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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Disorganized hebephrenic schizophrenia Paranoid schizophrenia Simple-type schizophrenia Childhood schizophrenia Pseudoneurotic chorer. Retrieved 5 May Bradley’s neurology in clinical practice 6th ed. Philosophical Transactions of the Royal Society of London.

Reported impairments range from short-term memory deficits to long-term memory difficulties, including deficits in episodic memory of one’s lifeprocedural memory of the body of how to perform an activity and working memory. Retrieved 8 September Milton Wexler was inspired to start the Hereditary Disease Foundation HDFwith the aim of curing genetic illnesses by coordinating and supporting research.

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. Research into the mechanism of HD has focused on identifying the functioning of HTT, how mHTT differs or interferes with it, and the brain pathology that the disease produces.


Retrieved 24 July Cerebral atrophy can be seen in the advanced stages of the disease. Top of the page – Article Outline.

Huntington’s disease

The remaining variation is attributed to environment and other genes that modify the mechanism of HD. Early symptoms are attributable to functions of the striatum and its cortical connections—namely control over movement, mood and higher cognitive function.

Radiology picture of the day. A physical examinationsometimes combined with a psychological examinationcan determine whether the onset of the disease has begun. American Journal of Human Genetics. A lower daily dose than those classicaly used in schizophrenia was sufficient to obtain efficient treatment.

Huntington’s disease – Wikipedia

Thus, the glutamines on CBP interact directly with the increased numbers of glutamine on the HTT chain and CBP gets pulled away from its typical location next to the nucleus. Retrieved 3 April The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in Cnoree, and not to disclose this data to third parties. Archived from the original on 8 Huntiington Journal of Neurology, Neurosurgery, and Psychiatry.

Archived from the original on 20 September There is consensus for testing only individuals who are considered cognitively mature, although there is a counter-argument that parents have a right to make the decision on their child’s behalf.


Rehabilitation during the late stage aims to compensate for motor and functional losses. List of Huntington’s disease media depictions.

Retrieved 19 July Adult personality and behavior Gender dysphoria Sexual maturation disorder Ego-dystonic sexual orientation Sexual relationship disorder Paraphilia Voyeurism Fetishism. Dissociative identity disorder Psychogenic amnesia Fugue state Depersonalization disorder.

The Journal of Clinical Investigation.

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. Modelling the disease in various types of animals, such as the transgenic mouse developed inenabled larger scale experiments. The rate of occurrence is highest in peoples of Western European descent, averaging around 7 perpeople, and is lower in the rest of the world; e.

Medical and Surgical Coree of Philadelphia.

Until the discovery of a genetic teststatistics could only include clinical diagnosis based on physical symptoms and a family history of HD, excluding those who died of other causes before diagnosis. This page was last edited on 29 Decemberat