Thursday, January 30, 2014

Case Report: Double Seronegative Myasthenia Gravis with Antiphospholipid Syndrome

Our daily search for updates regarding myasthenia gravis turned up the following case study from The Journal of Medical Case Reports. As noted in the introduction, myasthenia gravis is often related to other autoimmune diseases and outside conditions. It is the opinion of many that when myasthenia gravis presents itself, it can be a symptom of or associated with another disease. In the following case study the suspected associated condition is antiphospholipid syndrome.

Double seronegative myasthenia gravis with antiphospholipid syndrome: a case report

by Diana Dan, Pierre-Alexandre Bart, Jan Novy, Thierry Kuntzer and Carole Clair

Published: 1 January 2014 in Journal of Medical Case Reports
Abstract (provisional)

Myasthenia gravis is an autoimmune disease characterized by fluctuating muscle weakness. It is often associated with other autoimmune disorders, such as thyroid disease, rheumatoid arthritis, systemic lupus erythematosus, and antiphospholipid syndrome. Many aspects of autoimmune diseases are not completely understood, particularly when they occur in association, which suggests a common pathogenetic mechanism.

Case presentation
We report a case of a 42-year-old Caucasian woman with antiphospholipid syndrome, in whom myasthenia gravis developed years later. She tested negative for both antibodies against the acetylcholine receptor and against muscle-specific receptor tyrosine-kinase, but had typical decremental responses at the repetitive nerve stimulation testing, so that a generalized myasthenia gravis was diagnosed. Her thromboplastin time and activated partial thromboplastin time were high, anticardiolipin and anti-?2 glycoprotein-I antibodies were slightly elevated, as a manifestation of the antiphospholipid syndrome. She had a good clinical response when treated with a combination of pyridostigmine, prednisone and azathioprine.

Many patients with myasthenia gravis test positive for a large variety of auto-antibodies, testifying of an immune dysregulation, and some display mild T-cell lymphopenia associated with hypergammaglobulinemia and B-cell hyper-reactivity. Both of these mechanisms could explain the occurrence of another autoimmune condition, such as antiphospholipid syndrome, but further studies are necessary to shed light on this matter.Clinicians should be aware that patients with an autoimmune diagnosis such as antiphospholipid syndrome who develop signs and neurological symptoms suggestive of myasthenia gravis are at risk and should prompt an emergent evaluation by a specialist.

Source: The Journal of Medical Case Reports The complete article is available as a provisional PDF. The fully formatted PDF and HTML versions are in production.

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