Ocular Myositis Research Articles

Orbital Myositis after Herpes Zoster Ophthalmicus: A Case Report and a Narrative Review of the Literature.

Herpes zoster ophthalmicus results from the reactivation of the latent varicella zoster virus, affecting the first branch of the trigeminal nerve. In 20-70% of cases, Zoster Ophthalmicus can lead to ocular involvement, affecting various orbital structures. Orbital myositis is a rare but severe complication of herpes zoster ophthalmicus. We present a case of a 52-year-old man with no significant medical history who developed zoster-associated right ocular myositis and dacryocystitis. He was treated with intravenous acyclovir and oral steroids. A review of the literature identified 29 patients across 19 studies. The median age was 61 years, with a slight female predominance. In 55% of cases, the patients had no notable medical history. The most common presentation of myositis involved all oculomotor muscles. There were 22 cases who were treated with intravenous antiviral therapy and 19 received steroids. A full resolution of symptoms was achieved in 51.7% of patients. Zoster-related orbital myositis is a rare complication that should be considered even in immunocompetent individuals. It may occur either before or after the appearance of a vesicular rash. Magnetic resonance imaging is the preferred radiological exam for assessing orbital involvement. Intravenous antiviral therapy should be started within 72 h of symptom onset, and its combination with systemic corticosteroids appears to be an effective treatment for zoster-related ocular myositis.

Orbital myositis in a patient with Crohn's disease: A case report of two episodes

To describe a case report of a 34 years-old patient with Crohn's Disease and two episodes of Ocular Myositis. The research methodology employed in this study consisted of a retrospective review of the patient's complete medical history. Crohn's Disease is a chronic inflammatory bowel disorder known to be associated with a wide range of extraintestinal manifestations. Ocular abnormalities, such as episcleritis and uveitis, are commonly observed. However, orbital myositis is an extremely rare ocular extraintestinal manifestations characterized by acute ocular pain that worsens with eye movements and is often accompanied by diplopia. In this case report, we present the case of a 34-year-old woman with a confirmed diagnosis of Crohn's Disease, who experienced two episodes of acute orbital pain exacerbated by ocular movements and diplopia. The diagnosis was established through clinical evaluation and radiologic imaging, with confirmation after a good response to systemic corticosteroids. She responded favorably to systemic corticosteroid therapy on both episodes, and no additional treatment was required. As of now, she remains stable without any ocular sequelae. It is important to note that orbital myositis is an uncommon ocular manifestation associated with Crohn's Disease, and prompt recognition and management are crucial to achieve successful outcomes.

Immune checkpoint inhibitor-related myositis and myocarditis: diagnostic pitfalls and imaging contribution in a real-world, institutional case series

BackgroundImmune checkpoint inhibitors (ICIs) are reshaping the prognosis of many cancers, but often cause immune-related adverse events (irAEs). Among neurological irAEs, myositis is the most frequently reported. Our aim is to describe clinical and non-clinical characteristics, treatment and outcome of all irMyositis (skeletal limb-girdle and/or ocular myositis) and irMyocarditis cases in our reference center.MethodsWe retrospectively enrolled all irMyositis/irMyocarditis patients seen between 2018 and 2022. We reviewed demographics, clinical characteristics, biological, neurophysiological, imaging workup, treatment and outcome.ResultsWe included 14 consecutive patients. The most frequent treatments were pembrolizumab (35%) or ipilimumab–nivolumab combination (35%). Limb-girdle, ocular (non-fluctuating palpebral ptosis and/or diplopia with or without ophthalmoparesis) and cardiac phenotypes were equally distributed, overlapping in 40% of cases. Ocular involvement was frequently misdiagnosed; review of brain MRIs disclosed initially missed signs of skeletal myositis in one patient and ocular myositis in 3. Seven patients had other co-existing irAEs. When performed, myography showed a myogenic pattern. CK was elevated in 8/15 patients, troponin-T in 12/12 and troponin-I in 7/9 tested patients. ICI were discontinued in all cases, with further immunosuppressive treatment in nine patients. In most cases, neurological and cardiological outcome was good at last follow-up.ConclusionMyositis is a potentially severe irAE. Despite its heterogeneous presentation, some highly suggestive clinical symptoms, such as ocular involvement, or radiological signs should raise physicians’ attention to avoid misdiagnosis. We thus recommend a multidisciplinary assessment (including complete neuromuscular evaluation) even in case of isolated myocarditis. Our series underlines the importance of an early diagnosis, since suspension of ICI and adequate treatment are usually associated with good functional outcome.

Clinical heterogeneity between two subgroups of patients with idiopathic orbital inflammation

ObjectiveIdiopathic orbital inflammation (IOI) is a group of orbital inflammatory diseases of unknown etiopathogenesis. We investigated whether patients with IOI have clinical heterogeneity based on the presence (typical group) or...

Pembrolizumab-induced myasthenia gravis-like disorder, ocular myositis, and hepatitis: a case report

IntroductionPembrolizumab and other immune checkpoint inhibitors are the emerging treatment for selected, high-grade malignancies. However, a small number of patients are unable to tolerate its adverse effects, leading to discontinuation of this potentially life-changing therapy. In this study, we present a case of high-grade urothelial carcinoma patient, who experienced neurocomplications during the first pembrolizumab administration. However, we were able to limit the adverse effect by concomitant use of low-dose oral steroids.Case presentationA 75-year-old Taiwanese female with high-grade urothelial carcinoma of the left ureter came to the neurology clinic with complaints of acute onset of bilateral ptosis 16 days after her first infusion of pembrolizumab. It was found that she developed complete bilateral ptosis and limited extraocular muscle movements. Myasthenia gravis-related antibodies and repetitive stimulation test were negative. We diagnosed her with pembrolizumab-induced myasthenia gravis-like disorder and myositis based on clinical symptoms and elevation of muscle enzymes. We commenced methylprednisolone pulse therapy followed by oral steroid therapy with gradual resolution of the symptoms. Three months later, the patient received a second cycle of pembrolizumab with low-dose oral steroids without any complications.ConclusionPembrolizumab exerts its antitumor activity by interfering with the binding of programmed death 1 and its ligand, programmed death ligand 1. As a result, enhanced cytotoxic T cells can recognize tumor cells and induce cellular death. However, neurological complications may be severe and require prompt recognition and treatment. Our case demonstrated that concomitant use of low-dose steroids and pembrolizumab might prevent such complications.

EP.28Immune checkpoint inhibitors (ICIs)-related ocular myositis

EP.28Immune checkpoint inhibitors (ICIs)-related ocular myositis

Orbital autoimmune inflammatory disorders - Protein regional variability might explain specific lesion location

In ophthalmology, inflammatory diseases target different highly specific regions within the small confine of the orbit. Some entities even prefer a particular location or depth within the same tissue (ex. anterior, intermediate or posterior uveitides, chorioretinitides with unique topographic presentations). Though the location of a lesion strongly influences and helps us in our differential diagnosis, we still don’t understand why specific anatomic sites are susceptible to a disease while other areas are spared. We postulate that regional variability in tissue protein expression can sway the immune system’s capacity to trigger an autoimmune response. In addition to this site-specific quantitative and qualitative variability in potential antigen expression, we believe that other proteins implicated in the immune cascade, as well as geographic areas of relative resistance, tolerance and susceptibility, may be unequally distributed within the orbit. To illustrate our hypotheses, we review three major types of ocular myositis and describe how the extraocular muscles different embryologic origins and protein disparities might explain the fundamental clinical differences between these orbital inflammatory diseases. We hope that future differential genomics, proteinomics, epigenomics and analysis of RNA species of affected tissues, compared to their non-affected, yet microscopically similar, counterparts, will help us understand why diseases occur where they do. Hopefully, understanding these immune triggers will pave the way to new treatment options for ocular inflammatory diseases and for other auto-inflammatory conditions with a marked predilection for any given site.

Acute ocular myositis occurring under etanercept for rheumatoid arthritis

Ocular myositis is a rare disorder characterized by inflammation of single or multiple extra-ocular eye muscles presenting with painful diplopia and/or ophthalmoplegia. The etiology remains obscure and it is rarely associated with rheumatoid arthritis. We here reported the case of a 61-year-old woman treated by TNF-α blockade, namely etanercept, for rheumatoid arthritis for ten years who developed an acute ocular myositis. The patient improved after etanercept was stopped and initiation of high doses of corticosteroids. To our knowledge, this is the second report of ocular myositis occurring under TNF-α blockade treatment for rheumatoid arthritis.

Ocular Myositis Occurring with NOD2-associated Autoinflammatory Disease

NOD2-associated autoinflammatory disease (NAID) is an emerging disease1. We report a patient with ocular myositis (OM) whose clinical phenotype was consistent with NAID. A 34-year-old white woman developed eyelid swelling, pain on eye movement, and color vision loss 3 years ago. There was right eye pain with adduction and abduction with normal visual acuity and color vision on examination. A magnetic resonance imaging study revealed …

Ocular myositis

Patient 1: 13-year-old girl. Movement of right eye painful, particularly when looking to the left. MRT of orbit: inflamed swelling of the right m. rectus med. Therapy of 100 mg Urbason for 5 days, slowly reduced over a period of 8 weeks. Condition improved rapidly within a few days, MRT showed normal right m. rectus int. after 3 months. No relapse.

Ocular Myositis

Ocular myositis frequently manifests with orbital pain and diplopia. The diagnosis of ocular myositis falls within the overall classification of idiopathic orbital inflammatory diseases, defined as non-infective non-specific orbital inflammation without identifiable local or systemic causes. Orbital myositis may form part of more widespread systemic inflammatory processes such as Crohn's disease and the more recently described IgG4-related disease. There is also a broad range of ophthalmic differential diagnoses. Diagnosis, assessment and management of ocular myositis requires the cooperation of ophthalmologists and rheumatologists/immunologists in order to achieve the best patient outcomes. The current literature and avenues of future research are reviewed.

How to deal with diplopia

Diplopia is a frequent neuro-ophthalmologic symptom with diverse etiologies. This article describes elementary diagnostic tests and frequent causes of diplopia. Monocular diplopia persists when the other eye is closed and usually disappears when the patient looks through a pinhole. It is usually caused by errors in the optical media of the eye and has to be differentiated from spectacle-induced side effect and non-organic disorders. A sign of non-organic etiology is absence of change in image position when the head is tilted. Binocular diplopia disappears regardless of which eye is closed. Binocular diplopia occurs when the images of both eyes cannot be fused. The most frequent direct cause of diplopia is acquired strabismus. Knowledge of several specific types of strabismus enables efficient patient management. Congenital and decompensating strabismus like accommodative esotropia, pathophoria, strabismus surso- and deorsoadductorius, retraction syndrome, Brown's syndrome and esotropia in high myopia only need ophthalmologic treatment. Orbital injury, orbital tumor, ocular myositis, Graves orbitopathy and vascular disease usually require multidisciplinary management. Neurogenic paresis, superior oblique myokymia, ocular neuromyotonia, myasthenia, chronic progressive external ophthalmoplegia (CPEO), internuclear ophthalmoplegia (INO) and skew deviation require specific neurologic examination. Treatment of diplopia includes treatment of the fundamental disorder, monocular occlusion, prisms and strabismus surgery.

Diagnose und Differenzialdiagnose der endokrinen Orbitopathie in der MRT

Imaging of Graves' orbitopathy (GO) includes radiological and nuclear medicine procedures. Depending on the method used they provide information about the distribution and activity of the disease. Magnetic resonance imaging (MRI) is not only a helpful tool for making the diagnosis it also enables differentiation of the active and inactive forms of GO due to intramuscular edema. The modality is therefore appropriate to evaluate the disease activity and the course of therapy. The disease leads to the typical enlargement of the muscle bodies of the extraocular muscles. The inferior rectus, medial rectus and levator palpebrae muscles are mostly involved. Signal changes of the intraconal and extraconal fat tissue are possible and a bilateral manifestation is common. The differential diagnosis includes inflammatory diseases and tumors, of which orbital pseudotumor (idiopathic, unspecific orbital inflammation), ocular myositis and orbital lymphoma are the most important. The specific patterns (localization, involvement of orbital structures and signal changes) can be differentiated by MRI.

Subcutaneous immunoglobulins in ocular myositis

Subcutaneous immunoglobulins in ocular myositis

Topical Cyclosporine A as a Steroid-Sparing Agent in Steroid-Dependent Idiopathic Ocular Myositis With Scleritis: A Case Report and Review of the Literature

To report on a case of idiopathic orbital myositis with scleritis that was effectively controlled with topical 0.05% cyclosporine A and to provide a review of the literature on the treatment of ocular myositis with scleritis. A case report. A 35-year-old woman presented with a longstanding history of intractable periorbital pain, redness on her left eye, and diplopia during ocular movement. Her medical history revealed that she had the same symptoms for 5 years and had used numerous prescribed medications for migraine and ocular myositis. During this period, her symptoms and signs had been lessened on systemic steroid treatment, which recurred or worsened after discontinuing or tapering the therapy. Magnetic resonance imaging scans demonstrated an isolated enlargement of the left medial rectus muscle. Laboratory examination results showed no evidence of dysthyroid ophthalmopathy or another systemic disease. Because of adverse affects of systemic corticosteroid and cyclosporine treatments, topical cyclosporine A (0.05%) and dexamethasone were administered four times daily. The patient continued to use topical 0.05% cyclosporine A for 6 months. Using only topical cyclosporine A, she currently has no recurrences of disease on the last examination after 6 months of treatment. Moreover, magnetic resonance imaging revealed a completely normal extraocular muscle configuration. Topical 0.05% cyclosporine A may be a safe and effective long-term treatment of ocular myositis and scleritis. It should be considered as a steroid-sparing agent, particularly in recurrent disease and in those patients who experience adverse effects of systemic medications.

ASPECTOS CLÍNICOS DA LEISHMANIOSE VISCERAL CANINA NA CIDADE DE FORTALEZA, CE

A leishmaniose visceral é uma antropozoonose muito importante no Brasil. O presente trabalho teve como objetivo realizar a descrição clínica de 21 cães naturalmente acometidos por leishmaniose visceral na cidade de Fortaleza, Ceará. O diagnóstico de leishmaniose visceral foi realizado por meio de exames sorológicos. As alterações mais freqüentes foram as dermatológicas, seguidas por emaciação, hiporexia, linfadenomegalia e apatia. Também foram observadas alterações oculares, esplenomegalia, artrite, onicogrifose, emese, miosite, desidratação, epistaxe e comprometimento do sistema urinário.

Retrobulbar pain, diplopia and exophthalmus as manifestations of an isolated ocular myositis in a 15 year old boy

Aims: The idiopathic orbital Myositis is a rare disease. Typical symptoms are retrobulbar pain with painful eye movements, diplopia on deviation of eyeball, swelling of eyelid, exophthalmus and conjunctival redness. Vision, pupil reactions, fundus and intraocular pressure are always normal. Single or multiple muscle affection and uni- or bilateral eye affection have been described. Infectious causes like Herpes zoster, Borreliosis, Tuberculosis, cysticercosis, infections of the airways and autoimmune diseases like SLE, Crohn disease and paraneoplasitc syndromes should be considered in the differential diagnoses.

Extraocular Mitochondrial Myopathies and their Differential Diagnoses

The diagnosis of mitochondrial myopathy depends upon a constellation of findings, family history, type of muscle involvement, specific laboratory abnormalities, and the results of histological, pathobiochemical and genetic analysis. In the present paper, the authors describe the diagnostic approach to mitochondrial myopathies manifesting as extraocular muscle disease. The most common ocular manifestation of mitochondrial myopathy is progressive external ophthalmoplegia (PEO). To exclude myasthenia gravis, ocular myositis, thyroid associated orbitopathy, oculopharyngeal muscular dystrophy, and congenital fibrosis of the extraocular muscles in patients with an early onset or long-lasting very slowly progressive ptosis and external ophthalmoplegia, almost without any diplopia, and normal to mildly elevated serum creatine kinase and lactate, electromyography, nerve conduction studies and MRI of the orbits should be performed. A PEO phenotype forces one to look comprehensively for other multisystemic mitochondrial features (e.g., exercise induced weakness, encephalopathy, polyneuropathy, diabetes, heart disease). Thereafter, and presently even in familiar PEO, a diagnostic muscle biopsy should be taken. Histological and ultrastructural hallmarks are mitochondrial proliferations and structural abnormalities, lipid storage, ragged-red fibers, or cytochrome-C negative myofibers. In addition, Southern blotting may reveal the common deletion, or molecular analysis may verify specific mutations of distinct mitochondrial or nuclear genes.

291 Orbital Myositis Presenting as Cluster Headache

Background. Cluster headache affects men in the third decade of life. Episodes centers in and around eye, start acutely, last 1 to 2 hours, and occur several times a day. Associated features are ipsilateral conjunctival hyperemia, lacrimation, ptosis, miosis, and facial sweating. Orbital myositis belongs to the group of idiopathic orbital inflammatory syndromes. Clinical picture involves proptosis, conjunctival hyperemia, lid edema, and painful eye movements. Case report. We report on a 13 year-old girl presenting with recurrent and alternating orbital myositis mimicking a cluster headache. At the age of 10 years she first experienced an episode of ocular pain, diplopia, and palpebral edema involving the right eye. Lacrimation, conjunctival hyperemia, nasal rhinorrhea and headache were also observed. The episode lasted 1 month. Neurological examination was normal. Further four episodes affecting the right and the left eyes, alternatively, occurred during a period of 18 months. Cluster headache was initially diagnosed. Neither FANS nor tryptans were able to control clinical symptoms. Magnetic resonance imaging of the orbits showed evident edema and thickening of the correspondent rectus lateralis which subsided after corticosteroids. Idiopathic ocular myositis was therefore diagnosed. The following investigations were performed in several occasions and resulted normal or negative. They included: (i) levels of complement factors and of immunoglobulins; (ii) Raitest and Waaler-Rose test; (iii) research of antinuclear antibody, antibody to ds-DNA, antibodies to extractable nuclear antigens (RNP, Sm, SS/A, SS/B), antisynthetasis (Jo1), antineutrophil cytoplasmic antibody (IFI, antiproteinase 3, antimyeloperoxidase, antilactoferrin). Thyroid function was normal. Viral and bacterial infections were also excluded. Orbital myositis may clinically mimic cluster headache. Conclusions. Orbital myositis should be considered in those patients with atypical cluster headache signs such as painful eye movements and ocular pain which do not resolve after 3–4 hours. An early diagnosis is necessary to plan a proper therapeutic strategy.

Cyclic vertical deviation after ocular myositis and treatment by recession of the inferior rectus muscle

Cyclic strabismus is a rare disorder in which strabismus and orthotropia regularly alternate over a period of mostly 48 h. It may occur spontaneously, upon squint surgery, or in association with lesions of the central nervous system. In most cases the deviations are convergent. Clinical case report. A 34-year-old woman with bilateral recurrent ocular myositis for 2 years had developed cyclic vertical deviation 6 months after clinical remission. A hypotropia of the left eye alternated with an orthotropia, following a 48-h rhythm. Three months after recession of the inferior rectus muscle the alternating squint had disappeared. The aetiology of cyclic eye deviations, most of them occurring in a constant rhythm, is not known. The association with lesions of the central nervous system indicates a primary central dysregulation of a "biological clock". Their well-known occurrence, however, after squint surgery and, as in the present case, after orbital myositis, suggests that alteration of peripheral structures may contribute to a central dysregulation. Squint surgery seems to be the treatment of choice, even in rare cases with vertical deviations.