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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Excitotoxins may cause damage to numerous cellular structures.

Huntington’s disease

Insomnia Hypersomnia Sleep apnea Obstructive Congenital central hypoventilation syndrome Narcolepsy Cataplexy Kleine—Levin Circadian rhythm sleep disorder Advanced sleep phase disorder Delayed sleep ee disorder Nonhour sleep—wake disorder Jet lag.

Migraine Familial hemiplegic Cluster Tension. Histone modifications in Huntington’s disease”. Disorders typically diagnosed in childhood Intellectual disability X-linked intellectual disability Lujan—Fryns syndrome. Brain herniation Reye’s Hepatic encephalopathy Toxic encephalopathy Hashimoto’s encephalopathy.

The huntingtin protein interacts with over other proteins, and appears to have multiple biological functions.

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Huntington’s disease, particularly the application of the genetic test for the disease, has raised several ethical issues. Archived from the original on 27 July The issues for genetic testing include defining how mature an individual should be before being considered eligible for testing, ensuring the confidentiality of results, and whether companies should be allowed to use test results for decisions on employment, life insurance or other financial matters.


Archived from the original on 26 November Retrieved 14 April Seizures are also a common symptom of this form of HD. Disorders typically diagnosed in childhood. Outline Masquer le plan.

Gene silencing aims to reduce the production of the mutant protein, since HD is caused by a single dominant gene encoding a toxic protein. The clinical improvement was associated with plasma levels of clozapine which have been recognized as fhoree therapeutic ranges by previous studies.

Huntington’s disease – Wikipedia

Part of this gene is a repeated section called a trinucleotide repeatwhich varies in length between individuals and may change length between generations.

To initiate a particular movement, the cerebral cortex sends a signal to the basal ganglia that causes the inhibition to be released. Hunitngton CAG repeats over 28 are unstable during replicationand this instability increases with the number of repeats present.

The most characteristic initial physical symptoms are jerky, random, and uncontrollable movements called chorea. Some forms of preimplantation genetic diagnosis—non-disclosure or exclusion testing—allow at-risk huntinngton to have HD-free offspring without revealing their own parental genotype, giving no information about whether they themselves are destined to develop HD.

When either or both the parents have shown manifestations of the disease Generally, people have fewer than 36 repeated glutamines in the polyQ region which results in production of the cytoplasmic protein Huntingtin.


Personality disorder Impulse control disorder Kleptomania Trichotillomania Pyromania Dermatillomania Factitious disorder Munchausen syndrome.

Retrieved 1 April Journal page Archives Contents list.

Cerebral atrophy can be seen in huntintton advanced stages of the disease. Archived PDF from the original on 4 March The search for the cause of this condition was enhanced considerably inwhen the Hereditary Disease Foundation HDF was created by Milton Wexlerre psychoanalyst based in Los AngelesCaliforniawhose wife Leonore Sabin had been diagnosed earlier that year with Huntington’s disease. Degenerative SA Friedreich’s ataxia Ataxia-telangiectasia.

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. Neurology, Psychiatry and Brain Research 8: CCTG Myotonic dystrophy type 2.

Archived from the original on 18 February Archived from the original on 19 November Delirium Post-concussion syndrome Organic brain syndrome. The gene is also called HD and IT15hhuntington stands for ‘interesting transcript 15’. Brain Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic.

Encephalomyelitis Acute disseminated Myalgic Meningoencephalitis.

The Huntington’s disease mutation is genetically dominant and almost fully penetrant: Retrieved 12 May Radiology picture of the day.