Medical demography at stroke centers: Current situation in France.

INTRODUCTION: Following the 2010-2014 French national stroke action plan, the number of stroke center (SC) has gradually increased in France, allowing a homogeneous coverage and access to neurovascular care in organized and territorially defined structures. However, operational difficulties within SCs have been progressively reported over the last few years. The objective of this study was to identify the medical staff shortages in SC that may contribute to these difficulties. METHODS: A survey on the medical staffing level as of January 1, 2021 was sent to all French SC managers. Specific questions related on vacancies, need of interim medical staff, and participation in out-of-hour healthcare services. RESULTS: Among the 139 SC managers contacted, 122 (88%) filled in the questionnaire. Analysis of the data showed that over 879 physician positions opened, 163 (18.5%) remained vacant for a mean of two years, and that in 51 SCs (41.9%), more than two positions were unfilled. In 13 of these 51 SCs, the out-of-hour healthcare services relied on less than four practitioners, defining a critical situation, and three other SCs had to close temporarily (2) or permanently (1). Moreover, 39.2% of SCs with at least one vacancy used interim physicians, for a median period of 12.5 weeks/year (IQR 5-18). CONCLUSION: This study highlights the significant medical staff shortage in French SCs. In the absence of urgent measures, more SCs will close, jeopardizing the regional network and access to care for stroke patients.

Anatomical and neurophysiological basis of face recognition.

Face recognition is a highly developed and efficient human function that involves multiple neural networks. A main pathway links the occipital cortex, where an occipital face area (OFA) has been identified, to a fusiform face area (FFA) in the fusiform gyrus, which plays a critical role in face recognition. This core pathway deals with invariant aspects of the face. Another pathway, including the superior temporal sulcus, is involved in the perception of more changeable aspects of the face such as gaze orientation, face expression and lip movements. It has been defined by some authors as a "third pathway of visual recognition", i.e. a lateral pathway in addition to the "what" and "where" pathways. It deals with sociocognitive aspects of face perception. Many other accessory functional systems are connected to the core system of visual recognition to act in concert with it: the intraparietal sulcus (for the management of spatial attention), the primary auditory cortex (prelexical perception of speech), the amygdala, the insula and the limbic system (perception of emotions), the anterior temporal pole (access to the identity of the individual, his name, biographical information), etc. Functional brain imaging has made remarkable progress in the understanding of face perception, which in the early years was limited to the description of single cases of brain-damaged patients. This progress has made it possible to better analyse the many face recognition disorders, sometimes subtle, other times confusing, observed in human pathology.

Stroke Thrombectomy in Patients with COVID-19: Initial Experience in 13 Cases.

We performed a retrospective review in both comprehensive stroke units of a region affected early by the coronavirus disease 2019 (COVID-19) pandemic, between March 1 and April 26, 2020, including patients with COVID-19 who underwent mechanical thrombectomy for ischemic stroke. We identified 13 cases, representing 38.2% of 34 thrombectomies performed during this period. We observed increased mortality and a high incidence of thrombotic complications during hospitalization. Given the high rate of infected patients, systematic use of full personal protection measures seems justified.

Impact of the COVID-19 outbreak on acute stroke pathways - insights from the Alsace region in France.

BACKGROUND AND PURPOSE: To date, no study has attempted to quantify the impact of the COVID-19 outbreak on the incidence and treatment of acute stroke. METHODS: This was a retrospective review of acute stroke pathway parameters in all three stroke units in the Alsace region during the first month of the outbreak (1-31 March 2020), using the similar period from 2019 as a comparator. A secondary detailed analysis of all stroke alerts and stroke unit admissions was performed in the centre with the largest case volume. RESULTS: Compared to the same period in 2019, in March 2020 there were 39.6% fewer stroke alerts and 33.3% fewer acute revascularization treatments [40.9% less intravenous thrombolysis (IVT) and 27.6% less mechanical thrombectomy (MT)]. No marked variation was observed in the number of stroke unit admissions (-0.6%). The proportion of patients with acute revascularization treatments (IVT or MT) out of the total number of stroke unit admissions was significantly lower in March 2020 (21.3%) compared to 2019 (31.8%), P = 0.034. There were no significant differences in time delays or severity of clinical symptoms for patients treated by IVT or MT, nor in the distribution of final diagnosis amongst stroke alerts and stroke unit admissions. CONCLUSION: These results suggest that the overall incidence of stroke remained the same, but fewer patients presented within the therapeutic time window. Increased public awareness and corrective measures are needed to mitigate the deleterious effects of the COVID-19 outbreak on acute stroke care.

Cryptosporidiosis after treatment with fingolimod: a case report and pharmacovigilance review.

BACKGROUND: Cryptosporidium sp. are common intracellular parasites responsible of severe diarrhea in T-cell-immunocompromised patients. We report the first case of a woman who contracted cryptosporidiosis after treatment with fingolimod, a drug labeled for multiple sclerosis and responsible for marked lymphopenia. CASE PRESENTATION: A 60-year-old woman was admitted for abdominal pain diarrhea and fever. The patient suffered from multiple sclerosis and had been treated with fingolimod from august 2017 to september 2018 time of occurrence of the first digestive symptoms. Stool culture was negative but parasitological examination was positive for Cryptosporidium sp. Blood biological examination profound lymphopenia of 240/mm3 [17 CD4/mm3 (7%) and 32 CD8/mm3 (14%)]. Fingolimod was stopped, and the patient was put on nitazoxanide 500 mg bid for 7 days. The diarrhea resolved and no relapse was observed. Six other cases were found in the Pharmacovigilance database. CONCLUSION: Physicians should be aware of this association and screen for Cryptosporidium in cases of diarrhea in patients treated with fingolimod. Patients should be aware of this risk and advise to take appropriate measures to avoid such contamination.

Lyme borreliosis and other tick-borne diseases. Guidelines from the French scientific societies (II). Biological diagnosis, treatment, persistent symptoms after documented or suspected Lyme borreliosis.

The serodiagnosis of Lyme borreliosis is based on a two-tier strategy: a screening test using an immunoenzymatic technique (ELISA), followed if positive by a confirmatory test with a western blot technique for its better specificity. Lyme serology has poor sensitivity (30-40%) for erythema migrans and should not be performed. The seroconversion occurs after approximately 6 weeks, with IgG detection (sensitivity and specificity both>90%). Serological follow-up is not recommended as therapeutic success is defined by clinical criteria only. For neuroborreliosis, it is recommended to simultaneously perform ELISA tests in samples of blood and cerebrospinal fluid to test for intrathecal synthesis of Lyme antibodies. Given the continuum between early localized and disseminated borreliosis, and the efficacy of doxycycline for the treatment of neuroborreliosis, doxycycline is preferred as the first-line regimen of erythema migrans (duration, 14 days; alternative: amoxicillin) and neuroborreliosis (duration, 14 days if early, 21 days if late; alternative: ceftriaxone). Treatment of articular manifestations of Lyme borreliosis is based on doxycycline, ceftriaxone, or amoxicillin for 28 days. Patients with persistent symptoms after appropriate treatment of Lyme borreliosis should not be prescribed repeated or prolonged antibacterial treatment. Some patients present with persistent and pleomorphic symptoms after documented or suspected Lyme borreliosis. Another condition is eventually diagnosed in 80% of them.

Lyme borreliosis and other tick-borne diseases. Guidelines from the French Scientific Societies (I): prevention, epidemiology, diagnosis.

Lyme borreliosis is transmitted en France by the tick Ixodes ricinus, endemic in metropolitan France. In the absence of vaccine licensed for use in humans, primary prevention mostly relies on mechanical protection (clothes covering most parts of the body) that may be completed by chemical protection (repulsives). Secondary prevention relies on early detection of ticks after exposure, and mechanical extraction. There is currently no situation in France when prophylactic antibiotics would be recommended. The incidence of Lyme borreliosis in France, estimated through a network of general practitioners (réseau Sentinelles), and nationwide coding system for hospital stays, has not significantly changed between 2009 and 2017, with a mean incidence estimated at 53 cases/100,000 inhabitants/year, leading to 1.3 hospital admission/100,000 inhabitants/year. Other tick-borne diseases are much more seldom in France: tick-borne encephalitis (around 20 cases/year), spotted-fever rickettsiosis (primarily mediterranean spotted fever, around 10 cases/year), tularemia (50-100 cases/year, of which 20% are transmitted by ticks), human granulocytic anaplasmosis (<10 cases/year), and babesiosis (<5 cases/year). The main circumstances of diagnosis for Lyme borreliosis are cutaneous manifestations (primarily erythema migrans, much more rarely borrelial lymphocytoma and atrophic chronic acrodermatitis), neurological (<15% of cases, mostly meningoradiculitis and cranial nerve palsy, especially facial nerve) and rheumatologic (mostly knee monoarthritis, with recurrences). Cardiac and ophtalmologic manifestations are very rarely encountered.

Cervico-facial dystonia as depicted in sculpture before its scientific description.

The authors describe a sculpture from Daumier, called "Le Hargneux" (The peevish one), whose physiognomic study evokes hitherto unrecognized cranial-cervical dystonia. It is probably the first representation of dystonia in sculpture, before its scientific identification by Horatio Wood, in 1887.

Myelin oligodendrocyte glycoprotein antibodies in neuromyelitis optica spectrum disorder.

Neuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disease of the central nervous system characterized, in particular, by disabling episodes of optic neuritis and longitudinal extensive transverse myelitis. Its main pathogenic characteristic is the presence of anti-aquaporin-4 antibodies (AQP4-Abs) in the serum of affected patients. However, a proportion of patients with the typical NMOSD phenotype are, in fact, negative (seronegative) for AQP4-Abs and, within this category of patients, a proportion of them instead express antibodies to myelin oligodendrocyte glycoprotein (MOG-Abs). The presence of MOG-Abs in the sera of seronegative NMOSD patients is more frequently associated with monophasic disease and moderate symptom severity, and also appears to predict a better outcome. The present report is a review of the clinical and immunological features of MOG-Ab-positive NMOSD.

Do we know how to diagnose epilepsy early in Alzheimer's disease?

Epilepsy is an increasingly recognized comorbidity in Alzheimer's disease (AD). First described as generalized in dementia patients, epileptic AD patients are nowadays fully described in earlier stages of the disease (with mild or subjective cognitive impairment). At such early stages, patients may present not only with generalized seizures, but also with focal seizures (commonly localized in the frontal or temporal lobe). Thus, partial or generalized epilepsy is part of the semiological spectrum of AD that should be borne in mind at all stages of disease to ensure early identification and prevent the risk of repeated seizures (such as accidents, injury, progression of cognitive impairment). This review of the available (and still growing) literature shows that there are already sufficient data to inform physicians on seizure semiology, and on the diagnostic value of electroencephalography and brain imaging. Taken together, these tools can help to rapidly identify epilepsy in AD patients. Nevertheless, epilepsy diagnosis can be challenging, and test medication is sometimes necessary. Some cerebrospinal fluid biomarkers (or their ratios) may also prove to be good predictors of seizures in AD, but further studies are needed. Epilepsy in AD patients is frequently pharmacosensitive, and a good response can be obtained with standard doses of antiepileptic drugs. For all these reasons and based on our review of the literature, it appears that, at present, the diagnosis of epilepsy in AD is not only possible at any stage of the disease, but also to be recommended to improve the patient's prognosis.

French consensus procedure for assessing cognitive function in Parkinson's disease.

INTRODUCTION: One of the objectives of the French expert centers for Parkinson's disease (NS-Park) network was to determine a consensus procedure for assessing cognitive function in patients with Parkinson's. This article presents this procedure and briefly describes the selected tests. METHODS: A group of 13 experts used the Delphi method for consensus building to define the overall structure and components of the assessment procedure. For inclusion in the battery, tests had to be validated in the French language, require little motor participation, have normative data and be recognized by the international community. Experimental tasks and tests requiring specific devices were excluded. RESULTS: Two possibilities were identified, depending on whether an abbreviated or comprehensive assessment of cognitive function was necessary. For an abbreviated assessment, the experts recommended the Montreal Cognitive Assessment (MoCA) as a screening test for cognitive impairment or dementia. For a comprehensive neuropsychological assessment, the experts recommended assessing global efficiency plus the five main cognitive domains (attention and working memory, executive function, episodic memory, visuospatial function and language) that may be impaired in Parkinson's disease, using two tests for each domain. DISCUSSION AND CONCLUSION: A common procedure for assessing cognitive function is now available across the French network dedicated to Parkinson's disease, and is recommended for both research and clinical practice. It will also help to promote standardization of the neuropsychological assessment of Parkinson's disease.

[American Academy of Neurology, Washington, 18-25 April 2015]. / American Academy of Neurology, Washington, 18-25 avril 2015.

CEREBROVASCULAR DISEASES: The benefit of the thrombectomy using stents retrievers in the acute stroke phase is now demonstrated when there is a proximal occlusion of an intracranial artery, whatever its mechanism. The place of the anticoagulants in the management of cervical artery dissections remains uncertain, while the benefit of the blood pressure control in the secondary prevention of deep and lobar intracerebral hemorrhages is critical. The development of cardiac MRI, prolonged cardiac monitoring and transcranial doppler seems to improve the diagnosis of cardio-embolic sources of stroke. EPILEPSY: A specialized urgent-access single seizure clinic represents a model which reduces wait-times and improves patient access after a first fit. Co-locating a psychiatrist within outpatient epilepsy center leads to a reduction in psychiatric symptoms and people with psychogenic non-epileptic seizures. When neurologists around the world assess identical case scenarios for the diagnosis of epilepsy, concordance is between moderate and poor, showing that epilepsy diagnosis remains difficult. More than one third of elderly with new-onset epilepsy of unknown etiology exhibit temporal lobe atrophy on brain imaging. MOVEMENT DISORDERS: There is no major progress in the therapeutic approach of Parkinson's disease but the discovery of new genetic markers such as glucocerebrosidase mutations may greatly change our knowledge of the disease process and may induce new therapeutic strategies in the future. The natural history of the disease is also better understood from the prodromal phase to the post-mortem analysis of the brain and the classification of the processes based on abnormal protein deposits. DEMENTIA: The respective value of biomarkers (amyloid imaging versus CSF biomarkers) for in vivo diagnosis of Alzheimer's disease (AD) has been detailed. Therapeutic expectations mainly rely on anti-Aß immunization trials performed in preclinical (and no longer prodromal) stages of AD, with the aim of slowing the evolution of neuronal loss. Besides a lot of communications on dementia genetics or physiopathogeny, fascinating and promising results were presented on deep brain stimulation for depression resistant to medical treatment. PERIPHERAL NEUROPATHY: Ibudilast, administered with riluzole, is safe and tolerable in patients with amyotrophic lateral sclerosis (ALS), improves ALS function and delays progression. Patients with painful small fiber neuropathy have a high rate of mutations in the SCN9A gene, coding for Nav1.7 voltage-gated sodium-channels. Peripheral nerve lymphoma (NL) is a multifocal painful neuropathy that causes endoneurial inflammatory demyelination: primary NL is less severe than secondary NL, which occurs after remission, suggesting that nerve may be considered a "safe lymphoma haven". MULTIPLE SCLEROSIS (MS): Biotin in progressive forms of MS and daclizumab in relapsing-remitting forms appear to be promising treatments. In case of failure of current first-line and/or second-line therapeutics, alemtuzumab may be an interesting alternative treatment. Teriflunomide, dimethyl fumarate and fingolimod are oral treatments with confirmed efficacy and acceptable safety. Besides vitamin D insufficiency and smoking, which are confirmed risk factors for the disease, testosterone insufficiency (in males) and obesity are emerging risk factors, which could also be corrected.

Creatividad pictórica en demencia frontotemporal / Pictorial creativity in fronto-temporal dementia

Algunos pacientes con demencia frontotemporal (DFT) pueden presentar una notable conservación de su creatividad artística y, más a menudo, otros, en ausencia de antecedentes artísticos, desarrollan habilidades artísticas de novo. La creatividad artística se basa en mecanismos complejos que incluyen: el descubrimiento de la capacidad y necesidad de producir, el período de incubación, la percepción y la producción artística per se. La capacidad de copiar y las funciones visuoperceptivas están ampliamente conservadas en la DFT. Como muchos de los que desarrollan una creatividad artística tienen una difusión temporal anterior unilateral, junto con un deterioro del lenguaje y de las funciones sociales, su creatividad se ha conceptualizado como <>, es decir, la liberación del área posterior derecha del neocórtex implicada en la integración heteromodal y multisensorial. Sin embargo, aunque la creatividad se debe también a la confluencia de varias funciones altamente integradas, relacionadas con la funcionalidad prefrontal, tales como la memoria de trabajo, la atención mantenida, la planificación y la flexibilidad cognitiva, el incremento de dicha creatividad después de una lesión prefrontal resulta desconcertante. Esto puede ser explicado por el papel de la hipofrontalidad en un tipo de producción que es más intuitiva, menos ligada a las convenciones cognitivas, sociales y académicas. La difusión prefrontal y la del lenguaje podrían explicar el cambio en los puntos de vista de estos pacientes pero también en su conducta, marcada por una urgencia en producir, combinada con una reducción de las áreas de interés a la hora de realizar su trabajo (AU)

Some fronto-temporal dementia (FTD) patients may show remarkable preservation of their artistic creativity, and more often, in spite of the absence of an artistic background, others develop de novo artistic skills. Artistic creativity relies on complex mechanisms wich encompass the revelation of an ability and a need to produce, an incubation preiod, insight and artistic production per se. Copying skills and visuo-perceptive functions are largely preserved in FTD. As many of those who develop artistic creativity have unilateral anterior temporal dysfunction together with impaired language and social function, their creativity has been conceptualised as a'paradoxical facilitation', i.e., the release of right posterior neocortial areas, implicated in heteromodal and polysensory integration. However, although creativity stems as well from the confluence of several highly integrated functions such as working memory, sustained attention, planning, and cognitive flexibility, which are related to prefrontal functioning, its enhancement following prefrontal injury is puzzling. It may be explained by the role of hypofrontality in a type production which is more intuitive, less bound to cognitive, social and acedemic conventions. Prefrontal and language dysfunction may also explain the change in these patients'viewpoints but also in their behaviour, maked by an urge to produce combined with a contraction of their scope of interest in the accomplishment of their artistic work (AU)