  We would like to reply to Neuray et al. who report a series of five new
patients from four unrelated families with bi-allelic mutations of GRN. Their
work nicely completes the few existing reports of similar cases, and refers to
our recent publication describing six homozygous GRN pathogenic variant
carriers with divergent phenotypes and ages at onset (Huin et al., 2020). In
summary, the Letter from Neuray et al., reports valuable findings that lead to
better define CLN11 due to bi-allelic GRN pathogenic variants. Despite the
small sample number that does not allow statistical analysis, the authors
underlined the occurrence of cognitive deterioration and epilepsy. Further
study of the CLN11 families with functional brain imaging and
neuropsychological examinations may be highly informative for the understanding
and the clinical characterization of this rare disease.
