Paul Tessier ha creato negli anni 60 la chirurgia craniofacciale. Questa chirurgia richiede una perfetta collaborazione tra neurochirurgo, chirurgo maxillofacciale e anestesista-rianimatore pediatrico. La gestione di questi pazienti è svolta da equipe multidisciplinari al fine di adattare il trattamento ad ogni situazione. Per ragioni di pragmatismo, questo capitolo si limita all’esposizione di indicazioni e tecniche chirurgiche per le malformazioni più comuni dello scheletro craniofacciale: craniostenosi, facio-cranio-stenosi, ipertelorismo (e distopie orbitarie) e meningoencefalocele frontonasale.

ObjectiveThis study was undertaken to identify preoperative predictive factors of long‐term motor outcome in a large cohort of consecutive Parkinson disease (PD) patients with bilateral subthalamic nucleus deep brain stimulation (STN‐DBS).MethodsAll consecutive PD patients who underwent bilateral STN‐DBS at the Grenoble University Hospital (France) from 1993 to 2015 were evaluated before surgery, at 1 year (short‐term), and in the long term after surgery. All available demographic variables, neuroimaging data, and clinical characteristics were collected. Preoperative predictors of long‐term motor outcome were investigated by performing survival and univariate/multivariate Cox regression analyses. Loss of motor benefit from stimulation in the long term was defined as a reduction of less than 25% in the Movement Disorder Society–sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS‐UPDRS) part III scores compared to the baseline off‐medication scores. As a secondary objective, potential predictors of short‐term motor outcome after STN‐DBS were assessed by performing univariate and multivariate linear regression analyses.ResultsIn the long‐term analyses (mean follow‐up = 8.4 ± 6.26 years, median = 10 years, range = 1–17 years), 138 patients were included. Preoperative higher frontal score and off‐medication MDS‐UPDRS part III scores predicted a better long‐term motor response to stimulation, whereas the presence of vascular changes on neuroimaging predicted a worse motor outcome. In 357 patients with available 1‐year follow‐up, preoperative levodopa response, tremor dominant phenotype, baseline frontal score, and off‐medication MDS‐UPDRS part III scores predicted the short‐term motor outcome.InterpretationFrontal lobe dysfunction, disease severity in the off‐medication condition, and the presence of vascular changes on neuroimaging represent the main preoperative clinical predictors of long‐term motor STN‐DBS effects. ANN NEUROL 2021;89:587–597

Background: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is a well-established treatment for motor complications in Parkinson disease (PD). Since 2012, the nonrechargeable dual-channel neurostimulator available in France seems to have shorter battery longevity compared to the same manufacturer's previous model. Objective: The aim of this study was to evaluate the battery longevity of older and more recent neurostimulators from the same manufacturer and to explore factors associated with battery life variations. Materials and methods: We retrospectively studied our cohort of PD patients who underwent STN DBS between 1987 and 2017. We collected data concerning neurostimulator replacements and parameters. We compared the survival of the first device available, Kinetra (R) and the current one, Activa-PC (R) (Medtronic Inc.) and estimated the factors that had an impact on battery longevity through a Cox logistic regression. Results: Three hundred sixty-four PD patients received a total of 654 DBS STN neurostimulators: 317 Kinetra (R) and 337 Activa-PC (R). The survival analysis, using the Kaplan-Meier estimator, showed a difference between the curves of the two devices (log-rank test; p < 0.001). The median survival of an ActivaPC (R) neurostimulator was 1666 days, while it was 2379 days for a Kinetra (R). After adjustment, according to the multivariate analysis, the main factors associated with battery lifetime were: the neurostimulator type; the number of subsequent neurostimulator implantations; the total electrical energy delivered (TEED); and sex. Conclusion: The Kinetra (R) neurostimulator lifetime is 2.5 years longer than the Activa-PC (R). The type of the device, the high TEED and the number of subsequent neurostimulator implantations influence battery longevity most. These results have medical-economic implications since the survival of PD patients with DBS increases over years. (C) 2019 Elsevier Inc. All rights reserved.

Background Effects of DBS on freezing of gait and other axial signs in PD patients are unclear. Objective Secondary analysis to assess whether DBS affects these symptoms within a large randomized controlled trial comparing DBS of the STN combined with best medical treatment and best medical treatment alone in patients with early motor complications (EARLYSTIM-trial). Methods One hundred twenty-four patients were randomized in the stimulation group and 127 patients in the best medical treatment group. Presence of freezing of gait was assessed in the worst condition based on item-14 of the UPDRS-II at baseline and follow-up. The posture, instability, and gait-difficulty subscore of the UPDRS-III, and a gait test including quantification of freezing of gait and number of steps, were performed in both medication-off and medication-on conditions. Results Fifty-two percent in both groups had freezing of gait at baseline based on UPDRS-II. This proportion decreased in the stimulation group to 34%, but did not change in the best medical treatment group at 24 months (P = 0.018). The steps needed to complete the gait test decreased in the stimulation group and was superior to the best medical treatment group (P = 0.016). The axial signs improved in the stimulation group compared to the best medical treatment group (P < 0.01) in both medication-off and medication-on conditions. Conclusions Within the first 2 years of DBS, freezing of gait and other axial signs improved in the medication-off condition compared to best medical treatment in these patients. (c) 2019 International Parkinson and Movement Disorder Society

Paul Tessier ha creato la chirurgia craniofacciale negli anni ′60. Questa chirurgia richiede una perfetta collaborazione tra neurochirurgo, chirurgo plastico o maxillofacciale e anestesista-rianimatore pediatrico. A scopo di pragmatismo, questo capitolo si limita all’esposizione delle indicazioni e delle tecniche chirurgiche delle malformazioni più comuni dello scheletro craniofacciale: le craniostenosi, le faciocraniostenosi, gli ipertelorismi (e le distopie orbitarie) e il meningoencefalocele frontonasale.

Paul Tessier fue el creador de la cirugía craneofacial en la década de 1960. Esta cirugía requiere una colaboración perfecta entre el neurocirujano, el cirujano maxilofacial y el anestesista-reanimador pediátrico. El tratamiento de estos pacientes lo realizan equipos pluridisciplinarios para adaptarlo a cada situación. Con un afán de pragmatismo, este artículo se limita a la descripción de las indicaciones y de las técnicas quirúrgicas de las malformaciones más frecuentes del esqueleto craneofacial: las craneoestenosis, las faciocraneoestenosis, los hipertelorismos (y distopias orbitarias) y los meningoencefaloceles frontonasales.

We examined executive functioning in patients with Parkinson's disease exhibiting, or not, levodopa-resistant freezing of gait (L-FOG). 38 advanced-stage patients with L-FOG were identified in a consecutive series of 400 patients. They were matched with 38 patients without L-FOG. All patients underwent prospective evaluations of cognitive and motor functioning before subthalamic nucleus surgery, and 1 year after. A composite frontal score, a measure of executive functioning, was compared between the two groups. We also examined correlations between the frontal score and the score on the FOG item of the Unified Parkinson Disease Rating Scale II. Results show that after surgery, patients with L-FOG, as a group, were more impaired in executive functioning than control patients. However, individual data analysis showed preserved executive functions in 11 patients with L-FOG. In addition, there was no correlation between L-FOG severity and the degree of executive impairment. Therefore, frontal dysfunction may be one mechanism underlying L-FOG in a number of patients with Parkinson's disease. However, since some patients develop L-FOG despite the preservation of executive functions, lesions or dysfunction of other neuronal structures are likely to be involved.

Stimulation of the subthalamic nucleus (STN) improves the cardinal features of Parkinson disease (PD). However, its efficacy on gait disorders is less satisfying in the long term. In recent years, the pedunculopontine (PPN) nucleus has emerged as a possible promising deep brain stimulation target for gait disorders in PD. In this review, we examine whether STN and PPN act synergistically or antagonistically. Results suggest that the combination of STN and PPN stimulations leads to a significant further improvement in gait as compared with STN stimulation alone, but additive effects on the classical motor triad are questionable. Thus, they highlight the specificity of STN stimulation over PPN’s for the PD cardinal features and the specificity of PPN stimulation over STN for gait disorders. In addition, low-frequency stimulation of the PPN may improve alertness. The additive rather than potentiating effects of STN and PPN stimulations suggest that they may be mediated by distinct pathways. Nevertheless, considering the inconsistencies in published results regarding the influence of PPN stimulation on gait disorders, work is still needed before one can know whether it will convert into a standard surgical treatment and to decipher its place beside STN stimulation.

In advanced Parkinson's disease, several therapeutical option including not only lesional surgery (VIM, GPi) and deep brain stimulation (STN, GPi, VIM) but also continuous subcutaneous apomorphine infusion therapy can be proposed to the patient. The choice depends on the hope of the patient, patient's general health condition and the experience and choice of the neurosurgical and neurologist team. Here we report our experience based on 400 STN-DBS cases and we discuss, on the basis of our experience and on the literature, the advantage and disadvantage of DBS strategy as compared with non-surgical option such as continuous subcutaneous apomorphine infusion therapy.

Gait disturbances are frequent and disabling in advanced Parkinson's disease. These symptoms respond poorly to usual medical and surgical treatments but were reported to be improved by stimulation of the pedunculopontine nucleus. We studied the effects of stimulating the pedunculopontine nucleus area in six patients with severe freezing of gait, unresponsive to levodopa and subthalamic nucleus stimulation. Electrodes were implanted bilaterally in the pedunculopontine nucleus area. Electrode placement was checked by postoperative magnetic resonance imaging. The primary outcome measures were a composite gait score, freezing of gait questionnaire score and duration of freezing episodes occurring during a walking protocol at baseline and one-year follow-up. A double-blind cross-over study was carried out from months 4 to 6 after surgery with or without pedunculopontine nucleus area stimulation. At one-year follow-up, the duration of freezing episodes under off-drug condition improved, as well as falls related to freezing. The other primary outcome measures did not significantly change, nor did the results during the double-blind evaluation. Individual results showed major improvement of all gait measures in one patient, moderate improvement of some tests in four patients and global worsening in one patient. Stimulation frequency ranged between 15 and 25 Hz. Oscillopsia and limb myoclonus could hinder voltage increase. No serious adverse events occurred. Although freezing of gait can be improved by low-frequency electrical stimulation of the pedunculopontine nucleus area in some patients with Parkinson's disease our overall results are disappointing compared to the high levels of expectation raised by previous open label studies. Further controlled studies are needed to determine whether optimization of patient selection, targeting and setting of stimulation parameters might improve the outcome to a point that could transform this experimental approach to a treatment with a reasonable risk-benefit ratio.

We previously demonstrated that patients with schizophrenia or bipolar disorder and their relatives are overrepresented in creative occupations. Here, we use a new dataset with a considerably larger sample of patients (n = 1,173,763) to survey other psychiatric diagnoses and to validate previous findings. The specific aims of this study were to i) investigate if creativity is associated with all psychiatric disorders or restricted to those with psychotic features, and ii) to specifically investigate authors in relationship to psychopathology. We conducted a nested case–control study using longitudinal Swedish total population registries, where the occurrence of creative occupations in patients and their non-diagnosed relatives was compared to that of matched population controls. Diagnoses included were schizophrenia, schizoaffective disorder, bipolar disorder, unipolar depression, anxiety disorders, alcohol abuse, drug abuse, autism, ADHD, anorexia nervosa, and completed suicide. Creative professions were defined as scientific and artistic occupations. Data were analyzed using conditional logistic regression. Except for bipolar disorder, individuals with overall creative professions were not more likely to suffer from investigated psychiatric disorders than controls. However, being an author was specifically associated with increased likelihood of schizophrenia, bipolar disorder, unipolar depression, anxiety disorders, substance abuse, and suicide. In addition, we found an association between creative professions and first-degree relatives of patients with schizophrenia, bipolar disorder, anorexia nervosa, and for siblings of patients with autism. We discuss the findings in relationship to some of the major components of creativity.