[Acute visual loss related to sphenoid meningioma]. / Troubles visuels aigus associés aux méningiomes sphénoïdaux.

Meningiomas represent about 20% of intracranial tumors. Involvement of the medial sphenoid wing includes anterior clinoid, cavernous sinus and superior orbital fissure meningiomas. Due to the proximity of these tumors to the optic nerve, typically progressive unilateral vision loss, over several months to years, is the classic clinical presentation. We report three cases of acute monocular vision loss, two transient and one permanent, ipsilateral to a sphenoid meningioma. Ophthalmological involvement with sphenoid meningiomas is most often chronic, due to interruption of axoplasmic flow and demyelination of the optic nerve by local compression. However, vascular involvement with ischemia of the optic nerve or transient low blood flow secondary to compression of the carotid branches vascularizing these structures is another possible mechanism. In our series, two patients had amaurosis fugax, and one patient had sudden, persistent visual loss in relation to acute anterior ischemic optic neuropathy on the side of the meningioma. The mean age of patients with acute visual manifestations was 62 years. These ischemic and non-compressive visual symptoms, ipsilateral to sphenoid meningiomas, are difficult to interpret. Whether these temporary visual disturbances of vascular origin should be considered an early sign of future severe or permanent visual impairment when no optic nerve compression is observed is not certain. The place of these acute visual disturbances in the therapeutic decision, particularly surgical, remains to be defined. Larger multicentric prospective studies are needed to better understand the role of local circulatory factors attributable to meningioma in the occurrence of these acute visual signs.

INFLIXIMAB and ADALIMUMAB in Uveitic Macular Edema.

PURPOSE: To compare the efficacy of infliximab and adalimumab in patients with refractory uveitis-related macular edema (ME). METHODS: A retrospective study was conducted in all patients with refractory uveitis-related ME treated with infliximab or adalimumab in Pitié-Salpêtrière hospital between January 1, 2006 and January 1, 2016. All patients underwent a complete ophthalmologic examination, OCT and retinal angiography at baseline, and 6 (M6) and 24 months (M24) after treatment initiation. Main outcome was a decrease in central foveal thickness (CFT) on OCT. RESULTS: Twenty-five patients were included: 12 treated with adalimumab and 13 treated with infliximab. The median baseline CFT was 381 µm (Q1 = 254; Q3 = 470) in the adalimumab group and 469 µm (307; 539) in the infliximab group. At M6, 6/12 adalimumab-treated patients (50%) and 8/13 infliximab-treated patients (61%) were responders. The median CFT decrease from baseline was 61 µm (17-136) and 66 µm (-59-119) respectively at M6 and M24 in the adalimumab group versus 92 µm (9-165) and 52 µm (33-130) respectively at M6 and M24 in the infliximab group (all p > 0.05). CONCLUSION: No significant difference in efficacy was observed between infliximab and adalimumab at M6 and M24.

Anakinra in the Treatment of Patients with Refractory Scleritis: A Pilot Study.

PURPOSE: This study aimed to evaluate the safety and efficacy of anakinra for severe and refractory scleritis. METHODS: Ten patients with severe (i.e. at least 2 ocular relapses per year despite treatment) and refractory [i.e. at least to one disease modifying antirheumatic drugs (DMARDS)] scleritis were treated with anakinra (100 mg/day subcutaneously). Scleritis was associated with inflammatory systemic diseases in 60% of cases. The remission rate defined the primary outcome. RESULTS: Ninety percent of patients were complete responders with a mean follow-up of 19.4 months after starting anakinra. The corticosteroids daily dose decreased from 18.3 ± 4.1 mg to 4.2 ± 4.9 mg, (p < 0.05), at initiation of anakinra and at end of follow-up, respectively. Associated immunosuppressants were stopped in all cases except one. Side effects were observed in 4 patients who did not need anakinra withdrawal. CONCLUSIONS: This pilot study suggests the efficacy of anakinra in patients with refractory scleritis.

Neuro-ophthalmological conditions: Study of the clinical care pathway.

INTRODUCTION: Neuro-ophthalmologic conditions require specialized multidisciplinary management, both medical and surgical, for patients affected by visual loss due to nervous system disease. The primary goal of this study is to define the specificity of neuro-ophthalmology within the realm of visual health. The secondary goal is to review clinical care pathways by studying the organization of management, in terms of accessibility to care and personalization of the care pathway. PATIENTS AND METHODS: A field study was carried out from February to June 2015, within the ophthalmology service of the Pitié-Salpêtrière University Medical Center in Paris. A 30-minute interview with the patient before or after his or her neuro-ophthalmology consultation was performed, to describe the clinical care pathway. The medical records of interviewed patients were also analyzed. RESULTS: Seventeen care pathways (10 women and 7 men) were reviewed. The mean age at appearance of visual involvement was 44.5 years (±8.4 years). If we exclude 3 patients over 66 years and retired, 35.71% were active, 35.71% were disabled, and 28.57% were on sick leave. Ten patients (58.82%) met the criteria for admission to long-term care. The first step had been carried out by local private practitioners. The first physician seen was the general medicine physician (59%), then the private ophthalmologist on an emergency basis (17%). On average, patients went through 8 steps during their care pathway (from 6 to 10 steps) and 14 medical departments were involved. The study showed collaboration with the other services of the University Hospital Department of Vision and Disabilities (notably with the Fondation Rothschild, the Quinze-Vingts National Ophthalmology Hospital, and the Fondation Sainte-Marie). In addition to rehabilitation services, health care professionals participating in the outpatient care of the patients included an orthoptist (11.7%), a psychologist (11.7%), and an optician specializing in low vision for visual aids. Finally, patient support groups, AFM-Téléthon (myasthenia) and the ARIBa association (visual disability) were solicited by 2 patients for their involvement. A disturbance in activities of daily living leading to disabilities with psychological repercussions was noted by a number of patients. The most frequent complaints involve mobility (29.41%) and reading (23.52%). In total, 77% of patients state that their well-being has been affected, and they are disturbed by the progression of their disease. DISCUSSION AND CONCLUSIONS: The review of the clinical care pathway of patients affected by neuro-ophthalmological conditions shows that these pathologies are, on the one hand, often poorly understood, and on the other hand complex, leading to an often significant number of steps for the patient. This also emphasizes the necessity of a care network, specialized and structured to improve the efficiency of this management. Finally, these results demonstrate the existence of a very frequent disability, which may affect all aspects of the patients' lives, highlighting the importance of rehabilitation services and individuals participating in the follow-up of these patients beyond their acute care.

[Neuro-ophthalmological conditions: Study of the clinical care pathway]. / Les affections neuro-ophtalmologiques : étude du parcours de soins du patient.

INTRODUCTION: Neuro-ophthalmologic conditions require specialized multidisciplinary management, both medical and surgical, for patients affected by visual loss due to nervous system disease. The primary goal of this study is to define the specificity of neuro-ophthalmology within the realm of visual health. The secondary goal is to review clinical care pathways by studying the organization of management, in terms of accessibility to care and personalization of the care pathway. PATIENTS AND METHODS: A field study was carried out from February to June 2015, within the ophthalmology service of the Pitié-Salpêtrière University Medical Center in Paris. A 30-minute interview with the patient before or after his or her neuro-ophthalmology consultation was performed, so as to describe the clinical care pathway. The medical records of interviewed patients were also analyzed. RESULTS: Seventeen care pathways (10 women and 7 men) were reviewed. The mean age at appearance of visual involvement was 44.5 years (±8.4 years). If we exclude 3 patients over 66 years and retired, 35.71% were active, 35.71% were disabled, and 28.57% were on sick leave. Ten patients (58.82%) met the criteria for admission to long-term care. The first step had been carried out by local private practitioners. The first physician seen was the general medicine physician (59%), then the private ophthalmologist on an emergency basis (17%). On average, patients went through 8 steps during their care pathway (from 6 to 10 steps) and 14 medical departments were involved. The study showed collaboration with the other services of the University Hospital Department of Vision and Disabilities (notably with the Fondation Rothschild, the Quinze-Vingts National Ophthalmology Hospital and the Fondation Sainte-Marie). In addition to rehabilitation services, health care professionals participating in the outpatient care of the patients included an orthoptist (11.7%), a psychologist (11.7%), and an optician specializing in low vision for visual aids. And finally, patient support groups, AFM-Téléthon (myasthenia) and the ARIBa Association (visual disability) were solicited by 2 patients for their involvement. A disturbance in activities of daily living leading to disabilities with psychological repercussions was noted by a number of patients. The most frequent complaints involve mobility (29.41%) and reading (23.52%). In total, 77% of patients state that their well-being has been affected and they are disturbed by the progression of their disease. DISCUSSION AND CONCLUSIONS: The review of the clinical care pathway of patients affected by neuro-ophthalmological conditions shows that these pathologies are, on the one hand, often poorly understood, and on the other hand complex, leading to an often significant number of steps for the patient. This also emphasizes the necessity of a care network, specialized and structured to improve the efficiency of this management. Finally, these results demonstrate the existence of a very frequent disability, which may affect all aspects of the patients' lives, highlighting the importance of rehabilitation services and individuals participating in the follow-up of these patients beyond their acute care.

Uveitic macular edema.

Macular edema (ME) may complicate anterior, intermediate, and posterior uveitis, which may be because of various infectious, neoplastic or autoimmune etiologies. BRB breakdown is involved in the pathogenesis of Uveitic ME (UME). Optical coherence tomography has become a standard tool to confirm the diagnosis of macular thickening, due to its non-invasive, reproducible, and sensitive features. Retinal fluorescein and indocyanine green angiography is helpful to study the macula and screen for associated vasculitis, detect ischemic areas and preretinal, prepapillary or choroidal neovascular complications, and it may provide information about the etiology and be needed to assess the therapeutic response. UME due to an infection or neoplastic infiltration may require a specific treatment. If it remains persistent or occurs in other etiologies, immunomodulatory treatments may be needed. Intravitreal, subconjunctival, or subtenon corticosteroids are widely used. Their local use is contraindicated in glaucoma patients and limited by their short-lasting action. In case of bilateral sight-threatening chronic posterior uveitis, systemic treatments are usually needed, and corticosteroids are used as the standard first-line therapy. In order to reduce the daily steroid dose, immunosuppressive or immunomodulatory agents may be added, some of them being now available intravitreally. Ongoing prospective studies are assessing biotherapies and immunomodulators to determine their safety and efficacy in this indication.

[Birdshot retinochoroidopathy: Key messages]. / Rétinochoroïdopathie de type Birdshot : les éléments clés.

Birdshot retinochoroidopathy (BRC) is a potentially blinding posterior autoimmune uveitis that affects Caucasian patients in their fifties. Strongly associated with the HLA A 29 phenotype, BRC is characterized by the presence of suggestive choroidal lesions on the fundus, but their apparition can be delayed. Visual acuity is not a good descriptive factor of visual function. Ancillary tests can therefore help establish the diagnosis and allow proper follow-up of BRC patients: optical coherence tomography can reveal either oedema or atrophy of the macula. Fluorescein and infracyanine green angiograms can show signs of inflammatory activity and give further diagnostic clues. Electroretinograms and visual fields can be useful for the diagnosis and follow-up. BCR patients should be examined periodically and undergo exhaustive clinical and paraclinical tests to insure the best prognosis. Corticosteroids should be associated with immunosuppressive treatments as early as possible in the presence of factors that are predictive of the worse prognoses.

Evaluation of the visual function of patients with locked-in syndrome: Report of 13 cases.

Although visual function is thought to be preserved in patients with locked-in syndrome (LIS), enabling them to communicate through vertical or lateral eye movements or blinking of the upper eyelid, nothing is known about the actual visual function of patients with LIS. The goal of this study is to evaluate the visual function of patients with LIS which may enable better evaluation of the state of consciousness of these patients. Patients with LIS seen in a single neurovascular unit of a tertiary center between 1997 and 2013 were retrospectively reviewed. Each patient had a specialized neuro-ophthalmological evaluation under optimal environmental conditions (light, contrast, examination distance, head position, best ergonomic adaptation, and establishment of a means of communication with help from the patient's friends/family). Visual acuity, extraocular eye movements, confrontational visual field, slit lamp and fundus examination were performed. Thirteen patients (6M/7F) were included in this study. Mean visual acuity was 20/60. Oculomotor examination was abnormal in 77%. Forty-six percent of patients presented binocular diplopia mainly related to a VIth nerve palsy. One patient presented complete ophthalmoplegia. Forty-six percent of patients had nystagmus responsible for oscillopsia (oculopalatal tremor). An abnormal visual field was observed in 17% of patients, and abnormal pupillary light response leading to photophobia was present in 22% of patients. Keratitis or dry eye syndrome was present in most patients and was a major cause of pain and visual impairment. Our results suggest that the visual function is impaired in all patients with LIS. This impairment is multifactorial including mostly binocular diplopia or oscillopsia but also refractive errors, dry eye syndrome, keratitis or visual field defect. This altered visual function may alter the ability of the patient to interact with his environment and lead to underestimation of their state of consciousness. An ophthalmologic evaluation would allow for improvement of these patients' comfort, their ability to communicate, and the assessment of their state of consciousness.

[Uveitic macular edema]. / Œdème maculaire au cours des uvéites.

Macular edema may complicate anterior, intermediate, and posterior uveitis, which may be due to various infectious, tumoral, or autoimmune etiologies. Breakdown of the internal or external blood-retinal barrier is involved in the pathogenesis of inflammatory macular edema. Optical coherence tomography has become standard in confirming the diagnosis of macular thickening, due to its non-invasive, reproducible and sensitivity characteristics. Fluorescein and indocyanine green angiography allows for, in addition to study of the macula, screening for associated vasculitis, detection of ischemic areas, easy diagnosis of preretinal, prepaillary or choroidal neovascular complications, and it can provide etiological information and may be required to evaluate the therapeutic response. Treatment of inflammatory macular edema requires specific treatment in cases of infectious or tumoral etiologies. If it remains persistent, or occurs in other etiologies, anti-inflammatory treatments are needed. Steroid treatment, available in intravitreal, subconjunctival and sub-Tenon's routes, are widely used. Limitations of local use include induced cataract and glaucoma, and their short-lasting action. Such products may reveal retinal infection. Thus, bilateral chronic sight-threatening posterior uveitis often requires systemic treatment, and steroids represent the classic first-line therapy. In order to reduce the daily steroid dose, immunosuppressant or immunomodulatory drugs may be added. Certain of these compounds are now available intravitreally.

[Carotid-cavernous fistula: clinical and pathological correlations]. / Fistules carotido-caverneuses: corrélation anatomo-clinique.

INTRODUCTION: Depending upon the type of communication between the internal carotid artery and the cavernous sinus, two types of carotid-cavernous fistulae can be distinguished: direct carotid-cavernous fistula (DCCF), for which diagnosis is usually easy because of the obvious signs of orbital congestion, and indirect carotid-cavernous fistula (ICCF) or dural fistula, with few symptoms, associated with a delay in diagnosis which can alter prognosis for function and even life. PURPOSE: The purpose of this study is to determine characteristic signs of both types of CCF and to study their correlation with the anatomical type of vessels involved on the angiogram. The prognosis for both types of fistula was also studied. METHOD: Retrospective single-center study, including patients with angiographically confirmed DCCF or ICCF. Demographic, clinical, and prognostic characteristics of these patients were analyzed and compared with the angiogram findings. RESULTS: Six patients (4 DCCF and 2 ICCF) were included in this study. Mean age at diagnosis was 62.7 years in the DCCF group and 62 years in the ICCF group. Mean time until diagnosis was 7 weeks in the DCCF group and 24 weeks in the ICCF group. Signs of orbital congestion were present in 100% of patients with DCCF and absent in all patients in the ICCF group. Mean proptosis was 3.5mm in the DCCF group and 0.5 in the ICCF group. All patients had an audible bruit on auscultation. The location of the bruit was orbital in all patients with DCCF, and pretragal or jugal in 50% of patients with ICCF. CONCLUSIONS: ICCF can impact mortality and are often underdiagnosed. The diagnostic delay observed in our study reflects the difficulties which persist in recognizing the clinical signs of this condition. ICCF must be suspected in the case of a history suggestive of CCF, even if the clinical examination appears normal. The presence of a bruit must be carefully sought, not only in the classical orbital location, but also in other facial locations as shown in our study. Our results demonstrate that there is indeed a good correlation between the site of the CCF, the location of the bruit, and the vessels involved.

Choroidal and adnexal extranodal marginal zone B-cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases.

PURPOSE: To describe the clinical and imaging presentation, pitfalls in the diagnosis of choroidal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), as well as the therapeutic management and prognosis. METHODS: A retrospective case review of nine choroidal MALT lymphomas was performed. Initial clinical presentation and imaging findings of these histologically confirmed cases of lymphoma were analyzed. Treatment methods, time to diagnosis, systemic work-up, and treatment prognosis were assessed. RESULTS: Initial presentation was essentially blurred vision. The features described on examination were: anterior and posterior scleritis, iridocyclitis, choroidal infiltration, and exudative retinal detachment. Fluorescein and indocyanine green angiography as well as ultrasonography and optic coherence tomography provided arguments in favor of the diagnosis. Biopsy sites included conjunctiva, Tenon's capsule, deep scleral tissue, episclera, lacrimal gland, and choroid. Treatment mostly consisted of a combination of chemotherapy and radiotherapy. The mean time to diagnosis was 12 months. CONCLUSIONS: Owing to the insidious onset of these tumors and their ability to simulate other conditions, the diagnosis is commonly delayed. The prognosis is generally good and treatment is effective in the case of localized lymphoma.

[An unusual cause of compressive optic neuropathy]. / Une cause inhabituelle de neuropathie optique compressive.

Clinical manifestations of hydrocephalus vary according to the level of intracranial pressure, the speed of onset, and the etiological mechanism involved. We report the case of a 32-year-old patient with isolated compressive optic neuropathy associated with a dilated third ventricle, revealing congenital hydrocephalus.

[Dural-cavernous fistulas revealed by bilateral alternating third and sixth nerve palsies]. / Fistule durale carotido-caverneuse révélée par une paralysie bilatérale à bascule des troisièmes et sixièmes nerfs crâniens.

A 74-year-old male was referred for disequilibrium, associated with right third and sixth nerve palsies observed 2weeks after head trauma with no loss of consciousness. On clinical examination, 4months after the injury, contralateral (left) third and sixth nerve palsies were observed, while ocular motility was now normal on the right side. The remainder of the ophthalmological examination was normal. Upon further history, tinnitus was found to have been present since the trauma, and auscultation of the preauricular area demonstrated a systolic bruit. Cerebral angiogram confirmed the presence of bilateral dural-cavernous fistulas. Clinical features of indirect or dural-cavernous fistulas and therapeutic options proposed in the literature are reviewed.

[Maculopathy and dengue fever]. / Maculopathie et fièvre dengue.

The authors report a case of a 20 year old female who presented a bilateral decrease in vision. Detailed history revealed that 15 days before the onset of symptoms, the patient presented a dengue fever during her stay in Martinique. On initial examination, the fundus exam revealed a bilateral loss of foveal reflection. The optical coherence tomography demonstrated the macular edema and the dengue serology was postive. The patient received an interferon2a-based therapy. The macular edema disappeared and we noticed a partial but fast improvement of visual acuity 12 days after the treatment initiation.

[Orbital cysticercosis mimicking a painful third nerve palsy]. / Cysticercose oculaire mimant une paralysie douloureuse du III.

We report an atypical case of cysticercosis of the left levator-superior rectus complex, mimicking a painful partial third nerve palsy. The patient presented with painful diplopia, with limitation of elevation, depression and adduction of the left eye, associated with a ptosis of the upper left eyelid. Magnetic resonance imaging revealed two cysts, located within the left levator-superior rectus complex, and nine parenchymal cysts located in both hemispheres of the brain. Antibody titers directed against Cysticercus cellulosae were elevated as confirmed by Elisa and Immunoblot. Diplopia and local inflammation resolved after albendazole and systemic steroid treatment. Surgical resection of the cysts was unnecessary.

Diagnostic approach in optic neuropathy.

The diagnostic of optic neuropathy (ON) is a clinical diagnostic, relying on a detailed medical history, and a thorough clinical examination. In some cases, the attribution of the vision loss to a lesion of the optic nerve can be challenging, and further work-up is required to confirm the optic neuropathy. Once the diagnostic of optic neuropathy is stated, the pathophysiological mechanism of the ON has to be determined so that the appropriate therapeutic strategy can be initiated as promptly as possible. The diagnostic work-up must be as targeted as possible, oriented by the clinical examination. The different steps leading to the positive diagnostic of ON, and the etiologic work-up are detailed hereafter in order to achieve the most targeted work-up as possible. Differentials and current pitfalls are being reviewed.

Toxoplasmosis: new challenges for an old disease.

More than a century after the identification of Toxoplasma gondii, major issues need to be addressed for the optimal management of ocular disease. Toxoplasmic retinochoroiditis is the main cause of posterior uveitis in several geographical areas. The parasite establishes a love-hate relationship with the eye, manipulating the immune response and inducing variable initial lesions and further relapses. It is now well established that most cases are acquired after birth and not congenital. The severity of the disease is mainly due to the parasite genotype and the host immune status. Diagnosis is based on clinical features, but may be confirmed by biological tools applied to ocular fluids. Combining several techniques improves the diagnostic yield in equivocal cases. Therapeutic management is the most important challenge. Even though evidence-based data on the efficacy of anti-parasitic drugs are still missing, new strategies with a good safety profile are available and may be proposed earlier during the course of the disease, but also in selected cases, to reduce sight-threatening relapses. Revisiting the therapeutic options and indications may be an important step towards long-term maintenance of the visual function and avoidance of major complications.

Intravascular lymphoma mimicking a Vogt-Koyanagi-Harada disease.

PURPOSE: To report a case of intravascular lymphoma (IVL) mimicking a Vogt-Koyanagi-Harada disease (VKH). DESIGN: Case report. METHODS: A 38-year-old man was referred for blurred vision, headache, and hearing loss. Examination demonstrated vitritis and subretinal detachments in each eye. Cerebral fluid analysis showed lymphocytic meningitis. Cerebral MRI was normal. A diagnosis of VKH disease was made. RESULTS: Steroid treatment was introduced, after which all symptoms disappeared. Six months later, the patient returned with paraplegia and confusion. Cerebral MRI revealed hypodense periventricular lesions. A stereotaxic biopsy confirmed the diagnosis of IVL. The patient died a few months later. CONCLUSION: IVL may have many revealing aspects, including ophthalmologic symptoms.