title: Pallidal stimulation for primary generalised dystonia: effect on cognition, mood and quality of life.
abstract: We investigated the effect of pallidal deep brain stimulation (GPi-DBS) in dystonia on cognition, mood, and quality of life and also assessed if DYT1 gene status influenced cognitive outcome following GPi-DBS. Fourteen patients with primary generalized dystonia (PGD) were assessed, measuring their estimated premorbid and current IQ, memory for words and faces, and working memory, language, executive function, and sustained attention, one month before and one year or more after surgery. Changes in mood and behaviour and quality of life were also assessed. There was a significant improvement of dystonia with GPi-DBS (69 % improvement in Burke-Fahn-Marsden score, p < 0.0001). Performance on five cognitive tests either improved or declined at post-surgical follow-up. Calculation of a reliable change index suggested that deterioration in sustained attention on the PASAT was the only reliable change (worse after surgery) in cognition with GPi-DBS. DYT1 gene status did not influence cognitive outcome following GPi-DBS. Depression, anxiety and apathy were not significantly altered, and ratings of health status on the EQ5D remained unchanged. In our sample, GPi-DBS was only associated with an isolated deficit on a test of sustained attention, confirming that GPi-DBS in PGD is clinically effective and safe, without adverse effects on the main domains of cognitive function. The dissociation between GPi-DBS improving dystonia, but not having a significant positive impact on the patients' QoL, warrants further investigation.
authors.affiliation: Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, The National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK, m.jahanshahi@ucl.ac.uk.
authors.forename: Marjan
authors.initials: M
authors.lastname: Jahanshahi
authors.forename: Mariam
authors.initials: M
authors.lastname: Torkamani
authors.forename: Mazda
authors.initials: M
authors.lastname: Beigi
authors.forename: Leonora
authors.initials: L
authors.lastname: Wilkinson
authors.forename: Donna
authors.initials: D
authors.lastname: Page
authors.forename: Laura
authors.initials: L
authors.lastname: Madeley
authors.forename: Kailash
authors.initials: K
authors.lastname: Bhatia
authors.forename: Marwan
authors.initials: M
authors.lastname: Hariz
authors.forename: Ludvic
authors.initials: L
authors.lastname: Zrinzo
authors.forename: Patricia
authors.initials: P
authors.lastname: Limousin
authors.forename: Diane
authors.initials: D
authors.lastname: Ruge
authors.forename: Stephen
authors.initials: S
authors.lastname: Tisch
mesh_terms: D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D003072:Cognition Disorders / Q000209:etiology* / Q000628:therapy; D046690:Deep Brain Stimulation / Q000379:methods*; D004421:Dystonia / Q000150:complications / Q000235:genetics / Q000523:psychology / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D005917:Globus Pallidus / Q000502:physiology*; D006801:Humans; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D018832:Molecular Chaperones / Q000235:genetics; D019964:Mood Disorders / Q000209:etiology* / Q000628:therapy; D009483:Neuropsychological Tests; D055815:Young Adult
chemical_list: D018832:Molecular Chaperones; C108175:TOR1A protein, human