GBA-Associated Parkinson's Disease: Progression in a Deep Brain Stimulation Cohort.
J Parkinsons Dis
; 7(4): 635-644, 2017.
Article
em En
| MEDLINE
| ID: mdl-28777757
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.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doença de Parkinson
/
Estimulação Encefálica Profunda
/
Glucosilceramidase
/
Mutação
Tipo de estudo:
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Aspecto:
Patient_preference
Limite:
Female
/
Humans
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Male
/
Middle aged
Idioma:
En
Revista:
J Parkinsons Dis
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Reino Unido