Giant cell arteritis with ocular involvement successfully treated with tocilizumab and very short-course glucocorticoids: A case report.

PURPOSE: To report the case of a patient with arteritic anterior ischemic optic neuropathy (AAION) with a history of glucocorticoids (GC) hypersensitivity treated with monthly intravenous tocilizumab (IV TCZ) and short course of GC. CASE REPORT: A 71-year-old Caucasian patient presented with AAION related to giant cell arteritis (GCA) confirmed by temporal artery biopsy. Past medical history was significant for GC hypersensitivity proven by allergy evaluation and the patient was managed with IV TCZ and very short-course GC. CONCLUSION: TCZ therapy with very short-course GC could be effective in GCA with ophthalmic involvement as a first-line strategy. Clinical trials are needed for thorough evaluation of the efficiency of TCZ as a first-line treatment to induce and maintain remission in patients with GCA and ocular involvement.

[Neurological complications of JC virus infection: A review]. / Complications neurologiques de l'infection par le virus JC : revue générale.

Although human polyomavirus JC (JCV) seroprevalence in the general population is high, its neurological complications are rare and progressive multifocal leukoencephalopathy (PML), a lethal central nervous system (CNS) demyelinating disease, is the most well-known. After an usually asymptomatic primary infection during late childhood, a latent JCV form persists in different sites, notably the kidneys and lymphocytes. Rearrangement of that archetype into the prototypical neurotropic strain can reactivate JCV, thereby enabling its CNS penetration and infection of glial cells. In a context of defective immune defenses (HIV infection, cancer or immunosuppressant therapies) this infection leads to oligodendrocyte death that contributes, via demyelinization, to PML but also, as more recently described, to other CNS complications, e.g., JCV granule cell neuronopathy, meningitis or encephalitis. Clinical manifestations depend on the localization of the lesions. The increasingly widespread use of new immunomodulatory monoclonal antibodies to treat multiple sclerosis and other inflammatory systemic diseases has increased PML frequency in those previously rarely affected entities. Diagnosis relies on magnetic resonance imaging, JCV detection in cerebrospinal fluid and, when necessary, brain histology. PML is often lethal. No specific, evidence-based treatment with clinically relevant efficacy is available. The therapeutic objective is to restore host immune responses to JCV, while avoiding immune-reconstitution inflammatory syndrome.

[Delays in the management of ocular complications of giant cell arteritis: A retrospective monocentric study of 33 patients]. / Délais de prise en charge des complications visuelles d'artérite à cellules géantes : étude rétrospective monocentrique de 33 patients.

INTRODUCTION: Ocular complications of giant cell arteritis (GCA) can lead to irreversible bilateral blindness and represent a therapeutic emergency. Recommendations for the management of GCA have recently been updated. The objective of the study was to evaluate delays in appropriate management of the ocular complications of GCA and its determinants. METHOD: Retrospective, monocentric study, conducted over the period January 2013-November 2018. All consecutive patients with a final diagnosis of GCA and related visual impairment (permanent visual loss and/or alteration of visual field) were included. RESULTS: Thirty-three patients were included (women: 21, men: 12; mean age at diagnosis: 79). Twenty-seven patients (82%) presented with symptoms suggestive of ACG prior to the visual complication, ranging from a few weeks to several months. Seventeen patients (52%) had a known biological inflammatory syndrome (median CRP at 64 mg/L) prior to hospital consultation. The median time from the onset of permanent ophthalmologic manifestations to appropriate corticosteroid management was 3 days (range: 0-134). Two of the 21 patients who consulted an out-of-hospital ophthalmologist received corticosteroid therapy before referral to hospital. Three patients (9%) were treated within 24 h of the onset of the disorders. CONCLUSION: There is a significant delay in the appropriate management of ophthalmological complications of ACG and deviations from current recommendations. Numerous actions must therefore be taken to improve the visual prognosis of patients with ACG, both preventively (i.e. early diagnosis and treatment of ACG before the possible occurrence of visual complications), and curatively (rapid recognition and immediate treatment of ocular complications). These elements support the relevance of specific fast-track pathways for GCA.

Advanced multiparametric magnetic resonance imaging of multinodular and vacuolating neuronal tumor.

BACKGROUND AND PURPOSE: Multinodular and vacuolating neuronal tumor (MVNT) of the cerebrum is a rare brain lesion with suggestive imaging features. The aim of our study was to report the largest series of MVNTs so far and to evaluate the utility of advanced multiparametric magnetic resonance (MR) techniques. METHODS: This multicenter retrospective study was approved by our institutional research ethics board. From July 2014 to May 2019, two radiologists read in consensus the MR examinations of patients presenting with a lesion suggestive of an MVNT. They analyzed the lesions' MR characteristics on structural images and advanced multiparametric MR imaging. RESULTS: A total of 64 patients (29 women and 35 men, mean age 44.2 ± 15.1 years) from 25 centers were included. Lesions were all hyperintense on fluid-attenuated inversion recovery and T2-weighted imaging without post-contrast enhancement. The median relative apparent diffusion coefficient on diffusion-weighted imaging was 1.13 [interquartile range (IQR), 0.2]. Perfusion-weighted imaging showed no increase in perfusion, with a relative cerebral blood volume of 1.02 (IQR, 0.05) and a relative cerebral blood flow of 1.01 (IQR, 0.08). MR spectroscopy showed no abnormal peaks. Median follow-up was 2 (IQR, 1.2) years, without any changes in size. CONCLUSIONS: A comprehensive characterization protocol including advanced multiparametric magnetic resonance imaging sequences showed no imaging patterns suggestive of malignancy in MVNTs. It might be useful to better characterize MVNTs.

Clinical, imaging and follow-up study of optic neuritis associated with myelin oligodendrocyte glycoprotein antibody: a multicentre study of 62 adult patients.

BACKGROUND AND PURPOSE: There are few clinico-radiological data on optic neuritis (ON) with myelin oligodendrocyte glycoprotein antibody (MOG-IgG). The objective was to characterize the clinico-radiological phenotype and outcome of patients with MOG-IgG-related ON. METHODS: The records of all adult patients admitted in three medical centres with MOG-IgG-associated ON who underwent orbital and brain magnetic resonance imaging (MRI) at the acute phase were reviewed. Spinal cord MRI within 1 month from the ON and all of the follow-up MRI were reviewed. RESULTS: Of 62 patients, 41.9% had bilateral ON and 66.2% optic disc swelling. On initial MRI, lesions were anterior (92%), extensive (63%) and associated with optic perineuritis (46.6%). Silent brain lesions were found in 51.8% of patients but were mainly non-specific (81%). Of 39 individuals with spinal MRI at onset, nine had abnormal findings (four were asymptomatic). Two symptomatic patients had longitudinally extensive myelitis with concurrent H-sign. At last follow-up, 5% of patients had visual acuity ≤0.1. Brain MRI remained unchanged in 41 patients (87%). CONCLUSIONS: Our study supports a mostly benign ophthalmological course of MOG-IgG-associated ON, despite initially longitudinally extensive lesions and development of optic nerve atrophy on orbital MRI. Spinal MRI could be of interest in detecting silent suggestive lesions.

Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance.

Multinodular and vacuolating neuronal tumor of the cerebrum is a rare supratentorial brain tumor described for the first time in 2013. Here, we report 11 cases of infratentorial lesions showing similar striking imaging features consisting of a cluster of low T1-weighted imaging and high T2-FLAIR signal intensity nodules, which we referred to as multinodular and vacuolating posterior fossa lesions of unknown significance. No relationship was found between the location of the lesion and clinical symptoms. A T2-FLAIR hypointense central dot sign was present in images of 9/11 (82%) patients. Cortical involvement was present in 2/11 (18%) of patients. Only 1 nodule of 1 multinodular and vacuolating posterior fossa lesion of unknown significance showed enhancement on postcontrast T1WI. DWI, SWI, MRS, and PWI showed no malignant pattern. Lesions did not change in size or signal during a median follow-up of 3 years, suggesting that multinodular and vacuolating posterior fossa lesions of unknown significance are benign malformative lesions that do not require surgical intervention or removal.

Improving Detection of Multiple Sclerosis Lesions in the Posterior Fossa Using an Optimized 3D-FLAIR Sequence at 3T.

BACKGROUND AND PURPOSE: There is no consensus regarding the best MR imaging sequence for detecting MS lesions. The aim of our study was to assess the diagnostic value of optimized 3D-FLAIR in the detection of infratentorial MS lesions compared with an axial T2-weighted imaging, a 3D-FLAIR with factory settings, and a 3D double inversion recovery sequence. MATERIALS AND METHODS: In this prospective study, 27 patients with confirmed MS were included. Two radiologists blinded to clinical data independently read the following sequences: axial T2WI, 3D double inversion recovery, standard 3D-FLAIR with factory settings, and optimized 3D-FLAIR. The main judgment criterion was the overall number of high-signal-intensity lesions in the posterior fossa; secondary objectives were the assessment of the reading confidence and the measurement of the contrast. A nonparametric Wilcoxon test was used to compare the MR images. RESULTS: Twenty-two patients had at least 1 lesion in the posterior fossa. The optimized FLAIR sequence detected significantly more posterior fossa lesions than any other sequence: 7.5 versus 5.8, 4.8, and 4.1 (P values of .04, .03, and .03) with the T2WI, the double inversion recovery, and the standard FLAIR, respectively. The reading confidence index was significantly higher with the optimized FLAIR, and the contrast was significantly higher with the optimized FLAIR than with the standard FLAIR and the double inversion recovery. CONCLUSIONS: An optimized 3D-FLAIR sequence improved posterior fossa lesion detection in patients with MS.

Diagnosis and Prediction of Relapses in Susac Syndrome: A New Use for MR Postcontrast FLAIR Leptomeningeal Enhancement.

BACKGROUND AND PURPOSE: Leptomeningeal enhancement can be found in a variety of neurologic diseases such as Susac Syndrome. Our aim was to assess its prevalence and significance of leptomeningeal enhancement in Susac syndrome using 3T postcontrast fluid-attenuated inversion recovery MR imaging. MATERIALS AND METHODS: From January 2011 to December 2017, nine consecutive patients with Susac syndrome and a control group of 73 patients with multiple sclerosis or clinically isolated syndrome were included. Two neuroradiologists blinded to the clinical and ophthalmologic data independently reviewed MRIs and assessed leptomeningeal enhancement and parenchymal abnormalities. Follow-up MRIs (5.9 MRIs is the mean number per patient over a median period of 46 months) of patients with Susac syndrome were reviewed and compared with clinical and retinal fluorescein angiographic data evaluated by an independent ophthalmologist. Fisher tests were used to compare the 2 groups, and mixed-effects logistic models were used for analysis of clinical and imaging follow-up of patients with Susac syndrome. RESULTS: Patients with Susac syndrome were significantly more likely to present with leptomeningeal enhancement: 5/9 (56%) versus 6/73 (8%) in the control group (P = .002). They had a significantly higher leptomeningeal enhancement burden with ≥3 lesions in 5/9 patients versus 0/73 (P < .001). Regions of leptomeningeal enhancement were significantly more likely to be located in the posterior fossa: 5/9 versus 0/73 (P < .001). Interobserver agreement for leptomeningeal enhancement was good (κ = 0.79). There was a significant association between clinical relapses and increase of both leptomeningeal enhancement and parenchymal lesion load: OR = 6.15 (P = .01) and OR = 5 (P = .02), respectively. CONCLUSIONS: Leptomeningeal enhancement occurs frequently in Susac syndrome and could be helpful for diagnosis and in predicting clinical relapse.

Combining Multiple Magnetic Resonance Imaging Sequences Provides Independent Reproducible Radiomics Features.

To evaluate the relative contribution of different Magnetic Resonance Imaging (MRI) sequences for the extraction of radiomics features in a cohort of patients with lacrimal gland tumors. This prospective study was approved by the Institutional Review Board and signed informed consent was obtained from all participants. From December 2015 to April 2017, 37 patients with lacrimal gland lesions underwent MRI before surgery, including axial T1-WI, axial Diffusion-WI, coronal DIXON-T2-WI and coronal post-contrast DIXON-T1-WI. Two readers manually delineated both lacrimal glands to assess inter-observer reproducibility, and one reader performed two successive delineations to assess intra-observer reproducibility. Radiomics features were extracted using an in-house software to calculate 85 features per region-of-interest (510 features/patient). Reproducible features were defined as features presenting both an intra-class correlation coefficient ≥0.8 and a concordance correlation coefficient ≥0.9 across combinations of the three delineations. Among these features, the ones yielding redundant information were identified as clusters using hierarchical clustering based on the Spearman correlation coefficient. All the MR sequences provided reproducible radiomics features (range 14(16%)-37(44%)) and non-redundant clusters (range 5-14). The highest numbers of features and clusters were provided by the water and in-phase DIXON T2-WI and water and in-phase post-contrast DIXON T1-WI (37, 26, 26 and 26 features and 14,12, 9 and 11 clusters, respectively). A total of 145 reproducible features grouped into 51 independent clusters was provided by pooling all the MR sequences. All MRI sequences provided reproducible radiomics features yielding independent information which could potentially serve as biomarkers.

A 3T Phase-Sensitive Inversion Recovery MRI Sequence Improves Detection of Cervical Spinal Cord Lesions and Shows Active Lesions in Patients with Multiple Sclerosis.

BACKGROUND AND PURPOSE: Magnetic Resonance Imaging is the modality of choice to detect spinal cord lesions in patients with Multiple Sclerosis (MS). However, this imaging is challenging. New sequences such as phase-sensitive inversion recovery have been developed to improve detection. Our aim was to compare a 3D phase-sensitive inversion recovery and a conventional imaging dataset including postcontrast T2WI and T1WI to detect MS spinal cord lesions. MATERIALS AND METHODS: This retrospective single-center study included 100 consecutive patients with MS (mean age, 41 years) from January 2015 to June 2016. One senior neuroradiologist and 1 junior radiologist blinded to clinical data checked for new spinal cord lesions, individually analyzing conventional and 3D phase-sensitive inversion recovery datasets separately, placing a 3-week delay between the 2 readings. A consensus reading was done with a third senior neuroradiologist. A Wilcoxon test was used to compare the 2 imaging datasets. Intra- and interobserver agreement was assessed by the κ coefficient. RESULTS: 3D phase-sensitive inversion recovery detected significantly more lesions than conventional imaging (480 versus 168, P < .001). Eleven patients had no detected lesions on T2WI, whereas 3D phase-sensitive inversion recovery detected at least 1 lesion. All postcontrast T1WI enhancing lesions were also visible on 3D phase-sensitive inversion recovery. The signal-to-noise ratio was significantly higher using 3D phase-sensitive inversion recovery (0.63 versus 0.46, P = .03). Mean reading confidence was significantly higher using 3D phase-sensitive inversion recovery. Inter- and intraobserver agreement was good for both datasets. CONCLUSIONS: Our study showed that 3D phase-sensitive inversion recovery significantly improved detection of cervical spinal cord lesions, including both enhancing and nonenhancing lesions in patients with MS.

Hemorrhagic transformation after stroke: inter- and intrarater agreement.

BACKGROUND AND PURPOSE: Hemorrhagic transformation (HT) is a complication of stroke that can occur spontaneously or after treatment. We aimed to assess the inter- and intrarater reliability of HT diagnosis. METHODS: Studies assessing the reliability of the European Cooperative Acute Stroke Study (ECASS) classification of HT or of the presence (yes/no) of HT were systematically reviewed. A total of 18 raters independently examined 30 post-thrombectomy computed tomography scans selected from the Aspiration versus STEnt-Retriever (ASTER) trial. They were asked whether there was HT (yes/no), what the ECASS classification of the particular scan (0/HI1/HI2/PH1/PH2) (HI indicates hemorrhagic infarctions and PH indicates parenchymal hematomas) was and whether they would prescribe an antiplatelet agent if it was otherwise indicated. Agreement was measured with Fleiss' and Cohen's κ statistics. RESULTS: The systematic review yielded four studies involving few (≤3) raters with heterogeneous results. In our 18-rater study, agreement for the presence of HT was moderate [κ = 0.55; 95% confidence interval (CI), 0.41-0.68]. Agreement for ECASS classification was only fair for all five categories, but agreement improved to substantial (κ = 0.72; 95% CI, 0.69-0.75) after dichotomizing the ECASS classification into 0/HI1/HI2/PH1 versus PH2. The inter-rater agreement for the decision to reintroduce antiplatelet therapy was moderate for all raters, but substantial among vascular neurologists (κ = 0.70; 95% CI, 0.57-0.84). CONCLUSION: The ECASS classification may involve too many categories and the diagnosis of HT may not be easily replicable, except in the presence of a large parenchymal hematoma.

Acute idiopathic optic neuritis: not always benign.

BACKGROUND AND PURPOSE: Few recent data are available concerning idiopathic optic neuritis (ON). We aimed to describe a large cohort of patients with idiopathic ON. We compared this cohort with patients with ON related to myelin oligodendrocyte glycoprotein (MOG) or ON related to aquaporin-4 (AQP4) antibodies. METHODS: This was a monocentric retrospective observational study. Inclusion criteria for idiopathic ON were as follows: age ≥ 16 years, follow-up of at least 2 years, negative for antibodies against MOG and AQP4 immunoglobulin G, and no magnetic resonance imaging (MRI) lesions suggestive of demyelination (two brain MRI scans, one at baseline and one during follow-up, and one spinal cord MRI scan). RESULTS: Among 23 patients with idiopathic ON (female, 82.6%; median age, 36 years; median follow-up time, 41.4 months), 56.5% had recurrent ON (median time to a second ON episode, 6 months). The final visual acuity in this group (median, 0; mean, 0.43; range, 0-3) was similar to that in the AQP4 group (n = 18; P-value after Bonferroni correction = 0.936) but worse than that in the MOG group (n = 25; P-value after Bonferroni correction = 0.019). At the last evaluation, visual acuity levels were ≤0.5 and <0.2, respectively, in 36.8% and 21% of the idiopathic ON group, 58.3% and 26.7% of the AQP4 group, and 0% and 0% of the MOG group. CONCLUSION: The recovery of visual acuity among patients with idiopathic ON was poor, similar to that observed in the AQP4 group.