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.

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.

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.

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.

Central nervous system Aquaporin4 autoimmunity revealed by a single pseudotumoral encephalic lesion.

The radiological spectrum of neuromyelitis optica has become broader since the detection of aquaporin4 antibodies. We report a case of neuromyelitis optica patient with pseudotumoral encephalic lesion. A 66 year-old woman presented with sudden left lateral homonymous hemianopsia. A brain MRI showed an isolated and extensive right temporo-parieto-occipital lesion, involving periventricular white matter and the corpus callosum, with strong enhancement on post-gadolinium T1 weighted images, highly suggestive of lymphoma. Spinal cord MRI and body CT scan were unremarkable. Lumbar puncture showed pleocytosis, raised total protein level without abnormal cells or oligoclonal bands. A brain biopsy demonstrated non-specific demyelination. Serum aquaporin4 antibodies were positive, which was consistent with the diagnosis of neuromyelitis optica. Cases of central nervous system aquaporin4 autoimmunity presenting with an isolated brain lesion without optic neuritis or myelitis are extremely rare: this is the second case so far and the first one with advanced magnetic resonance characterization. Pseudotumoral encephalic lesions should include a large differential diagnosis, and testing aquaporin4 antibodies must be considered in order to avoid brain biopsy.

TIPIC Syndrome: Beyond the Myth of Carotidynia, a New Distinct Unclassified Entity.

BACKGROUND AND PURPOSE: The differential diagnosis of acute cervical pain includes nonvascular and vascular causes such as carotid dissection, carotid occlusion, or vasculitis. However, some patients present with unclassified vascular and perivascular changes on imaging previously reported as carotidynia. The aim of our study was to improve the description of this as yet unclassified clinico-radiologic entity. MATERIALS AND METHODS: From January 2009 through April 2016, 47 patients from 10 centers presenting with acute neck pain or tenderness and at least 1 cervical image showing unclassified carotid abnormalities were included. We conducted a systematic, retrospective study of their medical charts and diagnostic and follow-up imaging. Two neuroradiologists independently analyzed the blinded image datasets. RESULTS: The median patient age was 48 years. All patients presented with acute neck pain, and 8 presented with transient neurologic symptoms. Imaging showed an eccentric pericarotidian infiltration in all patients. An intimal soft plaque was noted in 16 patients, and a mild luminal narrowing was noted in 16 patients. Interreader reproducibility was excellent. All patients had complete pain resolution within a median of 13 days. At 3-month follow-up, imaging showed complete disappearance of vascular abnormalities in 8 patients, and a marked decrease in all others. CONCLUSIONS: Our study improved the description of an unclassified, clinico-radiologic entity, which could be described by the proposed acronym: TransIent Perivascular Inflammation of the Carotid artery (TIPIC) syndrome.

Evisceration and ocular tumors: What are the consequences?

PURPOSE: Evisceration can be performed for blind, painful eyes. This surgery can promote the dissemination of tumor cells within the orbit if an ocular tumor has been missed preoperatively. METHODS: We reviewed the medical records of patients who were eviscerated for blind, painful eyes between 2009 and 2014 and who were referred after the surgery to the Institut Curie or the Rothschild Foundation in Paris. We included the patients with a histological diagnosis of ocular tumor or orbital recurrence. Cytogenetic analysis was performed whenever possible. RESULTS: Four patients turned out to have an ocular tumor after evisceration (two choroidal melanomas, a rhabdoid tumor and an adenocarcinoma of the retinal pigment epithelium); two had a history of prior ocular trauma. The tumors were diagnosed either on histological analysis of the intraocular contents (2 patients) or biopsy of orbital recurrence (2 patients). Prior to evisceration, fundus examination was not performed in 3 patients. One had preoperative imaging but no intraocular tumor was suspected. At the time of this study, 3 patients had had an orbital recurrence and died. We also found 2 patients who had an evisceration despite a past history of choroidal melanoma treated with proton beam therapy. CONCLUSION: We showed that evisceration of eyes with unsuspected ocular malignancies was associated with a poor prognosis due to orbital recurrence and metastasis. The evisceration specimen should therefore always be sent for histological analysis in order to perform prompt adjuvant orbital radiotherapy if an ocular tumor is found.

Usefulness of colour Doppler flow imaging in the management of lacrimal gland lesions.

OBJECTIVE: To assess the role of colour Doppler flow imaging (CDFI) in the diagnosis and management of lacrimal fossa lesions. METHODS: Institutional ethical committee approval was obtained. Fifty-one patients with 62 lacrimal fossa lesions were retrospectively included from 2003-2015. All patients underwent conventional ultrasonography and CDFI, with a qualitative and quantitative analysis of the vascularization. All patients had lacrimal gland surgery. Definitive diagnosis was based on pathological examination. RESULTS: The study included 47 non-epithelial lesions (NEL) and 15 epithelial lesions (EL), with 24 (39 %) malignant lesions and 38 (61 %) benign lesions. NEL were significantly more likely to present with septa (p < 0.001), hypoechogenicity (p < 0.001), high vascular intensity (p < 0.001), both central and peripheral vascularization (p < 0.001), tree-shape vascularization (p < 0.05) and a low resistance index (RI) (p < 0.0001). EL were significantly more likely to present with the presence of cysts (p < 0.001), and a higher RI. Receiver operating characteristic curves identified a RI value of 0.72 as the best cut-off to differentiate NEL from EL, with a sensitivity and specificity of 100 %. CONCLUSION: CDFI is a valuable tool in the differential diagnosis of lacrimal fossa lesions. Resistance index measurement enables substantial distinction between EL and NEL, thus providing crucial data for surgical management. KEY POINTS: • CDFI is a valuable tool in lacrimal fossa lesions. • Resistance Index measurement enables substantial distinction between epithelial and non-epithelial lesions. • Management of patients becomes more appropriate.

Infraorbital nerve involvement on magnetic resonance imaging in European patients with IgG4-related ophthalmic disease: a specific sign.

OBJECTIVES: To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD). METHODS: From January 2006 through April 2015, 132 patients were admitted for non-lymphoma, non-thyroid-related orbital inflammation. Thirty-eight had both pre-therapeutic orbital MRI and histopathological IgG4 immunostaining. Fifteen patients were classified as cases of IgG4-ROD and 23 patients as cases of non-IgG4-ROD. Two readers performed blinded analyses of MRI images. The main criterion was the presence of an IONE, defined as the infraorbital nerve diameter being greater than the optic nerve diameter in the coronal section. RESULTS: IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P < 0.0001). IONE was only present in cases where, on MRI, the inflammation of the inferior quadrant was present and in direct contact with the ION canal. CONCLUSIONS: In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. Recognition of this pattern may facilitate the accurate diagnosis for clinicians and allow for the adequate management and appropriate care of their patients. KEY POINTS: • IONE on an MRI is a specific sign of IgG4-ROD. • IONE recognition allows for a quicker diagnosis and appropriate management. • IONE appears when inflammation is in direct contact with the ION canal.

Magnetic resonance imaging at one year for detection of postoperative residual cholesteatoma in children: Is it too early?

OBJECTIVE: To compare the residual cholesteatoma detection accuracy of diffusion-weighted (DW) and T1 delayed sequences for magnetic resonance at one year postoperative with second-look surgery in pediatric patients who have undergone primary middle ear surgery for cholesteatoma. METHODS: This was a prospective monocentric consecutive study conducted in a tertiary academic referral center. Children were referred for MR imaging (MRI) one year after surgery. A 1.5T MRI was utilized, using nonecho-planar DW images and delayed gadolinium-enhanced T1-weighted images. Accuracy of magnetic resonance imaging was assessed by two radiologists before surgery. Interobserver and intraobserver agreements were assessed using the κ test. Magnetic resonance imaging data were compared with surgery, which was considered as the gold standard. RESULTS: Twenty-four consecutive unselected pediatric patients were included. Sensitivity, specificity, positive predictive value, and negative predictive value for the first observer were of 40%, 86%, 67%, and 67%, respectively, and those for the second observer were 30%, 86%, 60%, and 63%, respectively. The only two cholesteatoma with a size superior to 3mm were diagnosed before surgery, but the majority of small cholesteatoma were not detected. CONCLUSIONS: MRI is a key examen to diagnosed the residual cholesteatoma but is limited by the size of the lesion under 3mm. Delaying the realization of MRI during follow-up could increase sensitivity, thus avoiding misdiagnosis as well as unnecessary second look surgery.