[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.

[Raxone in the Leber optical neuropathy: Parisian experience]. / Raxone dans la neuropathie optique de Leber : retour d'expérience parisienne.

INTRODUCTION: Leber's Hereditary Optic Neuropathy (LHON) causes a rapid and severe decrease in visual acuity. Raxone® (Idebenone, Santhera) is the only drug to have a European Marketing Authorization for the treatment of this optic neuropathy. It can be proposed in the first months after the onset of this optic neuropathy, according to an international consensus meeting. PATIENTS AND METHODS: Retrospective study of the efficacy of Raxone® on the visual acuity of patients with genetically confirmed LHON who were followed in four Parisian hospitals. The primary endpoint is the best recovery of LogMar visual acuity between baseline and the end of follow-up. The secondary endpoints are the evolution of LogMar visual acuity of the best eye at baseline and change in LogMar visual acuity for each eye considered separately. RESULTS: Seventeen patients, three women and 14 men, mean age 34.2 years, naive to treatment with Raxone® were included in this study. The mean duration of treatment was 11.0±6.6 months. A mitochondrial DNA mutation was found in all patients. Only 2 had the 14484 mutation. A recovery of better LogMar visual acuity was found at the end of the treatment for 4 eyes (23.5 %), and a deterioration was observed for 8 (47.0 %). Only 2 eyes (11.7 %) with the best visual acuity at baseline improved. On the other hand, 17.6 % of the eyes considered separately had an improvement in their LogMar visual acuity at the end of the treatment. CONCLUSION: The results confirm the trend of Raxone® treatment to improve patients' visual acuity. Given the recommendations of a consensus conference, this treatment should be started early after the onset of LHON. It is therefore important to look for this diagnosis in the presence of any hereditary optic neuropathy, in order to be able to initiate this treatment.

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.

Etiologies of acute demyelinating optic neuritis: an observational study of 110 patients.

BACKGROUND AND PURPOSE: New criteria for the diagnosis of multiple sclerosis (MS) and discovery of myelin oligodendrocyte glycoprotein (MOG) or aquaporin-4 (AQP4) antibodies (Abs) have changed the management of optic neuritis (ON). Our aim was to specify, in view of these recent advances, the etiologies of acute demyelinating ON for consecutive patients. METHODS: Retrospective database analysis was undertaken of consecutive adult patients with acute ON admitted from 1 December 2014 to 31 January 2016. Diagnosis of MS was made according to the 2010 McDonald criteria. Patients with Abs to AQP4 or MOG were classified as ON-AQP4 and ON-MOG, respectively. Patients who did not fulfill the diagnostic criteria and were negative for AQP4 and MOG Ab tests were classified as having idiopathic ON. RESULTS: Of 110 patients assessed, 78 had ON related to MS (70.9%). All patients without MS were tested for AQP4 and MOG Abs: 11 had MOG Ab (10%), 5 had AQP4 Ab (4.5%) and 16 were considered as having idiopathic ON (14.5%). Presence of intrathecal IgG oligoclonal bands was strongly associated with MS (mean, 88.4% vs. 34.4% in patients without MS; after Bonferroni correction, P < 0.0001). CONCLUSIONS: Optic neuritis related to MOG Ab was the second cause identified of demyelinating ON in our center. Idiopathic ON was as frequent as both ON-AQP4 and ON-MOG combined.

Optical coherence tomography (OCT) in optic neuritis and multiple sclerosis.

Optical coherence tomography (OCT) is a non-invasive imaging technique routinely used in ophthalmology to visualize and quantify the layers of the retina. It also provides information on optic nerve head topography, peripapillary retinal nerve fiber layer thickness, and macular volume, which correlate with axonal loss. These measurements are of particular interest in optic neuropathies and in multiple sclerosis, and OCT parameters are now used as endpoints in neurologic clinical trials.

[Vascular transient monocular visual loss]. / Les troubles visuels monoculaires transitoires d'origine vasculaire.

Diagnosis and management of transient monocular visual loss is an emergency. Ocular conditions causing transient visual loss are routinely managed by ophthalmologists. Vascular transient monocular visual loss may result from emboli, hypoperfusion, vasospasm, or venous congestion. Evaluation focuses on the carotid arteries, ophthalmic arteries, the aortic arch, the heart, and rarely hypercoagulable states. Secondary prevention of ischemic events is essential in order to prevent permanent visual loss as well as cerebral ischemic and cardiovascular death. Aggressive treatment of vascular risk factors is usually associated with antiplatelet agents. Anticoagulant and carotid surgery are only rarely required after vascular transient monocular visual loss.

[Transient visual loss: a practical approach]. / Les troubles visuels transitoires : démarche diagnostique.

Transient vision loss results from a variety of disorders including benign dry eye as well as vision- or life-threatening disorders such as giant cell arteritis. The goal of the initial evaluation is to determine the most likely mechanism based on the past medical history, characteristics of visual loss, and ophthalmologic and neurologic examinations. The ophthalmologist is often the first physician consulted and plays an essential role. Indeed, identification of the mechanism of transient visual loss allows the care-taker to properly decide on the work-up, its timing (emergency or otherwise), and subsequent treatment.

[Ocular disturbances in neuromuscular disorders]. / Manifestations oculaires des maladies du muscle et de la jonction neuromusculaire.

Compared with other skeletal muscles, extraocular muscles have fundamentally distinct properties that make them selectively vulnerable to certain neuromuscular disorders. When the oculomotor signs are predominant, their temporal progression allows the clinician to make the distinction between a muscular disease (mitochondrial disorder, oculopharyngeal muscular dystrophy...) and a disorder of the neuromuscular junction (myasthenia gravis, botulism...). In other instances, such as myotonic dystrophy or facioscapulohumeral dystrophy, the ocular signs are not in the forefront but must be recognized by the ophthalmologist as hallmarks of a muscular disorder. In all cases, the collaboration between the neurologist and the ophthalmologist is fruitful.

[Neuro-ophthalmologic initial presentation of sarcoidosis]. / Manifestations neuro-ophtalmologiques révélatrices d'une neuro-sarcoïdose.

PURPOSE: To describe different forms of neuro-ophthalmologic onset of sarcoidosis: clinical signs, means of diagnosis, treatment, and progression. PATIENTS AND METHODS: Retrospective study of 13 patients with neuro-ophthalmologic initial onset of sarcoidosis diagnosed in three departments between 1997 and 2003. RESULTS: There were ten women and three men, with a mean age of 36 years. Six patients suffered from diplopia. In three cases, the cavernous sinus was involved; the three other patients with diplopia had meningoradiculitis. Nine patients had infiltration of the anterior visual pathway: the optic nerve was involved in five cases, the chiasm in two cases, and two patients had papilledema. Two patients also had both symptoms. The dosage of the angiotensin-converting enzyme level was evaluated in 11 patients and was elevated in six cases. Nine patients underwent a lumbar puncture; the cerebrospinal fluid protein was high in seven cases. Chest radiography and CT were abnormal in nine cases of 11. Ten patients had histological proof of sarcoidosis; the three others had enough evidence to support this diagnosis. All of them were treated with systemic corticosteroids. The diplopia improved for the six patients. Among the seven patients with optic nerve or chiasmal infiltration, one recovered completely, two were partially improved, and four remained stable. CONCLUSIONS: Diplopia and anterior visual pathway abnormalities can be the manifestation of initial onset of sarcoidosis; therefore this diagnosis must be kept in mind when these frequent neuro-ophthalmologic signs are encountered. Complementary exams, mainly biopsy of the involved areas with histological analysis, are needed to confirm this diagnosis. Corticosteroid treatment is generally followed by improvement, but relapses may occur.