Article ; Online: New knowledge on anti-IgLON5 disease.
2024 Volume 37, Issue 3, Page(s) 316–321
Abstract: Purpose of review: Anti-IgLON5 disease is characterized by a distinctive sleep disorder, associated with a heterogeneous spectrum of neurological symptoms. Initial autopsies showed a novel neuronal tauopathy predominantly located in the tegmentum of the ...
Abstract | Purpose of review: Anti-IgLON5 disease is characterized by a distinctive sleep disorder, associated with a heterogeneous spectrum of neurological symptoms. Initial autopsies showed a novel neuronal tauopathy predominantly located in the tegmentum of the brainstem. Recently, new diagnostic red flags, biomarkers predictors of response to immunotherapy, and novel insights into the autoimmune pathogenesis of the disease have been reported. Recent findings: Patients with diagnosis of neurodegenerative dementia, progressive supranuclear palsy (PSP) or with motor-neuron disease (MND)-like syndrome have been reported to have IgLON5 antibodies, which are the hallmark of anti-IgLON5 disease. Second, low levels of neurofilament light chain in serum and cerebrospinal fluid of patients at disease onset could be a predictor of immunotherapy response. Recent neuropathological studies indicate that the neuronal tau deposits occur late in the course of the disease. Moreover, IgLON5 antibodies induce cytoskeletal changes in cultured hippocampal neurons suggesting that the tauopathy could be secondary of the IgLON5 antibody effects. Summary: Anti-IgLON5 disease can mimic and should be considered in atypical presentations of MND, neurodegenerative dementia and PSP. Neurofilament light chain levels seem promising biomarker for disease prognosis. Finally, the neuropathological and in vitro experimental studies strengthen the autoimmune hypothesis of the disease. |
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MeSH term(s) | Humans ; Cell Adhesion Molecules, Neuronal/immunology ; Cell Adhesion Molecules, Neuronal/metabolism ; Autoantibodies/immunology ; Supranuclear Palsy, Progressive/immunology ; Supranuclear Palsy, Progressive/diagnosis ; Animals ; Neurofilament Proteins/immunology ; Biomarkers/cerebrospinal fluid ; Biomarkers/metabolism |
Chemical Substances | IgLON5 protein, human ; Cell Adhesion Molecules, Neuronal ; Autoantibodies ; Neurofilament Proteins ; Biomarkers |
Language | English |
Publishing date | 2024-04-01 |
Publishing country | England |
Document type | Journal Article ; Review ; Research Support, Non-U.S. Gov't |
ZDB-ID | 1182686-1 |
ISSN | 1473-6551 ; 1350-7540 |
ISSN (online) | 1473-6551 |
ISSN | 1350-7540 |
DOI | 10.1097/WCO.0000000000001271 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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