Autoimmune encephalopathy (Q84126): Difference between revisions
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A encefalopatia autoimune é semelhante à encefalite autoimune [ver definição de encefalite autoimune], mas reflete um início mais subagudo a insidioso que tende a flutuar e frequentemente imita demências neurodegenerativas (doença de Creutzfeldt-Jakob ou doença de Lewy Body). Uma história pessoal ou familiar de autoimunidade é freqüentemente encontrada. As investigações geralmente mostram um líquido cefalorraquiano inflamatório, um eletroencefalograma epileptiforme e anormalidades de ressonância magnética nos lobos temporais mesiais. Podem ser encontrados autoanticorpos neurais (por exemplo, autoanticorpos complexos de canal de potássio controlados por voltagem) e não órgão-específicos (anticorpos de peroxidase da tireoide [TPO]). A exclusão de etiologias alternativas (por exemplo, infecções) é importante. A resposta à imunoterapia pode ser usada para confirmar o diagnóstico._x000D_ _x000D_ Autoanticorpos associados:_x000D_ _x000D_ DPPX (proteína semelhante a dipeptidil peptidase-6); GABAAR (autoanticorpos de ácido gama-aminobutírico tipo A); gAChR (auto-anticorpos ganglionares para receptores de acetilcolina (subunidade alfa-3)); GAD65 (anticorpos descarboxilase-65 do ácido glutâmico); VGKC (LGI1, CASPR2) (anticorpos do complexo do canal de potássio controlado por voltagem). | |||
| description / en | description / en | ||
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 ). | |||
Revision as of 09:29, 15 August 2026
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 ).
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1445382314 |
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| English | Autoimmune encephalopathy |
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 ). |
