Detecting myasthenia gravis as a cause of unclear dysphagia with an endoscopic tensilon test

Aims: The flexible endoscopic evaluation of swallowing-tensilon test (FTT) was developed to diagnose myasthenia gravis (MG) in patients with unclear pharyngeal dysphagia. The purpose of this study was to determine sensitivity and specificity of the FTT and compare its diagnostic validity with that of other diagnostic markers. Methods: In this single-centre pragmatic clinical cohort study, a total of 100 patients with unclear pharyngeal dysphagia were eligible to undergo FTT. All patients were subjected to FTT and subsequently followed up clinically. FTT was considered positive if a significant improvement of pharyngeal swallowing function could be objectified endoscopically upon administration of edrophonium chloride. In addition, repetitive nerve stimulation test and serum MG antibody analysis were conducted. Results: All subjects (mean age 62.5 ± 14.1 years, female 33) underwent FTT without any complications. According to the results of the diagnostic procedures and based on long-term clinical follow-up for at least 3 years, 51 patients were finally diagnosed with MG. The sensitivity and specificity for the FTT was 88.2% and 95.9%, respectively. Application of the Cochran’s Q test showed statistically significant heterogeneity among the diagnostic tests, with results indicating FTT performance to be more accurate than the repetitive nerve stimulation results ( p < 0.001) and comparable with serum antibody tests ( p > 0.99). Conclusion: FTT has excellent clinical properties to be used routinely in the assessment of dysphagia with isolated or predominant pharyngeal muscle involvement allowing rapid and accurate diagnosis of MG.

Case 17

Myasthenia gravis is a relatively common neuromuscular junction disorder. The diagnosis of myasthenia gravis may be challenging particularly in seronegative patients. This case starts by discussing the diagnostic modalities available to confirm the diagnosis of myasthenia gravis including serum antibodies, the Tensilon test, and the ice pack test. After a detailed discussion of the physiology of neuromuscular transmission, the case emphasizes the role of electrodiagnostic studies in the diagnosis of myasthenic gravis. This includes detailed findings on repetitive nerve stimulation recording distal and proximal muscles as well as single-fiber electromyography jitter studies. Finally, the diagnostic sensitivity of the available tests in myasthenia gravis is compared and a suggested electrodiagnostic strategy for patients with suspected myasthenic gravis is outlined.

Isolated dysphagia as initial sign of anti-IgLON5 syndrome

Objective:To report on dysphagia as initial sign in a case of anti-IgLON5 syndrome and provide an overview of the current literature.Methods:The diagnostic workup included cerebral MRI, fiber optic endoscopic evaluation of swallowing (FEES) with the FEES tensilon test, a videofluoroscopic swallowing study, evoked potentials and peripheral nerve conduction studies, polysomnography, lumbar puncture, and screening for neural autoantibodies. A systematic review of all published cases of IgLON5 syndrome is provided.Results:We report a case of anti-IgLON5 syndrome presenting with slowly progressive neurogenic dysphagia. FEES revealed severe neurogenic dysphagia and bilateral palsy of the vocal cords. Autoantibody screening was positive for IgLON5 IgG (+++, 1:1,000) serum levels but no other known neural autoantibody. Polysomnography was highly suggestive of non-REM parasomnia. Symptoms were partially responsive to immunotherapy.Conclusions:Slowly progressive neurogenic dysphagia may occur as initial sign of anti-IgLON5 syndrome highlighting another clinical presentation of this rare disease.

Specific features of anesthesia in patients with myasthenia gravis

Introduction. Myasthenia gravis is an autoimmune disease caused by antibodies leading to the destruction of nicotinic acetylcholine receptors on the neuromuscular junction. It is characterized by muscle weakness that gets aggravated with physical activity and improves at rest. Myasthenia Gravis Foundation of America made the clinical classification of Myasthenia gravis which is still in use today. ?Tensilon test? is still the gold standard for the diagnosis of Myasthenia gravis. In addition to this test repeated muscular stimulation can be used as well as the analysis of specific autoantibodies. Treatment of Myasthenia Gravis. In conservative treatment of Mysthenia gravis anticholinesterases, immunosuppressants and plasmapheresis can be used. If conservative treatment does not lead to the desired remission, surgical treatment is indicated. The most accepted indication for thymectomy is the presence of thymoma with generalized form of Myasthenia gravis in adults. How to Distinguish Myasthenic From Cholinergic Crisis. The following is important to make a difference between these two crises: knowledge of the events that preceded the crisis, the size of pupils as well as the presence of muscarinic signs and tensilon test. Specific Features of Anesthesia in Patients with Myasthenia Gravis. Mechanism of the disease development is the reason for the increased sensitivity or resistance of these patients to certain types of drugs used in anesthesia. Protocol of Perioperative Anesthesia in Patients with Myasthenia Gravis. Based on 35 years of experience in the surgical treatment of patients with Myasthenia gravis anesthesiologists at the Department of Thoracic Surgery, Institute for Pulmonary Diseases of Vojvodina, made the protocol of anesthesia and perioperative treatment for these patients. Conclusion. Anesthesiologists may have to deal with a patient with myasthenia gravis in different types of surgical interventions. The protocol for anesthesia and perioperative management of these patients herewith presented may greatly help them in their clinical practice.

Ice-on-Eyes Test in the Diagnosis of Myasthenia Gravis

Myasthenia gravis is the most frequent autoimmune neuromuscular transmission disorder with incidence of 2-20 patients per million. Its pathophysiology is autoimmune, with acetylcholine receptor auto antibodies damaging the post-synaptic fold at the muscle membrane. The diagnostic confirmation of myasthenia gravis is often challenging. Ice-oneyes test can be used to diagnose this disease for its simplicity, safety and cost-effectiveness. Here we report a case of myasthenia gravis in Enam Medical College Hospital, Savar, Dhaka where ice-on-eyes test was done with improvement of ptosis of the patient. Aim of this case report is to make aware our physicians to apply this simple bed side test instead of common traditional edrophonium (tensilon test) test for confirmation of the diagnosis of ocular myasthenia gravis. DOI: http://dx.doi.org/10.3329/jemc.v2i2.12845 J Enam Med Col 2012; 2(2): 92-93

Dandy-Walker syndrome in adult mimicking myasthenia gravis

The Dandy-Walker syndrome (DWS) is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-year-old woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed.