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Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy
- Source :
- Journal of Alzheimer's disease : JAD. 59(1)
- Publication Year :
- 2017
-
Abstract
- 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.
- Subjects :
- 0301 basic medicine
Pathology
medicine.medical_specialty
Cell Adhesion Molecules, Neuronal
Thalamus
IgLON5
tau Proteins
Nucleus basalis
03 medical and health sciences
0302 clinical medicine
TDP-43 pathology
microglia
non-cell autonomous neurodegeneration
tauopathy
Basal ganglia
Tegmentum
Medicine
Humans
Aged
Autoantibodies
Neurons
business.industry
General Neuroscience
Neurodegeneration
Brain
General Medicine
medicine.disease
DNA-Binding Proteins
Psychiatry and Mental health
Clinical Psychology
030104 developmental biology
nervous system
Tauopathies
Locus coeruleus
Female
Brainstem
Tauopathy
Microglia
Geriatrics and Gerontology
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 18758908
- Volume :
- 59
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Journal of Alzheimer's disease : JAD
- Accession number :
- edsair.doi.dedup.....d80be9b4c40900607410df5a8b7d67b7