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Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature

A 29-year-old man presented with a high-grade fever, headache, and urinary retention, in addition to meningeal irritation and myoclonus in his upper extremities. A cerebrospinal fluid (CSF) examination showed pleocytosis and high adenosine deaminase (ADA) levels with no evidence of bacterial infecti...

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Autores principales: Nakamura, Sota, Fujioka, Teppei, Kawashima, Shoji, Kawaguchi, Takatsune, Mizuno, Masayuki, Omura, Masahiro, Okita, Kenji, Kimura, Akio, Shimohata, Takayoshi, Matsukawa, Noriyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8502650/
https://www.ncbi.nlm.nih.gov/pubmed/33814491
http://dx.doi.org/10.2169/internalmedicine.6457-20
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author Nakamura, Sota
Fujioka, Teppei
Kawashima, Shoji
Kawaguchi, Takatsune
Mizuno, Masayuki
Omura, Masahiro
Okita, Kenji
Kimura, Akio
Shimohata, Takayoshi
Matsukawa, Noriyuki
author_facet Nakamura, Sota
Fujioka, Teppei
Kawashima, Shoji
Kawaguchi, Takatsune
Mizuno, Masayuki
Omura, Masahiro
Okita, Kenji
Kimura, Akio
Shimohata, Takayoshi
Matsukawa, Noriyuki
author_sort Nakamura, Sota
collection PubMed
description A 29-year-old man presented with a high-grade fever, headache, and urinary retention, in addition to meningeal irritation and myoclonus in his upper extremities. A cerebrospinal fluid (CSF) examination showed pleocytosis and high adenosine deaminase (ADA) levels with no evidence of bacterial infection, including Mycobacterium tuberculosis. T2-weighted brain magnetic resonance imaging showed transient hyper-intensity lesions at the splenium of the corpus callosum (SCC), bilateral putamen, and pons during the course of the disease. The CSF was positive for anti-glial fibrillary acidic protein (GFAP) antibodies. He was diagnosed with autoimmune GFAP astrocytopathy. The present case shows that the combination of an elevated ADA level in the CSF and reversible T2-weighted hyper-intensity on the SCC supports the diagnosis of autoimmune GFAP encephalopathy.
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spelling pubmed-85026502021-10-26 Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature Nakamura, Sota Fujioka, Teppei Kawashima, Shoji Kawaguchi, Takatsune Mizuno, Masayuki Omura, Masahiro Okita, Kenji Kimura, Akio Shimohata, Takayoshi Matsukawa, Noriyuki Intern Med Case Report A 29-year-old man presented with a high-grade fever, headache, and urinary retention, in addition to meningeal irritation and myoclonus in his upper extremities. A cerebrospinal fluid (CSF) examination showed pleocytosis and high adenosine deaminase (ADA) levels with no evidence of bacterial infection, including Mycobacterium tuberculosis. T2-weighted brain magnetic resonance imaging showed transient hyper-intensity lesions at the splenium of the corpus callosum (SCC), bilateral putamen, and pons during the course of the disease. The CSF was positive for anti-glial fibrillary acidic protein (GFAP) antibodies. He was diagnosed with autoimmune GFAP astrocytopathy. The present case shows that the combination of an elevated ADA level in the CSF and reversible T2-weighted hyper-intensity on the SCC supports the diagnosis of autoimmune GFAP encephalopathy. The Japanese Society of Internal Medicine 2021-04-05 2021-09-15 /pmc/articles/PMC8502650/ /pubmed/33814491 http://dx.doi.org/10.2169/internalmedicine.6457-20 Text en Copyright © 2021 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Nakamura, Sota
Fujioka, Teppei
Kawashima, Shoji
Kawaguchi, Takatsune
Mizuno, Masayuki
Omura, Masahiro
Okita, Kenji
Kimura, Akio
Shimohata, Takayoshi
Matsukawa, Noriyuki
Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature
title Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature
title_full Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature
title_fullStr Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature
title_full_unstemmed Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature
title_short Self-remitting Elevation of Adenosine Deaminase Levels in the Cerebrospinal Fluid with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report and Review of the Literature
title_sort self-remitting elevation of adenosine deaminase levels in the cerebrospinal fluid with autoimmune glial fibrillary acidic protein astrocytopathy: a case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8502650/
https://www.ncbi.nlm.nih.gov/pubmed/33814491
http://dx.doi.org/10.2169/internalmedicine.6457-20
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