Pediatric Ocular Myasthenia Gravis: Single-Center Experience.

BACKGROUND: Currently, there is no universally accepted standard treatment for ocular myasthenia gravis (OMG) in children. We aimed to investigate the possible proper regimens and timing of treatment for pediatric OMG cases based on the clinical manifestations: OMG with ptosis only and OMG with other features. METHODS: One hundred and forty two OMG cases attended at the Department of Pediatrics, Xiangya Hospital, Central South University, from 2010 to 2019 were included, and information from medical records was reviewed and recorded. Comparisons of clinical characteristics between patients with OMG with ptosis only and patients with OMG with other features as well as between patients treated with glucocorticoid (GC) within or after six months from disease onset were performed. RESULTS: OMG with other features constituted about 54.9% of the cases, and 66.2% of the patients achieved optimal outcome. Patients with OMG with ptosis only responded to pyridostigmine alone more than patients with OMG with other features who required several therapies (P < 0.001). Patients with OMG with ptosis only had a larger proportion of optimal outcome than the patients with OMG with other features (P = 0.002), and the difference remained significant even when the individual outcome groups were compared (P < 0.001). Patients who received GC within six months had a greater proportion of optimal outcome than those who received it after six months (P < 0.001). CONCLUSIONS: Although OMG with other features is a more common subtype of OMG, it is also more severe than OMG with ptosis only. An earlier addition of GC leads to optimal outcome.

A controlled clinical study on efficacy and safety of periocular triamcinolone acetonide injection for treating ocular myasthenia gravis.

OBJECTIVE: To evaluate the efficacy and safety of peribulbar triamcinolone acetonide injection for treating ocular myasthenia gravis (OMG), with a comparison of traditional oral drug therapy. METHODS: A total of 22 patients with OMG who received periocular triamcinolone acetonide injection (initially 20 mg weekly, then once per month later if symptoms were improved) from July 2019 to July 2022 were evaluated by a comparison of symptom degree before and after treatment. Adverse reactions were also monitored during the period of treatment. The period of follow-up was more than 6 months. Additionally, a comparison of the treatment efficacy between this periocular injection and traditional oral administration was performed in OMG patients. RESULTS: After 4 weeks of treatment, the degree of ptosis in OMG patients decreased to -3.00 ± 0.69, compared to the value (-0.86 ± 1.32) before treatment. The degree of ophthalmoplegia also decreased from 3.12 ± 0.72 to 0.86 ± 0.88 (P < 0.001) after treatment. The achievement rates of minimal manifestations status (MMS)for ptosis and ophthalmoplegia after 4 week-treatment were 86.3% and 75%, respectively, while they were 50% and 30% in patients with traditional oral administration. There was statistically significant difference only in MMS (rather than symptom relief rate and generalization conversion rate) between two groups. No serious complications (except for intraorbital hematoma) were found in OMG patients during the treatment period. CONCLUSION: Repeated peribulbar injection of triamcinolone acetonide can effectively alleviate the initial symptoms of OMG patients. However, the evaluation of its long-term efficacy is still needed. CLINICAL TRIAL REGISTRY: This study has been clinically registered by Chinese Clinical Trial Registry (ChiCTR), first trial registration date:05/07/2019, registration number: ChiCTR1900024285.

Cogan's sign in a patient with suspected post-COVID-19 vaccine-associated myasthenia gravis.

The Cogan's sign is indicative of myasthenia gravis. This is the first report of neurological signs in a patient with post-COVID-19 vaccine-associated myasthenia gravis in Brazil. In this case, a previously healthy 68-year-old woman presented with proximal limb weakness, left ptosis, and diplopia 1 month after receiving her fourth dose of the COVID-19 vaccine. Neurological examination revealed the presence of Cogan's sign, and she recovered rapidly after treatment. To our knowledge, this is the first reported case of myasthenia gravis associated with the COVID-19 vaccine in Brazil.

The effects of topical oxymetazoline on eyelid position, eye redness, and patient-reported eye appearance: A randomized controlled trial.

PURPOSE: This study assesses the effects of topical oxymetazoline 0.1% on eyelid position, eye redness, and patient-perceived eye appearance in patients without severe ptosis. METHODS: This is a randomized double-blinded controlled trial conducted at a single institute. Patients aged 18-100 years were randomized to receive one drop of oxymetazoline hydrochloride 0.1% or placebo bilaterally. Marginal reflex distance (MRD) 1 and 2, palpebral fissure height, eye redness, and patient-perceived eye appearance were assessed at baseline and two hours after drop instillation. Primary outcome measures included the change in MRD1, MRD2, and palpebral fissure height. Secondary outcome measures included changes in eye redness and patient-perceived eye appearance after drop instillation. RESULTS: In total, 114 patients were included, 57 treatment patients (mean age 36.4 ± 12.7 years, 31.6% male) and 57 controls (mean age 31.3 ± 10.1 years, 33.3% male). Baseline mean MRD1, MRD2, and palpebral fissure were similar between groups (p = 0.24, 0.45, and 0.23, respectively). Changes in MRD1 and eye redness in the treatment group were significantly greater than those in the control group (0.9 ± 0.9 mm vs. - 0.3 ± 0.4 mm, p < 0.001; - 2.6 ± 4.4 vs. - 0.5 ± 2.3, p = 0.002, respectively). Patient-perceived eye appearance was significantly improved in the treatment group compared to the controls (p = 0.002), with more treatment group patients also reporting increased eye size and decreased eye redness (p = 0.008, p = 0.003, respectively). There were 9 treatment-emergent adverse events (TEAEs) in 7 treatment group patients and 5 TEAEs in 5 control patients (p = 0.25), all of which were mild in severity. CONCLUSIONS: Topical oxymetazoline 0.1% increases MRD1 and palpebral fissure height, decreases eye redness, and improves patient-perceived eye appearance.

Efficacy and safety of two different botulinum toxin type A dilutions in chronic migraineurs.

Botulinum toxin type A is an effective preventive therapy for chronic migraine. Although the guidelines suggest a 50U/ml dilution of OnabotulinumtoxinA (BoNT/A), many clinicians use more concentrated solutions. However, there are no studies regarding the effect and safety of 100U/ml BoNT/A dilution with the saline solution following the PREEMPT paradigm. Our primary goal was to evaluate the efficacy, in reducing migraine frequency, and safety of two different BoNT/A dilutions (100U/ml vs 50U/ml) in the treatment of Chronic migraine. Our secondary goal was to determine the predictors of BoNT/A response. We retrospectively collected data from 113 chronic migraine patients treated with 3 rounds of BoNT/A according to the PREEMPT protocol as a preventive therapy. Patients were divided into two groups, based on BoNT/A dilution: 50U/ml (49 patients) vs. 100U/ml (64 patients) of sodium chloride 0.9%. We compared the migraine days/month, intensity, and intake of symptomatic medications at the baseline with the data obtained after the treatment; moreover, we evaluated the occurrence of adverse effects observed in the two groups. There was no difference regarding efficacy and safety between the two groups except for eyelid ptosis, which was more common in the 50U/ml BoNT/A group (p 0.018). Unilateral localization of migraine was associated with a more favorable outcome (OR 5.593, C.I. 2.358-13.268; p < 0.001) while Major Depressive Disorder predicted a less favorable response (OR 0.213, C.I. 0.087-0.523; p < 0.001). In our study, BoNT/A dilution did not influence the response to the therapy, but 100U/ml dilution could reduce the risk of eyelid ptosis. Unilateral localization of migraine pain might predict a more favorable response to the therapy, while the presence of a Major Depressive Disorder might predict a less favorable response.

Management of Severe Botulinum-Induced Eyelid Ptosis With Pretarsal Botulinum Toxin and Oxymetazoline Hydrochloride 0.1.

BACKGROUND: Eyelid ptosis following periocular onabotulinumtoxinA (BoNT-A) treatment is a known complication that can be frustrating for both patients and practitioners. Iatrogenic blepharoptosis occurs due to local spread of the BoNT-A from the periocular region into the levator palpebrae superioris muscle. Although injectors should have a thorough understanding of the relevant anatomy in order to prevent it, BoNT-A induced ptosis can occur even in the most experienced hands. OBJECTIVES: The aim of this study was to describe a case series of patients treated effectively with topical oxymetazoline HCl 0.1% and pretarsal BoNT-A injections in the setting of botox-induced ptosis. METHODS: The study group consisted of 8 patients who had undergone recent cosmetic BoNT-A treatment preceding the sudden onset of unilateral upper eyelid ptosis. RESULTS: A diagnosis of severe ptosis (>3 mm) was made in all the cases in this series. Pretarsal BoNT-A injections alone or in association with topical administration of Upneeq eyedrops (Upneeq, Osmotica Pharmaceuticals, Marietta, GA) significantly reversed the ptosis in all treated cases. CONCLUSIONS: This is the first documented case series of patients treated effectively with topical oxymetazoline HCl 0.1% and pretarsal BoNT-A injections in the setting of botox-induced ptosis. This treatment combination is a safe and effective option in these cases.

Vincristine-induced ptosis in a leukemia patient treated with pyridoxine and pyridostigmine.

INTRODUCTION: Blepharoptosis, commonly referred to as ptosis or eyelid sagging, is a condition where the upper eyelid droops over the eye. It can be congenital or acquired and is caused by the weakening of the eyelid muscles. CASE REPORT: We present a case of a 3-year-old boy with T-cell acute lymphoblastic leukemia who developed bilateral ptosis while on treatment with Berlin-Frankfurt Munster-98 protocol. MANAGEMENT & OUTCOME: The patient was diagnosed with bilateral ptosis due to vincristine, the primary agent in the induction phase of the protocol. The addition of the neuroregenerative agents and supportive measures led to marked improvement, followed by complete resolution within 3 weeks. DISCUSSION: Vincristine is an anticancer agent with known neurotoxicity, which has a significant role in treating hematological malignancies and sarcoma. In many studies, the addition of neuroregenerative agents such as pyridoxine and pyridostigmine has been noted to hasten recovery without any documented side effects. Similar findings were also drawn from our research due to India's higher incidence of vincristine-induced neurotoxicity. It is essential to promptly diagnose and manage symptoms at the earliest to prevent the risk of permanent nerve damage and inferior quality of life for the patient.

Current management of myasthenia gravis in Belgium: a single-center experience.

INTRODUCTION: As new treatments are becoming available for patients with myasthenia gravis (MG), it is worth reflecting on the actual status of MG treatment to determine which patients would most likely benefit from the new treatments. METHODS: We reviewed the clinical files of all MG patients seen at the Department of Neurology of the Antwerp University Hospital during the years 2019, 2020 and 2021. RESULTS: 163 patients were included. Age at diagnosis varied from the first to the eighth decades, with a peak of incidence from 60 to 70 years for both genders, and an additional peak from 20 to 30 years in women. Diplopia and ptosis were by far the most common onset symptom. At maximum disease severity, 24% of the patients still had purely ocular symptoms and 4% needed mechanical ventilation. 97% of the patients received a treatment with pyridostigmine and 68% with corticosteroids, often in combination with immunosuppressants. More than half reported side effects. At the latest visit, 50% of the patients were symptom-free. Also, half of the symptomatic patients were fulltime at work or retired with no or mild limitations in daily living. The remaining patients were working part-time, on sick leave, or retired with severe limitations. DISCUSSION AND CONCLUSION: The majority of MG patients are doing well with currently available treatments, but often at the cost of side effects in the short and in the long term. A significant group is in need of better treatments.

Orbital apex syndrome secondary to herpes zoster ophthalmicus: Clinical features and outcomes case report and systematic review.

BACKGROUND: This study aimed to describe the clinical features of patients with orbital apex syndrome (OAS) as a complication of herpes zoster ophthalmicus (HZO) and to identify factors associated with poor visual acuity outcomes. METHODS: We performed a systematic review and retrospective analysis of the clinical characteristics and outcomes of patients with OAS secondary to HZO reported in the literature over 42 years (1978-2020). RESULTS: We analysed 21 cases, 20 of which were identified in the literature, together with our patient. Their median age was 65 years, with equal involvement in both sexes. The median onset of OAS due to HZO was 10 days (range 1-28 days). The median time of treatment initiation was five days (range 1-21 days). All patients presented with reduced visual acuity, complete ophthalmoplegia, and ptosis. Most patients (17/21, 80.95%) were treated with systemic antiviral and corticosteroid therapy. Three (3/21, 14.29%) patients were immunocompromised. Recovery for ophthalmoplegia (19/21, 90.48%) and ptosis (16/21, 76.19%) was good. Half of the patients (9/18, 50%) showed poor vision recovery. Starting treatment more than 72 h after HZO onset (p = 0.045) was more likely to cause poor vision recovery. CONCLUSION: OAS is a rare, serious, and potentially late complication of HZO and continued observation up to and perhaps beyond four weeks is justifiable, if not encouraged. Early initiation of treatment with systemic antiviral and/or corticosteroids within 72 h of onset of HZO appears beneficial for the recovery of visual acuity.

Lacrimal gland botulinum toxin injection for epiphora management.

PURPOSE: Epiphora remains an often difficult to manage ocular complaint for ophthalmologists in all subspecialties. This review seeks to examine the safety and efficacy of botulinum toxin injection for management of chronic epiphora. METHODS: The authors conducted a Pubmed search for studies on the use of lacrimal and transplanted salivary gland botulinum toxin injections for the management of epiphora within the past 20 years. Studies included had a minimum of four glandular injections. RESULTS: The authors identified 14 studies and divided them by indication for injection; either functional epiphora, non-functional epiphora, or mixed studies. Seven studies examined injections for cases of functional epiphora, four for non-functional epiphora, and four for mixed cases. The number of glandular injections reported ranged from 4 to 65. Side effects reported were limited to diplopia, eyelid or lacrimal gland hematoma, papillary conjunctivitis, dry eye, ptosis, and bleeding. CONCLUSIONS: Glandular botulinum toxin injection should be considered as a viable treatment strategy for both functional and nonfunctional epiphora. From the studies reviewed, botulinum toxin injection was shown to be effective in both children and adults. Injection can be performed in the outpatient setting, is minimally invasive, technically easy to administer, has a favorable side effect profile, and good efficacy. Furthermore, repeat injections can be performed with similar efficacy.

Case Report: Treatment of Myasthenic Ptosis with Topical Ocular Oxymetazoline.

SIGNIFICANCE: Ptosis is often the hallmark finding in ocular and general myasthenia gravis. Reduction of ptosis has been achieved with oral and topical ocular medications. However, these medications can result in systemic and ocular adverse effects. A novel eye drop seems to be effective in reducing ptosis while minimizing adverse effects. PURPOSE: This case report aimed to demonstrate the efficacy of topical oxymetazoline hydrochloride 0.1%, an α-adrenergic agonist, in temporary elimination of ptosis associated with myasthenia gravis. CASE REPORT: A 68-year-old woman with a history of myasthenia gravis and long-standing ptosis in the right eye presented to improve the asymmetrical appearance of her eyelids. One drop of oxymetazoline hydrochloride 0.1% was instilled in the right eye of the patient. Within 2 hours, the ptosis was eliminated, the margin-reflex distance 1 increased by 2.0 mm, and the superior visual field measured by a superior 36-point screening test increased by 15 points. The effect lasted for at least 7 hours. Of note, there was a decrease in elevation of the contralateral nonptotic eyelid that did not receive a drop of oxymetazoline, which might occur only in myasthenia gravis. Further evaluation is warranted. CONCLUSIONS: Oxymetazoline 0.1% is effective in reducing and potentially eliminating ptosis related to ocular myasthenia gravis for up to 7 hours.

Diagnostic yields and clinical features of ocular myasthenia gravis.

ABSTRACT: To investigate clinical features and diagnosis process of ocular myasthenia gravis (OMG) in ophthalmology department.A total of 36 patients with ptosis or diplopia who had follow-up for at least 3 months between March 2016 and December 2019 were included in this study. Clinical symptoms of patients and the test results were analyzed. According to the positivity of serologic test, these patients were divided into 2 groups (confirmed OMG and possible OMG with relief of symptoms after antimyasthenic treatment) for comparison.Ptosis was present in 12 (33.33%) patients, diplopia was present in 14 (38.89%) patients, and both ptosis and diplopia were present in 10 (27.78%) patients. Acetylcholine receptor auto-antibody (AchR Ab) was positive in 14 (38.89%) of 36 patients and ice test was positive in 15 (71.43%) of 21 patients with ptosis. Unequivocal response to pyridostigmine was observed in 31 (86.11%) patients. For seropositive cases, AchR Ab titer was significantly higher in the group with 2 clinical symptoms than that in the 1 clinical symptom (P = .011).This study presents the usefulness and diagnostic validity of antimyasthenic treatment for OMG, especially seronegative OMG, with detailed symptom analysis.

Ophthalmologic manifestations in myasthenia gravis: presentation and prognosis.

We investigated the ophthalmologic manifestations and factors that influence outcomes in patients with myasthenia gravis (MG). We retrospectively analyzed the prevalence of neuro-ophthalmologic findings and clinical and outcome measures of 100 consecutive patients (53 males, 47 females), aged 55.7 ± 17.5 (range 15-85) years with an established diagnosis of MG. Forty-eight patients had purely ocular symptoms at the onset of disease (OMG) and 52 patients presented with generalized symptoms (GMG). Overall, 21 patients presented with extraocular muscle (EOM) weakness. Bilateral EOM weakness was seen in 12 patients, and unilateral EOM weakness was seen in nine patients. Diplopia responded partially to immunosuppressive treatments in 60% of patients with ophthalmoparesis. Twenty-five (52.1%) patients with ocular-onset MG converted to secondary GMG at a mean time of 14.5 months. Patients who developed secondary GMG were younger and had an earlier age of disease onset when compared with patients with pure OMG (p < 0.05). Patients with secondary GMG presented more frequently with ptosis and diplopia (72% vs. 28%) compared with patients with pure ocular MG who presented more frequently with isolated ptosis (66.7% vs. 33.3%) (p = 0.02). Remission and minimal manifestation status were achieved in 50 (79.3%) of all patients with a clinical follow-up ≥ 3 years. Poor outcome was associated with the presence of thymoma (p < 0.05). Myasthenic ophthalmoparesis is bilateral and heterogeneous and partly responds to treatment with immunotherapy. Younger patients with ptosis and diplopia at disease onset had an increased risk of secondary GMG. The presence of thymoma increases the risk for poor prognosis.

Juvenile Generalized Myasthenia Gravis: Presented as Unilateral Blepharoptosis and Successfully Managed with Pulse Intravenous Methylprednisolone.

Myasthenia Gravis is a rare autoimmune disorder of childhood and this is rarer in South Asia. We present a pre-pubertal 7 year old female child of seropositive Generalized Juvenile Myasthenia Gravis. She presented with unilateral blepharoptosis and later generalized symptoms appeared. Ice-pack test, Neostigmine challenge test and acetylcholine receptor antibody test were positive. Serum muscle specific tyrosine kinase antibody test was normal. She did not have thymic abnormalities. She did not respond to high dose (26 mg/kg/day) of Pyridostigmine and oral Prednisolone (2 mg/kg/day), but was successfully treated with a combination of pulse intravenous Methylprednisolone (30 mg/kg once a month for 6 months) and daily doses of oral Prednisolone (2 mg/kg/day) along with Pyridostigmine without significant side effects. This combination can be considered a potential inexpensive treatment for Juvenile Myasthenia Gravis in a resource limited area where other immunosuppressive treatments such as intravenous immunoglobulin is expensive and unaffordable.

Disseminated lymphoblastic lymphoma revealed by an isolated third cranial nerve palsy.

Dysfunction of the third cranial nerve can be provoked by a number of different conditions. An isolated cranial neuropathy as a first clinical sign of a non-Hodgkin lymphoma is very infrequent. We represent here an atypical case of lymphoblastic lymphoma revealed by an isolated third cranial nerve palsy. The patient was managed by alternating cycles of cyclophosphamide, vincristine, and prednisone. She made a full recovery with a complete resolution of the symptomatology.

"Mummy, my eyelids are heavy": A case series of juvenile myasthenia gravis.

PURPOSE: To report three cases of juvenile myasthenia gravis aged between 18 and 24 months with ocular symptoms as their first presentation. METHOD: A case series. RESULTS: We present a case series of juvenile myasthenia gravis in a tertiary centre in Malaysia. Two of the three cases consist of a pair of twins who presented with ptosis of bilateral eyes; the first twin presented 4 months later than the second twin. These two cases were positive for anti-acetylcholine receptor antibodies and had generalized myasthenia gravis, whereas the other case was negative for receptor antibodies and was purely ocular myasthenia gravis. CONCLUSION: Juvenile myasthenia gravis is relatively rare in toddlers. Early diagnosis and commencement of treatment is important to slow the progression of the disease and avoiding life-threatening events.

Effect of Initial Prednisone Dosing on Ocular Myasthenia Gravis Control.

BACKGROUND: Ocular myasthenia is an autoimmune condition that results in double vision or ptosis. It often requires treatment with prednisone for immunosuppression, but there have been no prospective trials to help clinicians determine ideal dosing. METHODS: This was a retrospective study comparing myasthenia symptom control at 1 month between patients treated with a maximum daily equivalent dose of prednisone less than 20 mg (low-dose group) vs 20 mg or more (medium-dose group). RESULTS: Thirty-nine patients were identified: 19 patients in the low-dose group with mean maximum daily dose of 10 mg and 20 patients in the medium-dose group with a mean maximum daily dose of 29 mg. The low-dose group had 75% controlled or significantly improved at 1 month, and the medium-dose group had 74% controlled or significantly improved at 1 month, P = 0.94. The overall seropositivity rate was 64%, with 84% of the antibody-positive group being controlled or significantly improved at 1 month and 57% of the antibody-negative group being controlled or significantly improved at 1 month, P = 0.07, and no difference in prednisone dosing between the 2 groups. CONCLUSION: Based on the results of this small retrospective study, it seems initial treatment for ocular myasthenia gravis with a mean maximum daily prednisone dose of 10 mg is similarly effective compared with mean maximum daily dose of 29 mg for control at 1 month.