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MRI findings of OPN patients with Graves' disease. (A) Coronal contrast enhanced T1-weighted MRI showing extraocular muscle belly enlargement (arrow). (B) Coronal contrast enhanced T1-weighted MRI showing thickening and enhancement of the left optic nerve sheath (arrow).
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Background: Optic perineuritis (OPN) is a special optic neuropathy that has a distinct etiology from neuromyelitis optica spectrum disorders (NMOSDs) or multiple sclerosis (MS)-related optic neuritis (ON). The mechanisms of how this inflammation developed and invaded the nerve sheath remain unknown. This study is aimed to analyze the etiology and d...
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Abstract This study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 fema...
Citations
... This aspect produces the "tram-track" sign on axial imaging and the "doughnut" sign on coronal imaging [1]. OPN is usually idiopathic, but infections, autoimmune diseases, neoplasia or drugs can cause secondary OPN [1,5,6]. Treatment consists in steroid therapy in high doses with rapid improvement of the clinical features [1], though spontaneous resolution of the disease has been reported [7,8]. ...
... When OPN was eventually diagnosed based on gadolinium-enhanced MRI, another important aspect was to determine whether the OPN is idiopathic or there is an underlying condition causing it. Even if OPN is frequently encountered as a primary disorder, a study published in 2021 included 44 cases of OPN, where only one patient had idiopathic OPN and the other 43 patients were found to have an underlying cause [5]. These results should raise concern in terms of further management of OPN, as most cases (primary and secondary OPN) respond rapidly to steroid therapy [2,5,8], but a serious systemic condition may go unnoticed and lead to subsequent complications. ...
... Even if OPN is frequently encountered as a primary disorder, a study published in 2021 included 44 cases of OPN, where only one patient had idiopathic OPN and the other 43 patients were found to have an underlying cause [5]. These results should raise concern in terms of further management of OPN, as most cases (primary and secondary OPN) respond rapidly to steroid therapy [2,5,8], but a serious systemic condition may go unnoticed and lead to subsequent complications. ...
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.
... Enhancement of the optic nerve sheath on axial and coronal post-contrast images suggested optic perineuritis. 12 The superior orbital fissure ( SOF ) and orbital apex were examined for soft tissue infiltration and fat stranding on axial and coronal T2 fat-saturated and post-contrast MR images. In addition, the SOF was partitioned into superolateral and the intra-annular middle parts and the presence of infiltrates and necrosis in the two parts was assessed. ...
We describe presenting clinical and imaging manifestations of SARS-CoV-19-associated rhino-oculo-cerebral mucormycosis (ROCM) in a hospital setting during the second wave of SARS-CoV-19 pandemic in India. Data on the presenting manifestations was collected from March 01 to May 31, 2021. Associations between clinical and imaging findings were explored, specifically: (1) the presence or absence of orbital pain and infiltration of superior orbital fissure on imaging; (2) the presence of unilateral facial nerve palsy and pterygopalatine fossa infiltration and geniculate ganglion signal on contrast magnetic resonance imaging and (3) vision loss and optic nerve findings on imaging. Orbital pain was reported by six of 36 subjects. A fixed, frozen eye with proptosis and congestion was documented in 26 (72%), complete vision loss in 23 (64%) and a unilateral lower motor neuron facial nerve palsy in 18 (50%). No association was found between the presence of orbital pain and superior orbital fissure infiltration on imaging. The ipsilateral geniculate ganglion was found to enhance more profoundly in seven out of 11 subjects with facial palsy and available MR imaging, and the ipsilateral pterygopalatine fossa was found infiltrated in 14. Among 23 subjects with complete loss of vision, nine (39%) demonstrated long-segment bright signal in posterior optic nerve on diffusion MR images. We conclude that orbital pain might be absent in SARS-CoV-19-associated ROCM. Facial nerve palsy is more common than previously appreciated and ishaemic lesions of the posterior portion of the optic nerve underlie complete vision loss.
... Pract Neurol: first published as 10.1136/practneurol-2021-003228 on 2 May 2022. Downloaded fromReview based inflammation, in particular neurosarcoidosis25 and IgG4-related disease.33 ...
The Optic Neuritis Treatment Trial previously reported that corticosteroids accelerated visual recovery in optic neuritis (ON) without improving outcome. This finding related largely to multiple sclerosis (MS), and subsequently neurologists tended to await spontaneous recovery in ON. Since then, non-MS cases of ON have been identified with antibodies to aquaporin-4 (AQP4) or myelin oligodendrocyte glycoprotein (MOG). These disorders can closely mimic multiple sclerosis-associated or idiopathic demyelinating optic neuritis (MS/IDON) initially but risk a worse visual outcome. Scrutinising the clinical features and neuroimaging often enables differentiation between MS/IDON and other causes of ON. Early treatment with high-dose corticosteroids is an important determinant of visual outcome in non-MS/IDON. Prompt use of plasma exchange may also save sight. In this review, we contrast the presentations of myelin oligodendrocyte glycoprotein associated optic neuritis (MOG-ON) and aquaporin 4 associated optic neuritis (AQP4-ON) with MS/IDON and provide an approach to acute management while awaiting results of antibody testing.
... Thus, meningeal lesions affect the ONS. 12 Previous cases have indicated that the ONS and optic nerve are associated with carcinoma through paraneoplastic syndrome 13 and metastasis. 14,15 Metastasis of meningeal lesions also affects the ONS and optic nerve. ...
Latent intracranial meningeal metastases (IMM) of lung cancer is difficult to determine, yet it is critical to do so given that it impacts the treatment agent. Studies on this disease are rare, thus necessitating further investigation. As a case study, we will explore the application of optic neuroimaging in IMM. A 62-year-old female patient was diagnosed with lung adenocarcinoma, which had progressed to osseous metastasis. During the course of chemotherapy, the patient had bilateral vision loss and paralysis of extraocular muscles. Ophthalmologists ruled out disease of the retina and suspected intracranial metastasis; however, brain-enhanced magnetic resonance angiography and magnetic resonance venography were normal. Given the patient's severe osteoarthropathy and poor physical condition, she refused to undergo a lumbar puncture examination. Optic neuro-ophthalmology imaging was ultimately used. Utilizing optical coherence tomography, we found that the basement membrane layer in the papilledema was protruding up towards the vitreous cavity. To assist in visualization, the optic nerve sheath was enhanced with optic magnetic resonance imaging. With these methods, the dural metastasis was identified, the treatment agent was changed for the patient, and she had a successful recovery. Thus, optic neuro-ophthalmology imaging should be recommended for patients who are in the latent course of dural metastasis, and it could also be used to evaluate therapeutic efficacy.
... Any lesion of the optic nerves may be more common in NMOSD than in MS [55]. Optic perineuritis may be of particular value in distinguishing NMOSD and MS from other autoimmune causes of optic neuritis [61] including MOGAD [62]. ...
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease of the central nervous system (CNS) associated with antibodies to aquaporin-4 (AQP4) which has distinct clinical, radiological and pathological features, but also has some overlap with multiple sclerosis and MOG-antibody associated disease. Early recognition of NMOSD is important because of differing responses to both acute and preventive therapy. Magnetic resonance (MR) imaging has proven essential in this process. Key MR imaging clues to the diagnosis of NMOSD are longitudinally extensive lesions of the optic nerve (more than half the length) and spinal cord (3 or more vertebral segments), bilateral optic nerve lesions and lesions of the optic chiasm, area postrema, floor of the IV ventricle, periaqueductal grey matter, hypothalamus and walls of the III ventricle. Other NMOSD specific lesions are denoted by their unique morphology: heterogeneous lesions of the corpus callosum, “cloud-like” Gd-enhancing white matter lesions and “bright spotty” lesions of the spinal cord. Other lesions described in NMOSD, including linear periventricular periependymal lesions and patch subcortical white matter lesions, may be less specific. The use of advanced MR imaging techniques is yielding further useful information about focal degeneration of the thalamus and optic radiation in NMOSD and suggests that paramagnetic rim patterns and changes in normal appearing white matter are specific to MS. MR imaging is crucial in the early recognition of NMOSD and in directing testing for AQP4 antibodies and guiding immediate acute treatment decisions. Increasingly MR imaging is playing a role in diagnosing seronegative cases of NMOSD.
Purpose:
To assess the association of optic nerve sheath (ONS) infiltration, fat infiltration, and scleral enhancement with active thyroid eye disease (TED) and dysthyroid optic neuropathy (DON).
Methods:
Thyroid eye disease patients who had axial and coronal fat-suppressed contrast enhanced T1-weighted magnetic resonance imaging (MRI) imaging performed were included. Optic nerve sheath infiltration was defined by the presence of thickening and circumferential enhancement of the optic nerve sheath. Clinical assessments were performed by orbital surgeons or neuro-ophthalmologists and the disease activity (active/inactive) and presence or absence of dysthyroid optic neuropathy were recorded.
Results:
The study population consisted of 76 orbits from 38 patients with a mean age of 53 ± 15 years, with 25 (66%) being female. Optic nerve sheath infiltration was present in 28 (37%) orbits, fat infiltration in 37 (49%) and scleral enhancement in 14 (18%) orbits. ONS infiltration (OR 19.8, p < 0.01), fat infiltration (OR 5.2, p < 0.01) and scleral enhancement (OR 12.2, p = 0.01) were all significantly associated with active clinical disease. Patients with ONS infiltration had a significantly higher odds of dysthyroid optic neuropathy (OR 3.4, p < 0.05). Fat infiltration (OR 2.8, p = 0.1) and scleral enhancement (OR 2.3, p = 0.23) were not significantly associated with DON.
Conclusions:
Optic nerve sheath infiltration may be a predictor of dysthyroid optic neuropathy. Intraorbital fat infiltration and scleral enhancement may be used to detect active TED. These radiological findings may serve as useful diagnostic and stratification tools in evaluating TED patients.
To evaluate the diagnostic performance of the extraocular muscle volume index at the orbital apex (AMI) and the signal intensity ratio (SIR) of the optic nerve in dysthyroid optic neuropathy (DON).
Clinical data and magnetic resonance imaging were collected retrospectively from 63 Graves’ ophthalmopathy patients, including 24 patients with DON and 39 without DON. The volume of these structures was obtained by reconstructing their orbital fat and extraocular muscles. The SIR of the optic nerve and axial length of eyeball were also measured. The posterior 3/5 of the retrobulbar space volume was used as the orbital apex to compare parameters in patients with or without DON. Area under the receiver operating characteristic curve (AUC) analysis was used to select the morphological and inflammatory parameters with the highest diagnostic value. A logistic regression was performed to identify the risk factors of DON.
One hundred twenty-six orbits (35 with DON and 91 without DON) were analyzed. Most of the parameters in DON patients were significantly higher than in non-DON patients. However, the SIR 3 mm behind the eyeball of the optic nerve and AMI had the highest diagnostic value in these parameters and are independent risk factors of DON by stepwise multivariate logistic regression analysis. Combining AMI and SIR had a higher diagnostic value than a single index.
Combining AMI with SIR 3 mm behind the eyeball’s orbital nerve can be a potential parameter for diagnosing DON.
The present study provided a quantitative index based on morphological and signal changes to assess the DON, allowing clinicians and radiologists to monitor DON patients timely.
The extraocular muscle volume index at the orbital apex (AMI) has excellent diagnostic performance for dysthyroid optic neuropathy.
A signal intensity ratio (SIR) of 3 mm behind the eyeball has a higher AUC compared to other slices.
Combining AMI and SIR has a higher diagnostic value than a single index.
Transient visual loss (TVL) is a common complaint in the Emergency Department, with numerous possible etiologies. Prompt evaluation and management of TVL can potentially prevent progression to permanent visual loss. In this case, a 62-year-old female presented with acute, painless, unilateral TVL. Two weeks prior to presentation, the patient reported bitemporal headaches and paresthesia of the distal extremities. A review of systems revealed chronic fatigue, cough, diffuse arthralgias, and decreased appetite for the previous six months. This case highlights the diagnostic approach to patients with TVL. Some common and rare causes associated with this clinical manifestation are briefly reviewed.
