Search

Objective: To investigate cognitive and behavioural effects of bilateral lead implants for high frequency stimulation (HFS) of the subthalamic nucleus in patients with Parkinson's disease; and to discriminate between HFS [...]

Objective Cerebral palsy (CP) can be classified as spastic, dyskinetic, ataxic or combined. Correct classification is essential for symptom-targeted treatment. This study aimed to investigate agreement among professionals on the phenotype of children with CP based on standardized videos. Methods In a prospective, observational pilot study, videos of fifteen CP patients (8 boys, mean age 11 ± 5 y) were rated by three pediatric neurologists, three rehabilitation physicians and three movement disorder specialists. They scored the presence and severity of spasticity, ataxia or dyskinesias/dystonia. Inter- and intraobserver agreement were calculated using Cohen's and Fleiss' kappa. Results We found a fair inter-observer (κ = 0.36) and moderate intra-observer agreement (κ = 0.51) for the predominant motor symptom. This only slightly differed within the three groups of specialists (κ = 0.33–0.55). Conclusion A large variability in the phenotyping of CP children was detected, not only between but also within clinicians, calling for a discussing on the operational definitions of spasticity, dystonia and ataxia. In addition, the low agreement found in our study questions the reliability of use of videos to measure intervention outcomes, such as deep brain stimulation in dystonic CP. Future studies should include functional domains to assess the true impact of management options in this highly challenging patient population. [ABSTRACT FROM AUTHOR]

Objective To investigate the Burke-Fahn-Marsden Movement Scale (BFMMS) for the influence of age and for the reliability of the scores. Methods Nine movement disorder specialists from different backgrounds (3 paediatric neurologists, 3 movement disorder-research students and 3 adult neurologists) cross-sectionally scored the BFMMS independently in 52 healthy children (4–16 years; m:f = 1). We associated outcomes with age with multivariate regression analysis and we additionally investigated the reliability of the scale by calculating the Intraclass Correlation Coefficients (ICC's) of inter- and intra-observer agreement and test-retest reliability. Results In healthy children, total BFMMS scores were significantly predicted by age (β=–0.71; p<0.001). The effect of age persisted at least until 16 years of age. ICC's were statistically significant (p<0.05) and varied between fair to almost perfect [inter-observer agreement: 0.33; intra-observer agreement: 0.69; test-retest reliability: 0.94]. The inter-observer agreement appeared statistically significant (p<0.05), varying from substantial to only slight [paediatric neurologists: 0.65; research students: 0.45; neurologists: 0.20]. Conclusion In healthy children, BFMMS is age-dependent, at least until 16 years of age. For reliable interpretation and longitudinal surveillance of quantitative dystonia rating scales in children, international paediatric normal values are needed, first. [ABSTRACT FROM AUTHOR]

Objective: Although subthalamic Deep Brain Stimulation (STN DBS) is proven effective in improving symptoms of Parkinson's Disease (PD), previous literature demonstrates a discrepancy between objective improvement and patients' perception thereof. We aimed to examine whether postoperative stimulation challenge tests (SCT) alters patients' satisfaction after STN DBS for PD.Methods: Fifty-four PD patients underwent preoperative levodopa challenge tests and were routinely invited for SCT 1-2 years postoperatively. SEverity of predominantly Nondopaminergic Symptoms in PD (SENS-PD) scores quantified non-dopaminergic disease severity. Motor functioning was quantified using Movement Disorders Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) III scores; a ratio between conditions ON and OFF (preoperative Med-ON vs. Med-OFF, and postoperative Med-ON/Stim-ON vs. Med-OFF/Stim-OFF) reflected treatment benefit. 'Global Impression of Change' (GIC) and 'Global Satisfaction with Surgery' (GSS) Likert scales were filled out before and immediately after SCT.Results: Postoperative Med-ON/Stim-ON severity was lower than preoperative ON severity. Disease severity scores were not different between assessments. GIC and GSS scores were higher after SCT versus before (GIC: Z = -3.80, r = 0.37, subjects indicating maximum scores before SCT: 32.1%, after SCT: 57.1%; GSS: Z = -3.69, r = 0.35, maximum scores before SCT: 25.0%, after SCT: 46.4%). Higher non-dopaminergic disease severity was associated with lower GIC and GSS scores (GIC: OR 1.2 (95%CI 1.0-1.3); GSS: OR 1.2 (95%CI 1.1-1.3), while motor-scores and magnitude of DBS-effects were not.Conclusion: SCT improves patients' satisfaction and is recommended especially in case of suboptimal subjective valuations. This information should be considered in clinical practice and in the context of clinical trials. [ABSTRACT FROM AUTHOR]

Patients with advanced Parkinson's Disease (PD) may be eligible for Deep Brain Stimulation (DBS) in case of medication-related motor fluctuations or tremor refractory to oral medication. However, several PD symptoms are unresponsive to DBS and constitute relative contra-indications for DBS. Patients referred for DBS undergo an eligibility screening during which motor functioning and contra-indications for surgery are assessed. During this pre-screening the potential benefits and drawbacks of surgery are discussed, together with patients' expectations of the results of DBS. Unrealistic expectations on the benefits of DBS may contribute to reduced patient satisfaction and poor clinical outcomes after surgery. The aim of this multicenter study (289 patients) was to assess the reasons for rejection after an outpatient-based pre-screening visit for DBS referrals, with particular emphasis on the role of patient expectations of DBS. The most frequent reason contributing to rejection was suboptimal oral treatment or satisfying symptom-control with oral medication (50% of rejections). Unrealistic expectations were identified in 38% of rejected patients and were the singular reason for rejection in 4%. Incorporating the assessment of unrealistic expectations increased the accuracy (Area Under the Curve) of determining DBS eligibility from 0.92 ((95% confidence interval (95%CI) 0.88-0.97) to 0.97 (95%CI 0.96-0.99). Patients' expectations of DBS are easily checked, and better education of patients and treating neurologists with regard to unrealistic expectations of this procedure may improve efficiency of referrals and avoid unnecessary stress and disappointments during screening. [ABSTRACT FROM AUTHOR]

Introduction Parkinson’s Disease (PD) patients are eligible for Deep Brain Stimulation (DBS) when oral medication offers insufficient benefit; however, accurate assessment of disease severity is crucial as non-dopaminergic features may deteriorate post-operatively. There is currently no reliable biomarker for non-dopaminergic disease severity. We therefore aimed to investigate whether quantitative EEG reflects non-dopaminergic disease severity. Methods Sixty-three consecutive PD patients screened for DBS between September 2015 and July 2017 were included (mean age 62.4 ± 7.2 years, 32% females). Relative spectral powers and the Phase-Lag-Index (PLI) reflecting functional connectivity were analysed.. The SENS-PD score and its subdomains quantified non-dopaminergic disease severity, whereas the MDS-UPDRS III quantified motor-severity. Results The SENS-PD composite score correlated with a spectral ratio ( δ  +  θ / α 1 +  α 1 +  β powers) (global spectral ratio Pearson r = 0.4, 95% Confidence Interval (95%CI) 0.1 to 0.6), as well as PLI in the α 2 band (10–13 Hz) (r = −0.3, 95%CI −0.5 to −0.1). These correlations seem driven by cognition (global spectral ratio: r = 0.4, 95%CI 0.2 to 0.6; global α 2 PLI r = −0.3, 95%CI −0.5 to −0.2) and psychotic symptoms (global spectral ratio: r = 0.5, 95%CI 0.2 to 0.8; global α 2 PLI: r = −0.2, 95%CI −0.3 to 0.0). MDS-UPDRS III was not significantly correlated with EEG parameters. Conclusion Both EEG slowing and reduced functional connectivity in the α 2 band were associated with advanced non-dopaminergic disease severity in PD. Further study is needed to evaluate the complementary utility of EEG parameters as a predictive tool of non-dopaminergic involvement in PD. [ABSTRACT FROM AUTHOR]

Question STN-DBS could be improved by specifically targeting the dorsolateral part of the STN. However, the correspondence between the location of the STN on T2 MRI images used for targeting and the location of the neurophysiological STN remains unclear. In this study, we compared the STN borders on T2 MRI images with the STN borders identified by microelectrode recordings (MER), measured during DBS surgery. We study the differences between MER-based STN and MRI-based STN in 1.5 T, 3.0 T and 7.0 T images. Methods An atlas-based 3D model of the STN was automatically fitted onto the exact stereotactic locations of the MER points using their classifications as inside or outside the STN. The agreement between STN models and the MRI images of the STN was evaluated by an experienced neurosurgeon. T2 MRI images of 1.5 T ( n = 14), 3.0 T ( n = 10) and 7.0 T ( n = 8) scans were compared. Results The dorsal border of the STN was identified more dorsal on the MRI than it was by MER. This was significant in the 3.0 T and 7.0 T images, with similar mismatch patterns decreasing towards the medial side of the dorsal border ( Fig. 1 ). The lateral border was identified significantly more medial on the MRI than it was by MER, for both 1.5 T and 3.0 T images. The STN borders on 7.0 T images showed no significant differences with MER ( Fig. 2 ). Conclusions MER helps to refine the delineation of the dorsal and lateral border of the STN in MRI-based targeting. Increasing MRI field strength to 7.0 T may improve identification of the lateral, but not the dorsal STN border. [ABSTRACT FROM AUTHOR]

Background The Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS), involving a movement scale (BFMMS) and a disability scale (BFMDS), is a universally applied instrument for quantitative assessment of dystonia in children and adults. However, immature movements by healthy young children may also reveal “dystonic characteristics” as a consequence of physiologically incomplete brain maturation. In young dystonic children, an age-related effect could theoretically confound both the BFMMS and the BFMDS. Objective In healthy young children, we aimed to determine whether physiologically immature movements and postures can induce an age-related effect on BFMMS and BFMDS scores. Methods Nine assessors, specialized in movement disorders (3 adult-, 3 pediatric-neurologists and 3 MD/PhD students) independently scored BFMMS in 52 healthy children (4–16 years; 4 children/year of age; male/female = 1). In another 52 healthy children (4–16 years; 4 children/year of age; male/female = 1), parents scored their children’s functional motor development according to the BFMDS. By regression analysis, we determined the association between BFMMS and BFMDS outcomes and age and compared the regression coefficients using the Z -test. Results In healthy children, assessment of physiologically immature motor performances by the BFMMS and BFMDS revealed an association with age (until 16 and 12 years of age, β = −0.72 and β = −0.60, respectively (both p < 0.001)). The unstandardized regression coefficients between BFMMS and BFMDS revealed no significant difference ( p = 0.753). Conclusions Both the quantitative BFMMS and the functional BFMDS are influenced by the age of the child in a similar pattern. For accurate interpretation of longitudinal BFMDRS scores in young dystonic children, consideration of pediatric age-relatedness appears advisor [ABSTRACT FROM AUTHOR]