Remote neurological evaluation reliably identifies patients eligible to endovascular therapy while non-eligible to intravenous thrombolysis.

INTRODUCTION/BACKGROUND: Early identification of suspected stroke patients who might be eligible for a reperfusion strategy is a daily challenge in the management of patient referrals. The aim of this study was to evaluate the performance of a remote medical assessment in identifying patients eligible for endovascular therapy (EVT) while not eligible for intravenous thrombolysis (IVT), compared with a decision based on bedside clinico-radiological data. METHODS: Patients admitted to the emergency department for acute neurological symptoms lasting for less than 24h were prospectively included. Assessment of the clinical severity and medical history was performed simultaneously by two vascular neurologists (VNs), one remotely using a mobile telemedicine solution (NOMADEEC), the other at the bedside. RACE score was calculated from the NIHSS score. At the end of the evaluation, both VNs quoted their treatment convictions (IVT/EVT). Final therapeutic decision following brain and vascular imaging was recorded and compared to remote and bedside predictions. The performances of three different conditions were evaluated: complete medical evaluation (NIHSS+medical history), NIHSS score alone, and RACE score alone. Remote and bedside performances were also compared. Diagnostic accuracy parameters (sensitivity, specificity, positive and negative predictive values) of each condition were estimated, along with their two-sided 95% binomial confidence intervals. RESULTS: Out of 215 enrolled patients, 186 had a complete evaluation, 91 (54.3%) were diagnosed with an ischemic stroke or transient ischemic attack and 46 (24.7%) had an intracranial occlusion. Considering the three conditions evaluated remotely, RACE score-based decision provided the best sensitivity 54.6% [95% CI 23.4; 83.2]/specificity 80.6% [73.9; 86.2] combination. However, the complete medical evaluation had the best specificity (88.6% [82.9; 92.9] compared to RACE scores alone (P=0.038). Remote and bedside performances did not differ (κ=0.68 [0.59; 0.77]). DISCUSSION/CONCLUSION: This real-life study performed in the setting of emergency demonstrates that remote medical evaluations including recording of extensive medical information and NIHSS examination to address patient's eligibility to revascularization treatment is swiftly feasible and is as effective as bedside prediction to EVT and/or IVT. Remote standardized medical evaluation might improve the decision of patients' primary orientation and avoid overcrowding of comprehensive stroke centres.

High glycemic variability: An underestimated determinant of stroke functional outcome following large vessel occlusion.

BACKGROUND AND PURPOSE: Early glycemic variability (GV) in diabetic patients is a poor prognosis factor following cardiovascular events. However, its influence on the course of acute ischemic stroke (AIS) with large vessel occlusion remains unclear. We investigated the relationship between high GV during acute stroke and three-month functional outcome among patients treated with combined intravenous thrombolysis and endovascular therapy for large vessel occlusion. METHODS: A single-center retrospective analysis of AIS patients with proximal intracranial occlusion who underwent thrombolysis and mechanical thrombectomy between January 2015 and May 2017. Early GV was assessed using standard deviation (SD) of blood glucose levels for the first 24hours. The main outcome was functional status at three months as defined by the modified Rankin scale (mRS). Secondary outcomes were change in NIHSS score from baseline to 24hours and occurrence of severe hemorrhagic transformation. Multivariate logistic regression analyses including GV, admission glycemia and mean glycemia were performed. RESULTS: Among the 93 patients evaluated, 26 had early high GV (≥20.9mg/dl). High GV was associated with poor functional outcome (OR=8.00; 95%CI [1.34-47.89]; P=0.02) unlike admission glycemia and mean glycemia (OR=2.92; 95%CI [0.51-16.60]; P=0.23 and OR=0.36; 95%CI [0.05-2.6]; p=0.31, respectively). High GV was not associated with NIHSS at 24hours or hemorrhagic transformation. CONCLUSION: Acute high GV contributes to poorer functional outcome following AIS related to large vessel occlusion and should be considered as a new target in acute stroke management.

Influence of inflammatory status in the acute phase of stroke on post-stroke depression.

BACKGROUND: Thirty percent of stroke patients will suffer from post-stroke depression (PSD). Recent data suggest that inflammation accounts for a substantial amount of depression. Our primary objective was to assess the association between standard inflammation biomarkers in the acute phase of stroke and PSD at three months. The secondary objective was to elaborate a predictive model of PSD from clinical, biological and radiological data. METHODS: We performed a retrospective analysis of a single-centre cohort of stroke patients with a three-month follow-up. Serum levels of C-reactive protein (CRP), fibrinogen, leukocyte count and neutrophil to lymphocyte ratio (NLR) were tested at admission and at peak. Mood was assessed at three months using the depression sub-scale of the Hospital Anxiety and Depression Scale (HADS). Association between inflammation biomarkers and HADS was evaluated with multi-linear regression adjusted on clinical and radiological parameters. Logistic predictive models of PSD at three months, with and without inflammation biomarkers, were compared. RESULTS: Three hundred and forty-eight patients were included, of whom 20.06% developed PSD. Baseline and peak values of all inflammatory markers were associated with the severity of PSD at three months. Area under the curve for the receiver operating characteristic curve of PSD prediction was 0.746 (CI 95% 0.592-0.803) with selected inflammation biomarkers and 0.744 (CI 95% 0.587-0.799) without. CONCLUSION: Most inflammation biomarkers are weakly associated with PSD, adding negligible value to predictive models. While they suggest the implication of inflammation in PSD pathogenesis, they are useless for the prediction of PSD, underscoring the need for more specific biomarkers.

Contribution of routine cardiac biological markers to the etiological workup of ischemic stroke.

BACKGROUND: Optimization of the detection of atrial fibrillation following stroke is mandatory. Unfortunately, access to long-term cardiac monitoring is limited in many centers. The aim of this study was to assess the potential usefulness of three routine biological markers, troponin, D-dimers and BNP, measured in acute stroke phase in the selection of patients at risk of cardio-embolic stroke. METHODS: Troponin, D-Dimers and BNP were measured within 48 h after admission for ischemic stroke in 634 patients. Stroke mechanism was defined at the 3 months follow-up visit using ASCOD classification using a standardized work-up. Association between clinical, radiological and biological markers and stroke mechanism was evaluated using logistic regression analyses. RESULTS: 159 patients (25.1% of total study population) had a cardiac mechanism. On multivariate analysis, admission initial stroke severity (OR 1.04, 95 CI% 1.004-1.07, p < 0.05) history of heart failure (OR 3.03, 95% CI 1.19-7.73, p < 0.05), ECG abnormalities and high BNP value (OR 4.34, 95% CI 2.59-7.29, p < 0.05) were associated with pure cardiac stroke mechanism. CONCLUSION: High BNP value measured within 48 h after stroke admission is an independent predictor of cardiac stroke mechanism. Its measurement might be used to improve the selection of patients for whom further cardiologic investigations such as continuous long term ECG monitoring would be the most useful. BNP should be added to the standard admission-work-up for stroke patients.

Evaluation of the application of the European guidelines for the diagnosis and clinical care of amyotrophic lateral sclerosis (ALS) patients in six French ALS centres.

BACKGROUND AND PURPOSE: Our objective was to evaluate the extent to which the 2005 recommendations of the European Federation of Neurological Sciences (EFNS) on the multidisciplinary management of amyotrophic lateral sclerosis (ALS) are followed in clinical practice. METHODS: This was a multicentre observational study involving six French ALS referral centres receiving prevalent and incident cases. Recommendations were translated into ad hoc questions referring to key aspects of management, and their application was evaluated by a clinical research assistant who independently examined the medical charts (MCs). When necessary, an independent board-certified neurologist answered the questions based on examination of the MC and interview of the caring neurologist. Questions regarding diagnosis and communication were put to patients in a self-administered questionnaire. RESULTS: In all, 376 patients [176 incident, 200 prevalent cases; median age at diagnosis 62.8 years (interquartile range 55.7-72.3); sex ratio 1.37; 27.3% bulbar onset] were included. All the topics covered in the recommendations were evaluated: diagnostic delay (e.g. mean 13.6 months, associated with age and onset); breaking the news (e.g. criteria for communication quality were satisfactory in more than 90%); multidisciplinary and sustained support (e.g. clinic visits were scheduled every 2-3 months in 90%). Also considered were whether riluzole had been offered, symptom management, genetic testing, ventilation, communication defects, enteral nutrition, palliative and end-of-life care. Characteristics associated with poor compliance with some guidelines (schedule of visits, delayed riluzole initiation) were also identified. CONCLUSION: This is the first evaluation of the application of the EFNS recommendations for the management of ALS in a nationwide sample. The results allow us to highlight areas for improvement.

The impact of intravenous thrombolysis on outcome of patients with acute ischemic stroke after 90 years old.

BACKGROUND: Age increases the risk of mortality and poor prognosis following stroke. The benefit of intravenous thrombolysis in very old patients remains uncertain. The purpose of the study was to evaluate the efficacy and safety of thrombolysis in very old patients considering their perfusion-imaging profile. METHODS: We conducted a retrospective study including patients older than 90 y.o. admitted for an acute ischemic stroke. A computed tomography perfusion-imaging (CTP) was performed in patients who received thrombolysis. Primary outcome was the functional status at 3 months, assessed by the modified Rankin scale (mRS). Secondary outcomes were the rate of hemorrhagic transformations, duration of hospitalization and the rate of death in the first 7 days. Patients receiving thrombolysis were compared with an age-matched group of non-thrombolysed patients. RESULTS: 78 patients were included (31 % male, aged 92 ± 1.7 y.o). 37 patients received thrombolysis and among them, 30 had CTP with a mismatch. The three months mRS was not significantly different in the two groups (mRS 0-2: 5 % and 7 % in the thrombolysed and non-thrombolysed group, respectively). Hemorrhagic transformations were more frequent in the thrombolysed group (54 % versus 12 %, p = 0.002) and symptomatic intracranial hemorrhage tended to be associated with mRS at three months and death in the first 7 days. Duration of hospitalization was longer in the thrombolysed group (10 days ± 12 versus 7 days ± 9, p = 0.046). CONCLUSIONS: Patients who received thrombolysis did not have a better functional prognosis than non-thrombolysed patients.

Lucid dreams, an atypical sleep disturbance in anterior and mediodorsal thalamic strokes.

BACKGROUND: Cognitive, affective, and behavioural disturbances are commonly reported following thalamic strokes. Conversely, sleep disorders are rarely reported in this context. OBSERVATIONS: Herein, we report the cases of two young patients admitted for an ischemic stroke located in the territories of the left pre-mammillary and paramedian arteries. Together with aphasia, memory complaint, impaired attention and executive functions, they reported lucid dreams with catastrophic content or conflicting situations. CONCLUSION: Lucid dreams are an atypical presentation in thalamic strokes. These cases enlarge the clinical spectrum of sleep-wake disturbances potentially observed after an acute cerebrovascular event.

Caseous calcification of the mitral annulus associated with stroke: report of two cases.

OBJECTIVE: Caseous calcification of the mitral annulus (CCAM) is a rare complication of mitral annulus calcification (MAC). Whether CCAM should be considered as a cardiac source of stroke or a simple marker of atherosclerosis remains a matter of debate. METHOD: Herein, we report two patients with stroke and CCAM. RESULTS: The first one was associated with extensive aortic arch atheroma, while CCAM was the only potential cause in the second case. Transthoracic echocardiography was normal in both cases and CCAM was diagnosed on transesophageal echocardiography. CONCLUSION: These observations suggest that CCAM should be added to the list of minor cardioembolic sources of stroke but also requires a perfect control of vascular risk factors.

[Intracranial hemorrhage in three haemophilic adults: A severe complication of hemophilia]. / Hémorragie intracérébrale chez trois adultes hémophiles : une complication sévère de l'hémophilie.

BACKGROUND: Intracranial hemorrhage (ICH) complications are uncommon at hemophilic patients. OBSERVATIONS: We report three cases of ICH occurring in hemophilic patients. Contributing factors were identified in two patients: hemophilia severity, presence of inhibitor, hepatitis C virus infection, and high blood pressure. No contributing factor was identified in the last patient. CONCLUSION: Rapid diagnosis of ICH is crucial in hemophilic patients. A search for contributing factors, both those specific to hemophilia, and those favoring ICH in the general population, is essential to optimize therapeutic care. Specific substitutive treatment for the deficient factor is a difficult management challenge.

Atraumatic nonaneurysmal sulcal subarachnoid hemorrhages: a diagnostic workup based on a case series.

INTRODUCTION: Atraumatic and nonaneurysmal sulcal subarachnoid hemorrhage (sSAH) is a rare type of cerebrovascular disease with various etiologies previously reported in small case reports. In this study, we propose to analyze clinical presentations, imaging patterns and etiologies in a large case series of such patients in order to propose a diagnostic workup. METHODS: We retrospectively analyzed clinical and radiological data of consecutive patients with a diagnosis of atraumatic and nonaneurysmal sSAH, admitted to our institution between 2008 and 2011. All patients had both computed tomography (CT) and magnetic resonance imaging (MRI) as a part of their initial evaluation. RESULTS: 30 patients (18 women and 12 men, mean age: 60 years) were identified. The main clinical symptoms at presentation were focal and transient neurological deficit (n = 22) and thunderclap headache (n = 10). Four patients had progressive headache and 4 other had partial or generalized epileptic seizures. MRI abnormalities associated with sSAH were prior hemorrhages, microbleeds, severe leukoencephalopathy and hemosiderosis suggesting cerebral amyloid angiopathy (CAA; n = 9), vasogenic edema in parieto-occipital areas compatible with a posterior reversible encephalopathy syndrome (PRES; n = 3), cortical venous thrombosis (n = 2) and concomitant acute cortical stroke (n = 3). Other underlying causes of sSAH, not diagnosed on MRI, were reversible cerebral vasoconstriction syndrome (RCVS) based on clinical criteria and conventional angiography (n = 4), angiitis diagnosed by skin biopsy (n = 1), vascular malformation diagnosed on CT and digital subtraction angiographies (n = 3), and overanticoagulation (n = 1). Four cases remained unresolved. CONCLUSION: This study confirmed that sSAH is a rare condition related to a wide spectrum of etiologies. Combination of brain MRI and magnetic resonance angiography and eventually digital subtraction angiography allowed the identification of an underlying etiology for 87% of patients. CAA, RCVS and PRES represented more than 50% of the etiological mechanisms. Among older patients, sSAH was mainly related to CAA while in younger patients, RCVS represented the most frequent etiology.

Bilateral periventricular nodular heterotopia in France: frequency of mutations in FLNA, phenotypic heterogeneity and spectrum of mutations.

Bilateral periventricular nodular heterotopia (BPNH) is the most common form of periventricular heterotopia. Mutations in FLNA, encoding filamin A, are responsible for the X linked dominant form of BPNH (FLNA-BPNH). Recently, atypical phenotypes including BPNH with Ehlers-Danlos syndrome (BPNH-EDS) have been recognised. A total of 44 FLNA mutations have so far been reported in this phenotype. Most of these mutations lead to a truncated protein, but few missense mutations have also been described. Here, the results of a mutation screening conducted in a series of 32 BPNH patients with the identification of 12 novel point mutations in 15 patients are reported. Nine mutations were truncating, while three were missense. Three additional patients with BPNH-EDS and a mutation in FLNA are described. No phenotype-genotype correlations could be established, but these clinical data sustain the importance of cardiovascular monitoring in FLNA-BPNH patients.

[Actinomycosis: an unusual cause of spinal cord compression. Case report and review of the literature]. / Actinomycose : une cause rare de compression médullaire.

Actinomycosis is an unusual and unrecognized cause of spinal cord compression of infectious origin. We report the case of a 57-year-old immunocompetent woman admitted for sub-acute lower limb ataxia. The diagnosis of spinal cord compression secondary to actinomyces infectious arthritis was established. Surgical decompression and long-term antibiotic treatment enabled complete recovery. Data from the literature indicate that actinomycosis is a potential cause of several neurological manifestations. Unusual but treatable, actinomycosis is a potential alternative when the main etiologies have been ruled out.

Atrial fibrillation is a major cause of stroke in apneic patients: a prospective study.

OBJECTIVES: Large prospective studies have established that sleep-disordered breathing (SDB) is associated with an increased incidence of cardiovascular and cerebrovascular diseases. Heterogeneous results have been published about SDB and ischaemic stroke mechanism. The aim of this study was to evaluate the relationship between poststroke apneic syndrome and stroke aetiologies according to the ASCO classification. METHODS: A total of 134 patients with acute ischaemic cerebrovascular events were prospectively included. Patients with severe or infratentorial infarctions were excluded. Stroke risk factors and clinical characteristics were recorded in all patients. An overnight polygraphy was recorded (Embletta PDS). Apneic patients were defined if the polygraphy reported an apnea-hypopnea index ≥15. A standardized diagnosis workup, including serum biological investigation, evaluation of extra and intracranial arteries and heart morphology and function, was performed. Stroke mechanism was defined using ASCO and TOAST classifications. RESULTS: A proportion of 42% (56 of 134) of the patients were diagnosed as having sleep apneas. Apneic patients were older (p < 0.001), had higher BMI (p = 0.02), and more were hypertensive (p < 0.001). Using ASCO classification, a major cardioembolic source of stroke, mainly atrial fibrillation, was more frequently observed in apneic patients (41.1% vs. 20.5%, p < 0.05), while no difference was observed when considering atherosclerosis or small-vessel disease mechanisms. Univariate analysis showed that cardioembolic stroke depends on sex, age, left atrial size and OSA; however, age remained the only significant factor in multivariate analysis. CONCLUSION: This study confirms the high prevalence of sleep apneas in stroke-affected patients and identifies atrial fibrillation as a major source of stroke in this population. The strong correlation between age and SDB seems to drive the increased frequency of stroke related to atrial fibrillation in this population.

[Cerebral infraction by calcified embolism: a spontaneous complication of calcified aortic stenosis]. / Infarctus cérébral par embolie calcaire: complication spontanée révélatrice d'un rétrécissement aortique calcifié.

Calcified aortic stenosis (CAS) is an unusual cause of cerebral infarct. The presence of cerebral intra-vascular or intra-parenchymatous calcifications, symptomatic or not, is suggestive of the diagnosis of CAS. We report two patients who experienced stroke induced by spontaneous calcic emboli from a calcified aortic valve and underline the importance of brain CT scan.

Leber's optic neuropathy associated with disseminated white matter disease: a case report and review.

Leber's hereditary optic neuropathy (LHON), a mitochondrial disease, is clinically characterized by a bilateral subacute loss of central vision consecutive to optic nerve involvement. In some cases of LHON, neurological features are reported including multiple sclerosis-like (MSL) phenotype. We report one additional male patient displaying LHON-MSL associated with the prevalent G11778A mutation and review the cases with expendable data published so far in the literature. We discuss the respective roles of inflammation and energetic metabolism dysregulation in the development of brain lesions. We propose to treat these patients early with both antioxidative and immunosuppressive drugs in order to avoid further handicap.