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Publication Detail
GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort.
  • Publication Type:
    Journal article
  • Publication Sub Type:
  • Authors:
    Lythe V, Athauda D, Foley J, Mencacci NE, Jahanshahi M, Cipolotti L, Hyam J, Zrinzo L, Hariz M, Hardy J, Limousin P, Foltynie T
  • Publication date:
  • Journal:
    Journal of Parkinson's disease
  • Medium:
  • Print ISSN:
  • Language:
  • Addresses:
    Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.
Recent evidence suggests that glucosidase beta acid (GBA) mutations predispose Parkinson's disease (PD) patients to a greater burden of cognitive impairment and non-motor symptoms. This emerging knowledge has not yet been considered in patients who have undergone deep brain stimulation (DBS); a surgery that is generally contraindicated in those with cognitive deficits.To explore the long-term phenotypic progression of GBA-associated PD, in a DBS cohort.Thirty-four PD patients who had undergone DBS surgery between 2002 and 2011 were included in this study; 17 patients with GBA mutations were matched to 17 non-carriers. Clinical evaluation involved the administration of four assessments: The Mattis Dementia Rating Scale was used to assess cognitive function; non-motor symptoms were assessed using the Non-Motor Symptom Assessment Scale for PD; quality of life was measured using the Parkinson's Disease Questionnaire; and motor symptoms were evaluated using part III of the Movement Disorders Society Unified Parkinson's Disease Rating Scale, in on-medication/on-stimulation conditions. Levodopa equivalent doses (LED) and DBS settings were compared with clinical outcomes.At a mean follow-up of 7.5 years after DBS, cognitive impairment was more prevalent (70% vs 19%) and more severe in GBA mutation carriers compared to non-carriers (60% vs 6% were severely impaired). Non-motor symptoms were also more severe and quality of life more impaired in GBA-associated PD. Motor symptoms, LED, and stimulation settings were not significantly different between groups at follow-up.GBA status appears to be an important predictor for non-motor symptom disease progression, after deep brain stimulation surgery.
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Clinical and Movement Neurosciences
Clinical and Movement Neurosciences
Neurodegenerative Diseases
Department of Neuromuscular Diseases
UCL Queen Square Institute of Neurology
Clinical and Movement Neurosciences
Clinical and Movement Neurosciences
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