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Case report: Pain in anti-DPPX encephalitis

Encephalitis due to antibodies targeting dipeptidyl-peptidase-like protein 6 (DPPX), a potassium channel subunit, is rare. The illness is typically characterized by a triad of weight loss, CNS hyperexcitability and cognitive symptoms, but recent reports suggest that the clinical picture may be more...

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Autores principales: Bjerknes, Tale L., Steihaug, Ole Martin, Haugen, Mette, Hjelland, Ina Elen, Vedeler, Christian Alexander
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9794743/
https://www.ncbi.nlm.nih.gov/pubmed/36588910
http://dx.doi.org/10.3389/fneur.2022.1091688
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author Bjerknes, Tale L.
Steihaug, Ole Martin
Haugen, Mette
Hjelland, Ina Elen
Vedeler, Christian Alexander
author_facet Bjerknes, Tale L.
Steihaug, Ole Martin
Haugen, Mette
Hjelland, Ina Elen
Vedeler, Christian Alexander
author_sort Bjerknes, Tale L.
collection PubMed
description Encephalitis due to antibodies targeting dipeptidyl-peptidase-like protein 6 (DPPX), a potassium channel subunit, is rare. The illness is typically characterized by a triad of weight loss, CNS hyperexcitability and cognitive symptoms, but recent reports suggest that the clinical picture may be more heterogeneous. Here, we describe the case of a 63-year-old female who was admitted to the hospital with severe extremity pain, which had been preceded by diarrhea and weight loss. She later developed cognitive changes, and her general condition rapidly deteriorated. Extensive workup did not reveal gastrointestinal illness or underlying malignancies. MRI of the brain was normal. Analyses of blood and cerebrospinal fluid showed normal cell counts but high titres of DPPX antibodies in blood and cerebrospinal fluid. The patient was treated with intravenous methylprednisolone followed by rituximab. At 1-year follow-up, she was without pain and had completely recovered. In this case, DPPX-associated autoimmune encephalitis was dominated by severe extremity pain, illustrating that sensory symptoms may be one of the main complaints in these patients. It is important for clinicians to be aware of the heterogeneous clinical picture in this serious condition, since correct diagnosis and treatment with immunosuppressants are associated with favorable prognosis.
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spelling pubmed-97947432022-12-29 Case report: Pain in anti-DPPX encephalitis Bjerknes, Tale L. Steihaug, Ole Martin Haugen, Mette Hjelland, Ina Elen Vedeler, Christian Alexander Front Neurol Neurology Encephalitis due to antibodies targeting dipeptidyl-peptidase-like protein 6 (DPPX), a potassium channel subunit, is rare. The illness is typically characterized by a triad of weight loss, CNS hyperexcitability and cognitive symptoms, but recent reports suggest that the clinical picture may be more heterogeneous. Here, we describe the case of a 63-year-old female who was admitted to the hospital with severe extremity pain, which had been preceded by diarrhea and weight loss. She later developed cognitive changes, and her general condition rapidly deteriorated. Extensive workup did not reveal gastrointestinal illness or underlying malignancies. MRI of the brain was normal. Analyses of blood and cerebrospinal fluid showed normal cell counts but high titres of DPPX antibodies in blood and cerebrospinal fluid. The patient was treated with intravenous methylprednisolone followed by rituximab. At 1-year follow-up, she was without pain and had completely recovered. In this case, DPPX-associated autoimmune encephalitis was dominated by severe extremity pain, illustrating that sensory symptoms may be one of the main complaints in these patients. It is important for clinicians to be aware of the heterogeneous clinical picture in this serious condition, since correct diagnosis and treatment with immunosuppressants are associated with favorable prognosis. Frontiers Media S.A. 2022-12-14 /pmc/articles/PMC9794743/ /pubmed/36588910 http://dx.doi.org/10.3389/fneur.2022.1091688 Text en Copyright © 2022 Bjerknes, Steihaug, Haugen, Hjelland and Vedeler. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Bjerknes, Tale L.
Steihaug, Ole Martin
Haugen, Mette
Hjelland, Ina Elen
Vedeler, Christian Alexander
Case report: Pain in anti-DPPX encephalitis
title Case report: Pain in anti-DPPX encephalitis
title_full Case report: Pain in anti-DPPX encephalitis
title_fullStr Case report: Pain in anti-DPPX encephalitis
title_full_unstemmed Case report: Pain in anti-DPPX encephalitis
title_short Case report: Pain in anti-DPPX encephalitis
title_sort case report: pain in anti-dppx encephalitis
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9794743/
https://www.ncbi.nlm.nih.gov/pubmed/36588910
http://dx.doi.org/10.3389/fneur.2022.1091688
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