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ocular myasthenia gravis

Xin Huang, Yingkai Li, Huiyu Feng, Pei Chen, Weibin Liu
Objectives: To describe the clinical profile, clinical outcomes and factors that may affect the outcome of juvenile myasthenia gravis (JMG) patients in southern China. Methods: We reviewed information relating to JMG patients treated and evaluated at the First Affiliated Hospital, Sun Yat-sen University, between 1998 and 2015. The study involved 327 JMG patients who had been followed up for ≥1 year. Results: Overall, 77.4% patients showed initial symptoms in the prepubertal period (<12 years)...
2018: Frontiers in Neurology
Kee Hong Park, Patrick Waters, Mark Woodhall, Bethan Lang, Thomas Smith, Jung-Joon Sung, Kwang-Kuk Kim, Young-Min Lim, Jee-Eun Kim, Byung-Jo Kim, Jin-Sung Park, Jeong-Geon Lim, Dae-Seong Kim, Ohyun Kwon, Eun Hee Sohn, Jong Seok Bae, Byung-Nam Yoon, Nam-Hee Kim, Suk-Won Ahn, Jeeyoung Oh, Hyung Jun Park, Kyong Jin Shin, Yoon-Ho Hong
Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA)...
2018: PloS One
Nermin Gorkem Sirin, Elif Kocasoy Orhan, Hacer Durmus, Feza Deymeer, Mehmet Baris Baslo
INTRODUCTION: The aim of this study was to define the diagnostic accuracy of concentric needle (CN)-jitter in newly diagnosed myasthenia gravis (MG) patients and to compare CN-jitter with repetitive nerve stimulation. METHODS: In 30 MG patients, repetitive nerve stimulation in 4 muscles (orbicularis oculi, nasalis, trapezius and abductor digiti minimi) and CN-jitter of extensor digitorum (ED) and frontalis muscles were evaluated. RESULTS: Twenty-eight of 30 patients (93%) had high jitter in at least one muscle...
February 25, 2018: Neurophysiologie Clinique, Clinical Neurophysiology
Enrique M San Norberto, Irene García-Saiz, Diana Gutiérrez, Liliana Domingos, Carlos Vaquero
The non-VKA oral anticoagulant rivaroxaban is indicated in prevention and treatment of venous thromboembolism. A 60-year-old male patient complained of bilateral ptosis after administration of rivaroxaban for deep vein thrombosis. Myasthenia gravis was confirmed by positive serum antiacetylcholine receptor antibody test. No mediastinal thymoma was found. The ocular myasthenia reversed after discontinuing rivaroxaban treatment. Nevertheless, ptosis recurred and chronic oral pyridostigmine bromide treatment was necessary...
February 15, 2018: Annals of Vascular Surgery
Florencia Aguirre, Andres M Villa
BACKGROUND: We investigated the prognosis of patients with myasthenia gravis (MG) initially presenting with only ocular symptoms in an Argentinian population. SUMMARY: We performed a retrospective analysis of 61 patients with MG with pure ocular involvement at onset. Generalized MG (gMG) developed in 73.7% of patients, while the rest only exhibited ocular symptoms throughout the course of the disease (ocular MG [oMG]). The AChR antibody (AChR-ab) was found in 81...
February 9, 2018: European Neurology
Christopher Tran, Vera Bril, Hans D Katzberg, Carolina Barnett
INTRODUCTION: Patients with myasthenia gravis often experience fatigue, but its effect on quality of life (QoL) is underestimated, and fatigue is rarely measured in clinical trials. METHODS: Two hundred fifty-seven myasthenic patients completed the Neuro-QoL-Fatigue and measures of disease severity and QoL. We studied the relationship between fatigue and clinical and demographic variables. Finally, we studied the responsiveness of the Neuro-QoL-Fatigue in 95 patients receiving treatments for myasthenia and estimated the minimal important difference (MID)...
January 17, 2018: Muscle & Nerve
Stephen Bacchi, Philippe Kramer, Colin Chalk
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which a clinical diagnosis may be confirmed with serological testing. The most common autoantibodies used to support a diagnosis of MG are anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies. In cases in which both of these autoantibodies are negative (termed double-seronegative [dSNMG]), other autoantibodies such as low-density lipoprotein receptor-related protein 4 (LRP4) may be used to aid in diagnosis...
January 2018: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Giorgia Sciacca, Ester Reggio, Giovanni Mostile, Alessandra Nicoletti, Filippo Drago, Salvatore Salomone, Mario Zappia
Neostigmine test (NT) is a pharmacological test, demonstrating a clinical improvement in patients affected by myasthenia gravis (MG). We aim to compare clinical evaluation and neurophysiological recordings by concentric-needle single-fiber electromyography (CN-SFEMG) in response to acute administration of neostigmine in ocular and generalized MG patients. Twenty-three MG patients (10 with ocular MG and 13 with generalized MG) were evaluated before and after 90 min neostigmine 0.5-mg administration. Clinical responsiveness was assessed by MG composite (MGC) scale...
January 12, 2018: Neurological Sciences
Sui Hsien Wong
No abstract text is available yet for this article.
December 27, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
Nidain Maneh, Kossivi Apetse, Bénédicte Marèbe Diatewa, Sidik Abou-Bakr Domingo, Aidé Isabelle Agba, Koffi Didier Ayena, Koffi Agnon Balogou, Komi Patrice Balo
Myasthenia gravis is a rare acquired autoimmune pathology causing neuromuscular transmission impairment. Juvenile onset of myasthenia gravis is often characterized by ocular involvement. We report two cases of ocular juvenile myasthenia gravis (JMG) in two siblings. They were two young girls, XA and XB, aged 11 and 9 years, of Malian origin, residing in Togo, born from first-degree of consanguinity presenting to Ophthalmology due to progressive decrease in visual acuity. XA showed visual acuity 8/10 on both eyes while XB showed improvement in visual acuity from 3/10 to 7/10 using a pinhole occluder, suggesting ametropia...
2017: Pan African Medical Journal
Chentao Liu, Xia Wang, Lili Xie, Jing Peng, Liwen Wu, Xiangrong Zheng
To evaluate the efficacy, recurrent risk factors and transferable ratio of treatments with 3 different regiments on children with systematic myasthenia gravis (MG).
 Methods: The data of 104 children with ocular MG from June 2010 to March 2014 were collected from Department of Pediatric Neurology of Xiangya Hospital and they were retrospectively studied. The patients were divided into 3 groups: a methylprednisolone group (n=44), a prednisone group (n=48) and a bromine pyridostigmine group (n=12). Evaluative system from American MG foundation was used to evaluate the efficacy of treatment and the ratio of ocular MG transformed into systematic MG...
November 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Tsubasa Takizawa, Marenori Kojima, Shigeaki Suzuki, Takashi Osada, Satoshi Kitagawa, Jin Nakahara, Shinichi Takahashi, Norihiro Suzuki
RATIONALE: Recently, drug-related myasthenia gravis (MG) has received attention, because the number of reported cases involving MG associated with immune checkpoint inhibitors, a new immunotherapy, is increasing. We present a case involving the new onset of MG, in which the symptoms started shortly after intravesical Bacillus Calmette-Guerin (BCG) for bladder cancer. PATIENT CONCERNS: A 69-year-old male with bladder cancer developed ptosis and diplopia 4 days after the completion of a treatment regimen with intravesical BCG weekly for 6 weeks...
November 2017: Medicine (Baltimore)
Maria E Farrugia, Marie Cleary, Caroline Carmichael
Ocular myasthenia is the milder end of the myasthenia gravis spectrum but treatment can be challenging especially in older patients. We retrospectively studied all patients on our database with ocular myasthenia (OMG), positive for acetylcholine receptor (AChR) antibodies. We identified 93 patients (64 men and 29 women). The mean age at disease onset was 63y, median 68y. Most (72%) experienced ptosis with diplopia; 19% experienced ptosis alone, while 7.5% complained of diplopia without ptosis. As expected, pyridostigmine was commenced early at diagnosis in the majority (69%) and 20% were still receiving pyridostigmine at final review...
November 15, 2017: Journal of the Neurological Sciences
Carolina Barnett, Vera Bril, Moira Kapral, Abhaya V Kulkarni, Aileen M Davis
OBJECTIVE: To study responsiveness and meaningful change of the Myasthenia Gravis Impairment Index (MGII) and its relative efficiency compared to other measures. METHODS: We enrolled 95 patients receiving prednisone, IV immunoglobulin (IVIg), or plasma exchange (PLEX) and 54 controls. Patients were assessed with the MGII and other measures-including the Quantitative Myasthenia Gravis Score, Myasthenia Gravis Composite, and Myasthenia Gravis Activities of Daily Living-at baseline and 3-4 weeks after treatment...
December 5, 2017: Neurology
Azita Chegini
BACKGROUND AND AIMS: Botulinum toxin (Botox) injections are used as a cosmetic treatment to decrease wrinkles in face and chin. Being a neurotoxic agent it minimizes muscle activity, while side effects are usually rare. This article subsequently presents one case of these rare effects. CASE: A 30-year-old woman presenting with ptosis, diplopia, dysarthria, dysphagia and muscle weakness was admitted to our hospital. She had no history of disease. For cosmetic reasons, she had three Botox injections during the preceding months...
November 2017: Atherosclerosis. Supplements
Isabel Illa, Elena Cortés-Vicente, María Ángeles Martínez, Eduard Gallardo
Patients with myasthenia gravis (MG) without antibodies to the acetylcholine receptor (AChR) or muscle-specific tyrosine kinase (MuSK) have been classified as having double-seronegative myasthenia gravis (dSNMG). We used the sera from six dSNMG patients with positive immunohistochemistry assays in a protein array to screen reactivity with 9000 human proteins. We identified cortactin, an intracellular protein that interacts with agrin/MuSK favoring AChR aggregation, as a new antigen in dSNMG. We then designed an in-house enzyme-linked immunosorbent assay as a screening assay and confirmed these results by western blot...
October 25, 2017: Annals of the New York Academy of Sciences
Lakshmi Leishangthem, Sudhakar Reddy Satti
71-year-old woman with progressive left-sided, monocular diplopia and ptosis. Her symptoms mimicked ocular myasthenia, but she had an indirect carotid cavernous fistula (CCF). She was diagnosed with monocular myasthenia gravis (negative acetylcholinesterase antibody) after a positive ice test and started on Mestinon and underwent a thymectomy complicated by a brachial plexus injury. Months later, she developed left-sided proptosis and ocular bruit. She was urgently referred to neuro-interventional surgery and was diagnosed with an indirect high-flow left CCF, which was treated with Onyx liquid and platinum coil embolisation...
October 19, 2017: BMJ Case Reports
F Jian, H B Wang, N Chen, S Yang, Y Liu, Y Z Zhao, L Zhang, H Pan, X H Zhang
Objective: To explore the clinical and electrophysiological features and prognosis of distal myasthenia gravis (MG). Methods: Data of MG patients in Beijing Tiantan Hospital from October 2016 to March 2017 were reviewed, including thorough neurological examinations, nerve conduction studies, repetitive nerve stimulations (RNS) and needle electromyographies. Distal MG patients were included and were analyzed as to their clinical and electrophysiological features and response to treatments. Results: Four patients (7...
October 10, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Kelvin Y Teo, Sharon L Tow, Benjamin Haaland, Tushar D Gosavi, Loo Jing-Liang, L O Yew Long, Dan Milea
INTRODUCTION: Ocular myasthenia gravis (OMG) is a common condition of the neuromuscular junction that may convert to generalized myasthenia gravis (GMG). Our aim in this study was to determine the conversion rate and predictive factors for generalization in OMG, in an Asian population. METHODS: The investigation consisted of a retrospective study of OMG patients with a minimum 2 years of follow-up. RESULTS: Among 191 patients with OMG, 155 had the minimum 2-year follow-up...
October 5, 2017: Muscle & Nerve
Shekhar Sehgal, Roshan Rebello, Louise Wolmarans, Marianne Elston
We present two patients with Graves' disease and concurrent myasthenia gravis. The impact of the dual diagnosis on the clinical course and the potential for a delayed diagnosis of myasthenia gravis is discussed. Patient 1, a 28-year-old man was diagnosed with Graves' disease following his second respiratory arrest. His history was strongly suggestive of a second pathology. Patient 2, a 66-year-old Cantonese woman with established Graves' disease presented with thionamide-related neutropaenia. Examination revealed bilateral ptosis and right lateral rectus palsy...
September 7, 2017: BMJ Case Reports
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