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Published online before print September 10, 2008, doi:10.1212/01.wnl.0000319701.50168.8c)
© 2008 American Academy of Neurology Impaired action knowledge in amyotrophic lateral sclerosisFrom the Departments of Neurology (M.G., C.A., A.K., L.E., L.M.) and Radiology (B.A.), University of Pennsylvania School of Medicine, Philadelphia. Address correspondence and reprint requests to Dr. Murray Grossman, Department of Neurology–2 Gibson, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104-4283 mgrossma{at}mail.med.upenn.edu Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition affecting the motor system, but recent work also shows more widespread cognitive impairment. This study examined performance on measures requiring knowledge of actions, and related performance to MRI cortical atrophy in ALS. Methods: A total of 34 patients with ALS performed measures requiring word-description matching and associativity judgments with actions and objects. Voxel-based morphometry was used to relate these measures to cortical atrophy using high resolution structural MRI. Results: Patients with ALS were significantly more impaired on measures requiring knowledge of actions than measures requiring knowledge of objects. Difficulty on measures requiring action knowledge correlated with cortical atrophy in motor cortex, implicating degraded knowledge of action features represented in motor cortex of patients with ALS. Performance on measures requiring object knowledge did not correlate with motor cortex atrophy. Several areas correlated with difficulty for both actions and objects, implicating these brain areas in components of semantic memory that are not dedicated to a specific category of knowledge. Conclusion: Patients with amyotrophic lateral sclerosis are impaired on measures involving action knowledge, and this appears to be due to at least two sources of impairment: degradation of knowledge about action features represented in motor cortex and impairment on multicategory cognitive components contributing more generally to semantic memory.
Abbreviations: ALS = amyotrophic lateral sclerosis; ALSFRS-R = ALS Functional Rating Scale–Revised; FTLD = frontotemporal lobar degeneration; VBM = voxel-based morphometry.
e-Pub ahead of print on September 10, 2008, at www.neurology.org. Supported in part by National Institutes of Health (AG17586, AG15116, NS44266, and NS53488) and the Dana Foundation. Disclosure: The authors report no disclosures. Received January 4, 2008. Accepted in final form May 1, 2008.
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