IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literature
ObjectiveWe present the case of a patient with clinical and imaging features of sporadic Creutzfeldt–Jakob disease (sCJD) and positive IgLON5 antibodies (Abs) in the serum and CSF.Case reportA 66-year-old Chinese man presented to the hospital with a stroke-like episode, followed by rapidly progressi...
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| Format: | Article |
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Frontiers Media S.A.
2024-03-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2024.1367361/full |
| _version_ | 1797254461238804480 |
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| author | Xiaofeng Li Yimin Chen Le Zhang Wei Zhang Bin Li José Fidel Baizabal-Carvallo Xingwang Song |
| author_facet | Xiaofeng Li Yimin Chen Le Zhang Wei Zhang Bin Li José Fidel Baizabal-Carvallo Xingwang Song |
| author_sort | Xiaofeng Li |
| collection | DOAJ |
| description | ObjectiveWe present the case of a patient with clinical and imaging features of sporadic Creutzfeldt–Jakob disease (sCJD) and positive IgLON5 antibodies (Abs) in the serum and CSF.Case reportA 66-year-old Chinese man presented to the hospital with a stroke-like episode, followed by rapidly progressive cognitive decline, mutism, and parkinsonism. The MRI results showed a cortical ribboning sign in diffusion-weighted MRI, periodic triphasic waves with a slow background in EEG, and positive protein 14–3-3 in CSF. There were matching IgLON5 Abs in the serum and CSF. A literature review showed positive autoimmune encephalitis Abs or autoimmune inflammatory disease between 0.5 and 8.6% among patients with clinical suspicion of CJD, most commonly anti-voltage-gated potassium channel (VGKC) complex and anti-N-methyl-D-aspartate receptor (NMDAR) Abs; however, IgLON5 autoimmunity in CJD has been rarely reported. This is an intriguing association as both conditions have been associated with brain deposits of phosphorylated tau protein.ConclusionIgLON5 Abs may be observed in patients with a diagnosis of CJD; it is unknown whether a synergistic effect of IgLON5 Abs with CJD exists, increasing neurodegenerative changes. |
| first_indexed | 2024-04-24T21:50:19Z |
| format | Article |
| id | doaj.art-9cd5da0732974134b777363cd050038a |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-24T21:50:19Z |
| publishDate | 2024-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-9cd5da0732974134b777363cd050038a2024-03-20T14:21:08ZengFrontiers Media S.A.Frontiers in Neurology1664-22952024-03-011510.3389/fneur.2024.13673611367361IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literatureXiaofeng Li0Yimin Chen1Le Zhang2Wei Zhang3Bin Li4José Fidel Baizabal-Carvallo5Xingwang Song6Department of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, ChinaDepartment of Neurology, Foshan Sanshui District People’s Hospital, Foshan, Guangdong, ChinaDepartment of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, ChinaDepartment of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, ChinaDepartment of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, ChinaDepartment of Sciences and Engineering, University of Guanajuato, León, MexicoDepartment of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, ChinaObjectiveWe present the case of a patient with clinical and imaging features of sporadic Creutzfeldt–Jakob disease (sCJD) and positive IgLON5 antibodies (Abs) in the serum and CSF.Case reportA 66-year-old Chinese man presented to the hospital with a stroke-like episode, followed by rapidly progressive cognitive decline, mutism, and parkinsonism. The MRI results showed a cortical ribboning sign in diffusion-weighted MRI, periodic triphasic waves with a slow background in EEG, and positive protein 14–3-3 in CSF. There were matching IgLON5 Abs in the serum and CSF. A literature review showed positive autoimmune encephalitis Abs or autoimmune inflammatory disease between 0.5 and 8.6% among patients with clinical suspicion of CJD, most commonly anti-voltage-gated potassium channel (VGKC) complex and anti-N-methyl-D-aspartate receptor (NMDAR) Abs; however, IgLON5 autoimmunity in CJD has been rarely reported. This is an intriguing association as both conditions have been associated with brain deposits of phosphorylated tau protein.ConclusionIgLON5 Abs may be observed in patients with a diagnosis of CJD; it is unknown whether a synergistic effect of IgLON5 Abs with CJD exists, increasing neurodegenerative changes.https://www.frontiersin.org/articles/10.3389/fneur.2024.1367361/fullCreutzfeldt–Jakob diseaseIgLON5 antibodiesautoimmune encephalitisneurodegenerative diseaserapid progressive dementia |
| spellingShingle | Xiaofeng Li Yimin Chen Le Zhang Wei Zhang Bin Li José Fidel Baizabal-Carvallo Xingwang Song IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literature Frontiers in Neurology Creutzfeldt–Jakob disease IgLON5 antibodies autoimmune encephalitis neurodegenerative disease rapid progressive dementia |
| title | IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literature |
| title_full | IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literature |
| title_fullStr | IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literature |
| title_full_unstemmed | IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literature |
| title_short | IgLON5 autoimmunity in a patient with Creutzfeldt–Jakob disease: case report and review of literature |
| title_sort | iglon5 autoimmunity in a patient with creutzfeldt jakob disease case report and review of literature |
| topic | Creutzfeldt–Jakob disease IgLON5 antibodies autoimmune encephalitis neurodegenerative disease rapid progressive dementia |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2024.1367361/full |
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