Objective: To determine the clinical course of velopharyngeal incompetence in children with myasthenia gravis (MG).
Methods: A 30-year retrospective study was performed using the medical records of 538 children who presented with velopharyngeal inadequacy (VPI) to a tertiary care academic pediatric center. Children with velopharyngeal incompetence due to myasthenia gravis were identified and their clinical courses were reviewed.
Results: Four children were identified with velopharyngeal incompetence associated with myasthenia gravis. All four children required intervention for improvement of speech intelligibility. A speech prosthesis was the uniform intervention.
Conclusion: Neonatal myasthenia gravis patients should be followed long-term as symptoms may recur as speech impairment. In addition, a high index of suspicion for this entity is required for early diagnosis due to the highly variable presentation and clinical course.
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http://dx.doi.org/10.1016/j.ijporl.2004.01.006 | DOI Listing |
Ann Clin Transl Neurol
December 2024
Transplant and Cell Therapy Program, Division of Hematology, Department of Medicine, The Ottawa Hospital, Ottawa, Ontario, Canada.
Objectives: Patients with refractory myasthenia gravis (MG) have few treatment options. Autologous hematopoietic stem cell transplantation (HSCT) has been used to treat immune diseases; however, its use in the treatment of MG is not broadly considered. Our objective is to report on the efficacy and safety of HSCT in refractory MG.
View Article and Find Full Text PDFTher Adv Neurol Disord
December 2024
Huashan Rare Disease Center and Department of Neurology, Huashan Hospital, Shanghai Medical College, National Center for Neurological Disorders, Fudan University, No.12 Urumqi Middle Road, Jing 'an District, Shanghai 200040, China.
Background: Thymoma-associated myasthenia gravis (TAMG) is a subtype of myasthenia gravis (MG) that is associated with more severe symptoms and a relatively poor prognosis. Eculizumab, an inhibitor to target human C5 component of the complement cascade, is considered a treatment option for refractory generalized MG (gMG).
Objectives: To explore the safety and efficacy of eculizumab in patients with TAMG.
Ther Adv Neurol Disord
December 2024
Department of Neurology, Faculty of Medicine, University of Augsburg, Stenglinstrasse 2, Augsburg 86156, Germany.
In acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (gMG), neonatal Fc-receptor (FcRn) inhibition has broadened the therapeutic spectrum. Myasthenic crisis (MC), heralded by an impending myasthenic crisis (iMC), is a critical condition requiring treatments with rapid onset and sustained efficacy. Currently treatments used for iMC, including intravenous immunoglobulins and plasma exchange/immunoadsorption, have limitations, such as delayed onset of action and potential side effects.
View Article and Find Full Text PDFTher Adv Neurol Disord
December 2024
Department of Neurology, Faculty of Medicine, University of Augsburg, Stenglinstrasse 2, Augsburg 86156, Germany.
In seropositive myasthenia gravis (MG), complement inhibition has been shown to be an effective and a fast-acting therapeutic option. Myasthenic crisis (MC), usually preceded by impending MC, is a life-threatening complication requiring highly effective treatments with rapid onset of action. Currently used treatment options of MC are limited, consisting mainly of symptomatic and immune therapies, that is, intravenous immunoglobulins and plasma exchange/immunoadsorption.
View Article and Find Full Text PDFCureus
November 2024
Neurology, Charles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, USA.
Myasthenia gravis (MG) is one of the most common neuromuscular disorders. It is an antibody-mediated autoimmune disease affecting the neuromuscular junction, presenting with fluctuating muscle weakness that commonly affects the ocular, bulbar, proximal, and respiratory muscles. Treating MG in the older population with preexisting comorbidities can be challenging.
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