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GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort.
J Parkinsons Dis 2017; 7(4):635-644JP

Abstract

BACKGROUND

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.

OBJECTIVE

To explore the long-term phenotypic progression of GBA-associated PD, in a DBS cohort.

METHODS

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.

RESULTS

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.

CONCLUSIONS

GBA status appears to be an important predictor for non-motor symptom disease progression, after deep brain stimulation surgery.

Authors+Show Affiliations

Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, London, UK.Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK. Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, London, UK.Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK.Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

28777757

Citation

Lythe, Vanessa, et al. "GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort." Journal of Parkinson's Disease, vol. 7, no. 4, 2017, pp. 635-644.
Lythe V, Athauda D, Foley J, et al. GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort. J Parkinsons Dis. 2017;7(4):635-644.
Lythe, V., Athauda, D., Foley, J., Mencacci, N. E., Jahanshahi, M., Cipolotti, L., ... Foltynie, T. (2017). GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort. Journal of Parkinson's Disease, 7(4), pp. 635-644. doi:10.3233/JPD-171172.
Lythe V, et al. GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort. J Parkinsons Dis. 2017;7(4):635-644. PubMed PMID: 28777757.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort. AU - Lythe,Vanessa, AU - Athauda,Dilan, AU - Foley,Jennifer, AU - Mencacci,Niccolò E, AU - Jahanshahi,Marjan, AU - Cipolotti,Lisa, AU - Hyam,Jonathan, AU - Zrinzo,Ludvic, AU - Hariz,Marwan, AU - Hardy,John, AU - Limousin,Patricia, AU - Foltynie,Tom, PY - 2017/8/5/pubmed PY - 2018/6/16/medline PY - 2017/8/5/entrez KW - Deep brain stimulation KW - Parkinson’s disease KW - glucosidase beta acid KW - phenotype SP - 635 EP - 644 JF - Journal of Parkinson's disease JO - J Parkinsons Dis VL - 7 IS - 4 N2 - BACKGROUND: 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. OBJECTIVE: To explore the long-term phenotypic progression of GBA-associated PD, in a DBS cohort. METHODS: 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. RESULTS: 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. CONCLUSIONS: GBA status appears to be an important predictor for non-motor symptom disease progression, after deep brain stimulation surgery. SN - 1877-718X UR - https://www.unboundmedicine.com/medline/citation/28777757/GBA_Associated_Parkinson's_Disease:_Progression_in_a_Deep_Brain_Stimulation_Cohort_ L2 - https://content.iospress.com/openurl?genre=article&id=doi:10.3233/JPD-171172 DB - PRIME DP - Unbound Medicine ER -