Primary Sjögren’s syndrome related optic neuritis

AIM:To determine the clinical features, diagnosis and treatment of the primary Sjögren syndrome (SS) related optic neuritis.METHODS:The clinical data of 8 patients (12 eyes) with primary SS related optic neuritis were analyzed retrospectively.RESULTS: Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS. They presented initially with the signs and symptoms of non-specific optic neuritis, and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland, and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients. There were serum positive titers for anti-Sjögren syndrome A (SSA) in 7 patients and anti-Sjögren syndrome B (SSB) in 8 patients. Anti-aquaporin-4 (AQP4) antibody was negative in all the 8 patients. Both glucocorticoids and immunosuppressive agent were administered, and visual acuity elevated in 8 eyes (66.7%), 3 patients (37.5%) recurred in the follow-up.CONCLUSION:Primary SS related optic neuritis is less common and easily misdiagnosed. The conventional therapies for optic neuritis could not control the recurrence.     

Primary Sj?gren’s syndrome related optic neuritis

AIM:To determine the clinical features, diagnosis and treatment of the primary Sjögren syndrome (SS) related optic neuritis.METHODS:The clinical data of 8 patients (12 eyes) with primary SS related optic neuritis were analyzed retrospectively.RESULTS: Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS. They presented initially with the signs and symptoms of non-specific optic neuritis, and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland, and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients. There were serum positive titers for anti-Sjögren syndrome A (SSA) in 7 patients and anti-Sjögren syndrome B (SSB) in 8 patients. Anti-aquaporin-4 (AQP4) antibody was negative in all the 8 patients. Both glucocorticoids and immunosuppressive agent were administered, and visual acuity elevated in 8 eyes (66.7%), 3 patients (37.5%) recurred in the follow-up.CONCLUSION:Primary SS related optic neuritis is less common and easily misdiagnosed. The conventional therapies for optic neuritis could not control the recurrence.     

Outcomes of toll-like receptors’ antagonism in steroid-resistant optic neuritis; a pilot study

Background

To investigate the safety and outcomes of off-label immunomodulator Mycobacterium w. (Mw), a TLR 9 antagonist in steroid-resistant idiopathic unilateral optic neuritis.

Methods

Case series. Eight patients with documented idiopathic unilateral optic neuritis who did not improve with methyl prednisolone followed by oral steroids as per the Optic Neuritis Treatment Trial (ONTT) were administered Mw 5?ml in 500?ml normal saline, 30?days after the last of dose of steroids had been administered. The dose was repeated at 3?months. Outcome measures monitoring the change in the best-corrected visual acuity (BCVA), pupillary reaction, colour vision, visual field (VF) examination (when possible), fundus examination and photography, visually evoked potential (VEP) testing. BCVA, pupillary reaction, and color vision were monitored immediately prior to steroid therapy, on days 1 and 7 post steroid therapy, pre Mw administration (i.e., 30?days after the last dose of steroids had been completed) and post Mw administration on days 1, 7, 30, 90, 120 and 180. VF, VEP and fundus photography was performed immediately prior to steroid administration, 30?days after the last dose of steroids (i.e., immediately prior to Mw), and days 30, 90,120 and 180 post Mw administration.

Results

There were five females and three males in an age range of 30–54?years. Six patients were available for follow-up at 6?months. All patients showed improvement in visual acuity, colour vision & pupillary reaction. Visual field monitoring was possible in four patients; all four had a centrocecal scotoma that persisted post steroid therapy but resolved 1?month post Mw therapy. Three out of five patients who had disc edema were available for all follow-ups, and showed resolution of disc edema post Mw therapy. The disc edema had persisted post steroid therapy. No adverse events were seen. The 2nd dose did not improve any of the said parameters. There was no recurrence of the disease up to the end of the follow-up period.

Conclusions

Mw appears to improve steroid resistant optic neuritis; future randomized clinical trials would help affirm this observation.     

【In Press】 Mechanism of delayed conduction of fellow eyes in patients with optic neuritis

To test the hypothesis that latency delay in the fellow eyes of optic neuritis (ON) patients is to compensate for delayed transmission of visual information, latency change of multi-focal visual evoked potential (VEP) traces in fellow eyes of 15 ON patients were analyzed. Patients with low risk (LR) for developing multiple sclerosis (MS) were examined separately from multiple sclerosis patients to isolate effect of cortical plasticity from potential pathological changes in disseminated disease. The small increase in latency in fellow eyes of LR group was statistically not significant. In MS patients, the latency was significantly delayed (P<0.02). The magnitude of the latency change in the fellow eyes did not correlate with the severity of latency delay in the affected eyes (R2<0.02, P=0.3). The differences between ON patients with and without MS, reported here, suggest that the presence of disseminated disease plays critical role in latency delay of the fellow eye.     

Isolated optic neuritis in acute disseminated encephalomyelitis—A case report

An 8-year-old boy presented with blurred vision in both eyes with intermittent pain over his brow area for 1–2 weeks. There was an episode of upper respiratory tract infection shortly after he had received H1N1 vaccination 1 month previously. Decreased visual acuity (especially in the right eye) with impaired color vision in both eyes was also noted. Results of his physical examination were all within normal limits except for a bilateral swollen disc. Brain magnetic resonance imaging revealed multiple hyperintense patches in the subcortical white matter of the bilateral frontal–parietal lobes. Methylprednisolone pulse therapy was prescribed then tapered with oral prednisolone for 6 months. Following magnetic resonance imaging performed 6 months after initial treatment, the previous lesions were no longer seen. A diagnosis of acute disseminated encephalomyelitis was confirmed. Visual acuity was recovered in the latest follow-up. Isolated optic neuritis is an uncommon feature of acute disseminated encephalomyelitis. It should be differentiated from multiple sclerosis, which will relapse, and permanent hyperintense lesions can be seen in the follow-up images.     

An association between Vogt-Koyanagi-Harada disease and Guillain-Barré syndrome

PURPOSE: To describe an association between Vogt-Koyanagi-Harada disease and Guillain-Barré syndrome. METHODS: Case series, describing three patients. RESULTS: In two patients, the disorders had their onsets within 2 weeks of each other; in the third patient, Vogt-Koyanagi-Harada disease occurred after 3 months, as Guillain-Barré syndrome resolved. All three patients had bilateral panuveitis typical of Vogt-Koyanagi-Harada disease. Each also developed well-accepted manifestations of Guillain-Barré syndrome, including paresis of the lower extremities (all patients), paresis of the upper extremities (two patients), paresis of cranial nerves (two patients), areflexia (all patients), and abnormal electromyography findings (two patients). CONCLUSIONS: Vogt-Koyanagi-Harada disease may follow or occur simultaneously with Guillain-Barré syndrome. The fact that these two autoimmune disorders occur together in some patients suggest that they may share common disease mechanisms.     

Multifocal VEP and OCT in optic neuritis: a topographical study of the structure–function relationship

Purpose To investigate topographical relationship between amplitude of multifocal visual evoked potentials (mfVEP) and retinal nerve fibre layer (RNFL) thickness following acute optic neuritis (ON). Patients and Methods Fifty patients with a clinical diagnosis of acute unilateral ON between 6 and 36 months prior to the study and 25 age-matched controls underwent mfVEP testing (Accumap V 2.1, ObjectiVision Pty Ltd, Sydney, Australia) and OCT imaging (fast RNFL protocol, Stratus™, software version 3.0, Carl Zeiss Meditec, Inc., Dublin, CA). RNFL thickness and mfVEP amplitude were measured for upper, temporal and lower retinal sectors and corresponding areas of the visual field in affected eyes of ON patients and control eyes. Inter-eye asymmetry coefficients for both RNFL thickness and mfVEP amplitude were calculated for each zone, and corresponding coefficients were correlated between each other. Results There was highly significant reduction of RNFL thickness and mean mfVEP amplitude in all three retinal sectors of the affected eye. Largest reduction of RNFL thickness was noticed in temporal sector and of mfVEP amplitude in corresponding central part of the visual field. RNFL thickness correlated highly with amplitude of the mfVEP derived from corresponding areas of the visual field in all three zones. Conclusions We demonstrated strong topographical associations between structural and functional measures of optic nerve integrity in patients with ON.     

The effect of interferon β-1a on optic neuritis relapse in patients with multiple sclerosis

Background  

To evaluate the clinical effect of interferon β-1a on optic neuritis (ON) relapse in patients with multiple sclerosis (MS) in Taiwan.     

Bilateral optic neuropathy in a case of primary Gougerot Sjögren's syndrome

INTRODUCTION: Primary Sj?gren's syndrome is frequently characterized by a sicca syndrome without associated connective tissue disease. Association with an optic neuropathy is uncommon. CASE REPORT: We report a case of optic neuropathy in a 59-year-old woman known to have primary Sj?gren's syndrome confirmed clinically and histologically 2 years ago. She suddenly presented an initial bilateral visual loss. The ophthalmological exam noted a visual acuity of 1/10 in the right eye and limited to light perception in the left eye, with bilateral optic ischemic neuropathy more developed in the left eye. Fluorescein angiography showed, signs of ischemic neuropathy. The diagnosis of Horton disease was suspected, but subnormal blood velocity and a negative biopsy of the temporal artery confirmed the diagnosis of optic neuropathy associated with primary Sj?gren's syndrome. General steroid therapy improved optic neuropathy in the right eye but was ineffective in the left eye. CONCLUSION: Optic neuropathy associated with Sj?gren's syndrome is rare but must be considered the most common ophthalmological manifestation of the disease. Visual prognosis depends on the rapidity of diagnosis and therapy.     

Effect of Nd∶YAG laser treatment for correlative capsular syndrome

ObjectiveTo study the clinical effect of Nd∶YAG laser treatment for correlative capsular syndrome after cataract phacoemulsification with foldable intraocular lenses （IOL） implants. MethodsThis was a retrospective case-controlled study. Thirty-three patients （33 eyes） diagnosed with correlative capsular syndrome who underwent cataract phacoemulsification with foldable IOL implantation were treated with Nd∶YAG laser. Twelve eyes were in the capsular contraction syndrome （CCS） group and 21 eyes were in the capsular block syndrome （CBS） group. In the CCS group, radial cuts were made in 10 eyes for anterior capsular fibrillar contraction rings and posterior capsulotomy with Nd∶YAG laser was used in 2 eyes. Seven eyes with early postoperative CBS underwent peripheral anterior capsulotomy with Nd∶YAG laser and fluid from the capsular bag was drained into the anterior chamber. Two of them had procedures that were combined with posterior capsulotomy. Fourteen eyes in the CBS group with late postoperative CBS underwent posterior capsulotomy with Nd∶YAG laser to liberate fluid from the capsular bag into the vitreous cavity. All cases were followed up for 6 months. ResultsIn the CCS group postoperatively, the anterior capsular fibrillar contraction rings were relaxed, capsular bag contraction disappeared, the anterior capsular transparent zones were enlarged, IOLs were in the central position, the lenses were stretched and there was no deviation in IOL tilt or capsular bag capture. In the CBS group postoperatively, IOLs were in good position, the posterior capsules were attached to the posterior surface of the lenses without spaces, and capsular bag block was relieved effectively. Pupillary block had disappeared, the depth of the anterior chamber had deepened and IOP was normal in all 7 eyes with early CBS. Uncorrected visual acuity （UCVA） improved at different degrees postoperatively in the CBS and CCS groups. ConclusionNd∶YAG laser is a good choice for correlative capsular syndrome after cataract phacoemulsification with foldable IOL implantation. It has a definite clinical effect and is a simple and safe procedure.     

Sodium hyaluronate eye drops of different osmolarity for the treatment of dry eye in Sjögren's syndrome patients

AIM: To study the effect of the treatment of dry eye in Sj?gren's syndrome patients with hypotonic or isotonic hyaluronate eye drops. METHODS: 40 Sj?gren's syndrome patients were divided in two groups and treated as follows: group 1 with hypotonic (150 mOsm/l) 0.4% hyaluronate eye drops; group 2 with isotonic 0.4% hyaluronate eye drops. The eye drops were instilled six times a day for 90 days. Grading of subjective symptoms, break up time (BUT), corneal fluorescein staining, conjunctival rose bengal staining, Schirmer's I test, and conjunctival impression cytology were carried out at 0 and 15, 30, 90 days from the beginning of the study. Patients were examined in a blind fashion. For the statistical analysis the Student's t test, Mann-Whitney U test, and chi(2) test were performed. RESULTS: Symptoms were statistically significantly improved at day 15 in both groups but group 1 patients had a global score statistically significantly better group 2 (p=0.02). At day 15 group 1 patients had an improvement from baseline values of BUT (p=0.003), fluorescein, and rose bengal score (p=0.000001 and p=0.0004 respectively). Group 2 patients had, at day 15, an improvement of BUT and fluorescein score compared to baseline values (p=0.05 and p=0.0001 respectively). A comparison between the two groups showed better results for group 1 patients at day 15 for rose bengal stain (p=0.01) and for BUT (p=0.05) and fluorescein score (p=0.0003) at day 90. The conjunctival impression cytology showed that group 1 had a statistically significant better total score than group 2 starting from day 15 and lasting throughout the study (p<0.02). Also group 2 patients showed an improvement from baseline values starting from day 30 (p=0.000005). CONCLUSION: Hyaluronate eye drops are useful for treating severe dry eye in Sj?gren's syndrome patients. The use of a formulation with pronounced hypotonicity showed better effects on corneoconjunctival epithelium than the isotonic solution.     

Structural-visual functional relationships detected by optical coherence tomography in varying age-cohorts’ patients with optic neuritis

AIM: To assess the relationships of final best-corrected visual acuity (BCVA) and the optic nerve structural loss in varying age-cohorts of optic neuritis (ON) patients.METHODS: This is a retrospective, cross-sectional study. Totally 130 ON subjects (200 eyes) without ON onset within 6mo were included, who underwent BCVA assessment, peripapillary retinal nerve fibre layer (pRNFL) and macular segmented layers evaluation by optical coherence tomography (OCT).RESULTS: For the 0-18y cohort, the final BCVA (logMAR) was significantly better and less frequent recurrences than adult cohorts (P=0.000). The final BCVA (logMAR) in all age-cohorts of the ON patients had negative and linear correlations to the pRNFL thicknesses and macular retinal ganglion cell layer (mRGCL) volumes, when the pRNFL thicknesses were reduced to the thresholds of 57.2-67.5 µm or 0.691-0.737 mm3 in mRGCL volumes, respectively, with the strongest interdependence in the 19-40y cohort. The ON patients from varying age cohorts would be threatened by blindness when their pRNFL thicknesses dropped 36.7-48.3 µm or the mRGCL volumes dropped to 0.495-0.613 mm3.CONCLUSION: The paediatric ON has best prognosis and young adult ON exhibits perfectly linear correlations of final vision and structural loss. The pRNFL and the mRGCL could be potential structural markers to predict the vision prognosis for varying-age ON patients.     

Leber’s hereditary optic neuropathy: course of disease in consideration of idebenone treatment and type of mutation

Graefe's Archive for Clinical and Experimental Ophthalmology - In September 2015, the first and so far only medication for treatment of Leber’s hereditary optic neuropathy (LHON) was...