Extraocular Muscle Involvement Research Articles

Magnetic Resonance Imaging of Idiopathic Orbital Myositis.

To characterize the qualitative and quantitative features of idiopathic orbital myositis (IOM) on MRI. This was a multicenter retrospective study of patients with active IOM with MRI. Patients with incomplete clinical records, poor-quality or interval scans without active myositis, and specific orbital myositis (i.e., orbital myositis secondary to an identified condition) were excluded. An enlargement ratio was calculated by dividing the diameters of the affected extraocular muscle (EOM) by the contralateral unaffected EOM. Twenty-four patients (mean age: 44.4 ± 17.8 years-old, male: 11) between 2011 and 2022 were included. One case (4.2%) was pediatric (17 years old), and 6 cases presented with recurrence. Active IOM was characterized by fusiform EOM enlargement, high T2 signal, and contrast enhancement. Average maximal EOM diameters ranged from 4.6 to 7.7 mm (enlargement ratio: 1.4-2.2). Eighteen (75%) patients had single EOM involvement, most commonly the medial rectus. Other ipsilateral structures affected included focal orbital fat (16/24, 66.7%) and lacrimal gland (8/24, 33.3%). Contralateral changes in the EOM and/or lacrimal gland were observed in 7 patients (29.2%). Patients presenting with recurrence were likely to develop ongoing recurrent episodes (p = 0.003). Various radiological patterns of involvement described including EOM enlargement, contrast enhancement, abnormal signal, and involvement of other orbital structures are indicative of active IOM. IOM remains a heterogeneous spectrum of acute and chronic clinico-radiological presentations. Inflammation may involve other ipsilateral or contralateral orbital structures or may be bilateral despite presenting clinically as unilateral disease. Quantitative measurements may have utility in differentiating IOM from other causes of orbital myositis.

Uncover the Hidden Veils: Ophthalmoplegia in Breast Cancer

Abstract
 Introduction : Breast cancer can metastasize to many sites, but the orbit is an infrequent location. Orbital metastases represent 1%–13% of all orbital tumors, and the prognosis is rather poor. We reported a case of orbital metastases of breast cancer that led to findings of other sites involvement.
 Case Illustration : A 50-year-old female presented with an inward squint and double vision for 9 months before admission. Esotropia, abduction, and elevation deficits in both eyes were observed. The visual acuity of both eyes was 6/6. Orbital MRI revealed an intraconal irregular lesion near the medial rectus muscle, which was hyperintense with contrast and thickened medial rectus muscles of both eyes. She underwent lumpectomy of breast mass two years before admission and no follow-up after surgery. Histopathology showed invasive lobular carcinoma (ILC). We referred her to hematologist- oncologist to have workup on metastases and treatment. Lung and extensive bone metastases were discovered. She was diagnosed with metastatic breast cancer (stage IV) and treated with hormonal therapy and bisphosphonates. On 3-month follow-up, diplopia and gaze limitations persist.
 Discussion : Diplopia and motility disturbances are the most common symptoms and signs of orbital metastases, including extraocular muscle involvement. The horizontal rectus muscles are more commonly involved. ILC is more frequent histological subtype involving orbital as the orbits are rich in fat and may attract disseminated ILC cells.
 Conclusion : Ocular symptoms in patients with a history of breast cancer should always be investigated for metastatic disease. A multidisciplinary approach is mandatory to achieve the best therapeutic management and long-term surveillance.

Investigation of the differential susceptibility of extraocular muscles in patients diagnosed with ocular myasthenia gravis based on the computerized diplopia test and the Ocular Motor Nerve Palsy Scale.

The pattern of extraocular muscle involvement in ocular myasthenia gravis varies across different reports, diverging from our own observations. Thus, we employed two novel tools to discern this pattern. A retrospective analysis was conducted to collect and organize clinical data from 43 patients diagnosed with ocular myasthenia gravis. Each patient underwent both the computerized diplopia test and the Ocular Motor Nerve Palsy Scale assessment to evaluate the involvement of extraocular muscles. Among the patients, there were 30 male and 13 female individuals, with a total of 113 affected extraocular muscles identified. Among all the affected extraocular muscles, the involvement of the levator palpebrae superioris muscle accounted for 35.40%, medial rectus muscle 7.7%, lateral rectus muscle 16.81%, superior rectus muscle 13.27%, inferior rectus muscle 12.39%, superior oblique muscle 1.77%, and inferior oblique muscle 2.65% of the total affected extraocular muscles. The positivity rates of the Neostigmine test were 89.19%, AChR antibody detection was 59.38%, and repetitive nerve stimulation was 34.38%. The AChR antibody positive rate among patients with only diplopia was 100%; among those with only ptosis, it was 80%; and among those with both diplopia and ptosis, it was 86.67%. The involvement of the extraocular muscles is not uniform. The levator palpebrae superioris exhibits the highest incidence rate, followed by the four rectus muscles and two oblique muscles. The inferior oblique involvement typically occurs when four or more EOMs are affected. Moreover, the levator palpebrae superioris and medial rectus show a higher tendency for bilateral involvement compared with other extraocular muscles.

Thyroid-associated ophthalmopathy: the role of oxidative stress.

Thyroid-associated ophthalmopathy (TAO) is an autoimmune condition affecting the eyes, characterized by proptosis, extraocular muscle involvement, and in severe cases, vision impairment including diplopia, optic neuropathy, and potential blindness. The exact etiology of TAO remains elusive; however, increased oxidative stress and decreased antioxidant capacity are pivotal in its pathogenesis. Elevated oxidative stress not only directly damages orbital tissues but also influences thyroid function and autoimmune responses, exacerbating tissue destruction. This review explores the role of oxidative stress in TAO, elucidates its mechanisms, and evaluates the efficacy and limitations of antioxidant therapies in managing TAO. The findings aim to enhance understanding of oxidative stress mechanisms in TAO and propose potential antioxidant strategies for future therapeutic development.

Mitochondrial myopathy without extraocular muscle involvement: a unique clinicopathologic profile.

Mitochondrial myopathy without extraocular muscles involvement (MiMy) represents a distinct form of mitochondrial disorder predominantly affecting proximal/distal or axial muscles, with its phenotypic, genotypic features, and long-term prognosis poorly understood. A cross-sectional study conducted at a national diagnostic center for mitochondrial disease involved 47 MiMy patients, from a cohort of 643 mitochondrial disease cases followed up at Qilu Hospital from January 1, 2000, to January 1, 2021. We compared the clinical, pathological, and genetic features of MiMy to progressive external ophthalmoplegia (PEO) and mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) patients. MiMy patients demonstrated a more pronounced muscle involvement syndrome, with lower 6MWT scores, higher FSS, and lower BMI compared to PEO and MELAS patients. Serum levels of creatinine kinase (CK), lactate, and growth and differentiation factor 15 (GDF15) were substantially elevated in MiMy patients. Nearly a third (31.9%) displayed signs of subclinical peripheral neuropathy, mostly axonal neuropathy. Muscle biopsies revealed that cytochrome c oxidase strong (COX-s) ragged-red fibers (RRFs) were a typical pathological feature in MiMy patients. Genetic analysis predominantly revealed mtDNA point pathogenic variants (59.6%) and less frequently single (12.8%) or multiple (4.2%) mtDNA deletions. During the follow-up, a majority (76.1%) of MiMy patients experienced stabilization or improvement after therapeutic intervention. This study provides a comprehensive profile of MiMy through a large patient cohort, elucidating its unique clinical, genetic, and pathological features. These findings offer significant insights into the diagnostic and therapeutic management of MiMy, ultimately aiming to ameliorate patient outcomes and enhance the quality of life.

Prevalence of Thyroid Eye Disease Among Thyroid Disorder Patients

Aim: To assess the prevalence of Thyroid Eye Disease among thyroid disorder patients. Introduction: Thyroid Eye Disease (TED) is a debilitating autoimmune disorder seen in hyperthyroid, hypothyroid and euthyroid patients. The global prevalence of TED is estimated to be 16 per 100,000 women and 2.9 per 100,000 men in the general population. Common symptoms include dry eyes, eyelid retraction, excessive watering, conjunctival redness and swelling, retro-orbital pain and blurred vision. This study aims to assess the prevalence of TED among dysthyroid patients attending Saveetha Medical College and Hospital, Chennai. Methodolody: The purpose of this study is to find the prevalence of TED among dysthyroid patients attending Saveetha Medical College and Hospital, Chennai during the months of July and August. A detailed history of each patient was taken after which a series of tests were conducted to identify and classify the severity of TED. This was done using NOSPECS (no signs, only signs, soft tissue involvement, proptosis, extraocular muscle involvement, corneal involvement, sight loss) classification. Schirmers test was performed to assess the prevalence of dry eyes among TED patients. Result: 122 patients consisting of 87 females and 35 males were included in this study. 43% (53 patients) were found to have Thyroid Eye Disease. There was no significant difference in prevalence between male and female subjects (43.6% and 42.8% respectively). The presence of TED was far greater in hyperthyroid than in hypothyroid patients. 44 out of 53 patients with TED were hyperthyroid (83%), 7 were hypothyroid (13.2%), and 2 patients were euthyroid (3.7%) . Schirmers test conducted on each patient revealed that 12% of the TED patients suffered from dry eyes. NCT test conducted to find the intraocular pressure in TED patients revealed no significant results. Conclusion: The high prevalence of TED seen in dysthyroid patients in this study calls for increased awareness regarding ocular manifestations in thyroid disorders. Patients should be encouraged to undergo regular checkups to ensure normal functioning of eyes and to prevent progression of the disease.

Rubrospinal activation during asleep subthalamic nucleus deep brain stimulation: a false localizing sign. Illustrative case.

Deep brain stimulation (DBS) can be a life-changing intervention for patients with Parkinson's disease (PD), but its success is largely dependent on precise lead placement. The subthalamic nucleus (STN) is one of the most common surgical targets of DBS, but the close anatomical and physiological resemblance of the STN to the mediocaudal red nucleus renders these landmarks difficult to distinguish. We present an atypical case in which targeted localization of the STN resulted in symptoms pathognomonic of rubrospinal tract (RST) stimulation. A 79-year-old female with a 12-year history of right-hand resting tremor due to medically refractory PD presented for asleep bilateral STN-DBS surgery. Right STN intraoperative testing revealed left hand and elbow flexion contractures, initially suggestive of corticospinal tract activation, despite imaging studies demonstrating reasonable lead placement in the central dorsolateral STN. The lead was moved anteromedially near the medial border of the STN, but stimulation at this location revealed similar but more robust flexor hand and arm contractures, without any extraocular muscle involvement. Thus, activation of the RST was suspected. Isolated activation of the RST is possible during STN-DBS surgery. Its identification can help avoid false localization and suboptimal lead placement.

A study on epidemiology, clinical profile, management, and outcome of Covid-19-associated rhino-orbital-cerebral mucormycosis in a tertiary hospital in Tamil Nadu

: The main objective of the study is to estimate determine the patient demographics, comorbidities, and medications used to treat COVID-19, presenting symptoms and signs, and the outcome of management of COVID-19-associated rhino-orbital-cerebral mucormycosis (ROCM).: A descriptive cross-sectional study was conducted among 50 patients admitted and suspected with Mucormycosis in 3 months duration from May 21 to July 21 Covid pandemic at a tertiary hospital in Villupuram. Written informed consentwas taken from patients or nearest of kin. This study was approved by Institutional Ethical Committee of Villupuram.: 84% patients among suspected mucormycosis were found to be diabetic, with 70% being males and 61.9% had covid history either covid positive or post covid status. About 60% of suspected mucor cases had h/o oxygen treatment and almost 44% had h/o steroid treatment for their covid treatment. Clinically 40% had extraocular muscle involvement in the form of restricted eye movement to total ophthalmoplegia and mortality was ranging from 37.5-57.14%. Those with proper glycemic control and initiation of treatment at appropriate time with endoscopic sinus debridement and antifungal therapy showed better recovery.: The study reveals that COVID-19 associated Mucormycosis has risen dramatically due to interplay of uncontrolled diabetes and in-appropriate corticosteroid use leading to pathogenic invasion and adverse outcomes. The infection has caused high morbidity and mortality among covid positive than post covid patients. It could be minimized by strict diabetic control under appropriate insulin dosage, wise use of steroids/oxygen and timely intervention based on clinical evaluation rather than waiting for investigations could minimize the complications from the infection and shorten the hospital stay.

The outcomes of endoscopic orbital decompression combined with fat decompression for thyroid-associated ophthalmopathy

PurposeTo present the clinical features of thyroid-associated ophthalmopathy (TAO) with different CT types, and to report the outcomes of endoscopic orbital decompression combined with fat decompression (EOD-FD).Patients and methodsThirty-four patients with TAO who underwent EOD-FD between December 2020 and March 2022 in the Ophthalmology Department of Li Huili Hospital Affiliated with Ningbo University, were included in this retrospective interventional case series. Patients were categorized into two groups based on the results of computerized tomography (CT) scans: muscle expansion type and fat hyperplasia type.ResultsThirty-four TAO patients (55 eyes) were included in this study, and the mean age was 38.62 years (range 22–60 years). The average eye protrusion (EP) reduced from preoperative 23.20 mm to postoperative 19.66 mm (p < 0.0001). Mean intraocular pressure (IOP) decreased from 20.11 mmHg at baseline to 17.29 mmHg postoperatively (p < 0.0001), with a reduction of 2.84 mmHg (14.12%). Twenty cases of muscle expansion and fourteen cases of fat hyperplasia were definite by CT imaging. The mean IOP in the muscle expansion group was higher than that in the fat hyperplasia group (p < 0.05). Elevated intraocular pressure (IOP) occurred in 23 eyes (36.11%), and it was associated with extraocular muscle involvement, gender, and EP. In 3 cases of impaired vision, the mean best corrected visual acuity (VA) improved from 0.4 preoperatively to 0.84 postoperatively (p < 0.01). There were 8 cases with visual field (VF) damage and/or corneal epithelium damage, and all these damages were reversible.ConclusionIn this study, we describe the clinical features and experience of EOD-FD in patients with TAO. EOD-FD is an effective technique in reducing IOP and proptosis, with a low incidence of postoperative diplopia.

Turbo Gradient and Spin-Echo BLADE-DWI for Extraocular Muscles in Thyroid-Associated Ophthalmopathy

Purpose: To investigate feasibility and diagnostic performance of turbo gradient and spin-echo BLADE (proprietary name for Periodically Rotated Overlapping ParallEL Lines with Enhanced Reconstruction [PROPELLER] in Siemens MR systems)-diffusion weighted imaging (TGSE-BLADE-DWI) for depicting extraocular muscle (EOM) involvement and activity in thyroid-associated ophthalmopathy (TAO), and to compare TGSE-BLADE-DWI with readout-segmented echo-planar imaging (RESOLVE). Materials and methods: Thirty-five patients with identified TAO underwent the two DWI scans. Two radiologists visually scored the image quality of the two DWIs with respect to the susceptibility artifacts and geometric distortions on a three-point scale. The maximum size (Sizemax) of EOMs and corresponding ADCs (cADCs) of each patient were compared between the active and inactive phases. The clinical activity score (CAS) was used as a reference to assess the diagnostic performance of EOM ADCs for grading TAO activity. ROC analysis, Pearson correlation, and Wilcoxon signed-rank test were used for statistical analyses. Results: For scores of EOMs, the image quality of TGSE-BLADE-DWI was significantly higher than that of RESOLVE. There were no statistically significant differences between the AUCs of the two DWIs, Sizemax, or cADCs between the active and inactive phases. TGSE-BLADE-DWI ADCs were significantly higher than the RESOLVE ADCs in the right superior rectus, right lateral rectus, left superior rectus, and left inferior rectus. There were no statistically significant correlations between the cADC or Sizemax, and CAS. The highest AUC was 0.697 for RESOLVE and 0.657 for TGSE-BLADE-DWI. The best performing ADC threshold was 1.85 × 10-3 mm2/s with 85.7% sensitivity, 58.8% specificity and 66.67% accuracy for RESOLVE and 1.99 × 10-3 mm2/s with 79.0% sensitivity, and 55.6% specificity and 65.27% accuracy for TGSE-BLADE-DWI. Conclusion: Compared to RESOLVE, TGSE-BLADE-DWI provided improved image quality with fewer susceptibility artifacts and geometric distortions for EOM visualization and showed an equivalent performance in detecting active TAO.

Clinical and imaging clues to the diagnosis and follow-up of ptosis and ophthalmoparesis.

Ophthalmoparesis and ptosis can be caused by a wide range of rare or more prevalent diseases, several of which can be successfully treated. In this review, we provide clues to aid in the diagnosis of these diseases, based on the clinical symptoms, the involvement pattern and imaging features of extra-ocular muscles (EOM). Dysfunction of EOM including the levator palpebrae can be due to muscle weakness, anatomical restrictions or pathology affecting the innervation. A comprehensive literature review was performed to find clinical and imaging clues for the diagnosis and follow-up of ptosis and ophthalmoparesis. We used five patterns as a framework for differential diagnostic reasoning and for pattern recognition in symptomatology, EOM involvement and imaging results of individual patients. The five patterns were characterized by the presence of combination of ptosis, ophthalmoparesis, diplopia, pain, proptosis, nystagmus, extra-orbital symptoms, symmetry or fluctuations in symptoms. Each pattern was linked to anatomical locations and either hereditary or acquired diseases. Hereditary muscle diseases often lead to ophthalmoparesis without diplopia as a predominant feature, while in acquired eye muscle diseases ophthalmoparesis is often asymmetrical and can be accompanied by proptosis and pain. Fluctuation is a hallmark of an acquired synaptic disease like myasthenia gravis. Nystagmus is indicative of a central nervous system lesion. Second, specific EOM involvement patterns can also provide valuable diagnostic clues. In hereditary muscle diseases like chronic progressive external ophthalmoplegia (CPEO) and oculo-pharyngeal muscular dystrophy (OPMD) the superior rectus is often involved. In neuropathic disease, the pattern of involvement of the EOM can be linked to specific cranial nerves. In myasthenia gravis this pattern is variable within patients over time. Lastly, orbital imaging can aid in the diagnosis. Fat replacement of the EOM is commonly observed in hereditary myopathic diseases, such as CPEO. In contrast, inflammation and volume increases are often observed in acquired muscle diseases such as Graves' orbitopathy. In diseases with ophthalmoparesis and ptosis specific patterns of clinical symptoms, the EOM involvement pattern and orbital imaging provide valuable information for diagnosis and could prove valuable in the follow-up of disease progression and the understanding of disease pathophysiology.

Primary mitochondrial myopathy: 12-month follow-up results of an Italian cohort

ObjectivesTo assess natural history and 12-month change of a series of scales and functional outcome measures in a cohort of 117 patients with primary mitochondrial myopathy (PMM).MethodsTwelve months follow-up data of 117 patients with PMM were collected. We analysed the 6-min walk test (6MWT), timed up-and-go test (× 3) (3TUG), five-times sit-to-stand test (5XSST), timed water swallow test (TWST), and test of masticating and swallowing solids (TOMASS) as functional outcome measures; the Fatigue Severity Scale and West Haven-Yale Multidimensional pain inventory as patient-reported outcome measures. PMM patients were divided into three phenotypic categories: mitochondrial myopathy (MiMy) without extraocular muscles involvement, pure chronic progressive external ophthalmoplegia (PEO) and PEO&MiMy. As 6MWT is recognized to have significant test–retest variability, we calculated MCID (minimal clinically important difference) as one third of baseline 6 min walking distance (6MWD) standard deviation.ResultsAt 12-month follow-up, 3TUG, 5XSST and FSS were stable, while TWST and the perceived pain severity (WHYMPI) worsened. 6MWD significantly increased in the entire cohort, especially in the higher percentiles and in PEO patients, while was substantially stable in the lower percentile (< 408 m) and MiMy patients. This increase in 6MWD was considered not significant, as inferior to MCID (33.3 m). NMDAS total score showed a slight but significant decline at 12 months (0.9 point). The perceived pain severity significantly worsened. Patients with PEO performed better in functional measures than patients with PEO&MiMy or MiMy, and had lower values of NMDAS.ConclusionsPMM patients showed a slow global decline valued by NMDAS at 12 months; 6MWT was a more reliable measurement below 408 m, substantially stable at 12 months. PEO patients had better motor performance and lower NMDAS than PEO&MiMy and MiMy also at 12 months of follow-up.

Ocular hypertension in patients with active thyroid-associated orbitopathy: a predictor of disease severity, particularly of extraocular muscle enlargement.

A total of 96 eyes and orbits of 48 adult patients with active TAO were investigated. All patients underwent magnetic resonance imaging of the orbit and measurement of all extraocular recti muscles (EOM). The obtained data was divided into two groups according to the IOP value: normal IOP ≤ 21mmHg; n = 47 and elevated IOP with IOP > 21mmHg; n = 49, and analyszed. A significant difference was found in the short diameter of medial rectus and inferior rectus muscles and in the sum of short parameters of all EOM. All these parameters were significantly higher in the elevated IOP group. Motility restriction in at least one gaze direction was also significantly more frequent (p < 0.0001) in the elevated IOP group. A positive moderate correlation was found between IOP and the sum of short parameters of EOM (r = 0.496). No correlation was found between the IOP and exophthalmos values (r = 0.267). During the follow-up, the frequency of strabismus surgery and orbital decompression was significantly higher in the elevated IOP group (p = 0.003; p = 0.002). Elevated IOP in the active TAO stage particularly correlates with extraocular muscle involvement. These patients are also more likely to require orbital decompression and strabismus surgery.

Primary Vitreoretinal Lymphoma: A Retrospective Study of 20 Eyes.

Purpose This study aimed to describe and analyze the clinical features of 20 eyes of 15 primary vitreoretinal lymphoma (PVRL) patients. Methods This was a retrospective case series and a review of the literature. Fifteen PVRL patients (20 affected eyes) referred between February 2011 and December 2019 were recruited, and their medical records were retrospectively reviewed. Results Among these 15 PVRL patients, seven were men (46.67%), and five had bilateral PVRL (33.33%). The median onset age was 66 ± 9.26 years and six (40%) patients had central nervous system (CNS) involvement, and two of them died of CNS-related complications. The ocular symptoms varied from decreased vision to binocular diplopia. The ocular manifestations were diverse and involved both the anterior and posterior segments, including the vitreous cells, subretinal white-yellow lesions, cotton-wool spots, and ophthalmoplegia. The rate of misdiagnosis and failure to diagnose was 100%, and 30% of them were misdiagnosed as uveitis. We found five cases revealing rare characteristics of this malignancy. Among them, there were two cases with mild hypertensive retinopathy exhibiting cotton-wool spots, one case mimicking age-related macular degeneration (AMD), one case with systemic lupus erythematosus (SLE), and one patient had extraocular muscle involvement. To the best of our knowledge, we reported PVRL exhibiting cotton-wool spots as the main manifestation and coexisting with extraocular myopathy for the first time. Conclusions PVRL is a rare intraocular malignancy that commonly masquerades as uveitis. As the clinical signs and symptoms are atypical, ophthalmologists must carefully examine patients to avoid misdiagnosis or a failure to diagnose. Cotton-wool spots and extraocular myopathy might be the dominant initial symptoms in PVRL patients, and AMD should be considered a differential diagnosis of PVRL. SLE patients under immunosuppressive treatment could have spontaneous PVRL.

Longitudinal association of thyroid-stimulating immunoglobulin levels with clinical characteristics in thyroid eye disease

ObjectivesThe clinical course of thyroid eye disease (TED) is heterogeneous and predicting patients who may develop the severe sequelae of the disease is difficult. In this study, we evaluated the...

Clinical characteristics and outcomes of myasthenia gravis patients with double positive antibodies against acetylcholine receptor and muscle-specific tyrosine kinase

Objective: To investigate the clinical manifestations, treatment characteristics and outcomes of myasthenia gravis (MG) dually positive for anti-acetylcholine receptor antibody (AChR-Ab) and anti-muscle-specific tyrosine kinase antibody (MuSK-Ab). Method: MG patients hospitalized in the First Affiliated Hospital of Sun Yat-sen University from August 2017 to November 2020 were retrospectively collected. Thirty-four MuSK-Ab positive MG (MuSK-MG) patients, 11 double-antibodies positive MG (DP-MG) patients, and 80 AChR-Ab positive MG (AChR-MG) patients were included and allocated to three different groups. The clinical data of patients in the three groups were collected, and the differences of demographic characteristics, clinical manifestations and treatment outcomes between DP-MG patients and AChR-MG and MuSK-MG patients were analyzed. Result: The proportion of female and male patients in DP-MG group was 7/11 and 4/11 respectively, and the onset age of DP-MG was (41±27) years.The difference in gender distribution between DP-MG and AChR-MG groups was statistically significant (P<0.05). The proportion of extraocular muscle involvement in the DP-MG and MuSK-MG groups (8/11 and 52.9%) was lower than that in the AChR-MG group (83.8%), and the difference was statistically significant (P<0.05). The incidence of myasthenia crisis in DP-MG and MuSK-MG groups (54.5% and 61.8%) were higher than that in AChR-MG group (20.0%), with astatistically significant difference(P<0.05). The positive rate of neostigmine test in DP-MG and MuSK-MG groups(8/11 and 74.2%) were lower than that of AChR-MG group (96.8%), and the positive rate of low frequency repetitive nerve stimulation (RNS) in DP-MG group (5/10) was lower than that in AChR-MG group (85.1%), with statistically significant differences (all P<0.05). MuSK-Ab titer was positively correlated with the course of disease (r=0.466, P<0.05), and antibody titer decreased after symptom improvement (P<0.05). The response of patients in DP-MG and MuSK-MG groups to cholinesterase inhibitors (2/11 and 9.1%) was worse than that in the AChR-MG group (66.3%), and the incidence of side effects in the two groups (5/11 and 39.4%) was higher than that in the AChR-MG group (15.0%), with statistically significant differences (all P<0.05). There were 4 DP-MG patients underwent thymectomy, and the pathological results detected two cases of thymoma and two cases of thymic hyperplasia. Subsequent follow-up showed that 5 (5/11) DP-MG patients achieved minimal manifestation status or better status. Conclusion: The gender distribution, age of onset, pharmacological characteristics and electrophysiological examination of DP-MG patients were similar to those of MuSK-MG patients, but the severity of DP-MG patients was between that of AChR-MG and MuSK-MG patients.

COVID-associated rhino-orbito-cerebral mucormycosis: A comparative study between computed tomography and magnetic resonance imaging for its early detection in suspected cases of rhino-orbito-cerebral mucormycosis

INTRODUCTION: Rhino-orbito-cerebral mucormycosis (ROCM), a life-threatening, acute fungal infection of the nasal cavities and paranasal sinuses, once considered a rare and lethal complication in immunocompromised patients, is now having a massive increase in ROCM incidence in India associated with COVID-19, i.e., COVID-associated mucormycosis with more than 15,000 cases as of May 2021. Early imaging by computed tomography (CT) and magnetic resonance imaging (MRI) is not only helpful in assessing the extent of involvement of this lethal disease but also helps in early diagnosis leading to prompt and aggressive treatment. The present study is aimed at determining and comparing the imaging findings on CT and MRI in ROCM patients, for early diagnosis in suspected cases of ROCM. MATERIALS AND METHODS: The present study is a comparative study of CT and MRI done on 11 patients suspected of ROCM, for 2 months from May to June 2021. RESULTS: Among 11 patients in the study group, ranging from 29 to 65 years of age, 45.4% belonged in 41–60 years of age group, with female predominance. 90.90% cases (10 patients) had maxillary sinus involvement among which 7 cases were diagnosed with ROCM, followed by 63.60% cases (7 patients) had ethmoidal sinus involvement among which 6 cases were diagnosed with ROCM. The involvement of retromaxillary space, pterygopalatine fossa space and masticator space, and cellulitis in premaxillary and preseptal spaces were findings seen only in diagnosed cases of ROCM. Erosions of bones were seen in ROCM cases, 27.20% cases (3 patients) had erosions of walls of maxillary sinus and lamina papyracea each. Orbital involvement in the form of intraconal space and extraocular muscles involvement was seen in 27.20% cases (3 patients) each. Optic nerve involvement was seen in 18.10% cases (2 patients) followed by extraconal space involvement in 9.01% cases (1 patient). Taking KOH staining and/or histopathology (HPR) as standard of reference, CT has a Sensitivity (Sn) of 71.40%, Specificity (Sp) of 100%, Positive predictive value (PPV) of 100%, Negative Predictive Value (NPV) of 66%, and Diagnostic accuracy (DA) of 81% as compared to MRI which has a Sn of 85.7%, Sp of 100%, PPV of 100%, NPV of 80%, and DA of 90.9%. CONCLUSIOIN: CT and MRI play a complementary role in diagnosis of ROCM, as CT is better in detecting bone erosions, whereas MRI is better in detecting soft tissue, orbital and central nervous system involvement. In the acute state of emergency as in the current pandemic with rising cases of ROCM and acute shortage of amphotericin-B, MRI is the single best modality for accurate detection of ROC, helping clinicians in the judicious use of Amphotericin-B.

Is Video-Oculography a Predictive Test for Myasthenia Gravis with Ocular Symptoms?

The aim of this study was to evaluate the contribution of extraocular muscle function testing with video-oculography (VOG), which is a noninvasive and easily applicable method of recording eye movement with digital cameras, to the diagnosis of myasthenia gravis (MG) in patients without any clinical eye movement abnormalities. The study included 18 patients prediagnosed with ocular MG: MG Group (N = 7) with abnormal, and non-MG Group (N = 11) with normal single-fiber electromyography. Control group included 50 healthy volunteers. Ocular movements were recorded with the EyeSeeCam VOG device. The inward latency of the 10° horizontal saccade and the downward latency of the 10° vertical saccade were significantly delayed; and the downward amplitude of the 10° vertical saccade was significantly lower in the MG group. Receiver operating characteristic curve analyses showed high specificity values for the discrimination of MG patients. This study supports the usefulness of the VOG device in revealing subclinical extraocular muscle involvement in MG.

English

BACKGROUND Computer tomography (CT) plays a crucial role in elucidating the etiological diagnosis and identifying the extent of orbital lesions related to thyroid eye disease, cellulitis, orbital osteomyelitis, bone tumours, mucocele, orbital varices and cavernous hemangioma. This study was intended to assess the diagnostic utility of CT in patients presenting with orbital lesions. METHODS We enrolled 30 consecutive patients presenting with orbital lesions, who underwent complete ophthalmological, systemic examination, computerized tomography, and other appropriate systemic investigations over one year period. RESULTS In our clinical cohort of 30 patients, the spectrum of encountered orbital lesions included thyroid eye disease in 8 (26.6 %), orbital fractures in 5 (16.6 %), pre-septal hematoma in 3 (10 %), cellulitis in 3 (10 %), lymphoproliferative lesions in 2 (6.6 %), orbital lymphomas in 2 (6.6 %), orbital mucocele in 2 (6.6 %) cavernous hemangioma in 1 (3.3 %), frontal bone osteomyelitis in 1 (3.3 %), orbital varices in 1 (3.3 %), osteoma in 1 (3.3 %) and orbital meningioma in 1 (3.3 %) patients. Proptosis was observed in 22 (77 %) patients. On CT imaging, lesion attenuation was hyperdense in 28 (92 %) and isodense in 2 (8 %) patients. Lesions were extraconal in 19 (63 %), conal in 8 (27 %) and intraconal in 3 (10 %) patients. Extraocular muscle involvement was seen in 8 (27 %) and bone involvement in 11 (37 %) cases. CONCLUSIONS Computerized tomography in the orbital lesions accurately established the nature and extent of lesions. In 100 % of cases, CT was helpful in deciding appropriate systemic investigations to establish the etiological diagnosis. KEY WORDS Computerized Tomography, Orbital Lesions, Etiological Diagnosis.

Pediatric Graves' orbitopathy: a multicentre study.

Graves' orbitopathy (GO) is a rare condition in children often considered to be a less severe condition than at an older age. The aim of our study was to analyse if there are any factors that distinguish paediatric from adult GO in order to provide guidelines for assessing and managing paediatric GO. Study design is a multicentre retrospective observational case series; 115 paediatric patients diagnosed with GO who visited our university medical centres in the Netherlands and Iran between 2003 and 2019 were submitted for complete ophthalmological examinations, serological testing and/or orbital imaging. Main outcome measures focussed on the natural course and clinical picture as well as medical and surgical treatment in paediatric GO. Clinical findings included proptosis (n = 97; 84.3%), eyelid retraction (n = 77; 67%) and diplopia (n = 13; 11.3%). Ninety-two patients (80%) presented with mild disease, 21 (18.3%) with moderate-severe disease and two (1.7%) with severe GO. Five patients (4.3%) underwent intravenous glucocorticoids and 25 patients underwent orbital decompression surgery. Strabismus surgery due to primary involvement of extraocular muscles was performed in two patients (1.7%). Overall, rehabilitative surgical treatment was planned in 31 patients (26.9%) with inactive disease. Two patients experienced reactivation of the disease. Despite the fact that paediatric and adult GO are considered two separate entities, they might be the same disease with two different clinical phenotypes. Paediatric GO population presents with a comparable clinical picture regarding both soft tissue involvement and proptosis, which may require surgical intervention. Proptosis was present in the majority of paediatric GO patients. Orbital decompression was performed in 21.7% of patients.