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Mutations in C9ORF72 resulting in expanded hexanucleotide repeats were recently reported to be the underlying genetic abnormality in chromosome 9p‐linked frontotemporal lobar degeneration with TAR DNA‐binding protein of 43 kD (TDP‐43) proteinopathy (FTLD‐TDP), amyotrophic lateral sclerosis (ALS), and frontotemporal lobar degeneration with motor neuron disease (FTLD‐MND). Several subsequent publications described the neuropathology as being similar to that of FTLD‐TDP and ALS without C9ORF72 mutations, except that cases with mutations have p62 and ubiquitin positive, TDP‐43 negative inclusions in cerebellum, hippocampus, neocortex, and basal ganglia. The identity of this protein is as yet unknown, and its significance is unclear. With the goal of potentially uncovering the significance of these inclusions, we compared the clinical, pathologic and genetic characteristics in cases with …