Outcome of children with acetylcholine receptor (AChR) antibody positive juvenile myasthenia gravis following thymectomy.
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.
Department of Paediatric Neurology, Evelina Children's Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK; Paediatric Neurology Unit, Institute of Paediatrics, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia.
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Heng
Department of Paediatric Neurology, Evelina Children's Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
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Lim
Department of Paediatric Neurology, Evelina Children's Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
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Absoud
Department of Cardiothoracic Surgery, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
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Austin
Department of Paediatric Neurology, Evelina Children's Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
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Clarke
Department of Paediatric Neurology, Evelina Children's Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
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Wraige
Department of Paediatric Nephrology, Evelina's Children Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
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Reid
Dubowitz Neuromuscular Centre, Great Ormond Street Hospital for Children, London, UK.
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Robb
Department of Paediatric Neurology, Evelina Children's Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK; Randall Division for Cell and Molecular Biophysics, Muscle Signalling Section, King's College, London, UK; Clinical Neuroscience Division, IoP, King's College, London, UK. Electronic address: Heinz.Jungbluth@gstt.nhs.uk.
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Jungbluth
D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009157:Myasthenia Gravis / Q000276:immunology / Q000601:surgery*; D011950:Receptors, Cholinergic / Q000276:immunology*; D012720:Severity of Illness Index; D013934:Thymectomy; D016896:Treatment Outcome
D001323:Autoantibodies; D011950:Receptors, Cholinergic
Acetylcholine receptor (AChR) antibodies; Juvenile myasthenia gravis (JMG); Thymectomy