Analysis of Magnetic Resonance Imaging in Paranasal Mucocele with Visual Disturbance.

INTRODUCTION: Ocular imaging strategies have evolved to facilitate the diagnosis of optic neuropathy. This study aimed to evaluate the pathogenesis of visual disturbance associated with paranasal mucocele via magnetic resonance imaging (MRI). METHODS: A total of 19 patients with mucocele and visual disturbance who underwent endoscopic sinus surgery, orbital MRI, and sinus computed tomography were included. The age, sex, days from onset to surgery, eye pain, and imaging findings were analyzed. The results were compared between two groups: 7 patients with preoperative visual acuity worse than 20/200 (the poor group) and 12 patients with equal or better than 20/200 (the fair group). RESULTS: Imaging showed a high compression rate of the orbit in 17 (89.5%) and enlargement of the subarachnoid space around the optic nerve in 15 (78.9%) of 19 patients. Preoperative vision was significantly poor in cases with hyperintense regions in the optic nerve on T2-weighted imaging, indicating the presence of optic neuritis. No cases showed severe inflammation of the cyst or the presence of intraorbital fat tissue. CONCLUSION: MRI-based diagnosis proved useful in evaluating pathological factors, such as orbital compression, ischemia, and optic neuritis, in individual cases. It can help in gaining insight into the pathogenesis and developing appropriate treatment strategies for visual disturbances associated with paranasal mucocele.

Beyond Myelin Oligodendrocyte Glycoprotein and Aquaporin-4 Antibodies: Alternative Causes of Optic Neuritis.

Optic neuritis (ON) is the most common cause of vision loss in young adults. It manifests as acute or subacute vision loss, often accompanied by retrobulbar discomfort or pain during eye movements. Typical ON is associated with Multiple Sclerosis (MS) and is generally mild and steroid-responsive. Atypical forms are characterized by unusual features, such as prominent optic disc edema, poor treatment response, and bilateral involvement, and they are often associated with autoantibodies against aquaporin-4 (AQP4) or Myelin Oligodendrocyte Glycoprotein (MOG). However, in some cases, AQP4 and MOG antibodies will return as negative, plunging the clinician into a diagnostic conundrum. AQP4- and MOG-seronegative ON warrants a broad differential diagnosis, including autoantibody-associated, granulomatous, and systemic disorders. These rare forms need to be identified promptly, as their management and prognosis are greatly different. The aim of this review is to describe the possible rarer etiologies of non-MS-related and AQP4- and MOG-IgG-seronegative inflammatory ON and discuss their diagnoses and treatments.

Optic Perineuritis Distinguishing Arteritic Ischaemic from Amiodarone-Associated Optic Neuropathy.

Giant cell arteritis (GCA) is a common medium to large vessel vasculitis of the elderly that can lead to permanent vision loss. Neuroimaging in GCA may reveal optic nerve sheath enhancement, which is a cardinal finding of optic perineuritis (OPN). The clinical manifestations of GCA can mimic that of other ocular disorders including amiodarone-associated optic neuropathy (AAON). We report a case of biopsy-proven GCA in a patient initially suspected to have AAON. This patient presented with bilateral optic disc oedema in conjunction with subacute predominately monocular vision loss and enhancement of the corresponding optic nerve sheath on neuroimaging. Clinicians should recognise that clinical and neuroimaging findings of GCA can mimic a variety of ocular and orbital pathologies including idiopathic OPN and AAON.

Comparison between optic neuritis associated with antibody against myelin oligodendrocyte glycoprotein and presumed idiopathic optic perineuritis.

BACKGROUND: The aim of this study was to determine important clinical and radiological features that aid in distinguishing presumed idiopathic optic perineuritis (OPN) from optic neuritis (ON) associated with antibody against myelin oligodendrocyte glycoprotein (MOG-Ab). METHODS: This retrospective, case-control study recruited patients with MOG-Ab-associated ON from 2011 through 2018 and idiopathic OPN from 2009 through 2011. The presence of MOG-Ab was not investigated in idiopathic OPN, because MOG-Ab testing was not available until 2011. The clinical and radiological features and the disease course were compared between the two patient groups. RESULTS: A total of 48 patients with MOG-Ab-associated ON were identified. These included 15 patients showing optic nerve sheath enhancement (ONSE) and 33 with only optic nerve enhancement. Ocular pain with ocular movement and optic disc swelling were more common in patients with ONSE, who also exhibited a poorer initial visual acuity than did those without ONSE. However, the response to steroid treatment, incidence of relapse after steroid treatment, and visual outcome at the last visit were similar between subgroups. The clinical and radiological features and treatment outcome were similar between these patients with OPN and patients with MOG-Ab-associated ON with ONSE. On the other hand, the clinical features of MOG-Ab-associated ON without ONSE differed from those of idiopathic OPN. CONCLUSION: Our findings showed a substantial proportion of ONSE in patients with MOG-Ab-associated ON. In view of the similarities between these patients and patients with OPN, MOG-Ab testing should be performed in all patients with idiopathic OPN.

[Five cases of optic neuropathy associated with varicella zoster virus infection].

Objective: To investigate the clinical characteristics, treatment and prognosis of optic neuropathy associated with varicella zoster virus (VZV). Methods: Five cases of optic neuropathy associated with VZV infection from Department of Neurology between January 1, 2014 and March 31, 2019 were retrospectively collected. The clinical manifestations, treatment and prognosis were analyzed. Results: There were 7 eyes involved in 5 cases, 3 cases (3/5) involved only one eye, and 2 cases (2/5) involved both optic nerves. During the follow-up time, no recurrence was found. Severe visual impairment occurred in 4 eyes (4/7) and non-severe visual impairment in 3 eyes (3/7). Visual acuity improved significantly in 1 eye (1/7), turned better in 2 eyes (2/7), and remained unchanged in 4 eyes (4/7). In acute phase, abnormal signals of optic nerve and/or sheath were observed on MR images. Case 3 received antiviral and hormone therapy on the second day after the onset of the disease, and the visual acuity recovered well; the other 4 cases had poor prognosis. Conclusions: Head and face VZV infection can cause serious optic neuropathy, leading to severe visual dysfunction, and poor prognosis, but recurrence is rare. Early intravenous administration of antiviral drugs (acyclovir is the best) and hormones are recommended for VZV infection in this area. It is best to use drugs within 72 hours in order to avoid and reduce secondary optic neuropathy as far as possible.

Incipient Syphilitic Papillitis.

Patients with syphilis can present with optic disc oedema (ODE) without visual compromise, which has been primarily attributed to papilloedema from raised intracranial pressure or optic perineuritis from optic nerve sheath inflammation. We report four cases of ODE in the setting of syphilis with preserved visual function, normal intracranial pressure, and no enhancement of the optic nerve or sheath on magnetic resonance imaging. We propose the term "incipient syphilitic papillitis" for cases presenting with ODE, preserved vision and absence of optic nerve sheath enhancement, which is likely a more common presentation than syphilitic optic perineuritis.

Myelin Oligodendrocyte Glycoprotein Antibody (MOG-IgG)-Positive Optic Perineuritis.

Optic perineuritis can be a manifestation of infectious and systemic inflammatory disorders, but the majority of cases are idiopathic. Myelin oligodendrocyte glycoprotein (MOG)-IgG-positive optic neuritis has been reported to be associated with optic nerve sheath enhancement. This report describes two MOG-IgG patients with clinical, radiological and therapeutic response consistent with optic perineuritis. MOG-IgG may account for many cases of previously described idiopathic optic perineuritis. Vision loss with optic nerve sheath enhancement on MRI should prompt testing for MOG-IgG.

Uveitis and optic perineuritis in the context of myelin oligodendrocyte glycoprotein antibody seropositivity.

BACKGROUND AND PURPOSE: Antibodies to myelin oligodendrocyte glycoprotein (MOG) have been identified in both children and adults with demyelination, with a strong association with bilateral or recurrent optic neuritis (ON). However, the full clinical spectrum of this newly described condition is unknown. We sought to describe non-ON inflammatory ophthalmological presentations such as uveitis and optic perineuritis in the context of MOG antibody seropositivity. METHODS: Using a live cell-based assay analysed by flow cytometry, we identified seropositive patients referred for MOG antibody testing in Australasia between 2014 and 2017. We identified four MOG antibody-positive patients with non-ON inflammatory ophthalmological presentations and present their detailed clinical information in this case series. RESULTS: Three patients had uveitis either in association with, or remote from, ON. One patient had optic perineuritis and peripheral ulcerative keratitis. We describe the presentation, examination, investigation findings and clinical course of these four patients. CONCLUSIONS: Recognition of these novel clinical associations may expand the clinical spectrum of MOG antibody-associated presentations. An expedited diagnosis may guide the management of these complex patients.

Optic perineuritis secondary to hyaluronic acid injections: a case report.

BACKGROUND: Although a safe, excellent administration method for hyaluronic acid derivatives has been documented; improper injections can lead to devastating and irreversible consequences. Here, we present the first known case of optic perineuritis caused by hyaluronic acid. CASE PRESENTATION: A young female experienced sudden orbital pain in the right eye after receiving hyaluronic acid injections to the eyebrows. She presented to the eye clinic two weeks later, after developing blurred vision in the right eye. Visual acuity was reduced significantly in the right eye. Automated visual field examination showed defects in both eyes. Fundus examination revealed bilateral swelling of optic discs. Magnetic resonance imaging of the brain demonstrated bilateral perineural enhancement consistent with optic perineuritis. The patient was treated with retrobulbar injection of hyaluronidase and oral prednisolone. Her vision improved with treatment. CONCLUSIONS: The prognosis for visual outcomes in patients with optic perineuritis is generally excellent. However, a poor prognosis is associated with delays to the initiation of treatment. Recognizing this condition is important, and treatment with corticosteroids should be initiated early.

Metastatic Melanoma of the Optic Nerve Sheath.

A 23-year-old man with a history of metastatic melanoma developed painful vision loss to counting fingers with enhancement of optic nerve on contrast-enhanced magnetic resonance imaging (MRI) and received a diagnosis of optic neuritis from an outside hospital. Despite empiric corticosteroid therapy, the patient worsened and developed secondary central retinal vein occlusion with further deterioration of vision. Repeat MRI demonstrated optic nerve sheath (ONS) involvement suggestive of optic perineuritis (OPN) and an ONS biopsy confirmed a rare case of isolated metastatic melanoma. Our case highlights the clinical and radiographic features that can mimic OPN and delay diagnosis and treatment.

Optic Perineuritis.

Optic perineuritis (OPN) is now defined as "a form of idiopathic orbital inflammatory disease, in which the specific target tissue is the optic nerve sheath". It may be idiopathic or may occur as part of an underlying systemic inflammatory disease. It is a rare disorder and information regarding its presentation and management is only available in case reports and small case series. This review will discuss the contribution of these recent articles to what is known about idiopathic and secondary OPN. Suggestions will also be made as to how to investigate and treat a patient presenting with OPN.

Evaluation of Retinal Nerve Fiber Layer in Patients with Idiopathic Optic Perineuritis using Optical Coherence Tomography.

The aim of this study was to assess the effect of idiopathic Optic perineuritis on the retinal nerve fiber layer, and determine the ability of optical coherence tomography to evaluate retinal nerve fiber loss after idiopathic Optic perineuritis. Four patients were assessed in this study. In all cases, average retinal nerve fiber layer was significantly thinner in the affected eye in comparison with the normal reference value and with the value for the contralateral normal eye at 12 months after the onset of optic perineuritis. Our study revealed that retinal nerve fiber layer loss occurs in idiopathic optic nerve sheath inflammation.

Recurrent Optic Perineuritis after Intranasal Cocaine Abuse.

Recurrent optic perineuritis can be related to orbital inflammation. Here we present the case of a 46-year-old male patient in whom recurrent episodes of optic perineuritis were related to chronic osteolytic sinusitis following intranasal cocaine abuse. Magnetic resonance imaging (MRI) demonstrated optic perineuritis adjacent to a soft tissue mass that intruded the orbit from the nasal cavity. Computed tomography (CT) confirmed destruction of the medial orbital wall. Staphylococcus aureus was cultured and biopsy showed granulomatous tissue. Visual outcome was poor. We review the literature and discuss the diagnostic pitfalls and management implications in relation to optic (peri)neuritis originating from the nasal sinuses.

The crucial role of gadolinium-enhanced MRI in a case of amaurosis fugax - a case report and literature review.

Optic perineuritis is the inflammation of the optic nerve sheath. This affliction can lead to visual field impairment and other signs and symptoms related to the orbital space, such as pain, disc edema, ophthalmoplegia, proptosis. However, not all patients present with such suggestive symptoms, requiring a thorough assessment. We report the case of a young male admitted to our hospital for recurrent episodes of monocular blindness. Amaurosis fugax is a well-known presentation of transient ischemic attacks (TIA) and it was ruled out. Gadolinium-enhanced MRI revealed a typical aspect of optic perineuritis. It was mandatory to consider all possible causes of secondary optic perineuritis as they all represent serious clinical conditions, even if the idiopathic form is more frequent. The clinical and paraclinical evaluation of the patient excluded an underlying disease and primary optic perineuritis was diagnosed. Corticosteroid therapy is usually curative and a course of methylprednisolone was initiated for our patient with good outcome. However, response to treatment is not diagnostic as both primary and secondary optic perineuritis are normally responsive, hence thorough differential diagnosis is necessary.

Optic Perineuritis Presenting with Transient Monocular Vision Loss (TMVL): Case Report.

Optic perineuritis is an inflammatory condition that presents with reduced visual acuity and painful eye movement. The presentation of optic perineuritis is similar of optic neuritis which results in delayed diagnosis and management. Up to this date, we found a single case of optic neuritis that presented with transient monocular vision loss (TMVL). No cases of optic perineuritis were associated with TMVL. Here, we report a case of a 30-year-old woman who presented with recurrent attacks of painless vision loss in her left eye, reaching up to 30 attacks per day. Ophthalmological examination was otherwise unremarkable. Lab investigations were normal. Magnetic resonance imaging was done, which showed left optic nerve sheath enhancement suggestive of left-sided focal optic perineuritis. Patient was managed with 1 mg IV methylprednisolone for 3 days. We report this case to shed light on the importance of accurate and early diagnosis of optic perineuritis presenting with TMVL. Prompt management of optic perineuritis is crucial in reducing morbidity and risk of relapse.

Concurrent Tuberculous Optic Neuritis and Optic Perineuritis in a Patient With Human Immunodeficiency Virus (HIV).

Concurrent tuberculous optic neuritis (ON) and optic perineuritis (OPN) in a patient with human immunodeficiency virus (HIV) is extremely rare. HIV-induced progressive CD4 depletion is associated with an increased risk of tuberculosis (TB), disseminated TB, and death. Early detection and initiation of anti-TB therapy with corticosteroid commencement helps in achieving better visual outcomes. Interestingly, we report a case of concurrent ON and OPN in a patient with HIV-TB co-infection. A 29-year-old lady, a prisoner, with newly diagnosed treatment-naive HIV, presented with acute-onset reduced vision in the left eye for 10 days. It was associated with pain in eye movement and headache. The patient was known to be a drug abuser since the age of 19 years and was a sexual worker. Her CD4 count was 292 cells/mm3.Visual acuity of the right eye was 6/12 with a pinhole of 6/9, and there was no perception of light (NPL) in all four quadrants of the left eye. Relative afferent pupillary defect (RAPD) was positive in the left eye. Both anterior segments were unremarkable. The right eye fundoscopy showed a normal optic disc, while the left eye showed a hyperemic disc. During subsequent follow-up, the patient had reduced right eye vision, and the vision dropped to 6/30 with a pinhole of 6/15. Her erythrocyte sedimentation rate (ESR) was raised to 88 mm/h. The Mantoux test was positive. Chest radiography was normal. MRI of the brain and orbit showed significant enhancement of the right optic nerve and left optic nerve sheath suggesting the diagnosis of right eye ON and left eye OPN secondary to TB. The patient was co-managed with an infectious disease team. She was started on highly active antiretroviral therapy (HAART) treatment (oral Tenvir-EM and efavirenz) upon presentation. Anti-TB therapy was commenced two months later. She was started on the intensive phase of the anti-TB regime followed by the maintenance phase. Oral dexamethasone was given concurrently according to the central nervous system (CNS) TB regime for six weeks. During follow-up, her right eye visual acuity was 6/9, and her left eye visual acuity improved to 6/12. Fundoscopy showed bilateral pale discs. To date, no episodes of recurrence have been seen.

Light After Darkness: A Case Report of Isolated Optic Perineuritis.

This is a report on remarkable visual recovery from blindness in a case of isolated optic perineuritis (OPN). A 68-year-old Chinese lady presented with a two-week history of progressive painless bilateral vision loss. Her vision was 6/18 on the right eye and no perception of light (NPL) on the left eye with positive relative afferent pupillary defect (RAPD). Fundus showed hyperaemic and swollen optic disc bilaterally. MRI of the brain and orbit revealed hyperintense periventricular white matter lesions, possibly early changes of multiple sclerosis (MS), and perineural enhancement of optic nerve bilaterally, consistent with OPN. All other investigations were negative. Intravenous methylprednisolone 1g/day for three days was started, followed with oral prednisolone, tapered in three months. At the third month of follow-up, her vision had improved to 6/12 on the left and 6/9 on the right. The hyperaemic and swollen disc has resolved. Intravenous megadose corticosteroid treatment is an effective first-line treatment for OPN.

A Case Report of Bilateral Optic Perineuritis With Idiopathic Intracranial Hypertension: Challenges in Diagnosis and Management.

Optic perineuritis (OPN) refers to the inflammation of the optic nerve sheath and it is a rare form of idiopathic orbital inflammatory disease. We report a rare case of bilateral OPN in an obese female teenager with idiopathic intracranial hypertension (IIH). She was initially presented with painless bilateral blurring of vision that was progressively worsening for three weeks duration. Visual acuity of both eyes was hand movement with no relative afferent pupillary defect detected. The confrontation visual field test showed central scotoma. Both anterior segments were unremarkable. Fundoscopy showed a swollen optic disc bilaterally, with extensive flame-shaped hemorrhages surrounding the disc area and dot blot hemorrhages in the posterior pole. A magnetic resonance imaging scan of the brain and orbit revealed the presence of bilateral optic nerve sheath enhancement with empty sella turcica. The patient was diagnosed with bilateral OPN with IIH. She received an initial high dose of systemic corticosteroid followed by a slow tapering dose. She was monitored by the neuromedical team for her IIH. She was followed up for about a year. The final best corrected visual acuity in the right eye was 6/36 and the left eye was 6/60. In conclusion, OPN poses challenges in diagnosis and management. This case emphasizes the importance of considering OPN in the differential diagnosis of optic nerve-related symptoms, as prompt recognition and intervention are crucial for favorable outcomes.

Bilateral retrobulbar optic neuritis combined with optic perineuritis associated with atezolizumab treatment for small cell lung carcinoma.

INTRODUCTION: Ocular immune-related adverse events (OirAEs) associated with novel cancer therapies of immune checkpoint inhibitors (ICIs) are emerging. Retrobulbar optic neuritis (ON) combined with optic perineuritis (OPN), associated with atezolizumab, has been rarely reported and has a unique clinical manifestation. CASE DESCRIPTION: A 67-year-old woman was diagnosed with small-cell lung cancer. As maintenance therapy, atezolizumab was administered continuously for 10 cycles for approximately 14 months. One week after the administration of the tenth dose of atezolizumab, the patient experienced a bilateral, successive painless visual decline. The fundus and the retinal nerve fiber layer revealed no abnormalities, but the ganglion cell of the macula disappeared loss. The concentric shrinking of the peripheral visual field of the left eye was noticed. Orbital MRI revealed bilateral optic nerve thickening and peripheral optic nerve sheath enhancement ("tram-track" and "doughnut" signs). Serology, cerebrospinal fluid results, and image examination ruled out common causes of vision decline, and the condition was identified as bilateral retrobulbar ON combined with OPN as a probable atezolizumab-related immune adverse event. Thereafter, atezolizumab was discontinued, and intravenous methylprednisolone pulse (IVMP) (160 mg/day for 5 days) plus intravenous immunoglobulin (20 g/day for 3 days) was administered. The patient's visual function considerably improved after three weeks. CONCLUSIONS: Retrobulbar ON and OPN associated with atezolizumab are rare side effects that are easily overlooked. Immune-related ON has unique features and requires early identification. The primary treatment for optic nerve irAEs is corticosteroids, but this is not standardized and should be used with caution.

Reversible Bilateral Vision Loss: An Unusual Presentation of Wernicke-Korsakoff Syndrome.

Neuro-ophthalmic manifestations of Wernicke encephalopathy (WE) are uncommon and vary from nystagmus, oculomotor palsies, anisocoria, and optic disc edema to vision loss. We describe a case of a 53-year-old woman presenting with subacute bilateral painless vision decline, lower-extremities weakness with impaired ambulation, headache, and abdominal pain. Neurological examination was pertinent for confabulation, bilateral decreased visual acuity with an absent blink to threat, absent afferent pupillary defect and fundus abnormalities, and significant allodynia in bilateral lower extremities. Besides elevated inflammatory marker with an erythrocyte sedimentation rate (ESR) of 130 mm/hr, her infectious, autoimmune, paraneoplastic, and neuromyelitis optica work-up was overall unremarkable. Brain MRI showed abnormal fluid-attenuated inversion recovery (FLAIR) signaling in bilateral mammillary bodies and around periaqueductal gray matter concerning WE. Due to concerns of Wernicke-Korsakoff syndrome (WKS), parenteral high-dose thiamine was initiated with significant clinical improvement. The patient was also later found to have a positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody, which was deemed false positive given the atypical phenotype and symptomatic improvement with thiamine supplementation. This case encourages the consideration of vision loss as a manifestation of WKS, especially in patients who have risk factors. Testing serum levels of thiamine is strongly encouraged; however, initiating empiric treatment is advocated for high clinical suspicion due to its reversible nature and minimal risk for side effects.