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Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy

Hashimoto's encephalopathy is an encephalitis of presumed autoimmune origin characterized by the presence of autoantibodies against thyroid proteins. We present a case of a young patient with pre-existing Hashimoto's thyroiditis and progressive cognitive complaints, absence-like episodes,...

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Autores principales: Mané-Damas, Marina, Vinke, Anita, Hoffmann, Carolin, Zong, Shenghua, Losen, Mario, Molenaar, Peter C., Damoiseaux, Jan, Koudijs, Suzanne, Rouhl, Rob P. W., Martinez-Martinez, Pilar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7358532/
https://www.ncbi.nlm.nih.gov/pubmed/32733453
http://dx.doi.org/10.3389/fimmu.2020.01358
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author Mané-Damas, Marina
Vinke, Anita
Hoffmann, Carolin
Zong, Shenghua
Losen, Mario
Molenaar, Peter C.
Damoiseaux, Jan
Koudijs, Suzanne
Rouhl, Rob P. W.
Martinez-Martinez, Pilar
author_facet Mané-Damas, Marina
Vinke, Anita
Hoffmann, Carolin
Zong, Shenghua
Losen, Mario
Molenaar, Peter C.
Damoiseaux, Jan
Koudijs, Suzanne
Rouhl, Rob P. W.
Martinez-Martinez, Pilar
author_sort Mané-Damas, Marina
collection PubMed
description Hashimoto's encephalopathy is an encephalitis of presumed autoimmune origin characterized by the presence of autoantibodies against thyroid proteins. We present a case of a young patient with pre-existing Hashimoto's thyroiditis and progressive cognitive complaints, absence-like episodes, and sporadic bilateral epileptiform frontal and frontotemporal activity. No abnormalities were observed during the neurological examination and on MRI. Antibodies to thyroid peroxidase (TPO) were elevated and remained positive while the symptoms were present. Levothyroxine and methylprednisolone did not ameliorate the complaints. Subsequent treatment with high-dose intravenous immunoglobulins (IVIG) led to improved cognitive functions and to the disappearance of the absence-like-episodes. Patient's serum, but not CSF, gave a characteristic IgG-specific hippocampal pattern in rat brain immunohistochemistry; this immunoreactivity was maintained after specific and complete depletion of TPO antibodies. Serum IgG bound to primary neurons in cell culture, likely targeting a yet unidentified neuronal surface antigen. The clinical response to IVIG suggests but does not prove, that the circulating novel autoantibodies may induce the encephalopathy. It would be of interest to investigate more patients with Hashimoto's encephalopathy for the presence of neuronal surface autoantibodies, to define their role in the disease and their target antigen(s).
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spelling pubmed-73585322020-07-29 Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy Mané-Damas, Marina Vinke, Anita Hoffmann, Carolin Zong, Shenghua Losen, Mario Molenaar, Peter C. Damoiseaux, Jan Koudijs, Suzanne Rouhl, Rob P. W. Martinez-Martinez, Pilar Front Immunol Immunology Hashimoto's encephalopathy is an encephalitis of presumed autoimmune origin characterized by the presence of autoantibodies against thyroid proteins. We present a case of a young patient with pre-existing Hashimoto's thyroiditis and progressive cognitive complaints, absence-like episodes, and sporadic bilateral epileptiform frontal and frontotemporal activity. No abnormalities were observed during the neurological examination and on MRI. Antibodies to thyroid peroxidase (TPO) were elevated and remained positive while the symptoms were present. Levothyroxine and methylprednisolone did not ameliorate the complaints. Subsequent treatment with high-dose intravenous immunoglobulins (IVIG) led to improved cognitive functions and to the disappearance of the absence-like-episodes. Patient's serum, but not CSF, gave a characteristic IgG-specific hippocampal pattern in rat brain immunohistochemistry; this immunoreactivity was maintained after specific and complete depletion of TPO antibodies. Serum IgG bound to primary neurons in cell culture, likely targeting a yet unidentified neuronal surface antigen. The clinical response to IVIG suggests but does not prove, that the circulating novel autoantibodies may induce the encephalopathy. It would be of interest to investigate more patients with Hashimoto's encephalopathy for the presence of neuronal surface autoantibodies, to define their role in the disease and their target antigen(s). Frontiers Media S.A. 2020-07-07 /pmc/articles/PMC7358532/ /pubmed/32733453 http://dx.doi.org/10.3389/fimmu.2020.01358 Text en Copyright © 2020 Mané-Damas, Vinke, Hoffmann, Zong, Losen, Molenaar, Damoiseaux, Koudijs, Rouhl and Martinez-Martinez. http://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 Immunology
Mané-Damas, Marina
Vinke, Anita
Hoffmann, Carolin
Zong, Shenghua
Losen, Mario
Molenaar, Peter C.
Damoiseaux, Jan
Koudijs, Suzanne
Rouhl, Rob P. W.
Martinez-Martinez, Pilar
Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy
title Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy
title_full Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy
title_fullStr Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy
title_full_unstemmed Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy
title_short Unidentified Neuronal Surface IgG Autoantibodies in a Case of Hashimoto's Encephalopathy
title_sort unidentified neuronal surface igg autoantibodies in a case of hashimoto's encephalopathy
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7358532/
https://www.ncbi.nlm.nih.gov/pubmed/32733453
http://dx.doi.org/10.3389/fimmu.2020.01358
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