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Neuromuscul Disord. 2014 Jan;24(1):25-30. doi: 10.1016/j.nmd.2013.09.013. Epub 2013 Oct 1.

Outcome of children with acetylcholine receptor (AChR) antibody positive juvenile myasthenia gravis following thymectomy.

Author information

1
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.
2
Department of Paediatric Neurology, Evelina Children's Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
3
Department of Cardiothoracic Surgery, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
4
Department of Paediatric Nephrology, Evelina's Children Hospital, Guy's & St. Thomas' Hospital NHS Foundation Trust, London, UK.
5
Dubowitz Neuromuscular Centre, Great Ormond Street Hospital for Children, London, UK.
6
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.

Abstract

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.

KEYWORDS:

Acetylcholine receptor (AChR) antibodies; Juvenile myasthenia gravis (JMG); Thymectomy

PMID:
24239058
DOI:
10.1016/j.nmd.2013.09.013
[Indexed for MEDLINE]

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