Characteristics and Prognosis of Binocular Diplopia in Patients With Giant Cell Arteritis.

BACKGROUND: Giant cell arteritis (GCA) is a large vessel vasculitis associated with a risk of permanent ophthalmologic complications. Data about diplopia prognosis in GCA are scarce. This study was designed to better characterize diplopia in newly diagnosed GCA patients. METHODS: All consecutive patients diagnosed with GCA from January 2015 to April 2021 in a French tertiary ophthalmologic center were retrospectively reviewed. GCA diagnosis relied on a positive temporal artery biopsy or high-definition MRI. RESULTS: Among 111 patients diagnosed with GCA, 30 patients (27%) had diplopia. Characteristics of patients with diplopia were similar to other GCA patients. Diplopia resolved spontaneously in 6 patients (20%). Diplopia was attributed to cranial nerve palsy in 21/24 patients (88%), especially third (46%) and sixth cranial nerve (42%). Ocular ischemic lesions occurred in 11 of the 30 patients with diplopia (37%); 2 patients developed vision loss after initiation of corticosteroids. In the remaining 13 patients, diplopia resolved after treatment onset in 12 patients (92%) with a median delay of 10 days. Patients treated intravenously tended to have a quicker improvement than those treated orally, but with a similar resolution rate of diplopia at 1 month. Two patients had relapse of diplopia at 4 and 6 weeks after an initial treatment course of 24 and 18 months, respectively. CONCLUSIONS: Diplopia is a rare feature at GCA diagnosis, but should raise clinician suspicion for GCA when associated with cephalic symptoms and prompt the initiation of corticosteroids to prevent ocular ischemic complications.

PARACENTRAL ACUTE MIDDLE MACULOPATHY IN GIANT CELL ARTERITIS.

PURPOSE: To report the occurrence of paracentral acute middle maculopathy (PAMM) in giant cell arteritis (GCA), describe its features and outcomes, and identify risk factors associated with PAMM in patients with GCA. METHODS: Review of medical records of patients with GCA who were examined in the Rothschild Foundation Hospital. Patients were divided into three groups: GCA with PAMM (Group 1), GCA with ophthalmic involvement but without PAMM (Group 2), and GCA without ophthalmic involvement (Group 3). We analyzed the data for age, sex, medical history, laboratory testing, visual acuity, and posterior segment vascular involvement. RESULTS: Among the 96 patients who met the inclusion criteria, 52 had ophthalmic involvement, and 16 patients were included in Group 1 (GCA with PAMM). In this subgroup, the mean age was 81.6 years and was found to be older than other groups. The visual prognosis was similar between Groups 1 and 2. Of the 20 eyes with PAMM, 35% were also associated with homolateral anterior ischemic optic neuropathy. No statistical difference was found in initial symptoms, signs, and laboratory testing. CONCLUSION: Paracentral acute middle maculopathy is frequently observed lesions in ocular GCA. Patients can present with isolated findings of PAMM as the only indication of GCA. Optical coherence tomography of the macula should be routinely performed in patients with suspected GCA, specifically if they complain of visual changes, to look for signs of ischemia in the middle layers of the retina. Isolated PAMM should raise suspicion for GCA in patients at risk.

Visual field loss and structure-function relationships in optic neuritis associated with myelin oligodendrocyte glycoprotein antibody.

BACKGROUND: A paradoxical discrepancy between severe peripapillary retinal nerve fiber layer (pRNFL) atrophy and good visual outcome had been reported in patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-associated optic neuritis (ON). However, only visual acuity (VA) was assessed. OBJECTIVES: To study visual field (VF) outcomes of patients with MOG-IgG-associated ON and evaluate the correlation between functional eye outcome and retinal structural changes assessed by optical coherence tomography. METHODS: The records of 32 patients with MOG-IgG-associated ON who underwent ophthalmological examination at least 12 months after ON onset were reviewed. Degree of VF disability was determined by mean deviation (MD). RESULTS: At final assessment (median, 35 months), 4.2% of 48 affected eyes (AE) had VA ⩽ 0.1, 40% had abnormal MD, and among AE with final VA ⩾ 1.0, 31% had mild to moderate damage. Thinning of the inner retinal layers was significantly correlated with MD impairment. Analysis demonstrated a threshold of pRNFL thickness (50 µm), below which MD was significantly worse (mean, -2.27 dB vs -17.72 dB; p = 0.0003). ON relapse was significantly associated with poor visual outcome assessed by MD. CONCLUSION: Functional impairment measured with VF is not rare, and MD assessment better reflects actual structural damage.

Basics, benefits, and pitfalls of pupillometers assessing visual function.

Numerous commercially and non-commercially available pupillometers are nowadays able to assess various biological functions in humans, by evaluating pupils' dynamics in response to specific stimuli. However, the use of pupillometers for ophthalmic afferent evaluations (i.e., photoreceptoral responses) in real-world settings is relatively limited. Recent scientific and technological advances, coupled with artificial intelligence methods have improved the performance of such devices to objectively detect, quantify, and classify functional disturbances of the retina and the optic nerve. This review aims to summarize the scientific principles, indications, outcomes, and current limitations of pupillometry used for evaluation of afferent pathways in ophthalmic clinical settings.

Are PHOMS a clinical sign of optic neuritis?

PURPOSE: Assess the prevalence and evolution of PHOMS in optic neuritis. METHODS: We analysed the medical files of 126 patients included in the OCTON cohort. Patients' medical files, digital retinal images and OCT examinations were reviewed, searching for optic nerve head oedema and PHOMS at the initial presentation and during the follow-up. RESULTS: We included 102 patients in the final analysis. Twenty-nine (29) eyes had optic nerve head oedema at the initial presentation. PHOMS were found to be present in 8 eyes affected with optic neuritis. All cases of PHOMS were associated with optic nerve head oedema. All the PHOMS decreased in size and disappeared with the improvement of the oedema. DISCUSSION: Our results show that PHOMS is not a common sign of optic neuritis. We didn't observe any case of PHOMS in the absence of optic nerve head oedema in eyes with optic neuritis. PHOMS seem to be a rare sign of optic neuritis associated to optic nerve head oedema, and they tend to disappear with the improvement of the optic nerve head oedema. We suggest that the presence of PHOMS in optic neuritis eyes with no optic nerve oedema should be a considered warning sign.

Spectralis High Magnification Module imaging in a case of Multiple Evanescent White Dot Syndrome.

PURPOSE: To report the use of Spectralis High Magnification Module (HMM) as part of multimodal imaging in Multiple Evanescent White Dot Syndrome (MEWDS). OBSERVATIONS: HMM imaging showed a blurry mosaic pattern corresponding to MEWDS-related photoreceptors' lesions. These abnormalities remained detectable at later stages of the disease while other imaging modalities were negative. CONCLUSIONS AND IMPORTANCE: HMM can be a useful technique to monitor the structure of the outer retina during the different stages of MEWDS.

Visual Field Defect Before and After Endovascular Treatment of Occipital Arteriovenous Malformations.

BACKGROUND: Occipital arteriovenous malformations (AVMs) carry a high risk of postoperative morbidity because of their anatomic relation to the visual cortex and optic radiations. Data regarding endovascular management of these lesions are scant. OBJECTIVE: To report our single-center experience with occipital AVMs, most of which were treated endovascularly, with a special interest for postoperative visual impairment. METHODS: From a prospective database, we assessed the clinical and radiological data of all patients with an occipital AVM managed between 1997 and 2018. The extension of the nidus to the primary visual cortex was assessed and correlated to the pre- and postintervention visual symptomatology. Modified Rankin Scale and visual fields (VFs) were assessed pre- and post-treatment and at the last follow-up. RESULTS: A total of 83 patients (47 males [56.6%]) with an occipital AVM were included in the study. Mean age at presentation was 33.5 ± 15.0 yr (min-max = 7-76). A total of 34 patients (41%) presented with hemorrhage related to the AVM. A total of 57 patients (68.7%) underwent endovascular treatment (EVT) alone, 20 (24.1%) underwent embolization and surgery, 3 (3.6%) underwent embolization and radiosurgery, and 3 (3.6%) were conservatively managed. A complete obliteration of the AVM was achieved in 53 patients (66.3%). A post-treatment worsening of the VF was found in 24 of the treated patients (30%), 3 patients (9%) for ruptured AVMs, and in 21 patients (46%) for unruptured AVMs. Morbidity rate was 3.7% and mortality rate was 2.5%. CONCLUSION: EVT of occipital AVM carries a non-negligible rate of complications, especially regarding visual functions.

Monitoring the short-term effect of intravenous immunoglobulins in multifocal motor neuropathy using motor unit number index.

OBJECTIVE: To determine whether motor unit number index (MUNIX) is pertinent to monitor the effect of intravenous immunoglobulins (IVIg) in multifocal motor neuropathy (MMN). METHODS: MUNIX was assessed longitudinally in 7 MMN patients and 17 healthy controls in the abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. A MUNIX sum-score and a compound muscle action potential (CMAP) sum-score were calculated by summing up the scores of APB and ADM. MMN patients were evaluated on the first day of IVIg infusion, 5 MMN patients were evaluated 22days after IVIg infusion, and 3 MMN patients were evaluated 1month after two IVIg infusions. RESULTS: Intraclass correlation coefficient of the MUNIX sum-score in healthy controls was 0.85, showing good test-retest reproducibility. MUNIX and CMAP sum-scores were lower in MMN patients than in healthy controls (p<0.01 and 0.02, respectively). MUNIX sum-score improved in three of the five patients 22days after IVIg infusion and in two of the three patients 1month after 2 IVIg infusions, whereas CMAP sum-score improved in only one patient in both evaluations. CONCLUSIONS: In this preliminary study, MUNIX seems to be a reliable and sensitive tool to monitor the short-term efficiency of IVIg in MMN. SIGNIFICANCE: MUNIX can help monitor IVIg treatment in MMN.

Motor unit number index (MUNIX): Is it relevant in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)?

OBJECTIVE: To determine the test-retest reliability of motor unit number index (MUNIX) technique and to explore if the MUNIX sumscore could be related with disability in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: The MUNIX technique was unilaterally assessed in the abductor digiti mini (ADM), the abductor pollicis brevi (APB) and the tibialis anterior (TA) muscles two different times by two blinded examiners. The MUNIX sumscore was calculated by adding the results of the ADM, APB and TA muscles. RESULTS: 14 CIDP patients were enrolled. The intraclass correlation coefficient (ICC) was great for inter and intra variability for ADM muscles (0.8 and 0.81), TA muscles (0.86 and 0.89) and MUNIX sumscore (0.76 and 0.83). The MUNIX sumscores from the first and second evaluations were strongly correlated (r=0.83, p<0.001). The MUNIX sumscore was significantly correlated with MRC testing (r=0.71, p<0.01), overall neuropathy limitation scale (ONLS) (r=-0.70, p<0.001), rasch-built overall disability scale (R-ODS) (r=0.71, p<0.001). CONCLUSIONS: The MUNIX technique has a good reproducibility and the MUNIX sumscore is related to the disability. SIGNIFICANCE: The MUNIX technique estimates the axonal loss and the number of functional motor units. The MUNIX sumscore may be a good instrument to evaluate the CIDP patients during their follow-up.