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In contrast, frontotemporal lobar degeneration with transactive response DNA\u2010binding protein type C (TDP\u2010C) shows a consistent predilection for the anterior temporal lobe (ATL). The relatively selective atrophy of ATL in TDP\u2010C patients has highlighted the importance of this region for complex cognitive and behavioral functions. This review includes observations on 28 TDP\u2010C patients, 18 with semantic primary progressive aphasia and 10 with other syndromes. Longitudinal imaging allowed the delineation of progression trajectories. At post\u2010mortem examination, the pathognomonic feature of TDP\u2010C consisted of long, thick neurites found predominantly in superficial cortical layers. These neurites may represent dystrophic apical dendrites of layer III and V pyramidal neurons that are known to play pivotal roles in complex cortical computations. Other types of frontotemporal lobar degeneration TDP, such as TDP\u2010A and TDP\u2010B, are not associated with long dystrophic neurites in the cerebral cortex, and do not show similar predilection patterns for ATL. Research is beginning to identify molecular, structural, and immunological differences between pathological TDP\u201043 in TDP\u2010C versus TDP\u2010A and B. Parallel investigations based on proteomics, somatic mutations, and genome\u2010wide association studies are detecting molecular features that could conceivably mediate the selective vulnerability of ATL to TDP\u2010C. Future work will focus on characterizing the distinctive features of the abnormal TDP\u2010C neurites, the mechanisms of neurotoxicity, initial cellular targets within the ATL, trajectory of spread, and the nature of ATL\u2010specific markers that modulate vulnerability to TDP\u2010C. 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