Frontotemporal lobar degeneration (FTLD) often overlaps clinically with corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), both of which have prominent eye movement abnormalities. To investigate the ability of oculomotor performance to differentiate between FTLD, Alzheimer's disease, CBS and PSP, saccades and smooth pursuit were measured in three FTLD subtypes, including 24 individuals with frontotemporal dementia (FTD), 19 with semantic dementia (SD) and six with progressive non-fluent aphasia (PA), as compared to 28 individuals with Alzheimer's disease, 15 with CBS, 10 with PSP and 27 control subjects. Different combinations of oculomotor abnormalities were identified in all clinical syndromes except for SD, which had oculomotor performance that was indistinguishable from age-matched controls. Only PSP patients displayed abnormalities in saccade velocity, whereas abnormalities in saccade gain were observed in PSP > CBS > Alzheimer's disease subjects. All patient groups except those with SD were impaired on the anti-saccade task, however only the FTLD subjects and not Alzheimer's disease, CBS or PSP groups, were able to spontaneously self-correct anti-saccade errors as well as controls. Receiver operating characteristic statistics demonstrated that oculomotor findings were superior to neuropsychological tests in differentiating PSP from other disorders, and comparable to neuropsychological tests in differentiating the other patient groups. These data suggest that oculomotor assessment may aid in the diagnosis of FTLD and related disorders.

Objective: To create and validate a simple, standardized version of the antisaccade (AS) task that requires no specialized equipment for use as a measure of executive function in multicenter clinical studies. Methods:The bedside AS (BAS) task consisted of 40 pseudorandomized AS trials presented on a laptop computer. BAS performance was compared with AS performance measured using an infrared eye tracker in normal elders (NE) and individuals with mild cognitive impairment (MCI) or dementia (n ϭ 33). The neuropsychological domain specificity of the BAS was then determined in a cohort of NE, MCI, and dementia (n ϭ 103) at UCSF, and the BAS was validated as a measure of executive function in a 6-center cohort (n ϭ 397) of normal adults and patients with a variety of brain diseases.

While patients with Alzheimer’s disease (AD) show deficits in attention, manifested by inefficient performance on visual search, new visual talents can emerge in patients with frontotemporal lobar degeneration (FTLD), suggesting that, at least in some of the patients, visual attention is spared, if not enhanced. To investigate the underlying mechanisms for visual talent in FTLD (behavioral variant FTD [bvFTD] and semantic dementia [SD]) patients, we measured performance on a visual search paradigm that includes both feature and conjunction search, while simultaneously monitoring saccadic eye movements. AD patients were impaired relative to healthy controls (NC) and FTLD patients on both feature and conjunction search. BvFTD patients showed less accurate performance only on the conjunction search task, but slower response times than NC on all three tasks. In contrast, SD patients were as accurate as controls and had faster response times when faced with the largest number of distracters in the conjunction search task. Measurement of saccades during visual search showed that AD patients explored more of the image, whereas SD patients explored less of the image before making a decision as to whether the target was present. Performance on the conjunction search task positively correlated with gray matter volume in the superior parietal lobe, precuneus, middle frontal gyrus and superior temporal gyrus. These data suggest that despite the presence of extensive temporal lobe degeneration, visual talent in SD may be facilitated by more efficient visual search under distracting conditions due to enhanced function in the dorsal frontoparietal attention network.