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Cerebellar presence of immune cells in patients with neuro-coeliac disease

Although various neurodegenerative disorders have been associated with coeliac disease (CD), the underlying neuropathological link between these brain and gut diseases remains unclear. We postulated that the neuronal damage sporadically observed in CD patients is immune-mediated. Our aim was to dete...

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Autores principales: Rouvroye, Maxine D., Bontkes, Hetty J., Bol, John G. J. M., Lissenberg-Witte, Birgit, Byrnes, Valerie, Bennani, Fadel, Jordanova, Ekaterina S., Wilhelmus, Micha M. M., Mulder, Chris J., van der Valk, Paul, Rozemuller, Annemieke J. M., Bouma, Gerd, Van Dam, Anne-Marie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10040112/
https://www.ncbi.nlm.nih.gov/pubmed/36966322
http://dx.doi.org/10.1186/s40478-023-01538-5
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author Rouvroye, Maxine D.
Bontkes, Hetty J.
Bol, John G. J. M.
Lissenberg-Witte, Birgit
Byrnes, Valerie
Bennani, Fadel
Jordanova, Ekaterina S.
Wilhelmus, Micha M. M.
Mulder, Chris J.
van der Valk, Paul
Rozemuller, Annemieke J. M.
Bouma, Gerd
Van Dam, Anne-Marie
author_facet Rouvroye, Maxine D.
Bontkes, Hetty J.
Bol, John G. J. M.
Lissenberg-Witte, Birgit
Byrnes, Valerie
Bennani, Fadel
Jordanova, Ekaterina S.
Wilhelmus, Micha M. M.
Mulder, Chris J.
van der Valk, Paul
Rozemuller, Annemieke J. M.
Bouma, Gerd
Van Dam, Anne-Marie
author_sort Rouvroye, Maxine D.
collection PubMed
description Although various neurodegenerative disorders have been associated with coeliac disease (CD), the underlying neuropathological link between these brain and gut diseases remains unclear. We postulated that the neuronal damage sporadically observed in CD patients is immune-mediated. Our aim was to determine if the loss of neurons, especially Purkinje cells, coincides with microglia activation and T- and B-cell infiltration in the cerebellum of patients with CD and a concomitant idiopathic neurological disease affecting the cerebellum (NeuroCD). Post-mortem cerebellar tissue was collected of validated NeuroCD cases. Gender- and age-matched genetic spinocerebellar ataxia (SCA) controls and non-neurological controls (NNC) were selected based on clinical reports and pathological findings. Cerebellar tissue of seventeen patients was included (6 NeuroCD, 5 SCA, 6 NNC). In SCA cases we found that the Purkinje cell layer was 58.6% reduced in comparison with NNC. In NeuroCD cases this reduction was even more prominent with a median reduction of 81.3% compared to NNC. Marked increased numbers of both CD3+ and CD8+ cells were observed in the NeuroCD but not in SCA patients. This coincided with significantly more microglial reactivity in NeuroCD patients. These findings demonstrate that the massive loss of Purkinje cells in the cerebellum of neuro CD patients is accompanied by local innate and T-cell mediated immune responses. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s40478-023-01538-5.
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spelling pubmed-100401122023-03-27 Cerebellar presence of immune cells in patients with neuro-coeliac disease Rouvroye, Maxine D. Bontkes, Hetty J. Bol, John G. J. M. Lissenberg-Witte, Birgit Byrnes, Valerie Bennani, Fadel Jordanova, Ekaterina S. Wilhelmus, Micha M. M. Mulder, Chris J. van der Valk, Paul Rozemuller, Annemieke J. M. Bouma, Gerd Van Dam, Anne-Marie Acta Neuropathol Commun Research Although various neurodegenerative disorders have been associated with coeliac disease (CD), the underlying neuropathological link between these brain and gut diseases remains unclear. We postulated that the neuronal damage sporadically observed in CD patients is immune-mediated. Our aim was to determine if the loss of neurons, especially Purkinje cells, coincides with microglia activation and T- and B-cell infiltration in the cerebellum of patients with CD and a concomitant idiopathic neurological disease affecting the cerebellum (NeuroCD). Post-mortem cerebellar tissue was collected of validated NeuroCD cases. Gender- and age-matched genetic spinocerebellar ataxia (SCA) controls and non-neurological controls (NNC) were selected based on clinical reports and pathological findings. Cerebellar tissue of seventeen patients was included (6 NeuroCD, 5 SCA, 6 NNC). In SCA cases we found that the Purkinje cell layer was 58.6% reduced in comparison with NNC. In NeuroCD cases this reduction was even more prominent with a median reduction of 81.3% compared to NNC. Marked increased numbers of both CD3+ and CD8+ cells were observed in the NeuroCD but not in SCA patients. This coincided with significantly more microglial reactivity in NeuroCD patients. These findings demonstrate that the massive loss of Purkinje cells in the cerebellum of neuro CD patients is accompanied by local innate and T-cell mediated immune responses. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s40478-023-01538-5. BioMed Central 2023-03-25 /pmc/articles/PMC10040112/ /pubmed/36966322 http://dx.doi.org/10.1186/s40478-023-01538-5 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research
Rouvroye, Maxine D.
Bontkes, Hetty J.
Bol, John G. J. M.
Lissenberg-Witte, Birgit
Byrnes, Valerie
Bennani, Fadel
Jordanova, Ekaterina S.
Wilhelmus, Micha M. M.
Mulder, Chris J.
van der Valk, Paul
Rozemuller, Annemieke J. M.
Bouma, Gerd
Van Dam, Anne-Marie
Cerebellar presence of immune cells in patients with neuro-coeliac disease
title Cerebellar presence of immune cells in patients with neuro-coeliac disease
title_full Cerebellar presence of immune cells in patients with neuro-coeliac disease
title_fullStr Cerebellar presence of immune cells in patients with neuro-coeliac disease
title_full_unstemmed Cerebellar presence of immune cells in patients with neuro-coeliac disease
title_short Cerebellar presence of immune cells in patients with neuro-coeliac disease
title_sort cerebellar presence of immune cells in patients with neuro-coeliac disease
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10040112/
https://www.ncbi.nlm.nih.gov/pubmed/36966322
http://dx.doi.org/10.1186/s40478-023-01538-5
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