New knowledge on anti-IgLON5 disease.
Curr Opin Neurol
; 37(3): 316-321, 2024 06 01.
Article
in En
| MEDLINE
| ID: mdl-38563128
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Motor Neuron Disease
/
Neurodegenerative Diseases
Limits:
Animals
/
Humans
Language:
En
Journal:
Curr Opin Neurol
Journal subject:
NEUROLOGIA
Year:
2024
Document type:
Article
Country of publication:
Reino Unido