Platt, Isobel Sarah;
Joseph, Albert;
Tsirka, Vasiliki;
Raja, Nazia;
Garcia, Maria;
Palace, Jacqueline;
Dobson, Ruth;
(2025)
Multiple autoimmunity: neuromyelitis optica spectrum disorder with Lambert-Eaton myasthenic syndrome.
Practical Neurology
10.1136/pn-2024-004446.
(In press).
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Abstract
A 56-year-old woman with a background of neuromyelitis optica spectrum disorder associated with aquaporin-4 antibodies (AQ4-NMOSD) treated with azathioprine, presented with a 2-month history of declining mobility, weight loss and hoarse voice. She had a history of autoimmune thyrotoxicosis and treated hypertension. Given her smoking history, the initial clinical concern was of malignancy. Neurophysiological examination identified severely attenuated responses from the upper and lower limb motor units, with significant neurophysiological incrementation postexercise, indicating a presynaptic neuromuscular junction disorder. Antibody testing showed markedly raised antibodies to the p/q subtype voltage-gated calcium channels, and she was diagnosed with Lambert-Eaton myasthenic syndrome (LEMS). She was treated with 3,4-diaminopyridine and rituximab to maintain remission. Follow-up serial fluorodeoxyglucose positron emission tomography (FDG-PET) CT scans have yet to identify an underlying malignancy. LEMS is associated with additional autoimmune diseases in about half of cases. This case illustrates the importance of recognising other treatable autoimmune conditions in the context of NMOSD.
| Type: | Article |
|---|---|
| Title: | Multiple autoimmunity: neuromyelitis optica spectrum disorder with Lambert-Eaton myasthenic syndrome |
| Location: | England |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1136/pn-2024-004446 |
| Publisher version: | https://doi.org/10.1136/pn-2024-004446 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Clinical neurology, EMG (single fibre), Lambert Eaton Syndrome, neuroimmunology, neuromuscular |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10205822 |
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