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Background and Purpose: In the settings of thrombectomy, the first-pass effect (FPE), defined by a complete recanalization after one pass with no rescue therapy, has been shown to be associated with an improved outcome. As this phenomenon has been predominantly described in anterior circulation strokes, we aimed to study the prevalence, outcomes, and predictors of FPE in patients with a basilar artery occlusion. Methods: From a prospective multicentric registry, we collected the data of all consecutive basilar artery occlusion patients who underwent thrombectomy and compared the outcomes of patients who achieved FPE and those who did not. We also compared FPE patients with those who achieved a complete recanalization with >1 pass. Finally, a multivariate analysis was performed to determine the predictors of FPE. Results: Data from 280 patients were analyzed in our study, including 84 of 280 patients (30%) with an atheromatous etiology. An FPE was achieved in 93 patients (33.2%), with a significantly higher proportion of good outcomes (modified Rankin Scale score 0-2 at 3 months) and lower mortality than non-FPE patients. An FPE was also associated with improved outcomes compared with patients who went on to have full recanalization with >1 pass. Contact aspiration as first-line strategy was a strong predictor of FPE, whereas baseline antiplatelets and atheromatous etiology were negative predictors. Conclusions: In our study, an FPE was achieved in approximately one-third of patients with a basilar artery occlusion and was associated with improved outcomes. More research is needed to improve devices and techniques to increase the incidence of FPE. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03776877.

ObjectivesTo assess the prevalence and the cumulative incidence of dementia at short-, medium- and long-term follow-up after deep brain stimulation (DBS) of the subthalamic nucleus (STN) (at 1, 5, and 10 years) and to evaluate potential risk factors for postoperative dementia.MethodsThe presence of dementia (according to the DSM-V) was retrospectively evaluated at each postoperative follow-up in patients with Parkinson disease (PD) who underwent bilateral STN-DBS. Preoperative and perioperative risk factors of developing postoperative dementia were also investigated. Demographic data, disease features, medications, comorbidities, nonmotor symptoms, PD motor scales, neuropsychological scales at baseline, and perioperative complications were collected for each patient.ResultsA total of 175 patients were included, and 104 were available at 10-year follow-up. Dementia prevalence was 2.3% at 1 year, 8.5% at 5 years, and 29.8% at 10 years. Dementia cumulative incidence at 1, 5, and 10 years was 2.3%, 10.9%, and 25.7%, respectively. The corresponding dementia incidence rate was 35.6 per 1,000 person-years. Male sex, higher age, hallucinations, lower frontal score at baseline, and perioperative cerebral hemorrhage were predictors of dementia.ConclusionsIn patients with PD with longstanding STN-DBS, dementia prevalence and incidence are not higher than those reported in the general PD population. Except for few patients with perioperative cerebral hemorrhage, STN-DBS is cognitively safe, and does not provide dementia risk factors in addition to those reported for PD itself. Identification of dementia predictors in this population may improve patient selection and information concerning the risk of poor cognitive outcome.

Background and Purpose: Approximately half of the patients with acute ischemic stroke due to anterior circulation large vessel occlusion do not achieve functional independence despite successful reperfusion. We aimed to determine influence of reperfusion strategy (bridging therapy, intravenous thrombolysis alone, or mechanical thrombectomy alone) on clinical outcomes in this population. Methods: From ongoing, prospective, multicenter, observational Endovascular Treatment in Ischemic Stroke registry in France, all patients with anterior circulation large vessel occlusion who achieved successful reperfusion (modified Thrombolysis in Cerebral Infarction 2b-3) following reperfusion therapy were included. Primary end point was favorable outcome, defined as 90-day modified Rankin Scale score ≤2. Patient groups were compared using those treated with bridging therapy as reference. Differences in baseline characteristics were reduced after propensity score-matching, with a maximum absolute standardized difference of 14% for occlusion site. Results: Among 1872 patients included, 970 (51.8%) received bridging therapy, 128 (6.8%) received intravenous thrombolysis alone, and the remaining 774 (41.4%) received MT alone. The rate of favorable outcome was comparable between groups. Excellent outcome (90-day modified Rankin Scale score 0–1) was achieved more frequently in the bridging therapy group compared with the MT alone (odds ratio after propensity score-matching, 0.70 [95% CI, 0.50–0.96]). Regarding safety outcomes, hemorrhagic complications were similar between the groups, but 90-day mortality was significantly higher in the MT alone group compared with the bridging therapy group (odds ratio, 1.60 [95% CI, 1.09–2.37]). Conclusions: This real-world observational study of patients with anterior circulation large vessel occlusion demonstrated a similar rate of favorable outcome following successful reperfusion with different therapeutic strategies. However, our results suggest that bridging therapy compared with MT alone is significantly associated with excellent clinical outcome and lower mortality. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03776877.

BackgroundCurrently available treatments for Parkinson’s disease (PD) do not slow clinical progression nor target alpha-synuclein, the main pathology associated with the disease.ObjectiveThe study objective was to evaluate the efficacy and safety of prasinezumab, a humanized monoclonal antibody that binds aggregated alpha-synuclein, in individuals with early PD. The study rationale, design, and baseline characteristics of enrolled subjects are presented here.MethodsThe PASADENA study is a multicenter, randomized, double-blind, placebo-controlled treatment study. Individuals with early PD, recruited across the US and Europe, received monthly intravenous doses of prasinezumab (1500 mg or 4500 mg) or placebo for a 52-week period (Part 1), followed by a 52-week extension (Part 2) in which all participants received active treatment. Key inclusion criteria were: aged 40–80 years; Hoehn & Yahr (H&Y) Stage I or II; time from diagnosis ≤2 years; having bradykinesia plus one other cardinal sign of PD (e.g. resting tremor, rigidity); DAT-SPECT imaging consistent with PD; and either treatment naïve or on a stable monoamine oxidase B (MAO-B) inhibitor dose. Study design assumptions for sample size and study duration were built using a patient cohort from the Parkinson’s Progression Marker Initiative (PPMI). In this report, baseline characteristics are compared between the treatment-naïve and MAO-B inhibitor-treated PASADENA cohorts and between the PASADENA and PPMI populations.ResultsOf the 443 patients screened, 316 were enrolled into the PASADENA study between June 2017 and November 2018, with an average age of 59.9 years and 67.4% being male. The mean time from diagnosis at baseline was 10.11 months, with 75.3% in H&Y Stage II. Baseline motor and non-motor symptoms (assessed using Movement Disorder Society – Unified Parkinson’s Disease Rating Scale [MDS-UPDRS]) were similar in severity between the MAO-B inhibitor-treated and treatment-naïve PASADENA cohorts (MDS-UPDRS Total score [standard deviation (SD)]; 30.21 [11.96], 32.10 [13.20], respectively). The overall PASADENA population (63.6% treatment naïve and 36.4% on MAO-B inhibitor) also showed a similar severity in MDS-UPDRS scores (e.g. MDS-UPDRS Total score [SD]; 31.41 [12.78], 32.63 [13.04], respectively) to the PPMI cohort (all treatment naïve).ConclusionsThe PASADENA study population is suitable to investigate the potential of prasinezumab to slow disease progression in individuals with early PD.Trial RegistrationNCT03100149

Human 4-hydroxyphenylpyruvate dioxygenase-like (HPDL) is a putative iron-containing non-heme oxygenase of unknown specificity and biological significance. We report 25 families containing 34 individuals with neurological disease associated with biallelic HPDL variants. Phenotypes ranged from juvenile-onset pure hereditary spastic paraplegia to infantile-onset spasticity and global developmental delays, sometimes complicated by episodes of neurological and respiratory decompensation. Variants included bona fide pathogenic truncating changes, although most were missense substitutions. Functionality of variants could not be determined directly as the enzymatic specificity of HPDL is unknown; however, when HPDL missense substitutions were introduced into 4-hydroxyphenylpyruvate dioxygenase (HPPD, an HPDL orthologue), they impaired the ability of HPPD to convert 4-hydroxyphenylpyruvate into homogentisate. Moreover, three additional sets of experiments provided evidence for a role of HPDL in the nervous system and further supported its link to neurological disease: (i) HPDL was expressed in the nervous system and expression increased during neural differentiation; (ii) knockdown of zebrafish hpdl led to abnormal motor behaviour, replicating aspects of the human disease; and (iii) HPDL localized to mitochondria, consistent with mitochondrial disease that is often associated with neurological manifestations. Our findings suggest that biallelic HPDL variants cause a syndrome varying from juvenile-onset pure hereditary spastic paraplegia to infantile-onset spastic tetraplegia associated with global developmental delays.

To evaluate outcomes of thrombectomy in patients with a basilar artery occlusion (BAO) and mild symptoms, defined by an initial National Institutes of Health Stroke Scale (NIHSS) score ≤6.We performed a retrospective analysis of a multicenter prospective cohort of consecutive patients with acute ischemic stroke with BAO who underwent thrombectomy. We compared baseline and procedural characteristics, as well as outcomes between patients with BAO with an NIHSS score ≤6 and6. Multivariate analyses were performed to determine baseline and procedural predictors of good outcome (modified Rankin Scale score 0-2) among patients with an NIHSS score ≤6.A total of 269 patients were included: 50 (19%) had an initial NIHSS score ≤6 and 219 (81%) had an NIHSS score6. Patients with mild strokes (NIHSS score ≤6) had better outcomes (68% of modified Rankin Scale score 0-2 vs. 27% for NIHSS score6; P0.0001), lower mortality (14% vs. 48; P0.0001) and fewer parenchymal hematomas at day 1 (0% vs. 10%; P = 0.016). A multivariate analysis identified the following predictors for good outcome among patients with BAO with an NIHSS score ≤6: younger age, fewer passes, a cardioembolic cause, and the absence of need for angioplasty/stenting.Thrombectomy seems to be safer and more effective for mild BAO strokes with NIHSS score ≤6 than for more severe patients. Even although thrombectomy showed high rates of recanalization, a substantial proportion (32%) nevertheless had a poor long-term clinical outcome. The number of passes, patient's age, and stroke cause seem to be predictors of clinical outcome.

Contains fulltext : 237881.pdf (Publisher’s version ) (Open Access) Human 4-hydroxyphenylpyruvate dioxygenase-like (HPDL) is a putative iron-containing non-heme oxygenase of unknown specificity and biological significance. We report 25 families containing 34 individuals with neurological disease associated with biallelic HPDL variants. Phenotypes ranged from juvenile-onset pure hereditary spastic paraplegia to infantile-onset spasticity and global developmental delays, sometimes complicated by episodes of neurological and respiratory decompensation. Variants included bona fide pathogenic truncating changes, although most were missense substitutions. Functionality of variants could not be determined directly as the enzymatic specificity of HPDL is unknown; however, when HPDL missense substitutions were introduced into 4-hydroxyphenylpyruvate dioxygenase (HPPD, an HPDL orthologue), they impaired the ability of HPPD to convert 4-hydroxyphenylpyruvate into homogentisate. Moreover, three additional sets of experiments provided evidence for a role of HPDL in the nervous system and further supported its link to neurological disease: (i) HPDL was expressed in the nervous system and expression increased during neural differentiation; (ii) knockdown of zebrafish hpdl led to abnormal motor behaviour, replicating aspects of the human disease; and (iii) HPDL localized to mitochondria, consistent with mitochondrial disease that is often associated with neurological manifestations. Our findings suggest that biallelic HPDL variants cause a syndrome varying from juvenile-onset pure hereditary spastic paraplegia to infantile-onset spastic tetraplegia associated with global developmental delays.

ObjectiveTo evaluate the effects of deep brain stimulation of the subthalamic nucleus (STN-DBS) on motor complications in patients with Parkinson disease (PD) beyond 15 years after surgery.MethodsData on motor complications, quality of life (QoL), activities of daily living, Unified Parkinson’s Disease Rating Scale motor scores, dopaminergic treatment, stimulation measures, and side effects of STN-DBS were retrospectively retrieved and compared before surgery, at 1 year, and beyond 15 years after bilateral STN-DBS.ResultsFifty-one patients with 17.06 ± 2.18 years STN-DBS follow-up were recruited. Compared to baseline, the time spent with dyskinesia and the time spent in the “off” state were reduced by 75% (p < 0.001) and by 58.7% (p < 0.001), respectively. Moreover, dopaminergic drugs were reduced by 50.6% (p < 0.001). Parkinson's Disease Quality of Life Questionnaire total score and the emotional function and social function domains improved 13.8% (p = 0.005), 13.6% (p = 0.01), and 29.9% (p < 0.001), respectively. Few and mostly manageable device-related adverse events were observed during the follow-up.ConclusionsSTN-DBS is effective beyond 15 years from the intervention, notably with significant improvement in motor complications and stable reduction of dopaminergic drugs. Furthermore, despite the natural continuous progression of PD with worsening of levodopa-resistant motor and nonmotor symptoms over the years, patients undergoing STN-DBS could maintain an improvement in QoL.Classification of EvidenceThis study provides Class IV evidence that, for patients with PD, STN-DBS remains effective at treating motor complications 15 years after surgery.

ObjectiveTo describe neuroimaging findings and to report the epidemiologic and clinical characteristics of patients with coronavirus disease 2019 (COVID-19) with neurologic manifestations.MethodsIn this retrospective multicenter study (11 hospitals), we included 64 patients with confirmed COVID-19 with neurologic manifestations who underwent a brain MRI.ResultsThe cohort included 43 men (67%) and 21 women (33%); their median age was 66 (range 20–92) years. Thirty-six (56%) brain MRIs were considered abnormal, possibly related to severe acute respiratory syndrome coronavirus. Ischemic strokes (27%), leptomeningeal enhancement (17%), and encephalitis (13%) were the most frequent neuroimaging findings. Confusion (53%) was the most common neurologic manifestation, followed by impaired consciousness (39%), presence of clinical signs of corticospinal tract involvement (31%), agitation (31%), and headache (16%). The profile of patients experiencing ischemic stroke was different from that of other patients with abnormal brain imaging: the former less frequently had acute respiratory distress syndrome (p= 0.006) and more frequently had corticospinal tract signs (p= 0.02). Patients with encephalitis were younger (p= 0.007), whereas agitation was more frequent for patients with leptomeningeal enhancement (p= 0.009).ConclusionsPatients with COVID-19 may develop a wide range of neurologic symptoms, which can be associated with severe and fatal complications such as ischemic stroke or encephalitis. In terms of meningoencephalitis involvement, even if a direct effect of the virus cannot be excluded, the pathophysiology seems to involve an immune or inflammatory process given the presence of signs of inflammation in both CSF and neuroimaging but the lack of virus in CSF.ClinicalTrials.gov identifierNCT04368390.

Background and Purpose: Although the efficacy of endovascular treatment (EVT) in patients with anterior circulation ischemic stroke (AIS) is well documented, early neurological deterioration after EVT remains a serious issue associated with poor outcome. Besides obvious causes, such as lack of reperfusion, procedural complications, or parenchymal hemorrhage, early neurological deterioration may remain unexplained (UnEND). Our aim was to investigate predictors of UnEND after EVT in patients with AIS. Methods: Patients who underwent EVT for AIS, with an initial National Institutes of Health Stroke Scale score >5, Alberta Stroke Program Early CT Score ≥6, and included in a multicenter prospective observational registry were analyzed. Predictors of UnEND, defined as ≥4-point increase in the National Institutes of Health Stroke Scale score between baseline and day 1 after EVT, were determined via center-adjusted analyses. Results: Among the 1925 included in the analysis, 128 UnEND (6.6%) were recorded. In multivariate analysis, predictors of UnEND were diabetes mellitus (odds ratio [OR], 2.17 [95% CI, 1.32–3.56]), prestroke modified Rankin Scale score ≥2 (OR, 2.22 [95% CI, 1.09–4.55]), general anesthesia (OR, 2.55 [95% CI, 1.51–4.30]), admission systolic blood pressure (OR, 1.10 [95% CI, 1.01–1.20]), age (OR, 1.38 [95% CI, 1.14–1.67]), number of passes (OR, 1.16 [95% CI, 1.04–1.28]), direct admission or not to a comprehensive stroke center (OR, 0.49 [95% CI, 0.30–0.81]), and initial National Institutes of Health Stroke Scale score (OR, 0.65 [95% CI, 0.52–0.81]). Conclusions: Severely impaired AIS patients with nonmodifiable factors are more likely to develop UnEND. Some modifiable predictors of UnEND such as the number of EVT passes could be the object of improvement in AIS management.

Background and Purpose: The efficiency of prehospital care chain response and the adequacy of hospital resources are challenged amid the coronavirus disease 2019 (COVID-19) outbreak, with suspected consequences for patients with ischemic stroke eligible for mechanical thrombectomy (MT). Methods: We conducted a prospective national-level data collection of patients treated with MT, ranging 45 days across epidemic containment measures instatement, and of patients treated during the same calendar period in 2019. The primary end point was the variation of patients receiving MT during the epidemic period. Secondary end points included care delays between onset, imaging, and groin puncture. To analyze the primary end point, we used a Poisson regression model. We then analyzed the correlation between the number of MTs and the number of COVID-19 cases hospitalizations, using the Pearson correlation coefficient (compared with the null value). Results: A total of 1513 patients were included at 32 centers, in all French administrative regions. There was a 21% significant decrease (0.79; [95%CI, 0.76–0.82]; P P P R 2 −0.51; P =0.04). Patients treated during the COVID outbreak were less likely to receive intravenous thrombolysis and to have unwitnessed strokes (both P Conclusions: Our study showed a significant decrease in patients treated with MTs during the first stages of the COVID epidemic in France and alarming indicators of lengthened care delays. These findings prompt immediate consideration of local and regional stroke networks preparedness in the varying contexts of COVID-19 pandemic evolution.

OBJECTIVE This study was undertaken to identify preoperative predictive factors of long-term motor outcome in a large cohort of consecutive Parkinson disease (PD) patients with bilateral subthalamic nucleus deep brain stimulation (STN-DBS). METHODS All consecutive PD patients who underwent bilateral STN-DBS at the Grenoble University Hospital (France) from 1993 to 2015 were evaluated before surgery, at 1 year (short-term), and in the long term after surgery. All available demographic variables, neuroimaging data, and clinical characteristics were collected. Preoperative predictors of long-term motor outcome were investigated by performing survival and univariate/multivariate Cox regression analyses. Loss of motor benefit from stimulation in the long term was defined as a reduction of less than 25% in the Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) part III scores compared to the baseline off-medication scores. As a secondary objective, potential predictors of short-term motor outcome after STN-DBS were assessed by performing univariate and multivariate linear regression analyses. RESULTS In the long-term analyses (mean follow-up = 8.4 ± 6.26 years, median = 10 years, range = 1-17 years), 138 patients were included. Preoperative higher frontal score and off-medication MDS-UPDRS part III scores predicted a better long-term motor response to stimulation, whereas the presence of vascular changes on neuroimaging predicted a worse motor outcome. In 357 patients with available 1-year follow-up, preoperative levodopa response, tremor dominant phenotype, baseline frontal score, and off-medication MDS-UPDRS part III scores predicted the short-term motor outcome. INTERPRETATION Frontal lobe dysfunction, disease severity in the off-medication condition, and the presence of vascular changes on neuroimaging represent the main preoperative clinical predictors of long-term motor STN-DBS effects. ANN NEUROL 2021.

Objectives: To describe the outcome of primary meningeal sarcoma (PMS) in a pediatric population. Methods: We conducted a retrospective study on patients harbouring a PMS based on the French registry of paediatric tumours. Questionnaires were sent to all French paediatric oncologists. Data on patient characteristics and treatments were collected. Pathology and imaging were centrally reviewed. Results: Between August 1989 and May 2010, 12 patients from 6 French centres, aged 3 months to 14.5 years (mean: 3.3 years) were treated for a PMS. Mean follow-up was 12 years (range: 3 months to 24 years). Tumour locations were: frontal (3), parieto-occipital (2), parietal (1), temporal (1), occipital (1), thalamic (1), pontocerebellar angle (1), cerebellar tentorium (1), ambient cistern (1). No metastasis was observed. The first-line treatment was surgery in 10 cases, chemotherapy in 2. Resection was total in 6 cases, partial in 6. Central pathology review concluded to: high-grade undifferentiated sarcoma (8), chondrosarcoma (2), fibrosarcoma (1), myxoid desmoplastic tumour (1). Seven tumours were smaller than 5 cm in size. Eleven patients received 2 to 10 courses of chemotherapy (median: 5) but no response was observed. Four out of 6 patients for whom a total resection was performed were still alive at time of study, compared to 2 when only partial resection was possible. Four out of 6 patients who received radiotherapy are still alive, compared to 1 out of 4 patients without radiotherapy. The 5-year Event-Free Survival (EFS) and Overall Survival (OS) rates were 50%. The median EFS in case of total resection was 39 months versus 16 months in case of partial resection. Neither correlation was found between tumour size and location or between age at diagnosis and EFS/OS. Conclusions: We report the first multicentre case series describing clinical, radiological presentation and outcome of PMS in children. In this short series of very rare tumours, age and tumour size did not seem to be prognostic factors. Total resection and radiotherapy seem to be essential. The role of chemotherapy remains unclear.

Ependymomas are rare central nervous system tumors. Half the patients experience recurrence and total resection is of major importance to obtain the most chance of cure. Thus, some patients undergo several repeated resections which may occasionally be at the origin of dissemination along the neuraxis and exceptionally to extraneural sites. We report the case of a boy who presented a multiple recurrence of his supratentorial ependymoma, first at initial site and then with cervical lymph nodes metastases, highlighting the fact that multiple surgical resections may confer an added risk for hematogeneous and lymphatic dissemination. The impact of adjuvant therapies on this risk is discussed.

Background Non-motor fluctuations represent a main source of disability in Parkinson's disease (PD). Among them, neuropsychiatric fluctuations are the most frequent and are often under-recognized by patients and physicians, partly because specific tools for assessment of neuropsychiatric fluctuations are lacking. Objective To develop a scale for detecting and evaluating the presence and the severity of neuropsychological symptoms during the ON and OFF phases of non-motor fluctuations. Methods Neuropsychiatric symptoms reported by PD patients in the OFF- and the ON-medication conditions were collected using different neuropsychiatric scales (BDI-II, BAI, Young, VAS, etc.). Subsequently, tree phases of a pilot study was performed for cognitive pretesting, identification of ambiguous or redundant items (item reduction), and to obtain preliminary data of acceptability of the new scale. In all the three phases, the scale was applied in both the OFF and ON condition during a levodopa challenge. Results Twenty items were selected for the final version of the neuropsychiatric fluctuation scale (NFS): ten items measured the ON neuropsychological symptoms and ten items the OFF neuropsychological manifestations. Each item rated from 0-3, providing respective subscores from 0 to 30. Conclusions Once validated, our NFS can be used to identify and quantify neuropsychiatric fluctuations during motor fluctuations. The main novelty is that it could be used in acute settings. As such, the NFS can assess the neuropsychiatric state of the patient at the time of examination. The next step will be to validate the NFS to be used in current practice.

BackgroundReports on behavioural outcomes after subthalamic nucleus deep brain stimulation in Parkinson’s disease are controversial and limited to short-term data. Long-term observation in a large cohort allows a better counselling and management.MethodsTo determine whether a long-term treatment with subthalamic stimulation induces or reduces impulse control behaviours, neuropsychiatric fluctuations and apathy, 69 patients treated with subthalamic stimulation are prospectively and retrospectively assessed using Ardouin Scale of Behavior in Parkinson’s Disease before and after 3–10 years of stimulation.ResultsAt a mean follow-up of 6 years, all impulse control disorders and dopaminergic addiction were significantly decreased, apart from eating behaviour and hypersexuality. Neuropsychiatric fluctuations also significantly improved (ON euphoria: 38% of the patients before surgery and 1% after surgery, PConclusionsBilateral subthalamic nucleus stimulation was overall very effective in improving impulse control disorders and neuropsychiatric fluctuations in parkinsonian patients in the long term despite a counteracting frequent apathy. Transient episodes of impulse control disorders still occurred within the follow-up. These findings recommend a close follow-up in parkinsonian patients presenting with neuropsychiatric symptoms before deep brain stimulation surgery.Clinical trial registrationNCT01705418;Post-results.

Congenital disorders of glycosylation (CDG) constitute an ever-growing group of genetic diseases affecting the glycosylation of proteins. CDG individuals usually present with severe multisystem disorders. MAN1B1-CDG is a CDG with nonspecific clinical symptoms such as intellectual deficiency and developmental delay. Although up to 40 affected individuals were described so far, its final diagnosis is not straightforward using common biochemical methods due to the trace-level accumulation of defective glycan structures. In this study, we present three unreported MAN1B1-CDG individuals and propose a decision tree to reach diagnosis using a panel of techniques ranging from exome sequencing to gel electrophoresis and mass spectrometry. The occurrence of MAN1B1-CDG in patients showing unexplained intellectual disability and development delay, as well as a particular transferrin glycosylation profile, can be ascertained notably using matrix assisted laser desorption/ionization – time of flight (MALDI-TOF) mass spectrometry analysis of endo-β-acetylglucosaminidase H-released serum N-glycans. In addition to reporting new pathogenic variants and additional clinical signs such as hypersialorrhea, we highlight particular biochemical features of MAN1B1-CDG with potential glycoprotein-specific glycosylation defects.

Ewing's sarcoma (ES) is the second most common malignant bone tumor in children and young adults. ES may also occur as a primary soft tissue neoplasm without involvement of the bone and is then referred to as extraosseous ES (EES). The paediatric central nervous system extraosseous Ewing's sarcoma (CNS-EES) is an extremely rare entity since less than 20 cases have been reported in literature. Moreover, only less than 10 cases correspond to brain parenchyma primary tumors. These cases have been often misdiagnosed with other varieties of primary brain tumors, particularly those which were called PNET (Primitive Neuro-Ectodermal Tumor). We present a case of true intraparenchymal CNS-EES and a comparison of epidemiology, behaviour, treatment, and prognosis of CNS-EES and intracranial PNET cases.

Background and Purpose: The best anesthetic management for mechanical thrombectomy of large vessel occlusion strokes is still uncertain and could impact the quality of reperfusion and clinical outcome. We aimed to compare the efficacy and safety outcomes between local anesthesia (LA) and conscious sedation in a large cohort of acute ischemic stroke patients with anterior circulation large vessel occlusion strokes treated with mechanical thrombectomy in current, everyday clinical practice. Methods: Patients undergoing mechanical thrombectomy for anterior large vessel occlusion strokes at 4 comprehensive stroke centers in France between January 1, 2018, and December 31, 2018, were pooled from the ongoing prospective multicenter observational Endovascular Treatment in Ischemic Stroke Registry in France. Intention-to-treat and per-protocol analyses were used. Results: Among the included 1034 patients, 762 were included in the conscious sedation group and 272 were included in the LA group. In the propensity score matched cohort, the rate of favorable outcome (90-day modified Rankin Scale score 0–2) was significantly lower in the LA group than in the conscious sedation group (40.0% versus 52.0%, matched relative risk=0.76 [95% CI, 0.60–0.97]), as well as the rate of successful reperfusion (modified Thrombolysis in Cerebral Infarction grade 2b–3; 76.6% versus 87.1%; matched relative risk=0.88 [95% CI, 0.79–0.98]). There was no difference in procedure time between the 2 groups. In the inverse probability of treatment weighting-propensity score-adjusted cohort, similar significant differences were found for favorable outcomes and successful reperfusion. In inverse probability of treatment weighting-propensity score-adjusted cohort, a higher rate of 90-day mortality and a lower parenchymal hematoma were observed after LA. The sensitivity analysis restricted to our per-protocol sample provided similar results in the matched- and inverse probability of treatment weighting-propensity cohorts. Conclusions: In the Endovascular Treatment in Ischemic Stroke registry mainly included patients in early time window (

Background: Metabolic and inflammatory conditions may lead to neurological disorders. Neuromyelitis optica spectrum disorders (NMOSDs) refer to a rare group of demyelinating diseases of the central nervous system which essentially involve the optic nerves and spinal cord. Methods: We report a case of biotinidase deficiency (BD) initially misdiagnosed as NMOSD in a pediatric patient. Results: An 8-year-old girl was initially diagnosed with NMOSD on the basis of optic neuritis (ON) associated with three episodes of longitudinally extensive transverse myelitis (LETM). Intravenous high-dose corticosteroids were effective during the first two episodes of LETM. The third acute episode which resulted in tetraplegia, respiratory distress, and blindness was refractory to corticosteroids, plasmapheresis, and rituximab. The unusual clinical course and persistent high levels of plasma and cerebrospinal fluid (CSF) lactate led to additional metabolic investigations being performed. Acylcarnitine profile revealed increased C5-OH acylcarnitine suggestive of BD. Diagnosis was confirmed by direct assessment of plasma enzyme activity (quantified as 5% of the control value). Genetic analysis revealed two mutations, c.643C>T (p.L215F) and c.1612C>T (p.R538C), in the BTD gene (3p25). Dramatic clinical improvement occurred after long-term oral biotin treatment. Conclusion: BD is a treatable condition that may closely mimic the neurological findings of LETM and NMOSD.

BackgroundThe effectiveness of mechanical thrombectomy (MT) in acute ischemic stroke due to large vessel occlusion is time-dependent. While only stroke centers with endovascular capabilities perform MT, many patients who had a stroke initially present to the closest primary stroke centers capable of administering earlier intravenous thrombolysis, and then require to be transferred to a comprehensive stroke center for MT.PurposeTo compare the outcomes of this care pathway (drip and ship (DS)) with that whereby patients are directly transferred to a comprehensive stroke center (mothership (MS)).MethodsWe performed a systematic review and meta-analysis of published studies using several electronic databases to determine whether successful reperfusion (modified Thrombolysis In Cerebral Infarction ≥2b), functional independence at 90 days (modified Rankin Scale score ≤2), symptomatic intracranial hemorrhage, and 90-day mortality differed between those who underwent MT with the DS or the MS treatment pathway. Outcomes were meta-analyzed and the results expressed as adjusted relative risk (aRR) for the primary analysis and unadjusted relative risk (uRR) for secondary analysis.ResultsEight studies including 2068 patients were selected, including one study reporting results fully adjusted for baseline characteristics. Patients undergoing MS had better functional independence than those undergoing DS (uRR=0.87, 95% CI 0.81 to 0.93; aRR=0.87, 95% CI 0.77 to 0.98). No difference was found between the treatment pathways in successful reperfusion (uRR=1.05, 95% CI 0.95 to 1.15; aRR=1.00, 95% CI 0.92 to 1.10), symptomatic intracranial hemorrhage (uRR=1.37, 95% CI 0.91 to 2.06; aRR, 1.53, 95% CI 0.79 to 2.98), and 90-day mortality (uRR=1.00, 95% CI 0.84 to 1.19; aRR=1.21, 95% CI 0.89 to 1.64).ConclusionsPatients who had an acute ischemic stroke admitted directly to a comprehensive stroke center (MS patients) with endovascular capacities may have better 90-day outcomes than those receiving DS treatment. However, major limitations of current evidence (ie, retrospective studies and selection bias) suggest a need for adequately powered studies. Multicenter randomized controlled trials are expected to answer this question.

International audience; Introduction: Visual impairment is a concern in premature infants as perinatal factors may alter maturation during visual development. This observational study aimed at evaluating visual maturation at term equivalent age and factors associated with impaired visual maturation.Methods: Infants born before 32 weeks' gestation were evaluated with routine brain MRI, visual acuity, refraction, fundus, and clinical eye examination. Environmental factors were collected from infant's files.Results: Fifty-four infants (29.5 ± 1.7 weeks' gestation, birth weight 1194 ± 288 g) were studied at term equivalent age. Visual acuity was higher in premature infants at term equivalent age than in a reference publication with the same method in term newborns at birth (1.54 ± 0.67 vs. 0.99 ± 0.40 cycles/degree, p = 0.008). In multivariate analysis, abnormal brain MRI was the only factor associated with visual acuity (r 2 = 0.203; p = 0.026). Incomplete retinal vascularization was observed in 29/53 of infants at term equivalent age and associated with MRI abnormalities of the posterior fossa (p = 0.027) and larger refractive sphere difference between both eyes (1.2 ± 0.8 vs. 0.6 ± 0.4 diopters; p = 0.0005). Retinopathy of prematurity was associated with indices of smaller cerebral volume (p = 0.035).Conclusion: Higher visual acuity in premature infants at term equivalent age than in term newborns at birth may be related to longer visual experience from birth. Lower visual acuity was correlated with abnormal MRI in preterm infants at term equivalent age.

ObjectiveTo determine the postoperative attempted and completed suicide rates after subthalamic nucleus deep brain stimulation (STN-DBS) in a single-center cohort and to determine factors associated with attempted and completed suicide.MethodsWe retrospectively included all patients with Parkinson disease (PD) who underwent bilateral STN-DBS surgery at the Grenoble University Hospital between 1993 and 2016. For each patient who committed or attempted suicide, 2 patients with PD with STN-DBS without any suicidal behaviors were matched for age (±1 year), sex, and year of surgery (±2 years). Clinical data were collected from medical records. Detailed preoperative and postoperative neuropsychological evaluations, including frontal and Beck Depression Inventory (BDI) scores, were gathered.ResultsA total of 534 patients with PD were included. Completed and attempted suicide percentages were 0.75% (4 of 534) and 4.11% (22 of 534), respectively. The observed suicide rate in the first postoperative year (187.20 of 100,000 per year, 1 of 534) was higher than the expected National Observatory on Suicide Risks rate adjusted for age and sex (standardized mortality ratio 8.1). This rate remained similar over the second and third postoperative years. In a comparison of the 26 patients completing/attempting suicide and the 52 controls, the first group showed more frequent history of suicidal ideation/suicide attempts and psychotic symptoms, higher percentage of family psychiatric history, higher psychiatric medication use, and higher preoperative frontal and BDI scores on neuropsychological evaluations.ConclusionsSuicide behaviors can occur after STN-DBS, especially during the first 3 years. A careful multidisciplinary assessment and long-term follow-up are recommended to recognize and treat this potentially preventable risk for mortality.

Background Recommended treatments for spontaneous intracranial hypotension (SIH) range from bed rest only to neurosurgery. However, the serious form of SIH is poorly defined. A better description of patient characteristics and their outcome may help define therapeutic options. Methods We reviewed 24 cases of patients with SIH and separated them into 2 groups according to whether or not they presented with signs of severity at admission: disturbance of consciousness, subdural hematomas (SDHs), and cerebral venous thrombosis. Results Nine patients (37%) were classified as having a serious form of SIH: six (25%) presented SDHs; three (12%) disturbance of consciousness; and one (4%) cerebral venous thrombosis. Bed rest and epidural blood patches (EBPs) were sufficient to treat all patients in the nonserious form group and 4 patients in the serious form group. Two patients (8%) had to undergo cerebrospinal fluid leak repair, and 3 others (12%) evacuation of SDHs. Outcome was good in both groups, with only one (4%) death due to extensive SDHs. Times to diagnosis in the serious form group (63 vs. 35 days, P = 0.052) and to recovery (9 months vs. 5 months, P = 0.088) tended to be higher without reaching difference. Conclusions The presence of SDHs, disturbance of consciousness, and a trend toward a longer time to diagnosis and recovery seem to define the serious form of SIH. These patients may require exploration and surgical repair of cerebrospinal fluid leak, only after failure of conservative measures—bed rest and time—and EBP, with good outcome.

International audience; We report the evaluation of an automated method for quantification of brain tissue damage, caused by a severe traumatic brain injury, using mean diffusivity computed from MR diffusion images. Our automatic results obtained on realistic phantoms and real patient images 10 days post-event provided by nine different centers were coherent with four expert manually identified lesions. For realistic phantoms automated method scores were equal to 0.77, 0.77 and 0.83 for Dice, Precision and Sensibility respectively compared to 0.78, 0.72 and 0.86 for the experts. The inter correlation class (ICC) was 0.79. For 7/9 real cases 0.57, 0.50 and 0.70 were respectively obtained for automated method compared to 0.60, 0.52 and 0.78 for experts with ICC=0.71. Additionally, we detail the quality control module used to pool data from various image provider centers. This study clearly demonstrates the validity of the proposed automated method to eventually compute in a multi-centre project, the lesional load following brain trauma based on MD changes.

Pituitary macroadenoma constitutes a frequently misdiagnosed benign tumor. We report herein a case where such macroadenoma, a prolactinoma, was incidentally discovered in a 63-year-old man who had been referred to F-FDG PET and F-FDOPA PET imaging for a pharmacoresistant epilepsy. An increased uptake was documented for both radiotracers within the sellar region, although with a much higher contrast for F-FDOPA than for F-FDG. This case presents an increased uptake documented within a prolactinoma owing to the high contrast and image quality provided by F-FDOPA PET.

International audience; PURPOSE: This study evaluates the correlation between injuries to deep gray matter nuclei, as quantitated by lesions in these nuclei on MR T2 Fast Spin Echo (T2 FSE) images, with 6-month neurological outcome after severe traumatic brain injury (TBI). MATERIALS AND METHODS: Ninety-five patients (80 males, mean age = 36.7y) with severe TBI were prospectively enrolled. All patients underwent a MR scan within the 45 days after the trauma that included a T2 FSE acquisition. A 3D deformable atlas of the deep gray matter was registered to this sequence; deep gray matter lesions (DGML) were evaluated using a semi-quantitative classification scheme. The 6-month outcome was dichotomized into unfavorable (death, vegetative or minimally conscious state) or favorable (minimal or no neurologic deficit) outcome. RESULTS: Sixty-six percent of the patients (63/95) had both satisfactory registration of the 3D atlas on T2 FSE and available clinical follow-up. Patients without DGML had an 89% chance (P = 0.0016) of favorable outcome while those with bilateral DGML had an 80% risk of unfavorable outcome (P = 0.00008). Multivariate analysis based on DGML accurately classified patients with unfavorable neurological outcome in 90.5% of the cases. CONCLUSION: Lesions in deep gray matter nuclei may predict long-term outcome after severe TBI with high sensitivity and specificity.

International audience; Peroxisome biogenesis disorders (PBD) are a heterogeneous group of disorders due to PEX genesmutations, with a broad clinical spectrum comprising severe neonatal disease to mild presentation. Recently, Berendse et al reported an improvement of peroxisomal functions with L-arginine supplementation in fibroblasts with specific mutations of PEX1, PEX6, and PEX12. We report the first treatment by L-arginine in a patient homozygous for the specific PEX12 mutation shown to be L-arginine responsive in fibroblasts. We described the effect of L-arginine on biochemical (decrease of some plasma peroxisomal parameters) and neurophysiological (improvement of deafness) parameters. Some subjective clinical effects have also been observed (no more sialorrhea, behavior improvement). More studies are needed to assess the efficacy of L-arginine in some PBD patients with specific mutations.

Background Existing methods to predict recovery after severe traumatic brain injury lack accuracy. The aim of this study is to determine the prognostic value of quantitative diffusion tensor imaging (DTI). Methods In a multicenter study, the authors prospectively enrolled 105 patients who remained comatose at least 7 days after traumatic brain injury. Patients underwent brain magnetic resonance imaging, including DTI in 20 preselected white matter tracts. Patients were evaluated at 1 yr with a modified Glasgow Outcome Scale. A composite DTI score was constructed for outcome prognostication on this training database and then validated on an independent database (n=38). DTI score was compared with the International Mission for Prognosis and Analysis of Clinical Trials Score. Results Using the DTI score for prediction of unfavorable outcome on the training database, the area under the receiver operating characteristic curve was 0.84 (95% CI: 0.75-0.91). The DTI score had a sensitivity of 64% and a specificity of 95% for the prediction of unfavorable outcome. On the validation-independent database, the area under the receiver operating characteristic curve was 0.80 (95% CI: 0.54-0.94). On the training database, reclassification methods showed significant improvement of classification accuracy (P < 0.05) compared with the International Mission for Prognosis and Analysis of Clinical Trials score. Similar results were observed on the validation database. Conclusions White matter assessment with quantitative DTI increases the accuracy of long-term outcome prediction compared with the available clinical/radiographic prognostic score.

We report two cases of salivary gland anlage tumor (SGAT), a nasopharyngeal lesion that affects newborns. The first case concerned a male newborn, presenting respiratory distress secondary to a nasopharyngeal mass. The second case was diagnosed in a 6-week-old girl, suffering from respiratory difficulties due to a nasal cavity mass. A magnetic resonance imaging (MRI) in the second case revealed the presence of several small round and linear fluid-like areas. Histologically, both lesions were suggestive of SGAT, characterized by epithelial structures that blended with spindle-cells, drawing highly cellular nodules. Connective tissue between nodules contained squamous cystic nests and ducts.

Cranial traumas from gunshot wounds are characterised by the impact of a high velocity projectile. There are therefore serious, life threatening traumas. CT Scan is essential in the emergency setting for initial evaluation of traumatic bone and parenchymatous injuries to determine the indication for neurosurgery and appropriate medical management. In case of survival, CT Scan and MRI can be used to monitor progress and any possible complications, in particular vascular or infectious complications which are specific to this type of injury.

There is a wide range of spinal cord pathologies (vascular, inflammatory, infectious, metabolic, degenerative). They present clinically as acute partial or complete cord syndromes, or chronic myelopathies (more than 4 weeks in duration). MRI examination should be undertaken with a very strict protocol. Spinal cord lesions should be evaluated with regards to their T1W and T2W signal characteristics, involvement of grey and/or white matter, axial and sagittal extension, cord volume changes, contrast uptake and associated lesions (perimedullary, radicular or brain). The correlation of MR imaging features with clinical and biological data (blood and CSF) should suggest a differential diagnosis.

Despite advances in diagnosis and treatment, atherosclerosis remains the second cause of death in the world. Due to technical advances, high resolution MRI (HR-MRI) allows depiction of the wall of cervical arteries, especially carotid atherosclerosis. HR-MRI allows visualization of the different components of atherosclerosis: necrotic lipid core, intraplaque hemorrhage, calcifications and fibrous cap. Global plaque volume as well as the volumes of individual plaque components can be calculated. Atherosclerotic plaque structure analysis, along with stenosis measurement, contribute to the stratification of the stroke risk. HR-MRI may also be used to assess treatment efficacy aimed at stabilizing or reducing plaque progression. Beyond the arterial lumen, direct evaluation of vessel wall should modify the management of atherosclerosis in the years to come.

The management of gliomas evolves towards more aggressive strategies with a combination of surgery, radiotherapy and chemotherapy. Follow-up imaging based on morphological MRI, with simple and reproducible protocols, may be associated with functional MRI and spectroscopy. Baseline postsurgical MRI must be performed within the first three days. Follow-up examinations should be done 2 months after radiotherapy and during chemotherapy, usually after each cycle of two or three treatments. Continued follow-up after therapy is recommended to assess response and detect recurrences or therapeutic complications.

Resume La maladie de Rendu Osler est une dysplasie vasculaire familiale hereditaire a forte penetrance et expression variable. Il en existe au moins deux formes : HHT1 ou la mutation porte sur le gene de l’endogline sur le chomosome 9 et HHT2 ou la mutation genetique porte sur le gene de l’activin receptor-like kinase 1 sur le chromosome 12. La maladie de Rendu Osler est caracterisee par des telangiectasies cutaneo-muqueuses et des malformations arterioveineuses viscerales. Les manifestations neurologiques touchent 8 a 10 % des patients. Il peut s’agir d’AVC ischemique ou d’abces compliquant une FAV pulmonaire ou de manifestations en rapport avec des malformations arterioveineuses (MAV) cerebrales ou medullaires. Ces MAV ont cependant des risques plus faibles que les MAV sporadiques et ne justifient pas un depistage systematique excepte chez l’enfant en particulier issu de famille porteuse de la forme HHT1.

Medical risk management has one main purpose: to ensure the safety of care. The law of March 2002 has generated a true cultural revolution. The radiologist is involved with new and difficult areas of medical liability due to technical advances, the increasing number of imaging techniques, the increasing complexity of imaging techniques, their efficiency and the need for multidisciplinary approach. Imaging recommendations requiring increasing levels of technical and clinical skills. The radiologist is liable with regards to the indications of imaging studies, and also with regards to informed consent. The prevention of medicolegal problems is achieved by competency, which must be combined to good liability insurance and ongoing vigilance supported by appropriate continuous medical education.

Resume L’imagerie a un role important pour le diagnostic et surtout dans l’evaluation des possibilites therapeutiques et du pronostic des tumeurs cranioencephaliques (TCE). En postoperatoire, elle intervient a la fois pour evaluer le geste operatoire lui-meme et prevoir les traitements associes. Dans le suivi, l’imagerie permet d’apprecier les reponses et les complications de ces therapeutiques. L’imagerie par resonance magnetique (IRM) est actuellement unanimement reconnue comme l’examen de choix au diagnostic et dans le suivi des TCE. Les techniques d’imagerie fonctionnelle de diffusion et de perfusion, disponibles sur les imageurs modernes sont aisement realisables en pratique clinique. Elles apportent des renseignements fondamentalement differents et complementaires a ceux fournis par l’imagerie morphologique, particulierement interessants au diagnostic mais aussi dans le cadre du suivi de ces lesions.

Resume Le melanome intra rachidien est une tumeur primitive de localisation inhabituelle. Nous rapportons un nouveau cas, atypique par sa primo localisation radiculaire, et par sa recidive metastatique precoce a 6 mois intra durale, extra medullaire. L’IRM est l’examen de choix, mettant en evidence une lesion spontanement hyper intense en ponderation T1, rehaussee intensement par le gadolinium, et dont le signal s’attenue en ponderation T2, orientant d’emblee le diagnostic vers un caractere melanique ou hemorragique. Les anomalies de signal ne sont cependant pas specifiques et le diagnostic de certitude est etabli par l’analyse anatomo-pathologique.

Two pilot studies on a small number of patients demonstrated the feasibility of transcranial Doppler ultrasound evaluation in the follow-up of intracranial aneurysms treated with detachable coils. This prospective study aimed to assess the sensitivity of contrast-enhanced transcranial echo-Doppler in detecting partial aneurysmal occlusion following endovascular treatment. Transcranial Doppler sonography (TCD) with contrast administration and magnetic resonance angiography (MRA) or angiography were carried out after endovascular treatment in 92 patients for 98 aneurysms of the anterior circulation. In 18% of cases, the quality of ultrasound exploration was deemed unsatisfactory for evaluation of the aneurysmal site. Results were concordant in 63% of patients (49/78). When occlusion appeared satisfactory at angiography or MRA, the result was concordant with TCD in 68% of cases (44/77). When a residual aneurysm was detected, TCD sensitivity was 38% (8/18). An analysis of subgroups failed to disclose anatomic or morphological aneurysm features in which transcranial echo-Doppler is sufficiently sensitive and specific to detect possible residual aneurysm or recanalisation. In our experience, even after contrast administration TCD is not sufficiently sensitive or specific in the detection of residual aneurysm to replace angiography or MRA.

Background and Purpose — Vasospasm secondary to subarachnoid hemorrhage (SAH) is responsible for severe ischemic complications. Although effective, angioplasty must be performed at a very early stage to produce any clinical recovery. Diagnostic investigations to assess arterial narrowing (transcranial Doppler, angiography) or cerebral perfusion (xenon CT, single-photon emission CT) do not provide evidence of the extent of parenchymal ischemia. In stroke, diffusion-weighted MR imaging (DWI) appears to be the most sensitive procedure to detect cerebral ischemia. We studied asymptomatic vasospasm in patients with aneurysmal SAH to assess whether DWI provides predictive markers of silent ischemic lesions and/or progression toward symptomatic ischemia. Methods — Seven asymptomatic vasospasm patients (average blood velocity rates >120 cm/s), 3 patients with symptomatic vasospasm, and 4 patients with SAH but without vasospasm were studied at regular intervals by DWI, and their apparent diffusion coefficients (ADCs) were calculated. Results — All patients with vasospasm including those without symptoms presented abnormalities on DWI with a reduction of the ADC prevalently in the white matter. No such abnormalities were observed in patients without vasospasm. The abnormalities on DWI resolved completely in 4 of the 7 patients, with no parenchymal lesion. Resolution was partial in 3 patients whose white matter still presented residual round, focal ischemic lesions. Conclusions — Being able to correlate abnormalities on DWI with parenchymal involvement in asymptomatic patients would be of considerable clinical significance. It is hoped that larger studies will be undertaken to determine whether the ADC has a reversibility threshold, because this would facilitate patient management.

The authors report a case of secondary worsening of neurological symptoms in a patient 2 months after cord injury at T5 causing paraplegia. The MRI showed myeolomalacia, which appears as cord oedema, located in the grey matter, extending increasingly from the initial lesion (eighth thoracic vertebra) to the bulb. This cord lesion known as grey matter cytotoxic oedema, evolved into a syringomyelic cavity.

Objective Watershed infarcts (WSI) are thought to result from hemodynamic mechanism, but studies have suggested that microemboli from unstable carotid plaques may distribute preferentially in watershed areas, i.e., between two cerebral arterial territories. Intraplaque haemorrhage (IPH) is an emerging marker of plaque instability and microembolic activity. We assessed the association between WSI and IPH in patients with recently symptomatic moderate carotid stenosis. Methods and Results We selected 65 patients with symptomatic moderate (median NASCET degree of stenosis = 31%) carotid stenosis and brain infarct on Diffusion-Weighted Imaging (DWI) on Magnetic Resonance Imaging (MRI) from a multicentre prospective study. Fourteen (22%) had WSI (cortical, n = 8; internal, n = 4; cortical and internal, n = 2). Patients with WSI were more likely to have IPH than those without WSI although the difference was not significant (50% vs. 31%, OR = 2.19; 95% CI, 0.66–7.29; P = 0.20). After adjustment for degree of stenosis, age and gender, the results remained unchanged. Conclusion About one in fifth of brain infarcts occurring in patients with moderate carotid stenosis were distributed in watershed areas. Albeit not significant, an association between IPH - more generally plaque component - and WSI, still remains possible.

Conventional magnetic resonance imaging (MRI) at term equivalent age (TEA) is suggested to be a reliable tool to predict the outcome of very premature infants. The objective of this study was to determine simple reproducible MRI indices, in premature infants and to analyze their neonatal determinants at TEA. A cohort of infants born before 32 weeks gestational age (GA) underwent a MRI at TEA in our center. Two axial images (T2 weighted), were chosen to realize nine measures. We defined 4 linear indices (MAfhlv: thickness of lateral ventricle; CSI: cortex-skull index; VCI: ventricular-cortex index; BOI: bi occipital index) and 1 surface index (VS.A: volume slice area). Perinatal data were recorded. Sixty-nine infants had a GA (median (interquartile range)) of 30.0 weeks GA (27.0; 30.0) and a birth weight of 1240 grams (986; 1477). MRI was done at 41.0 (40.0; 42.0) weeks post menstrual age (PMA). The inter-investigator reproducibility was good. Twenty one MRI (30.5%) were quoted abnormal. We observed an association with retinopathy of prematurity (OR [95CI] = 4.205 [1.231–14.368]; p = 0.017), surgery for patent ductus arteriosus (OR = 4.688 [1.01–21.89]; p = 0.036), early onset infection (OR = 4.688 [1.004–21.889]; p = 0.036) and neonatal treatment by cefotaxime (OR = 3.222 [1.093–9.497]; p = 0.03). There was a difference for VCI between normal and abnormal MRI (0.412 (0.388; 0.429) vs. 0.432 (0.418; 0.449); p = 0,019); BOI was higher when fossa posterior lesions were observed; VS.A seems to be the best surrogate for cerebral volume, 80% of VS.As’ variance being explained by a multiple linear regression model including 7 variables (head circumference at birth and at TEA, PMA, dopamine, ibuprofen treatment, blood and platelets transfusions). These indices, easily and rapidly achievable, seem to be useful but need to be validated in a large population to allow generalization for diagnosis and follow-up of former premature infants.

Background Syringomyelia due to intracranial hypotension is rarely described. As a consequence, intracranial hypotension is less recognized as a potential cause of syringomyelia or mistaken with Chiari type 1 malformation. The pathogeny is poorly understood, and we lack diagnostic and therapeutic strategies for this particular setting. Case Description We describe a 45-year-old patient who developed syringomyelia after about 10 years of undiagnosed intracranial hypotension caused by traumatic C6 cerebrospinal fluid (CSF) leak. Surgical closing of the leak was required to treat intracranial hypotension after failure of conservative measures and blind epidural patches. It led to a marked improvement of cerebral and spinal signs. We discuss the pathogeny of syringomyelia caused by intracranial hypotension and highlight a mechanical theory of hyperpressure against the cervical spine due to blockage of CSF flow by descent of cerebellar tonsils at the foramen magnum level. We describe discriminating clinical and radiologic signs to differentiate intracranial hypotension from Chiari type 1 malformation and discuss mechanisms and causality relating trauma and intracranial hypotension. Conclusions Syringomyelia can be a consequence of long-term progression of intracranial hypotension, which must be differentiated from Chiari type 1 malformation. In our case, resolution was achieved by detecting and closing the CSF leak causing the intracranial hypotension. Reports of similar cases are necessary to understand the origin of CSF leak in traumatic intracranial hypotension and assess the best therapeutic strategy.

PURPOSE: To evaluate the technical quality and the diagnostic performance of a protocol with use of low volumes of contrast medium (25 mL) at 64-detector spiral computed tomography (CT) in the diagnosis and management of adult, nontraumatic subarachnoid hemorrhage (SAH). MATERIALS AND METHODS: This study was performed outside the United States and was approved by the institutional review board. Intracranial CT angiography was performed in 73 consecutive patients with nontraumatic SAH diagnosed at nonenhanced CT. Image quality was evaluated by two observers using two criteria: degree of arterial enhancement and venous contamination. The two independent readers evaluated diagnostic performance (lesion detection and correct therapeutic decision-making process) by using rotational angiographic findings as the standard of reference. Sensitivity, specificity, and positive and negative predictive values were calculated for patients who underwent CT angiography and three-dimensional rotational angiography. The intraclass correlation coefficient was calculated to assess interobserver concordance concerning aneurysm measurements and therapeutic management. RESULTS: All aneurysms were detected, either ruptured or unruptured. Arterial opacification was excellent in 62 cases (85%), and venous contamination was absent or minor in 61 cases (84%). In 95% of cases, CT angiographic findings allowed optimal therapeutic management. The intraclass correlation coefficient ranged between 0.93 and 0.95, indicating excellent interobserver agreement. CONCLUSION: With only 25 mL of iodinated contrast medium focused on the arterial phase, 64-detector CT angiography allowed satisfactory diagnostic and therapeutic management of nontraumatic SAH.

The emergence of high-resolution rapid imaging methods has enabled magnetic resonance (MR) imagers to noninvasively image the fine internal structure of cervical arterial walls. In this article, a comprehensive guide to performing high-resolution MR imaging of cervical arteries is provided, including the choice of coils, sequences, and imaging parameters, as well as tips for optimal image quality. Explanations and illustrations are given of using high-resolution MR imaging to quantify plaque volume, determine atherosclerotic plaque burden, depict plaque composition, and ultimately identify unstable plaque before it leads to a clinical event. Finally, the role of high-resolution MR imaging in the diagnosis of cervical dissection and inflammatory disease of the arterial wall is emphasized.

Posterior reversible encephalopathy (PRES) represents an uncommon entity related to multiple pathologies, the most common of which is hypertensive crisis. PRES is classically characterized as symmetrical parieto-occipital edema, but may affect other areas of the brain. Diffusion-weighted magnetic resonance imaging (DWI) is important for differentiating between vasogenic and cytotoxic edema. We present here the case of a 43-year-old woman, known to suffer from arterial hypertension and severe renal failure, who developed PRES with restricted apparent diffusion coefficients (ADC) in various cerebral areas, suggesting irreversible tissue damage. Nevertheless, follow-up cranial MRI revealed complete remission, indicating that restricted diffusion does not always lead to cell death in this pathology. The underlying pathophysiological mechanism is not well understood. Such reversibility of diffusion anomalies has already been reported with transient ischemia, vasospasm after subarachnoid hemorrhage and epilepsy but, to our knowledge, never before in PRES.

OBJECTIVE: Angioleiomyomas are soft tissue tumors with smooth muscle and vascular components. They are extremely rare in intracranial locations and only three cases have been reported in the literature, including one in the cavernous sinus. Furthermore, long-term follow-up after surgery for a tumor at this site has not been described. We report a new case of intracavernous angioleiomyoma with complete surgical removal and no recurrence after 6 years of clinical and radiological follow-up. CLINICAL PRESENTATION: A 50-year-old patient presented with vertical diplopia resulting from left trochlear nerve palsy. Magnetic resonance examination showed a left intracavernous tumor with hypersignal on T2-weighted images, hyposignal on T1-weighted images, and delayed homogeneous enhancement after gadolinium injection. INTERVENTION: Surgical treatment was completed by a left pterional approach. There was a clear plane separating the tumor from the vascular and nervous elements of the cavernous sinus. Complete tumor resection was achieved. Diplopia improved after surgery. Follow-up did not reveal any recurrence. CONCLUSION: Angioleiomyomas are rare benign tumors with an excellent prognosis after total removal that justifies surgical treatment as the first-line treatment.