retrieved 24 July Huntington’s Disease — Third Edition.

Cognitive or behavioral symptoms are rarely the first symptoms diagnosed; they are usually only recognized in hindsight or when they develop further.

Modelling the disease in various types of animals, such as the transgenic mouse developed inenabled larger scale experiments.

Huntington’s disease

A longer repeat results in an earlier age of onset and a faster progression of symptoms. Caspasean enzyme which plays a role in catalyzing apoptosis, is thought to be activated by the mutated gene through damaging the ubiquitin-protease system.


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. The highest concentrations are found in the brain and testeswith moderate amounts in the liverheartand lungs. Journal of Neurology, Neurosurgery, and Psychiatry.

Problems with mood, mental abilities, coordination, jerky body movements [1] [2]. Focal Generalised Status epilepticus Myoclonic epilepsy. Embryos produced using in vitro fertilization may be genetically tested for HD using preimplantation genetic diagnosis PGD.

An choere theory that explains another way cell function may be disrupted by HD proposes that damage to mitochondria in striatal cells is of central importance numerous accounts of mitochondrial metabolism deficiency have been found. A number of these collaborate juntington umbrella organizations, like the International Huntington Association and the European HD network.

Delirium Post-concussion syndrome Organic brain syndrome.

Huntington’s disease – Wikipedia

Journal of Bioenergetics and Biomembranes. The gene is also called HD and IT15 huntignton, which stands for ‘interesting transcript 15’.

Archived from the original on 18 February The huntingtin protein interacts with over other proteins, and appears to have multiple biological functions. Archived from the original on 22 October