| ID |
http://id.who.int/icd/entity/1445382314 |
| Preferred Name |
Encéphalopathie auto-immune |
| Definitions |
Autoimmune encephalopathy is similar to autoimmune encephalitis [see autoimmune encephalitis definition] but reflects a more subacute to insidious onset which tends to fluctuate and often mimics neurodegenerative dementias (Creutzfeldt-Jakob disease or Lewy Body disease). A personal or family history of autoimmunity is often found. Investigations typically show an inflammatory spinal fluid, an epileptiform electroencephalogram and MRI abnormalities in the mesial temporal lobes. Accompanying neural autoantibodies (e.g. voltage gated potassium channel complex autoantibodies) and non-organ specific (thyroid peroxidase [TPO] antibodies) may be found. Exclusion of alternative etiologies (e.g. infections) is important. Response to immunotherapy may be used to confirm the diagnosis. Associated autoantibodies: DPPX (dipeptidyl-peptidase–like protein-6); GABAAR (Gamma-aminobutyric-acid type-A autoantibodies); gAChR (ganglionic acetylcholine receptor autoantibodies (alpha-3 subunit)); GAD65 (glutamic acid decarboxylase-65 antibodies); VGKC (LGI1, CASPR2) (voltage gated potassium channel complex antibodies ). |
| Type |
http://www.w3.org/2002/07/owl#Class |
| comment | Autoimmune encephalopathy is similar to autoimmune encephalitis [see autoimmune encephalitis definition] but reflects a more subacute to insidious onset which tends to fluctuate and often mimics neurodegenerative dementias (Creutzfeldt-Jakob disease or Lewy Body disease). A personal or family history of autoimmunity is often found. Investigations typically show an inflammatory spinal fluid, an epileptiform electroencephalogram and MRI abnormalities in the mesial temporal lobes. Accompanying neural autoantibodies (e.g. voltage gated potassium channel complex autoantibodies) and non-organ specific (thyroid peroxidase [TPO] antibodies) may be found. Exclusion of alternative etiologies (e.g. infections) is important. Response to immunotherapy may be used to confirm the diagnosis. Associated autoantibodies: DPPX (dipeptidyl-peptidase–like protein-6); GABAAR (Gamma-aminobutyric-acid type-A autoantibodies); gAChR (ganglionic acetylcholine receptor autoantibodies (alpha-3 subunit)); GAD65 (glutamic acid decarboxylase-65 antibodies); VGKC (LGI1, CASPR2) (voltage gated potassium channel complex antibodies ). αυτοάνοση εγκεφαλοπάθεια είναι παρόμοια με αυτοάνοση εγκεφαλίτιδα (βλ. αυτοάνοση εγκεφαλοπάθεια, αλλά αντανακλά μια πιο οξεία σε ύπουλη οίδημα που τείνει να κυμανθεί και συχνά μιics νευροεκφυλιστική άνοια (creutzfeldt-jakob ασθένεια ή ασθένεια του οργανισμού). μια προσωπική ή οικογενειακή ιστορία της αυτοανοσίας είναι σημαντικό. οι έρευνες εμφανίζουν συνήθως ένα φλεγμονώδες νωτιαίο υγρό, ένα επιγενετικό ηλεκτροεγκεφαλογράφημα και κάρη ανωμαλίες στην μεσοχρονική κροταφικές λοβούς. l'encéphalopathie auto-immune est semblable à l'encéphalite auto-immune (voir la définition de l'encéphalite auto-immune), mais reflète un début plus subaigu à insidieux qui tend à fluctuer et qui imite souvent des démences neurodégénératives (maladie de creutzfeldt-jakob ou maladie à corps de lewy). on trouve souvent des antécédents personnels ou familiaux d'auto-immunité. les enquêtes montrent généralement un liquide vertébral inflammatoire, un électroencéphalogramme éileptiforme et des anomalies de l'irm dans les lobes temporels du méso. on peut trouver des auto-anticorps neuronaux (p. ex., auto-anticorps du canal potassique bloqué par la tension) et des anticorps non organiques (anticorps de la peroxydase thyroïdienne [tpo]). l'exclusion d'autres étiologies (p. ex., infections) est importante. |
| broaderTransitive | |
| prefLabel | Encéphalopathie auto-immune |
| browserUrl | NA |
| label | Encéphalopathie auto-immune αυτοάνοση εγκεφαλοπάθεια encefalopatia autoimmunologiczna Autoimmune encephalopathy Autoimmunenzephalopathie |
| subClassOf |
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