Viruses belonging to the herpes family group, including HSV (herpes simplex virus) 1 and 2, VZV (varicella zoster virus) and CMV (cytomegalovirus) are the leading causes of necrotizing retinitis. These viral retinal necroses generally manifest in three forms, depending on the patient's immune status: acute retinal necrosis (ARN), progressive outer retinal necrosis (PORN) and CMV retinitis. Although specific, effective drug treatments are available today, early treatment initiation is essential to avoid sight-threatening complications.
View Article and Find Full Text PDFMult Scler Relat Disord
October 2023
Background: Uveitis may be associated with multiple sclerosis (MS) in 1% of cases. Prognosis of this association remains unknown.
Methods: We conducted a retrospective analysis in a cohort of 41 patients with MS (34 relapsing-remitting MS, and 7 secondary progressive MS) matched with 123 controls (MS without uveitis) followed in Department of Neurology, Pitié Salpêtrière Hospital, Paris.
Neurosarcoidosis (NS) is a rare but severe form of sarcoidosis. NS is associated with significant morbidity and mortality. Mortality is about 10% at 10 years with more than 30% of patients who have a significant disability.
View Article and Find Full Text PDFThe management of primary central nervous system (PCNSL) is one of the most controversial topics in neuro-oncology because of the complexity of the disease and the limited number of controlled studies available. In 2021, given recent advances and the publication of practice-changing randomized trials, the European Association of Neuro-Oncology (EANO) created a multidisciplinary task force to update the previously published evidence-based guidelines for immunocompetent adult patients with PCNSL and added a section on immunosuppressed patients. The guideline provides consensus considerations and recommendations for the treatment of PCNSL, including intraocular manifestations and specific management of the elderly.
View Article and Find Full Text PDFIntroduction: Vitreoretinal lymphoma is a rare ocular cancer with high morbidity and mortality despite treatment. Diagnosis by cytopathology is often delayed, and various molecular and image-based investigations have been developed. Diverse treatments are used, but there is a limited medical evidence to differentiate their effectiveness.
View Article and Find Full Text PDFJCO We previously reported the results of a randomized phase II study in patients with newly diagnosed primary CNS lymphoma (age 18-60 years). Patients were treated with high-dose methotrexate-based induction chemotherapy followed by whole-brain radiotherapy (WBRT) or high-dose chemotherapy (thiotepa-busulfan-cyclophosphamide) with autologous stem-cell transplantation (ASCT). The median follow-up was 33 months.
View Article and Find Full Text PDFErdheim-Chester disease (ECD) is a rare L-group histiocytosis. Orbital involvement is found in a third of cases, but few data are available concerning the radiological features of ECD-related orbital disease (ECD-ROD). Our aim was to characterize the initial radiological phenotype and outcome of patients with ECD-ROD.
View Article and Find Full Text PDFPurpose Of Review: The aim of this study was to highlight the diagnostic and management challenges of primary vitreoretinal lymphoma (PVRL) through a review of the literature and a European survey on real-life practices for PVRL.
Recent Findings: The care of PVRL patients is heterogeneous between specialists and countries. Upfront systemic treatment based on high-dose methotrexate chemotherapy, with or without local treatment, might reduce or delay the risk of brain relapse.
Expert Rev Clin Immunol
September 2021
: Noninfectious uveitis (NIU) is one of the leading causes of blindness worldwide. In adult patients, anterior NIU is usually managed with topical corticosteroids. In intermediate, posterior uveitis.
View Article and Find Full Text PDFThe term "vitritis" refers to the presence of a cellular infiltration of the vitreous body, usually in the context of an intraocular inflammation, but not exclusively. Intermediate uveitis is the most prominent cause of vitritis, including infectious and auto-immune/auto-inflammatory etiologies. Corticosteroids and immunosuppressive therapies should not be started before ruling out the infectious causes of vitritis, especially in immunosuppressed individuals.
View Article and Find Full Text PDFPurpose: The purpose of this study was to report retinal dystrophy as a novel clinical feature and expand the ocular phenotype in patients harboring biallelic candidate FDXR variants.
Methods: Patients carrying biallelic candidate FDXR variants were identified by whole genome sequencing (WGS) as part of the National Institute for Health Research BioResource rare-disease and the UK's 100,000 Genomes Project (100KGP) with an additional case identified by exome sequencing. Retrospective clinical data were collected from the medical records.
Background And Objective: The prognosis in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a matter of debate. Our aim was to assess the long-term outcomes of patients with MOGAD.
Methods: We retrospectively analysed the clinical and paraclinical data of patients from the French nationwide observatory study NOMADMUS who tested positive for MOG antibodies (MOG-IgG) and who had clinical follow-up of at least 8 years from their first episode.
Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a new retinal optical coherence tomography (OCT) finding. The had made recommendations to distinguish PHOMS from true optic disc drusen (ODD) in 2018. While publications on PHOMS have increased since then, the accuracy of the definition of PHOMS and reliability of detection is unknown.
View Article and Find Full Text PDFBackground: Neuro-ophthalmologic manifestations are uncommon in sarcoidosis. We aim to assess the prognostic factors and outcome of neuro-ophthalmic sarcoidosis.
Methods: We conducted a multicenter retrospective study on patients with neuro-ophthalmic sarcoidosis.
Background: During the last decade, our understanding of cerebrospinal fluid (CSF) physiology has dramatically improved, thanks to the discoveries of both the glymphatic system and lymphatic vessels lining the dura mater in human brains.
Evidence Acquisition: We detail the recent basic science findings in the field of CSF physiology and connect them with our current understanding of the pathophysiology of idiopathic intracranial hypertension (IIH).
Results: Transverse sinus (TS) stenoses seem to play a major causative role in the symptoms of IIH, as a result of a decrease in the pressure gradient between the venous system and the subarachnoid space.