Table 3.

Pathological criteria of anti-IgLON5 disease

Criteria of Anti-IgLON5 Disease
Possible
All of the following requirements:
1. Neurodegenerative features with neuronal loss and gliosis in brain areas showing hyperphosphorylated (hp)Tau pathology without the presence of inflammatory infiltrates.
2. Selective neuronal involvement by deposition of hp Tau in the form of neurofibrillary tangles, pretangles, and neuropil threads with both 3R-tau and 4R-tau isoforms contributing to the inclusions.
3. The hpTau pathology predominantly affects subcortical structures, including the hypothalamus, brain stem tegmentum, and upper spinal cord.
Probable
Criteria of “possible” AND at least one of the following:
1. Clinical history suggestive of a sleep disorder (NREM and REM parasomnia with sleep apnea), or brain stem, mainly bulbar dysfunction.
2. Presence of HLA-DRB1*1001 and HLA-DQB1*0501 alleles.
Definite
Criteria of “possible” AND presence of IgLON5 antibodies in CSF or serum.

See Gelpi et al. (104) for details.