Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients.
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.
Soukup
Georg Rüdiger
GR
Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany.
Sperfeld
Anne-Dorte
AD
Uttner
Ingo
I
Karitzky
Jochen
J
Ludolph
Albert Christian
AC
Kassubek
Jan
J
Schreiber
Herbert
H
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