The pathophysiology of penicillamine‐induced myasthenia gravis
Identifieur interne : 003413 ( Main/Exploration ); précédent : 003412; suivant : 003414The pathophysiology of penicillamine‐induced myasthenia gravis
Auteurs : Ralph W. Kuncl [États-Unis] ; Alan Pestronk [États-Unis] ; Daniel B. Drachman [États-Unis] ; Emanuel Rechthand [États-Unis]Source :
- Annals of Neurology [ 0364-5134 ] ; 1986-12.
English descriptors
- Teeft :
- Acetylcholine, Acetylcholine receptor, Acetylcholine receptors, Achr, Achr blockade, Achr degradation, Achr degradation rate, Achrs, Antibody titer, Autoimmune response, Autoimmune state, Available achrs, Binding sites, Biopsy, Bootstrap nonparametric method, Brain damage, Brief communication, Control cultures, Decremental responses, Degradation, Deltoid, Drachman, Dysregulatory state, Epileptic brain damage, Functional activities, Gravis, High antibody titer, Initial serum, Junctional, Junctional acetylcholine receptors, Junctional achrs, Magnetic resonance, Magnetic resonance imaging, Massachusetts institute, Muscle nerve, Myasthenia, Myasthenia gravis, Neurol, Neurology, Neuromuscular, Neuromuscular junction, Neuromuscular junctions, Normal number, Ongoing, Ongoing autoimmunity, Ongoing subclinical, Pars compacta, Pars compacta signal, Pars reticulata, Penicillamine, Penicillamine administration, Penicillamine myasthenia, Present patient, Receptor, Repetitive stimulation, Rheumatoid, Rheumatoid arthritis, Serial studies, Significant difference, Skeletal muscle, Status epilepticus, Systemic factors, Temporal course, Whitaker college.
Abstract
The temporal course and pathophysiology of penicillamine‐induced myasthenia gravis were studied in detail in a typical case. Our results suggest that this disorder and idiopathic autoimmune myasthenia gravis share the same essential pathophysiological features, including the presence of anti–acetylcholine receptor (AChR) antibody, serum‐induced blockade of AChRs, antibody‐mediated accelerated degradation of AChRs, and a resultant quantitative reduction in available junctional AChRs. An initial severe reduction in junctional AChRs was reversed and the patient recovered, both within 8 months of stopping penicillamine. Our data suggest that penicillamine probably produced myasthenia gravis by initiating a new autoimmune response rather than by enhancing ongoing autoimmunity.
Url:
DOI: 10.1002/ana.410200617
Affiliations:
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Our results suggest that this disorder and idiopathic autoimmune myasthenia gravis share the same essential pathophysiological features, including the presence of anti–acetylcholine receptor (AChR) antibody, serum‐induced blockade of AChRs, antibody‐mediated accelerated degradation of AChRs, and a resultant quantitative reduction in available junctional AChRs. An initial severe reduction in junctional AChRs was reversed and the patient recovered, both within 8 months of stopping penicillamine. Our data suggest that penicillamine probably produced myasthenia gravis by initiating a new autoimmune response rather than by enhancing ongoing autoimmunity.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Maryland</li></region></list><tree><country name="États-Unis"><region name="Maryland"><name sortKey="Kuncl, Ralph W" sort="Kuncl, Ralph W" uniqKey="Kuncl R" first="Ralph W." last="Kuncl">Ralph W. Kuncl</name></region><name sortKey="Drachman, Daniel B" sort="Drachman, Daniel B" uniqKey="Drachman D" first="Daniel B." last="Drachman">Daniel B. Drachman</name><name sortKey="Kuncl, Ralph W" sort="Kuncl, Ralph W" uniqKey="Kuncl R" first="Ralph W." last="Kuncl">Ralph W. Kuncl</name><name sortKey="Pestronk, Alan" sort="Pestronk, Alan" uniqKey="Pestronk A" first="Alan" last="Pestronk">Alan Pestronk</name><name sortKey="Rechthand, Emanuel" sort="Rechthand, Emanuel" uniqKey="Rechthand E" first="Emanuel" last="Rechthand">Emanuel Rechthand</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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