Abstract
We analyzed relationships among pubertal stage at disease onset, sex, disease severity, and acetylcholine receptor antibody (AChR Ab) levels in 46 patients with autoimmune juvenile myasthenia gravis (JMG). Female predominance was least in children with prepubertal disease onset (F:M = 1.3:1) and increased in patients with peripubertal (F:M = 1.8:1) and postpubertal (F:M = 14:1) onset. Seronegative JMG was most common in children with early disease onset: 4 of 9 (44%) with prepubertal, 4 of 22 (18%) with peripubertal, and 0 of 15 (0%) with postpubertal onset were seronegative. The rapid therapeutic response to plasmapheresis was useful in distinguishing some patients with seronegative JMG from those with congenital myasthenia gravis (CMG). The high frequency of seronegative JMG in patients with prepubertal onset indicates that AChR Ab assays do not adequately discriminate between JMG and CMG in young children. Furthermore, the different sex distribution in patients with different pubertal stages at disease onset suggests that sex hormones play an important modulating role in JMG.
- © 1993 by the American Academy of Neurology
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
