Le réseau de surveillance de la maladie de Creutzfeldt-Jakob

IntroductionTransmissible spongiform encephalopathies (TSE) have been under epidemiological surveillance in France and in Europe since the early 1990s. The observation of iatrogenic Creutzfeldt-Jakob disease (CJD), the outbreak of bovine spongiform encephalopathy (ESB) and its probable transmission to many species gave rise to the surveillance which remains warranted by the emergence of a variant of CJD (vCJD), in 1996.State of artIn France, epidemiological surveillance is coordinated by the InVS which receives input from cases notifications addressed to INSERM Unit 708 directly by clinicians or more often following requests for 14-3-3 detection in CSF. All suspected cases are followed up until a final diagnosis is established. Thanks to the effectiveness of the French network of neuropathology, autopsies are performed in more than half of patients who die with a diagnosis of suspected CJD. Diagnostic criteria allow comparison of the incidence of the different forms of the disease in all countries with a system of surveillance. Sporadic CJD is the most frequent form of the disease with more than 80% of the cases. Its origin remains unknown. To date, cases of iatrogenic CJD referred to the French surveillance network have been caused by dura mater grafts or human growth hormone treatments administrated in the 1980s. Ten percent of TSE are of genetic origin with an autosomic dominant transmission of a mutation or an insertion located on the PRNP gene. The most recent form of the disease is vCJD which is a new form, first described in the United Kingdom in 1994.Prospect and conclusionActive epidemiological surveillance remains a timely issue, particularly in France, because of the development of new cases of iatrogenic CJD after human growth hormone treatment. It is of importance in France and worldwide because of the emergence of post-transfusional cases of vCJD and the possible appearance of vCJD in persons with valine-valine or methionine-valine genotypes at codon 129.     

Consenso sobre guías de tratamiento de gliomas de bajo grado elaborado por el grupo de tumores de la SENEC

Adult low-grade gliomas (Low Grade Gliomas, LGG) are tumors that originate from the glial cells of the brain and whose management involves great controversy, starting from the diagnosis, to the treatment and subsequent follow-up. For this reason, the Tumor Group of the Spanish Society of Neurosurgery (GT-SENEC) has held a consensus meeting, in which the most relevant neurosurgical issues have been discussed, reaching recommendations based on the best scientific evidence. In order to obtain the maximum benefit from these treatments, an individualized assessment of each patient should be made by a multidisciplinary team.Experts in each LGG treatment field have briefly described it based in their experience and the reviewed of the literature.Each area has been summarized and focused on the best published evidence.LGG have been surrounded by treatment controversy, although during the last years more accurate data has been published in order to reach treatment consensus. Neurosurgeons must know treatment options, indications and risks to participate actively in the decision making and to offer the best surgical treatment in every case.     

Les programmes de soins ambulatoires contraints dans le cadre de la loi de 2011, modifiée. Point de vue de la direction

A Compulsory Outpatient Treatment (COT) can be organized by the consulting psychiatrist, at the end of the period of 72 hours of observation and care, if the patient requires no longer constant but regular monitoring. It may include exclusive ambulatory care, or short sequences of hospitalization if needed. This modality raises several questions concerning the psychiatrist, the Director of the facility, and the legal status of the patient in COT. The use of force is excluded if the patient does not comply with his compulsory treatment. At the same time, the COT is not subject to systematic control of the judge. The issues are both practical and legal, especially if the doctor decides that the patient needs urgently the return in inpatient facility for medical reasons. The quality of the information issued to the patient is fundamental, in this area where the Director's decision is bound by the decision of the psychiatrist, putting them both in an area of legal uncertainty.     

Perfil clínico y sociodemográfico de la epilepsia en adultos de un centro de referencia de Colombia

ObjectivesTo describe the sociodemographic and clinical characteristics of a cohort of patients with epilepsy from a reference centre in Colombia.MethodsCross-sectional study including patients diagnosed with epilepsy who attended our epilepsy centre (Neurocentro) between 2013 and 2016. Data were gathered from patients’ medical histories.ResultsWe gathered data from a total of 354 patients diagnosed with epilepsy. Median age was 37 years; 52% were men. Seizures were focal in 57% of the patients and generalised in 38%; seizure type was not determined in 6% of the sample. The most frequent aetiology was cryptogenic (21%), followed by traumatic (14%). Median time of disease progression and age at onset were 23 and 11 years, respectively. Psychiatric comorbidities were found in 18% of the patients and 40% had some degree of cognitive impairment. Around 40% of our sample reported adverse reactions to antiepileptic drugs at some point during treatment. Antiepileptic drugs were administered in monotherapy in 36% of the patients. Around 37% had drug-resistant epilepsy and 14% underwent surgery.ConclusionsPsychiatric comorbidities, cognitive impairment, adverse drug reactions, and drug-resistant epilepsy are common among epileptic patients in Colombia. Knowledge of the factors with an impact on epilepsy may lay the foundations for improving management of these patients on the administrative level and improving quality of life.     

Endoscopia endonasal de base de cráneo

Introduction and objectiveThe endoscopic endonasal techniques used in skull base surgery have evolved greatly in recent years. Our study objective was to perform a qualitative systematic review of the likewise systematic reviews in published English language literature, to examine the evidence and conclusions reached in these studies comparing transcranial and endoscopic approaches in skull base surgery.Material and methodsWe searched the references on the MEDLINE and EMBASE electronic databases selecting the systematic reviews, meta-analyses and evidence based medicine reviews on skull based pathologies published from January 2000 until January 2013. We focused on endoscopic impact and on microsurgical and endoscopic technique comparisons.ResultsFull endoscopic endonasal approaches achieved gross total removal rates of craniopharyngiomas and chordomas higher than those for transcranial approaches. In anterior skull base meningiomas, complete resections were more frequently achieved after transcranial approaches, with a trend in favour of endoscopy with respect to visual prognosis. Endoscopic endonasal approaches minimised the postoperative complications after the treatment of cerebrospinal fluid (CSF) leaks, encephaloceles, meningoceles, craniopharyngiomas and chordomas, with the exception of postoperative CSF leaks.ConclusionsRandomized multicenter studies are necessary to resolve the controversy over endoscopic and microsurgical approaches in skull base surgery.     

Influencia de la estimulación cerebral profunda en la carga de cuidadores de pacientes con enfermedad de Parkinson

Introduction

Our aim is to assess the burden on caregivers of patients with Parkinson's disease treated with deep brain stimulation (DBS) compared to those caring for patients at advanced stages and undergoing other treatments. We have also assessed the variables associated with presence of caregiver overload.

Evolución y tratamiento de la fase avanzada de una serie de pacientes con enfermedad de Parkinson LRRK2

IntroductionLRRK2 mutations have traditionally been associated with a benign phenotype of Parkinson's disease (PD). Favourable responses to deep brain stimulation (DBS) are reported in the advanced phase.MethodsWe performed a retrospective analysis of the clinical characteristics and progression of 13 patients with LRRK2-associated PD (13 with G2019S and one with I1371 V). Nine patients were in the advanced phase, with a mean progression time of 7.2 years before reaching this phase.ResultsSeven patients underwent bilateral subthalamic DBS implantation, and two received infusion treatment. Patients with mutation G2019S responded excellently to DBS, with Unified Parkinson's disease rating scale (UPDRS) II and III scores improving by 80% at six months. This response was sustained over time. The patient with mutation I1371 V had a severe phenotype of the disease, and presented a moderate response to DBS. Patients with advanced LRRK2-associated PD showed predominantly frontal cognitive involvement, with significant language impairment.ConclusionsIn these patients, progression was faster in the advanced stage of the disease. We emphasise the suitability of subthalamic DBS in the management of these patients.     

Determinación de la temperatura de respuesta nociceptiva sobre la cola de ratones albinos de la cepa Balb/c

IntroductionDesigns for determining nociceptive response in rodents are of great use in neurology and experimental neuroscience. Immersing mice's tails in warm water is one of the most widely used procedures to evaluate this response; however, a wide range of temperatures are used in different studies. Knowing the temperature that produces a powerful nociceptive response in the tail of BALB/c mice is extremely useful.MethodsEight 2-month-old male BALB/c mice were used. A 14-cm high beaker was filled with water up to 13 cm. The animals’ tails were immersed in the container with a starting temperature of 36 °C. The water temperature was raised in 1 °C increments until we identified the temperatures that produced nociceptive responses. That response was determined by counting the time taken before the mouse shook its tail to remove it from the water.ResultsSix of the 8 mice began shaking their tails at the temperature of 51 °C. All animals removed their tails from the water at the temperatures of 54 °C, 55 °C, and 56 °C, taking a mean time of 8.54, 7.99, and 5.33 seconds, respectively. ANOVA applied to the response times for each of the 3 temperatures indicated revealed a value of F=2.8 (P=.123).ConclusionsThe response time was statistically similar for the temperatures of 54 °C, 55 °C, and 56 °C; however, the data were less dispersed for the latter temperature.     

Impacts de la combinaison de programmes de soutien à l’emploi et de remédiation cognitive sur le maintien en emploi de personnes souffrant de schizophrénie : une méta-analyse

ObjectivesAbout 80% of people suffering from schizophrenia are not working despite their desire to obtain a job. The rate of employment and job tenure remain below the average of the non-clinical population even though supported employment programs (SE) were implemented. Cognitive deficits, among other factors, could influence the occupational functioning of people suffering from schizophrenia. Given that these deficits are highly prevalent in people who have experienced multiple episodes of psychosis, numerous studies have evaluated the efficacy of integrating cognitive remediation to SE programs. Our objective was to conduct a meta-analysis to evaluate the efficacy of these combined programs (SE+) in terms of job tenure.Material and methodsWe searched the literature in several databases (Medline, Embase, Pubmed, PsycInfo, et Cochrane Library) using different keywords (“schiz*”AND “vocation*” AND “rehabilitation or recovery” AND “cogniti*” AND “random* control*”). The selection of studies was limited to those written in French or English, using a randomized-controlled trial or prospective design, including participants with a schizophrenia-spectrum diagnosis, including and presenting work outcomes (e.g., work duration, job acquisition) of a program combining SE and cognitive remediation programs. Following data extraction, we calculated the Hedges’ g effect size for each study that reported job tenure outcomes. We used a random-effects model and evaluated heterogeneity with the Cochran's Q-statistic and the I2 index. Publication bias was estimated through the use of a funnel plot, the Rosenthal's fail-safe N and Egger's asymmetry test.ResultsWe identified 12 studies that presented different SE+ programs comprising 334 and 322 persons suffering from schizophrenia assigned to treatment and control conditions, respectively. Our quantitative results suggest that combining cognitive remediation and SE programs do not significantly impact job tenure. Although our analyses suggest the presence of heterogeneity and publication bias, it is still advisable to conduct a meta-analysis because it allows circumventing the biases introduced when using the vote counting technique (i.e., simply comparing the number of positive and negative studies). Our results should thus be considered as exploratory and future meta-analyses are encouraged when a significantly larger number of studies on the subject will be published.ConclusionsVarious improvements to the reviewed programs could be implemented in order to enhance job tenure, notably by integrating other components such as social skills training or by focusing the remediation on cognitive functions more closely related to job tenure.     

Biomarcadores de enfermedad de Alzheimer en deterioro cognitivo leve: experiencia en una clínica de memoria de Latinoamérica

ObjectiveThis study aimed to investigate the role and prognosis of Alzheimer disease biomarkers in patients with mild cognitive impairment (MCI) at a memory clinic in Latin America.MethodsWe studied 89 patients with MCI, 43 with Alzheimer-type dementia, and 18 healthy controls (matched for age, sex, and educational level) at our memory clinic (Instituto FLENI) in Buenos Aires, Argentina. Patients and controls underwent an extensive demographic, neurological, and neuropsychological assessment. All subjects underwent a brain MRI scan; FDG-PET scan; amyloid PET scan; apolipoprotein E genotyping; and cerebrospinal fluid concentrations of Aβ_1-42, tau, and phosphorylated tau. Patients were categorised as positive or negative for the presence of amyloid pathology and neurodegeneration.ResultsAmyloid pathology was observed in cerebrospinal fluid results in 18% of controls, 64% of patients with MCI, and 92% of patients with Alzheimer-type dementia. Suspected non–Alzheimer disease pathophysiology was found in 11% of controls, 6% of patients with MCI, and 8% of patients with Alzheimer-type dementia. At 30 months of follow-up, 45% of amyloid-positive patients with MCI and 20% of amyloid-negative patients with MCI showed progression to dementia.ConclusionsThis study demonstrates biomarker-based MCI prognosis and supports its role in clinical decision-making in daily practice.     

Corrélats anatomocliniques au cours de la démence de la maladie de Parkinson

IntroductionCognitive disorders such as deficit of attention and executive and visuoconstructive dysfunctions occur in Parkinson's disease dementia (PDD). Memory impairment is not an early feature and statement not well delimited.Case reportA 78-year-old man with PDD underwent neuropsychological assessment and moreover demonstrated memory decline. After death, pathology examination of the brain and immunohistochemy analysis confirmed PD and showed Lewy body pathology (LBP) in the insula, limbic and especially in CA3 hippocampus areas. Hippocampus and gyrus parahippocampic also exhibited neurofibrillary tangles. Lack of senile plaque and lack of beta A4 amyloid deposition were noticeable in the whole brain examination.ConclusionSevere executive dysfunctions are probably related to LBP and dysfunction in memory process may be related to DNF lesions in medial temporal area.     

Place de la toxine botulique dans le traitement de la spasticité de l’enfant

IntroductionSpasticity is a motor disorder, which can be treated by botulinum toxin (BT). We found no studies describing BT management of spasticity in Tunisian children. The aim of our study was to determine the frequency of spastic children treated with BT in the Tunisian hospital population and to evaluate treatment efficacy.MethodsWe conducted a prospective study over a 5-year period including all children diagnosed with spasticity treated with BT and attending the “Movement Disorders and Botulinum Toxin” outpatient clinic of the National Institute of Neurology of Tunis.ResultsHundred and fifteen patients were included (31% of patients attending the “Movement Disorders and Botulinum Toxin” outpatient clinic). Mean age was 7.6 years and M:F sex ratio 1.7. Main clinical features were: spastic quadriplegia (48%), equinus deformity (70.4%) and cerebral palsy (88%). All patients were evaluated with the modified Ashworth score and were treated with BT. Other treatments were associated with BT: baclofene, physiotherapy, ortheses, plaster, and sometimes surgical treatment. The average percentage of improvement after BT was > 50%. The Ashworth score was significantly lower for the majority of injected muscles.Discussion and conclusionOur study is the first to describe BT management of spasticity in Tunisian children. Treatments of spasticity are numerous and vary according to location and extent of spasticity. BT is the main treatment for focal spasticity. Associated with physical therapy, BT allows optimal management of spastic children.     

Syndrome de Susac : étude de cinq cas

IntroductionSusac syndrome is a rare microangiopathy, responsible for small cerebral, retinal and cochlear infarcts. The classic clinical triad includes multiple neurologic signs (from headaches to coma), retinal branch occlusions and sensorineural hearing loss.MethodsWe report a series of five patients with Susac syndrome followed in our department from 1997 to 2007.ResultsThere were four women and one man (mean age at onset: 35.2 years). Clinical symptoms at onset were neurological (n = 1), ophthalmological (n = 1), auditory (n = 1) and clinical triad (n = 2). Neurologic symptoms included encephalopathy (n = 2), headache (n = 5), transient ischemic attacks (n = 1). Brain MRI showed T2 lesions in the white and grey matter, corpus callosum and gadolinium-enhanced punctiform lesions. Cerebrospinal fluid contained an elevated protein level in three cases. Immunologic treatments (steroids [n = 4], cylophosphamid [n = 3], intravenous immunoglobulins [n = 5]) associated with aspirin and/or oral anticoagulants, despite early relapses (n = 2), led to dramatic clinical improvement (n = 5).ConclusionDue to its polymorphism the SS is difficult to diagnose when the clinical triad is lacking. In the absence of clinical trial and consensus treatment is empiric and based on supposed pathogenesis.     

Profil de personnalité de femmes auteures de crimes violents selon le lien à la victime

Objectives

Despite societal changes in recent decades, the criminality of women remains far less studied than that of men, even more so when it comes to violent crime. The present study examines specific psychological factors such as personality disorders, impulsivity, dissociative symptoms as well as putative differences in demographic and background characteristics between women who have victimised a family member versus an acquaintance or a stranger.