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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2022
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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. |
format | Online Article Text |
id | pubmed-9794743 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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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