Frontotemporal lobar degeneration is a progressive neurodegenerative disorder disproportionately affecting the frontal and anterior temporal lobes (2
). Some clinical subtypes of FTLD help illuminate the nature of artistic creativity. Previous case studies of patients with left-sided FTLD syndromes, whether temporally predominant (semantic dementia) or frontally predominant (progressive non-fluent aphasia) reveal that a new preoccupation with art, greater attention to visual stimuli, and increased visual creativity sometimes occur during the early stages of these kinds of dementia. In contrast, Alzheimer’s disease (AD), which typically first affects the posterior parietal and medial temporal areas, is associated with decreased visuo-constructive ability (7
). Of further interest, creativity in FTLD has been observed in visual art, music, mechanics, and mathematics, but not in writing or poetry (1
A number of other descriptions of artistically creative FTLD patients exist in the literature. In one series of case studies, left-temporal variant FTLD patients, who are known for their severe semantic loss, exhibited a newfound interest in art and preferred making representational drawings of landscapes, animals, and people. Abstract or symbolic art was notably absent (7
). In a second study, a 57-year-old right-handed female artist’s left-sided frontal and temporal atrophy correlated with her deteriorating language and social skills but increased creativity in her art-making. Unlike the prior case series, her painting evolved from more traditional landscapes and representational art to freer, more expressive, and abstract forms with the progression of her dementia (9
). In a third study, a 56-year-old right-handed businessman with no previous interest in art began painting for the first time with the onset of FTLD. He displayed heightened visual awareness to his environment, especially light and sound, even as his language and behavior deteriorated (10
While FTLD presents with a variety of clinical syndromes depending on the brain regions affected, these clinical syndromes cannot yet be used to definitively predict the underlying neuropathological subtype. The above reports of increased creativity in FTLD describe patients whose pathological subtype was either unknown or was consistent with one of the pure FTLD pathology types, i.e., either tau positive, or tau-negative ubiquitin and TDP43-positive inclusions with no motor neuron disease (MND) pathology. To our knowledge, no reports of increased creativity in patients with the FTLD-MND pathological subtype (ubiquitin and TDP43-positive inclusions with MND pathology) have yet been published. Clinically, patients with MND can gradually lose limb, bulbar, and respiratory muscle function as a result of gradual degeneration of both upper and lower motor neurons, and up to 50% of these patients manifest some degree of cognitive impairment (3
). Patients found to have mixed FTLD-MND pathology can present with clinical features anywhere on the spectrum between typical FTLD (behavior-predominant with few if any motor symptoms) and typical amyotrophic lateral sclerosis (ALS) (motor predominant with few behavior and cognitive changes).
Here we describe the case of a 53-year-old, right-handed man who first presented with the language and behavior impairments characteristic of FTLD, then developed upper and lower motor neuron deficits consistent with a second diagnosis of amyotrophic lateral sclerosis. Upon autopsy, the patient was confirmed to have combined FTLD-MND neuropathology. This case study provides a detailed description of VW’s cognitive, emotional, behavioral, and motor symptoms, which initially enabled but eventually limited his productivity as a visual artist. From another perspective, the evolution of the quality of VWs artwork provides another means of understanding his neurodegenerative decline.