Frontotemporal dementia (FID) is a rare form of dementia specific to the frontal and temporal lobes, that accounts for as much as 25% of the presenile dementias (Miller et al., 1998). A number of studies have now documented the bizarre finding that in relation to the temporal lobe variant of FTD, enhanced artistic ability often emerges amidst an otherwise progressive cognitive decline. In the temporal lobe variant of FTD, the anterior temporal and basal frontal lobes atrophy slowly while dorsolateral frontal areas remain intact (Miller et al., 1998). Miller, Ponton, Benson, Cummings and Mena (1996) describe a 56-year-old businessman with the temporal lobe variant of FTD who began painting for the first time, despite no previous interest in art. The subjective experience described by the patient was one of heightened visual awareness, whereby lights and sounds were experienced as either intensely painful or as producing a euphoria that enhanced creativity. Even more unusual was the finding that over the next decade, as the disease progressed, he drew with increasing precision and detail, to the extent that he started to win awards at local art shows. However, by the age of 67 his work began to deteriorate, and at the age of 68 a number of brain scans were taken. MRI revealed bitemporal atrophy, and a single photon emission computed tomography (SPECT) revealed bilateral temporal hypoperfusion, although frontal, occipital and parietal perfusion were found to be normal.
In further case studies of patients with the temporal variant of FTD, Miller et al. (1998) describe another three individuals with similar stories. All of them developing a interest in visual art during the early stages of the disease, displaying increasing proficiency as the disease progressed, and eventually becoming too incapacitated to continue. Miller et al. (1998) comment further on similarities between the patients, highlighting that all of them i) displayed creativity in visual, rather than verbal, forms, ii) they produced art that was largely reconstructed from memory iii) their works of art were realistic copies rather than abstract of symbolic depictions, iv) they displayed an increased interest in fine detail, v) there was an almost obsessional drive for perfection in their work. As with the first patient examined, a common finding from SPECT analysis during the late stages of the disease was that of bitemporal hypoperfusion. Miller et al. (1998) suggest that the reason the visuospatial abilities were spared in these patients was that the dorsolateral prefrontal cortex is spared in the temporal lobe variant of FTD. Further, they suggested that the selective degeneration of the anterior temporal and orbital frontal cortex caused an enhancement of artistic interests and aptitudes due to decreases in the number of inhibitory projections to the visual systems involved with perception. This type of behavioural improvement observed in patients with brain injury has been termed "paradoxical functional facilitation" by (Kapur, 1996).
The most accurate picture of the brain areas involved in the emergence of artistic ability associated with FTD was provided by Miller, Boone, Cummings, Reade and Mishkin (2000). In this study clinical, neuropsychological and neuro-imaging data from 69 patients with a clinical diagnosis of FTD were compared. The data from patients who displayed emerging artistic skills in FTD were compared to those who did not, in order to isolate the important brain areas involved. Interestingly, enhanced musical as well as visual skills were found in this sample, with creativity displayed in a diverse number of ways including invention, bridge or chess playing, composition, piano playing, photography, painting and crafts. However, as with the previous study it is noteworthy that none of these talents were manifest in a verbal form. Twelve FTD patients (17%) were found to display new or preserved musical or visual ability. Nine of these patients displayed left-sided predominant hypoperfusion using SPECT while only 12 of the 45 patients without artistic ability were left-sided. Further, eight of the twelve patients with ability displayed the temporal lobe variant pattern, while only nine of the 45 patients without artistic ability showed the temporal lobe variant. These differences were found to be significant. In summary, most of the FTD patients who displayed artistic ability were found to have a perfusion deficit specific to the temporal lobes, with asymmetrical left hemisphere degeneration. These findings lent further support to the hypothesis that selective degeneration of the temporal cortex leads to paradoxical functional facilitation of visual and musical systems. Specifically, degradation of the left anterior temporal cortex appears to be particularly involved in the disinhibition of these systems. It is also noteworthy that the motivation to engage in artistic ability was also observed to increase dramatically in these patients, which Miller et al. (2000) suggest is due to the reduction of inhibition to the dorsolateral frontal regions involved with working memory.
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