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

Huntington’s disease HDalso known as Huntington’s choreais an inherited disorder that results in death of brain cells. Rehabilitation during the late stage aims to compensate for motor and functional losses.

Archived from the original on 26 June Sydenham’s choreabenign hereditary chorealupusparaneoplastic syndromeWilson’s disease [6]. Retrieved 18 April Archived from the original on 21 October Damage to the basal ganglia can cause the release or reinstatement of the inhibitions to be erratic and uncontrolled, 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.

Huntington’s disease – Wikipedia

Retrieved 12 May Functional neuroimaging techniques, such as functional magnetic resonance imaging fMRI and positron emission tomography PETcan show changes in brain activity before the onset of physical symptoms, but they are experimental tools, and are not used clinically. Modelling the disease in various types of animals, such as the transgenic mouse developed inenabled larger scale experiments.


Caspasean enzyme which plays a role in catalyzing apoptosis, is thought to be activated by the mutated gene through damaging the ubiquitin-protease system. Nervous and Mental Disease. The interactions of the altered huntingtin protein with numerous proteins in neurons leads to an increased vulnerability of glutamine, which, in large amounts, has been found to be an excitotoxin.

The accumulating damage to this area causes the characteristic erratic movements associated with HD. Individuals with both genes affected are rare. There are also autosomal recessive disorders that resemble sporadic cases of HD.

Milton Wexler was inspired to start the Hereditary Disease Foundation HDFwith the aim of chlree genetic illnesses by coordinating and supporting research. American Journal of Insanity.

Retrieved 7 May How far the disease has progressed can be measured using the unified Huntington’s disease rating scalewhich provides an overall rating system huntkngton on motor, behavioral, cognitive, and functional assessments. Archived from the original on 20 September Journal page Archives Contents list. When either or both the parents have shown manifestations of the disease 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.

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.

Because of the basal ganglia’s inability to inhibit movements, individuals affected by it will inevitably experience a reduced ability to produce speech and swallow foods and liquids dysphagia.


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Archived from the original on 19 November Some HD research has ethical issues due to its use of animal testing and embryonic stem cells. Retrieved 15 November There are ethical concerns related to prenatal genetic testing or preimplantation genetic diagnosis to ensure a child is not born with a given disease. One X-linked disorder of this type is McLeod syndrome.

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Genetic counseling is available to provide advice and guidance throughout the huntingto procedure, and on the implications of a confirmed diagnosis. Archived from the original on 16 June Retrieved 24 July Reported neuropsychiatric manifestations are anxietydepressiona reduced display of emotions blunted affectegocentrismaggressionand compulsive huntinvtonthe latter of which can cause or worsen addictionsincluding alcoholismgamblingand hypersexuality.

The basal ganglia—the part of the brain most prominently affected in early HD—play a key role in movement and behavior control. Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic.

It is not inherited according to sex, but the length of the repeated huntingto of the gene and hence its severity can be influenced by the sex of the affected parent.