title: Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients.
abstract: A cross-sectional neuropsychological study of cognitive functions in 20 male patients with genetically proven spinal and bulbar muscular atrophy (SBMA) was performed, with a comparison of their cognitive performance with that of 20 age- and education-matched control subjects. Neuropsychological assessment covered executive functioning, memory, and attentional control. The SBMA patients revealed deficits in verbal and non-verbal fluency as well as concept formation. Additionally, they showed significant memory deficits in all of the investigated domains of working memory, short-term and long-term memory. With respect to attentional control, the SBMA patients underperformed in relevant subtests, although performance differences did not reach significance overall. We conclude that fronto-temporal cognitive functions are impaired in SMBA, although at a subclinical level. Thus, functional deficits in SBMA are not confined to motor neurons but also affect extramotor networks.
authors: Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany.
authors: Georg Rüdiger
authors: GR
authors: Soukup
authors: Anne-Dorte
authors: AD
authors: Sperfeld
authors: Ingo
authors: I
authors: Uttner
authors: Jochen
authors: J
authors: Karitzky
authors: Albert Christian
authors: AC
authors: Ludolph
authors: Jan
authors: J
authors: Kassubek
authors: Herbert
authors: H
authors: Schreiber
mesh_terms: D001288:Attention / Q000502:physiology; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000150:complications*; D016022:Case-Control Studies; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology*; D003430:Cross-Sectional Studies; D056344:Executive Function / Q000502:physiology; D006801:Humans; D008297:Male; D008568:Memory / Q000502:physiology; D008875:Middle Aged; D009483:Neuropsychological Tests; D018709:Statistics, Nonparametric