Journal
JOURNAL OF ALZHEIMERS DISEASE
Volume 59, Issue 1, Pages 13-20Publisher
IOS PRESS
DOI: 10.3233/JAD-170189
Keywords
IgLON5; microglia; non-cell autonomous neurodegeneration; tauopathy; TDP-43 pathology
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Funding
- Fondazione Cariverona (Biomarcatori predittivi e diagnostici in patologie neoplastiche, infiammatorie e neurodegenerative)
- Veneto Region Finalized Research (Experimental Study of a Clinical Network for Diagnosing Rapidly Progressive Dementias) [RP-2014-00000400]
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A novel neuronal tauopathy, mainly confined to hypothalamus and brainstem tegmentum, has recently been reported in patients with autoantibodies to the neuronal cell-adhesion molecule IgLON5. We describe a patient with anti-IgLON5 syndrome, who presented with dysautonomia and sleep disorder, followed by subacute dementia. Postmortem brain examination disclosed neuronal tau pathology prevailing in the hippocampus, amygdala, and locus coeruleus, in addition to microglial/neuronal TDP-43 pathology, with overexpression of aberrantly phosphorylated forms and neurotoxic truncated fragments, in basal ganglia, nucleus basalis, thalamus, and midbrain. These findings suggest that neurodegeneration in anti-IgLON5 syndrome might also occur via a microglia-triggered non-cell autonomous pathway.
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