Case Of Ocular Myasthenia Gravis Research Articles

E015 A case of ocular myasthenia gravis seen by rheumatology as suspected mononeuritis multiplex in an adult male shortly after Pfizer-BioNTech SARS-CoV-2 vaccination

Abstract Background/Aims Myasthenia gravis (MG) is an antibody-mediated autoimmune disease targeting proteins at the postsynaptic membrane of the neuromuscular junction. MG is thought to occur in genetically susceptible individuals following an environmental trigger. SARS-CoV-2 infection has been associated with new-onset autoimmune disease, new-onset MG, and exacerbations of pre-existing MG, with molecular mimicry between SARS-CoV-2 epitopes and autoantigen-induced autoreactivity thought to be part of the underlying mechanism. We report a case of new-onset ocular MG following first dose Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination which was referred to rheumatology as suspected mononeuritis multiplex. Methods A 53-year-old man of East Asian ethnicity presented to the emergency department (ED) with sudden onset diplopia and left lateral gaze restriction 7 days after receiving his first dose of the Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination. He had longstanding myopia and dry eyes but no other medical history, no regular medications or significant family history. He was a current smoker, with a 50-pack year history. He did not drink alcohol or use any recreational drugs. He was found to have an isolated left VI cranial nerve (CN) palsy with an otherwise normal ocular and physical examination. Blood tests were unremarkable apart from raised cholesterol, and he was discharged with a suspected self-limiting microvascular CN lesion. Three weeks later he presented to ED with worsening diplopia, increasingly restricted eye movements, headache, nausea, vomiting and blurred vision. Ophthalmology assessment noted new right sided CN III and VI palsy, persistent left CN VI palsy, and vertical diplopia in all fields of gaze. Neurological and physical examination were normal. Bloods including an autoimmune screen were unremarkable. SARS-CoV-2 Spike antibodies were positive consistent with SARS-CoV-2 vaccination but not infection. Intracranial and thoracic imaging were unremarkable. He was referred to and seen by both rheumatology and neurology as a case of suspected mononeuritis multiplex. Results A diagnosis of ocular MG was confirmed with positive serum acetylcholine receptor antibodies, and he was started on prednisolone, and pyridostigmine to good effect. Daily forced vital capacity (FVC) showed no respiratory muscle involvement, and nerve conduction studies and electromyography were normal, excluding secondary generalisation. Conclusion A review of the literature found 14 reported cases of new-onset MG all within 4 weeks following SARS-CoV-2 vaccine. Whilst these cases provide interesting insights into the pathogenesis of autoimmune conditions such as MG, they are not epidemiological studies to inform vaccine safety. Ultimately, current evidence suggests that the risks of SARS-COV-2 infection outweigh the risk of vaccine-related adverse events, therefore we suggest clinicians should be aware of potential new-onset autoimmune conditions, but support the safety of SARS-COV2 vaccination. Further, research into possible immunological mechanisms behind this phenomenon, including identifying potential epitopes inducing molecular mimicry, could help establish the likelihood of a causative link. Disclosure A. Storrie: None. R.M. Hum: None. J. Lilleker: None. P. Ho: None.

Ocular Myasthenia: Clinical Course and the Diagnostic Utility of Assaying Acetylcholine Receptor Antibodies

ABSTRACT Myasthenia gravis (MG) is an autoimmune disease that causes neuromuscular junction transmission defect and has a predilection for the with neuromuscular junction transmission defect and predilection for extra-ocular and eyelid muscles. Most cases of ocular MG (OMG) convert later to generalised MG (GMG). Assaying acetylcholine receptor antibodies (AchRA) has been used to diagnose MG, but the reported sensitivity in OMG is lower (50%) than in GMG. We report the clinical course and the diagnostic yield of assaying AchRA in a Kuwaiti cohort of patients with OMG. We carried out a retrospective review of 47 patients diagnosed with OMG who were tested for AchRA. Ancillary tests included the ice test, single-fibre electromyography (SFMEG), and repetitive nerve stimulation electromyography (RNS). Progression to GMG occurred in 51% of OMG patients with a mean time to progression of 12.1 months (range 4 to 20 months). AchRAs were positive in 46 of 47 cases (98%), while SFEMG was positive in 31 of 34 cases (91.1%). Older age (44.25 years versus 38 years, p < .05) and higher AchRA titre (2.0 nmol/L versus 1.27 nmol/L, p < .05) were significantly associated with conversion to GMG. We have found a high rate of AchRA seropositivity in relatively younger subjects of OMG. Higher AchRA titres and older age were associated with conversion to GMG, usually within the first 2 years.

Factors Affecting Generalization of Ocular Myasthenia Gravis in Palembang

Introduction. Ocular myasthenia gravis (OMG) is an autoimmune disease which is characterized by weakness of extraocular muscles, levator palpebrae and orbicularis oculi, resulting in ptosis and binocular diplopia. Nearly all patients present with eyelid and extra ocular muscles involvement. Approximately 30% to 80% of patients with OMG experience a conversion to generalized myasthenia gravis (GMG) within 2 years. There are not only have ptosis and diplopia but also limb weakness,bulbar symptoms, or even respiratory failure. This study was aimed to observe the clinical features of OMG to GMG and risk factors and median time to conversion of OMG to GMG of myasthenia gravis patients in Mohammad Hoesin General Hospital Palembang.&#x0D; Method. This study is a cohort retrospective study and the data were collected from the medical records of 91 patients who were registered as myasthenia gravis patients during September 2018 to March 2020. Sosiodemographic and clinical characteristics, including onset of OMG to GMG, history of smoking, presence of thymic abnormalities, and medications received were reviewed retrospectively.&#x0D; Result. A total of 91 OMG patients were observed in this study with 32 (35,2%) patients converted from ocular myastenia gravis to general myastenia gravis. Median conversion time to GMG was 34 months. Risk factor for convertion cases of OMG to MGG was receiving immunosupressive agents (Risk: 14.7, 95% CI 4.83, 44.7), thymus hyperplasia (Risk: 3.36, CI 95% 0.33, 33.6), Female (Risk: 2.41, 95% CI 0.94, 6.17), Smoking (Risk: 1.56, 95% CI 0.31, 7.81).&#x0D; Conclusion. Ptosis was the definitive sign for OMG in this study, with all patients had ptosis, thus it needs the colaboration from neuroophthalmologist and neurologist to diagnose and manage this case. Most of converted case was female and those who receive an immunosupressive agent therapy.

External ophthalmoparesis as part of generalised myasthenia gravis in a dog: are there more similarities to the human counterpart than originally thought?

AbstractMyasthenia gravis in dogs is an autoimmune disease affecting the neuromuscular junction and resembling its human counterpart. Classification of the disease in humans includes an ocular and generalised form. Isolated ocular myasthenia gravis is confined to the extraocular muscles which most commonly results in ptosis, due to an impaired levator palpebrae superioris muscle responsible for elevating the upper eyelid, and diplopia (double vision) due to weakness of the extraocular bulbar muscles. Isolated ocular myasthenia gravis frequently becomes generalized over time or can present as part of generalised myasthenia gravis. Myasthenia gravis in dogs is classified by severity and distribution of clinical signs, as well as, serology and thymic status. However, it does not include the extraocular muscles. This is the first description of a case of ocular myasthenia gravis in a dog, progressing to generalised myasthenia gravis as seen in humans.

Pediatric ocular myasthenia gravis: Case report and literature review

Objective: The objective was to describe a case of ocular myasthenia gravis (MG) in a pediatric patient. Design: This is a descriptive, retrospective study and case report. Methodology: For the purpose of the case report, we searched and analyzed the literature referring to the diagnosis and treatment of ocular MG (OMG) in children. Description: A previously healthy 5-year-old girl presented to the emergency room because of sudden-onset ptosis of the right upper lid without other symptoms. Her examination demonstrated fluctuating ptosis and limitation to downgaze in the right eye. Because of the fluctuation of the ptosis, OMG was suggested, and the patient was tested with ice-pack test and neostigmine test with positive results. Treatment with pyridostigmine was initiated with a good response. Discussion: OMG has a very low incidence in pediatric patients. It is a disorder characterized by impairing neurotransmission at the neuromuscular junction that generates extraocular muscle weakness. It is important that ophthalmologists have knowledge about this disease because it is a differential diagnosis when there is a patient who has ptosis, strabismus, or limitation of movements. There are no standard diagnostic criteria for this disease; the diagnosis is based on clinical presentation and pharmacologic, serologic, and electrophysiologic tests. The treatment is based on steroids and anticholinesterase drugs. Some patients will require plasmapheresis or immunomodulators.

A 49-Year-Old Man with Combined Myasthenia Gravis, Autoimmune Thyroiditis and IgG4 Orbitopathy

Background: Patients with autoimmune thyroid disease have higher risk of myasthenia gravis, especially the ocular form of myasthenia gravis. IgG4-related disease and autoimmune thyroiditis have been reported to coexist; and IgG4- related thyroiditis pertain higher autoantibodies when compared with non-IgG4 thyroiditis. However, combined occurrence of myasthenia gravis, autoimmune thyroiditis, and IgG4-related disease has not been reported before. Methods: Here we report a case of ocular myasthenia gravis combined with autoimmune thyroiditis and elevated serum IgG4 that initially deteriorated to generalized myasthenia gravis after 3 days of intravenous (IV) pulse methylprednisolone treatment, but was rescued after plasma exchange therapy. The patient was placed on oral prednisolone, azathioprine, pentoxifylline, hydroxychloroquine, but the ocular symptoms relapsed occasionally when he felt tired. Another eight sessions of weekly IV pulse methylprednisolone were administered during outpatient clinic follow-ups. Findings: There was no more deterioration to generalized myasthenia gravis, and his ocular symptoms fully recovered after the treatment. He finally underwent thoracoscopic thymectomy, and pathology shows atrophic thymus. All immunosuppressants were tapered off after surgery, and he remained symptomfree on the last follow-up, about half a year after surgery. Conclusion: Since thyroid associated orbitopathy and IgG4-related orbitopathy respond well to steroid treatment, initial deterioration of concomittent ocular myasthenia gravis to generalized form after IV pulse steroid therapy can be rescued by plasmapheresis, and the follow-up IV pulse steroid therapy should not be precluded as the definitive treatment in this situation. Finally, all immunosuppressants were successfully discontinued and he remained symptomfree after surgical removal of the atrophic thymus.

Ocular myasthenia gravis in a person with tetraplegia presenting challenges in diagnosis and management

We report the first case of ocular myasthenia gravis (OMG) in a patient with complete tetraplegia, highlighting diagnostic and management challenges. Spinal multidisciplinary rural clinic and specialised inpatient Spinal Cord Injury Unit, NSW, Australia. A 61-year-old man with established C5 AIS A tetraplegia, presented with sudden onset of diplopia and bilateral ptosis, later diagnosed as OMG, in context of other complex co-morbidities, including a cervical cord syrinx, obstructive sleep apnoea and labile blood pressure. Clinical findings were consistent with fluctuating bilateral partial third and sixth nerve palsies. Acetylcholine receptor antibodies were negative, but electromyography demonstrated muscle fatigue. The ocular signs responded well to pyridostigmine. Medications taken before diagnosis, including solifenacin for neurogenic bladder overactivity, were ceased to avoid attenuating the anti-cholinesterase effect. However, the unopposed anti-cholinesterase activity led to frequent and painful abdominal spasms, associated with uncontrolled detrusor hyperreflexia and worsening autonomic dysreflexia (AD). A trans-vesical phenol block to treat this provided only short-lasting benefit. Pyridostigmine was ceased to avoid provoking his abdominal spasms and his regular medications were recommenced. It was decided that the most appropriate treatment for his distressing diplopia was an eye patch. After discharge home, he continued to experience problems with recurrent urinary tract infections, abdominal spasms, episodic postural hypotension and AD. After 5 months, the patient died from an acute myocardial infarction. This case report contributes new knowledge about the rare presentation of OMG in a person with chronic tetraplegia.

Ocular myasthenia gravis: Side effect of urografin

Ocular myasthenia gravis is an autoimmune disorder of the neuromuscular junction. Diplopia and ptosis are common symptoms at the onset of ocular myasthenia gravis. It may occur due to the antibodies developed against various drugs. We are reporting a case of ocular myasthenia gravis which was developed in a patient post angiography which may be due to antibody developed against the dye used in angiography.

Sixty cases of ocular myasthenia gravis treated with acupuncture and moxibustion at extra-meridian and collateral

ObjectiveTo explore effective therapeutic method for the treatment of ocular myasthenia gravis. MethodsSixty cases of ocular myasthenia gravis were selected. The acupuncture was applied at Dàzhuī ▪ GV 14), B▪ihuì (▪) GV 20), Shàngxīng (▪ GV 23) of Governor Vessel, and its crossing points with Yinqiao and Yangqiao Meridian, such as Jīngmíng (▪ BL 1), shēnmài (▪ BL 62), Dìcāng (▪ ST 4), Jūliáo (▪ ST 3), Chéngqì (▪ ST 1), etc. Besides, ginger-partition moxibustion was applied at Yángbái (▪ GB 14) and Sìbái (▪ ST 2). The plum-blossom needle was applied at upper limb distribution of lung channel of hand taiyin, heart channel of hand shaoyin and pericardium channel of hand jueyin. The patients’ clinical symptoms, potential degradation percentages of repetitive nerve stimulation (RNS) in low frequency were observed. Results8 cases were clinically cured, 20 cases were basically cured, 12 cases were remarkably effective, 15 cases were effective and 5 cases were failed. The total effective rate was 91.7%. Compared with before the treatment, the potential degradation percentages of repetitive nerve stimulation in low frequency were lowered [(19.44±6.28)% vs (8.78±3.12)%, (P<0.05)]. ConclusionAcupuncture and moxibustion at extra-meridian and collateral for ocular myasthenia gravis could achieve positive efficacy.

Different Characteristic Phenotypes According to Antibody in Myasthenia Gravis

To find the characteristic phenotypes of 3 different types of myasthenia gravis (MG). The clinical and electrophysiological features among 15 cases of muscle-specific kinase antibody positive (MuSK Ab+) MG, 59 cases of double seronegative (DSN) MG, and 161 cases of acetylcholine receptor antibody (AChR Ab)+ MG in the University of Alabama at Birmingham were compared. AChR Ab was positive in 69% of cases and MuSK Ab in 6% of cases. MuSK Ab+ MG was more common (14%) in African Americans compared with whites (4%). AChR Ab+ MG is characterized by male predominance, later onset, a fewer cases of ocular MG, and a higher association with thymoma. DSN-MG is characterized by a greater prevalence of ocular MG, milder forms of MG with less number of crisis, and fewer abnormalities in the repetitive nerve stimulation test. MuSK Ab+ MG is characterized by younger age at onset, severe and bulbar forms of MG, predominant faciobulbar neck weakness, and a poor response to edrophonium, anticholinesterase, and intravenous immunoglobulin. Long-term outcome showed no difference among 3 types of MG. AChR Ab+ MG and DSN-MG are similar, with the exception of less severity in the latter. MuSK Ab+ MG has distinct clinical and electrophysiological features.

An Ocular Form of Myasthenia Gravis with a High Titer of Anti-MuSK Antibodies during a Long-term Follow-up

We herein report a case of ocular myasthenia gravis (MG) that was highly positive for anti-muscle-specific tyrosine kinase (MuSK) antibodies. The examined patient exhibited bilateral ptosis and lateral gaze palsy without any generalized symptoms and was diagnosed with ocular MG with anti-MuSK antibodies. She responded to treatment with prednisolone and immunosuppressants and experienced only ocular symptoms for four years and eight months after onset. Ocular MG with anti-MuSK antibodies lasting for a long term has rarely been described. Our findings suggest that it may be reasonable to test for the presence of anti-MuSK antibodies in patients who present with external ophthalmoplegia.

A case of ocular myasthenia gravis and Miller-Fisher syndrome

A40-year-old Chinese man with myasthenia gravis presented to the emergency department with worsening of ocular symptoms and the authors were asked for an opinion. He first presented 7 years ago with diplopia and ptosis that were responsive to edrophonium. His acetylcholine receptor antibody titre was elevated at 0.8 optical density unit (ODU) (laboratory reference &lt;0.43 ODU), although repetitive nerve stimulation study did not produce a significant motor potential decrement. He improved with regular pyridostigmine (40 mg three times a day) but there was still mild ptosis and limited abduction of his left eye. His condition did not change in the last few years. For the current presentation, he had progressive deterioration of ocular movements over 1 week. Physical examination showed complete ophthalmoplegia bilaterally, partial ptosis of the left eye and absent pupillary light reflex in the right. Intravenous edrophonium 10 mg produced minimal improvement in right eye abduction. On further questioning, he also complained of numbness of his extremities, difficulties in fine hand functions, and unsteadiness while walking. He recalled a flu-like illness 2 weeks before the neurological symptoms. All his tendon reflexes were unobtainable and he had an ataxic gait. There was no demonstrable limb or facial weakness. Cerebrospinal fluid analysis showed increased protein concentration at 1.33 g/litre (reference range 0.12–0.6 g/litre) with total cell count of &lt;1×106/litre. Anti-GQ1b antibody level was elevated at 43 enzyme immunoassay (EIA) Units (reference range &lt;10 EIA). Nerve conduction studies only showed marginal reduction of F-wave persistence in the upper limbs. Magnetic resonance imaging of his brain revealed no abnormalities. Miller-Fisher syndrome (MFS) was diagnosed. He was treated with three sessions of plasmapheresis. His symptoms started to improve over the next week and he subsequently made a full clinical recovery. Moreover, he was completely free from his myasthenia gravis-related ophthalmoplegia in the following 2 months. Pyridostigmine was later recommenced when his baseline symptoms returned.

Thyrotoxic myopathy and myasthenia gravis: a case report.

ALTHOUGH muscular weakness is characteristic of hyperthyroidism, L the simultaneous occurrence of this condition and myasthenia gravis in the same patient is relatively rare (1–4). However, 6 per cent of patients at the Mayo Clinic with myasthenia gravis either had associated hyperthyroidism or had had this disease before the onset of myasthenia gravis (5). In the Johns Hopkins series of 270 cases of ocular myasthenia gravis (220 generalized and 50 localized), 12 showed some abnormality of the thyroid gland; and of these, 8 had hyperthyroidism (6). The myasthenia of chronic thyrotoxic myopathy superficially resembles myasthenia gravis and the two conditions may be easily confused, particularly when exophthalmic ophthalmoplegia is present (4). In 1908, Rennie (7, 8) published the first report of hyperthyroidism and associated myasthenia gravis. In 1946, Cohen (4) suggested that perhaps this was an instance of exophthalmic ophthalmoplegia and not of the combined diseases. Since then, several cases of this d...