Table 1.

Neuropathological findings from patients with anti-IgLON5 disease since 2014

Reference	Patient	Age (yr)	Sex	Neuropathology	Autopsy/biopsy/PET
Sabater et al., 2014	1	53	Male	P-tau deposition mainly involving the hypothalamus and brainstem tegmentum.	Autopsy
	2	76	Female
Gelpi et al., 2016	3	53	Male	Tau pathology with predominant involvement of the hypothalamus and brainstem tegmentum, with a rostrocaudal gradient of severity to include the upper cervical cord.	Autopsy
	4	76	Female
	5	54	Female
	6#	77	Female
	7#	48	Male
	8#	49	Male
Cagnin et al., 2017	9	69	Female	Tau deposition in the nucleus basalis, amygdala, hypothalamus, hippocampus, and locus coeruleus. TDP-43 aggregation in various microglial populations.	Autopsy
Montagna et al., 2018	10	75	Female	Extensive white matter destruction and the presence of numerous macrophages and lymphocytosis. No tau deposition.	Biopsy
Morales-Briceño et al., 2018	11	49	Male	Frontal cortex: meningeal thickening, lymphocyte and microglia infiltration, edema, gliosis. Cerebellum: edema, gliosis, and loss of Purkinje cells. No tau deposition.	Biopsy
Schöberl et al., 2018	12	70	Female	Tau-PET: increased tau deposition in the cerebellar hemispheres and midline, upper and lower brainstem. TSPO-PET: microglia activation in the leptomeninges	PET
Erro et al., 2019	13	71	Male	Few perivascular CD8+ T cell infiltrates and increased microglial activation in the posterior hypothalamus, amygdala, hippocampus, and brainstem. P-tau deposition consistent with AD, no p-tau found in the brainstem.	Autopsy

#: IgLON5 antibody test was not done. Patients 3 and 4 are the same as the patients 1 and 2, respectively. AD: Alzheimer’s disease; CSF: cerebrospinal fluid; PET: positron emission tomography; TSPO: translocator protein.