A single case had, in addition to the anti-IgLON5 disease-related brainstem tauopathy and TDP-43 aggregates in brainstem motor neurons as also seen in ALS, frequent intranuclear hyaline inclusions in an extent and distribution that were consistent with neuronal intranuclear hyaline inclusion body disease (NIHID) [25]; genetic testing to exclude fragile-X tremor ataxia syndrome or NOTCH2NLC gene alterations for NIHID was not possible. Here, IGLON5 is linked to amyotrophic lateral sclerosis.