Optineurin (OPTN) is a ubiquitous cytoplasmic protein with various functions including membrane trafficking and signal transduction [9]. OPTN mutations are associated with adult-onset glaucoma and familial and sporadic amyotrophic lateral sclerosis (ALS)[4]. OPTN immunoreactivity has been reported in a variety of ubiquitinated inclusions including ALS-associated neuronal cytoplasmic inclusions, neurofibrillary tangles in Alzheimer’s disease, Pick bodies in Pick disease, Lewy bodies in Parkinson’s disease and glial cytoplasmic inclusions in multiple system atrophy [3, 6]. With respect to polyglutamine diseases, neuronal nuclear inclusions (NNIs) in Huntington’s disease (HD) and spinocerebellar ataxia type 3 (SCA3) are reported to be immunonegative for OPTN [2].