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  1. Outcome of children with acetylcholine receptor (AChR) antibody positive juvenile myasthenia gravis following thymectomy
    Heng HS, Lim M, Absoud M, Austin C, Clarke D, Wraige E, et al.
    Neuromuscul Disord, 2014 Jan;24(1):25-30.
    PMID: 24239058 DOI: 10.1016/j.nmd.2013.09.013
    Most evidence supporting the benefit of thymectomy in juvenile myasthenia gravis (JMG) is extrapolated from adult studies, with only little data concerning paediatric populations. Here we evaluate the outcome of children with generalized JMG who underwent thymectomy between 1996 and 2010 at 2 tertiary paediatric neurology referral centres in the United Kingdom. Twenty patients (15 female, 5 male), aged 13months to 15.5years (median 10.4years) at disease onset, were identified. Prior to thymectomy, disease severity was graded as IIb in 3, III in 11, and IV in 6 patients according to the Osserman classification. All demonstrated positive anti-acetylcholine receptor (AChR) antibody titres. All patients received pyridostigmine and 14 received additional steroid therapy. Transternal thymectomy was performed at the age of 2.7-16.6years (median 11.1years). At the last follow-up (10months to 10.9years, median 2.7years, after thymectomy), the majority of children demonstrated substantial improvement, although some had required additional immune-modulatory therapies. About one third achieved complete remission. The postoperative morbidity was low. No benefit was observed in one patient with thymoma. We conclude that thymectomy should be considered as a treatment option early in the course of generalised AChR antibody-positive JMG.
    Matched MeSH terms: Receptors, Cholinergic/immunology*
  2. "Mummy, my eyelids are heavy": A case series of juvenile myasthenia gravis
    Krishna L, Abdul Jalil NF, Lott PW, Singh S, Choo MM
    Eur J Ophthalmol, 2021 Mar;31(2):NP119-NP122.
    PMID: 31390886 DOI: 10.1177/1120672119867605
    PURPOSE: To report three cases of juvenile myasthenia gravis aged between 18 and 24 months with ocular symptoms as their first presentation.

    METHOD: A case series.

    RESULTS: We present a case series of juvenile myasthenia gravis in a tertiary centre in Malaysia. Two of the three cases consist of a pair of twins who presented with ptosis of bilateral eyes; the first twin presented 4 months later than the second twin. These two cases were positive for anti-acetylcholine receptor antibodies and had generalized myasthenia gravis, whereas the other case was negative for receptor antibodies and was purely ocular myasthenia gravis.

    CONCLUSION: Juvenile myasthenia gravis is relatively rare in toddlers. Early diagnosis and commencement of treatment is important to slow the progression of the disease and avoiding life-threatening events.

    Matched MeSH terms: Receptors, Cholinergic/immunology
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