Brain Advance Access originally published online on June 30, 2008
Brain 2008 131(8):e99; doi:10.1093/brain/awn122
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Reply: A few comments on Ravel's diseases
Department of Neurology, University of California, San Francisco, CA, USA
Correspondence to: William W. Seeley, Department of Neurology, University of California, San Francisco, CA, USA E-mail: wseeley{at}memory.ucsf.edu
Received May 16, 2008. Accepted May 16, 2008.
Sir, We thank Dr Sellal for his interest in our work and for raising several important issues in neurodegenerative disease research. Frontotemporal dementia (FTD), the primary progressive aphasias (PPAs) and corticobasal syndrome (CBS) represent an evolving, complex, and overlapping clinical spectrum (Kumar-Singh and Van Broeckhoven, 2007
). These syndromes result from a group of underlying histopathologies, including frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy and rarely Alzheimer's disease. Noting the prominent speech, praxis and writing deficits that arose early in Ravel's disease, we and others have suggested that his illness may have fallen within the broad PPA spectrum (Baeck, 1996; Amaducci et al., 2002; Seeley et al., 2008). Deciding whether Ravel's disease began with apraxia or aphasia does not impact the conclusion that he most likely suffered an asymmetric, dominant hemisphere cortical neurodegeneration syndrome. Research criteria for PPA, moreover, allow for ideomotor apraxia and perseveration within the first 2 years of symptoms (Mesulam, 2003).
Our report of Anne Adams (1940–2007) and previous work suggest that dominant hemisphere degeneration can be associated with gains in artistic creativity (Miller et al., 1996; Seeley et al., 2008). When might such gains be expected to peak? This question is embedded within Dr Sellal's doubts that Ravel, whose first deficits arose between 1931 and 1933, could have been influenced by incipient disease in 1928, when he wrote Boléro. Investigating pre-symptomatic PPA spectrum illness is challenging. One study assessed patients with inherited FTD and found evidence for subtle executive dysfunction decades before diagnosis (Geschwind et al., 2001). In Alzheimer's disease, neuroimaging (Reiman et al., 2004) and neuropathological (Braak and Braak, 1991) methods detect Alzheimer's disease-like changes 10–30 years before expected symptom onset. Family members of patients diagnosed with FTD and PPA often look back on a period of increased vivacity just before first symptoms emerged. In agreement with Dr Sellal, we doubt that Ravel's Boléro reflects a deficit related to incipient disease. Rather, we conceptualize Boléro as an intensification of Ravel's prodigious innate musical creativity.
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