Combined Therapy of Orthopedic Surgery after Deep Brain Stimulation in Cerebral Palsy Mixed Type - A Case Report -

Dystonia is a symptom defined by involuntary and irregular contractions of the muscles, which cause movement disorders and postural problems. Deep brain stimulation (DBS) in globus pallidus interna (GPi) is a good option for controlling dystonia. DBS has already been shown to have significant effects on primary dystonia as well as Parkinson's disease. Dystonia is very difficult to manage, as seen in cerebral palsy (CP) mixed with spasticity. As CP patients grow, their musculoskeletal problems may require orthopedic surgery. However, the outcome of orthopedic surgery is not usually suitable due to dystonia. Therefore, we attempted to control dystonia through DBS initially and perform orthopedic surgery to correct musculoskeletal deformities after treatment of dystonia. Herein, we report a case that showed remarkable improvement in terms of the dystonia rating scale and gait pattern after combined therapy of DBS and orthopedic surgery

Le trou de la vitamine D : pourquoi ne pas supplémenter tous les aliments chez les enfants

Le rachitisme carentiel du nourrisson a pratiquement disparu en France depuis l’enrichissement systématique des laits infantiles en vitamine D, en 1992. Beaucoup d’arguments suggèrent qu’après cet âge, l’apport total de vitamine D, qu’il soit assuré par photosynthèse cutanée (en été) ou par voie orale devrait être au moins de 1 000 UI/j (25 µg/j). Les apports oraux observés chez les enfants et adolescents aux États-Unis (8 µg/j) et dans les pays du nord de l’Europe (5 à 7 µg/j), sont plus élevés que dans les pays du centre et du sud de l’Europe (< 5 µg/j) mais restent insuffisants comparativement au Besoin Nutritionnel Moyen (BNM = 10 µg/j). En France, ils ne sont que de 2 µg/j de 2 à 17 ans. Cette situation oblige à recourir à une supplémentation hivernale systématique. Du fait de la mauvaise observance d’une supplémentation quotidienne, le Comité de Nutrition de la Société Française de Pédiatrie recommande une supplémentation orale trimestrielle par une dose de charge de 80 ou 100 000 UI de vitamine D3 administrée, l’une en novembre, l’autre au mois de février.Infantile nutritional rickets nearly disappeared in France thanks to systematic enrichment of infantile formulas in 1992. A lot of arguments suggest that after infancy, total vitamin D supply by cutaneous photosynthesis (in summer) and oral intake should be at least 1 000 IU/d (25 µg/d). Oral vitamin D supplies observed in children in United-States (8 µg/d) and in Nordic European countries (5 to 7 µg/d) are higher than in central and south European countries (< 5µg/d), but remain below the Estimated Average Requirements (EAR = 10 µg/d). In France they are only 2 µg/d at the age from 2 to 17 years. This situation requires systematic oral supplementation in winter. Because of bad observance of daily supplementation in childhood, the Nutrition Committee of the French Pediatric Society recommends to use twice single loading doses of 80 or 100 000 IU of vitamin D3 in winter, the first in November and the second in February

Learning disease progression models with longitudinal data and missing values

International audienceStatistical methods have been developed for the analysis of longitudinal data in neurodegenerative diseases. To cope with the lack of temporal markers-i.e. to account for subject-specific disease progression in regard to age-a common strategy consists in realigning the individual sequence data in time. Patient's specific trajectories can indeed be seen as spatiotemporal perturbations of the same normative disease trajectory. However, these models do not easily allow one to account for multimodal data, which more than often include missing values. Indeed, it is rare that imaging and clinical examinations for instance are performed at the same frequency in clinical protocols. Multimodal models also need to allow a different profile of progression for data with different structure and representation. We propose to use a generative mixed effect model that considers the progression trajectories as curves on a Rieman-nian Manifold. We use the concept of product manifold to handle multimodal data, and leverage the generative aspect of our model to handle missing values. We assess the robuste-ness of our methods toward missing values frequency on both synthetic and real data. Finally we apply our model on a real-world dataset to model Parkinson's disease progression from data derived from clinical examination and imaging

Functional Movement Disorders During the COVID ‐19 Pandemic: Back to Charcot's Era at the Salpêtrière

info:eu-repo/semantics/publishedVersio

Evaluation of Thiel cadaveric model for MRI-guided stereotactic procedures in neurosurgery

BACKGROUND: Magnetic resonance imaging (MRI)-guided deep brain stimulation (DBS) and high frequency focused ultrasound (FUS) is an emerging modality to treat several neurological disorders of the brain. Developing reliable models to train and assess future neurosurgeons is paramount to ensure safety and adequate training of neurosurgeons of the future. METHODS: We evaluated the use of Thiel cadaveric model to practice MRI-guided DBS implantation and high frequency MRI-guided FUS in the human brain. We performed three training sessions for DBS and five sonications using high frequency MRI-guided FUS in five consecutive cadavers to assess the suitability of this model to use in training for stereotactic functional procedures. RESULTS: We found the brains of these cadavers preserved in an excellent anatomical condition up to 15 months after embalmment and they were excellent model to use, MRI-guided DBS implantation and FUS produced the desired lesions accurately and precisely in these cadaveric brains. CONCLUSION: Thiel cadavers provided a very good model to perform these procedures and a potential model to train and assess neurosurgeons of the future

Maturation of B Cells in the Lamina Propria of Human Gut and Bronchi in the First Months of Human Life

Little is known of the maturation of the mucosae-associated lymphoid tissue (MALT) in man, because, for ethical reasons, tissues from newborns are not easy to obtain. We used the opportunity provided by autopsies systematically performed in infants who died of Sudden Infant Death Syndrome (SIDS) to study the maturation of the MALT after birth. Gut and bronchus samples of 90 infants from postpartum to 90 months and who died from SIDS were collected and studied by histological and immunofluorescence examination. Plasma cells, absent at birth, appeared within a few hours after birth and initially were of the IgM isotype. IgA plasma cells appeared at 12 days. These cells were first observed in gut and later in bronchi, indicating that maturation of the gut precedes that of bronchi. The number of plasma cells increased rapidly over time and IgA plasma cells became predominant after 3 weeks in the gut and 6 weeks in bronchi. At birth, only small IgM bearing B-cell foci were seen and organized germinal centers appeared to develop over a few days, first in the gut and only later in bronchi. These results confirm that, in man, the MALT organization at birth is still in its fetal form and that maturation depends on intestinal challenges and evolves over several weeks before IgA becomes the predominant isotype secreted

Retrieval practice improves memory in patients with schizophrenia: new perspectives for cognitive remediation.

Schizophrenia is associated with severe cognitive deficits, particularly episodic memory deficits, that interfere with patients' socio-professional functioning. Retrieval practice (also known as testing effect) is a well-established episodic memory strategy that involves taking an initial memory test on a previously learned material. Testing later produces robust long-term memory improvements in comparison to the restudy of the same material both in healthy subjects and in some clinical populations with memory deficits. While retrieval practice might represent a relevant cognitive remediation strategy in patients with schizophrenia, studies using optimal procedures to explore the benefits of retrieval practice in this population are still lacking. Therefore, the purpose of our study was to investigate the benefits of retrieval practice in patients with schizophrenia. Nineteen stabilised outpatients with schizophrenia (DSM-5 criteria) and 20 healthy controls first studied a list of 60 word-pairs (30 pairs with weak semantic association and 30 non associated pairs). Half the pairs were studied again (restudy condition), while only the first word of the pair was presented and the subject had to recall the second word for the other half (retrieval practice condition). The final memory test consisted in a cued-recall which took place 2 days later. Statistical analyses were performed using Bayesian methods. Cognitive performances were globally altered in patients. However, in both groups, memory performances for word-pairs were significantly better after retrieval practice than after restudy (56.1% vs 35.7%, respectively, Pr(RP &gt; RS) &gt; 0.999), and when a weak semantic association was present (64.7% vs 27.1%, respectively; Pr(weak &gt; no) &gt; 0.999). Moreover, the positive effect of RP was observed in all patients but one. Our study is the first to demonstrate that retrieval practice efficiently improves episodic memory in comparison to restudy in patients with schizophrenia. This learning strategy should therefore be considered as a useful tool for cognitive remediation programs. In this perspective, future studies might explore retrieval practice using more ecological material

Criteria for the diagnosis of corticobasal degeneration

Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset ≥50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype. Future validation and refinement of the proposed criteria are needed

Energy and nutrient dietary reference values for children in Europe: methodological approaches and current nutritional recommendations

The Expert Group on the Methodological Approaches and Current Nutritional Recommendations in Children and Adolescents was convened to consider the current situation across Europe with regard to dietary recommendations and reference values for children aged 2-18 years. Information was obtained for twenty-nine of the thirty-nine countries in Europe and a comprehensive compilation was made of the dietary recommendations current up to September 2002. This report presents a review of the concepts of dietary reference values and a comparison of the methodological approaches used in each country. Attention is drawn to the special considerations that are needed for establishing dietary reference values for children and adolescents. Tables are provided of the current dietary reference values for energy and for the macronutrients, vitamins, minerals, trace elements and water. Brief critiques are included to indicate the scientific foundations of the reference values for children and to offer, where possible, an explanation for the wide differences that exist between countries. This compilation demonstrated that there are considerable disparities in the perceived nutritional requirements of European children and adolescents. Although some of this diversity can be attributed to real physiological and environmental differences, most is due to differences in philosophy about the best methodological approach to use and in the way the theoretical approaches are applied. The report highlights the main methodological and technological issues that will need to be resolved before harmonisation can be fully considered. Solving these issues may help to improve the quality and consistency of dietary reference values across Europe. However, there are also considerable scientific and political barriers that will need to be overcome and the question of whether harmonisation of dietary reference values for children and adolescents is a desirable or achievable goal for Europe requires further consideratio

A Prospective Flexible-Dose Study of Paliperidone Palmitate in Nonacute But Symptomatic Patients With Schizophrenia Previously Unsuccessfully Treated With Oral Antipsychotic Agents

AbstractPurposeThe goal of this study was to explore the tolerability, safety, and treatment response of flexible doses of once-monthly paliperidone palmitate (PP) in the subset of nonacute but symptomatic adult patients with schizophrenia previously unsuccessfully treated with oral antipsychotic agents in the PALMFlexS (Paliperidone Palmitate Flexible Dosing in Schizophrenia) study.MethodsThis was an interventional, single-arm, international, multicenter, unblinded, 6-month study performed in patients with schizophrenia. Patients were categorized according to reasons for switching. In patients switching because of lack of efficacy or for other reasons, primary efficacy outcomes were the proportion achieving treatment response (defined as ≥20% improvement in Positive and Negative Syndrome Scale [PANSS] total score from baseline to last-observation-carried-forward end point) and maintained efficacy (defined as noninferiority in the change in PANSS total score at end point versus baseline [Schuirmann's test]), respectively.FindingsA total of 593 patients (intention-to-treat population) were enrolled: 63.1% were male; their mean (SD) age was 38.4 (11.8) years; and 78.6% had paranoid schizophrenia. The main reasons for transition to PP were patient's wish (n = 259 [43.7%]), lack of efficacy (n = 144 [24.3%]), lack of compliance (n = 138 [23.3%]), and lack of tolerability (n = 52 [8.8%]) with the previous oral antipsychotic medication. The recommended PP initiation regimen (150 milligram equivalents [mg eq] day 1 and 100 mg eq day 8) was administered in 93.9% of patients. Mean PANSS total score decreased from 71.5 (14.6) at baseline to 59.7 (18.1) at end point (mean change, –11.7 [15.9]; 95% CI, –13.0 to –10.5; P < 0.0001). Sixty-four percent of patients showed an improvement of ≥20% in PANSS total score, and the percentage of patients rated mildly ill or less in Clinical Global Impression–Severity increased from 31.8% to 63.2%. Mean personal and social performance total score (SD) increased (ie, improved) significantly for all patients from baseline to end point (58.1 [13.4] to 66.1 [15.7]; P < 0.0001).ImplicationsThe PALMFlexS study is a pragmatic interventional study compared with randomized controlled trials, conducted in a large, more representative sample of patients with schizophrenia, and designed specifically to mimic real-world clinical situations. The findings support the results from randomized controlled studies. They also demonstrate that a clinically relevant treatment response is possible in patients who are considered to be clinically stable by their physician, supporting the use of flexibly dosed PP in such patients. Clinical trials.gov number: NCT01281527