Latin Americans and Caribbeans in Europe. A cross-country analysis

With the beginning of the 21st century, there has been an acceleration of migratory flows from Latin America and the Caribbean (LAC) to Europe. As a result, and despite the negative impact of the economic crisis, 4.6 million Latin American and Caribbean immigrants reside in Europe, half of them in Spain. This article analyses the recent evolution of these migratory flows, their territorial distribution, and their demographic profiles according to the 2011 European census data disseminated by a new tool -the Census Hub- implemented by the European Statistical System. The analysis shows the existence of a high LAC immigrant concentration in Spain and in certain European cities, a marked young and feminized demographic profile, a great variety of educational levels and a different insertion in each European labour market, although many LAC immigrants work in low-skill occupations, being overqualified and underemployed in most of the countries

WHO grade II meningioma: Epidemiology, survival and contribution of postoperative radiotherapy in a multicenter cohort of 88 patients

International audienceBackground: Meningioma is the most common primary intracranial tumor, representing 13-36.6% of all primary central nervous system tumors. Meningiomas are benign in about 90% of cases. World Health Organization (WHO) grade II meningioma is associated with a high rate of recurrence and poorer survival than in grade I. The reference treatment is surgery, which should be as complete as possible. Currently, in grade II, there are no recommendations for systematic adjuvant treatment such as radiotherapy. We studied a homogeneous series of grade II meningiomas treated by surgery in two university hospital centers to analyze use of radiotherapy and its efficacy.Methods: We retrospectively analyzed patients in our database with WHO grade II meningioma, operated on between 2007 and 2010 in the university hospitals of Montpellier and Bordeaux, France. Clinical and radiological data, treatments and survival were analyzed.Results: Eighty-eight patients were included. Five-year overall survival was 89.7%. Nineteen patients received radiotherapy during follow-up, without significant impact on survival (P=0.27).Conclusion: In WHO grade II meningioma, it is currently difficult to establish clear recommendations for radiotherapy. The present study is in accordance with the literature that early postoperative radiotherapy is not mandatory in grade II meningioma with macroscopically total resection

Pituitary metastasis of malignant melanoma misdiagnosed as pituitary adenoma: A case report and systematic review of the literature

International audienceWe report a case of malignant melanoma revealed by a metastasis to the pituitary gland. The tumor was misdiagnosed as a pituitary adenoma and aggressive transsphenoidal surgery was complicated by a cerebrospinal fluid fistula. Nine weeks later, the patient presented multiple leptomeningeal and brain metastases spreading from the sellar region. Regarding these observations, we conducted a systematic review of the literature in order to investigate clinicoradiological features that should lead clinicians to suspect pituitary metastasis and how it should impact the surgical management

Highlighting the Dystonic Phenotype Related to GNAO1

BACKGROUND: Most reported patients carrying GNAO1 mutations showed a severe phenotype characterized by early‐onset epileptic encephalopathy and/or chorea. OBJECTIVE: The aim was to characterize the clinical and genetic features of patients with mild GNAO1‐related phenotype with prominent movement disorders. METHODS: We included patients diagnosed with GNAO1‐related movement disorders of delayed onset (>2 years). Patients experiencing either severe or profound intellectual disability or early‐onset epileptic encephalopathy were excluded. RESULTS: Twenty‐four patients and 1 asymptomatic subject were included. All patients showed dystonia as prominent movement disorder. Dystonia was focal in 1, segmental in 6, multifocal in 4, and generalized in 13. Six patients showed adolescence or adulthood‐onset dystonia. Seven patients presented with parkinsonism and 3 with myoclonus. Dysarthria was observed in 19 patients. Mild and moderate ID were present in 10 and 2 patients, respectively. CONCLUSION: We highlighted a mild GNAO1‐related phenotype, including adolescent‐onset dystonia, broadening the clinical spectrum of this condition. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Societ

KMT2B-related disorders: expansion of the phenotypic spectrum and long-term efficacy of deep brain stimulation

Heterozygous mutations in KMT2B are associated with an early-onset, progressive and often complex dystonia (DYT28). Key characteristics of typical disease include focal motor features at disease presentation, evolving through a caudocranial pattern into generalized dystonia, with prominent oromandibular, laryngeal and cervical involvement. Although KMT2B-related disease is emerging as one of the most common causes of early-onset genetic dystonia, much remains to be understood about the full spectrum of the disease. We describe a cohort of 53 patients with KMT2B mutations, with detailed delineation of their clinical phenotype and molecular genetic features. We report new disease presentations, including atypical patterns of dystonia evolution and a subgroup of patients with a non-dystonic neurodevelopmental phenotype. In addition to the previously reported systemic features, our study has identified co-morbidities, including the risk of status dystonicus, intrauterine growth retardation, and endocrinopathies. Analysis of this study cohort (n = 53) in tandem with published cases (n = 80) revealed that patients with chromosomal deletions and protein truncating variants had a significantly higher burden of systemic disease (with earlier onset of dystonia) than those with missense variants. Eighteen individuals had detailed longitudinal data available after insertion of deep brain stimulation for medically refractory dystonia. Median age at deep brain stimulation was 11.5 years (range: 4.5–37.0 years). Follow-up after deep brain stimulation ranged from 0.25 to 22 years. Significant improvement of motor function and disability (as assessed by the Burke Fahn Marsden’s Dystonia Rating Scales, BFMDRS-M and BFMDRS-D) was evident at 6 months, 1 year and last follow-up (motor, P = 0.001, P = 0.004, and P = 0.012; disability, P = 0.009, P = 0.002 and P = 0.012). At 1 year post-deep brain stimulation, >50% of subjects showed BFMDRS-M and BFMDRS-D improvements of >30%. In the long-term deep brain stimulation cohort (deep brain stimulation inserted for >5 years, n = 8), improvement of >30% was maintained in 5/8 and 3/8 subjects for the BFMDRS-M and BFMDRS-D, respectively. The greatest BFMDRS-M improvements were observed for trunk (53.2%) and cervical (50.5%) dystonia, with less clinical impact on laryngeal dystonia. Improvements in gait dystonia decreased from 20.9% at 1 year to 16.2% at last assessment; no patient maintained a fully independent gait. Reduction of BFMDRS-D was maintained for swallowing (52.9%). Five patients developed mild parkinsonism following deep brain stimulation. KMT2B-related disease comprises an expanding continuum from infancy to adulthood, with early evidence of genotype-phenotype correlations. Except for laryngeal dysphonia, deep brain stimulation provides a significant improvement in quality of life and function with sustained clinical benefit depending on symptoms distribution