@article {Sune8, author = {Bo Sun and Victor Mgbachi and Mark Woodhall and Zanna Voysey and Laura Azzopardi and Prabha Rajan and Stephen Wroe and Patrick Waters and M Isabel Leite and Sarosh Irani}, title = {09.36 A tumultuous case of episodic apnoea}, volume = {90}, number = {12}, pages = {e8--e8}, year = {2019}, doi = {10.1136/jnnp-2019-ABN-2.24}, publisher = {BMJ Publishing Group Ltd}, abstract = {Glycine-receptor(GlyR) antibodies are well defined in progressive encephalomyelitis with rigidity and myoclonus (PERM), and are associated with a limited spectrum of immunotherapy-responsive syndromes. Whilst paradigms of autoantibody pathogenicity have been proposed, the trigger and propagation of GlyR antibodies remains unclear. We herein describe and define clinical and immunobiological observations of a GlyR antibody positive patient with PERM with the rare association of an ovarian teratoma.A 36-year-old woman presented with subacute axial and limb rigidity, stimulus-sensitive myoclonus, startle, ataxia, anxiety and episodic apnoea. GlyR antibodies were present in serum and CSF. CT revealed a 53 mm left ovarian teratoma. Despite improvement following immunotherapy, she relapsed with respiratory arrest, therefore removal of the left ovarian teratoma was performed.The fresh teratoma tissue was cultured in B-cell stimulating and plasma cell maintaining conditions, based on similar work with ovarian teratomas from patients with NMDAR-antibody encephalitis (Makuch et al., Annals of Neurology 2018).This teratoma secreted GlyR antibodies into culture supernatants consistent with intra-tumoral synthesis of glycine-receptor antibodies. Whilst marked post-surgical improvement was observed, relapses persisted in correspondence with persistent glycine-receptor antibodies.Our case highlights key areas for clinical consideration; GlyR antibody-mediated disease can be paraneoplastic in nature; ovarian teratomas can produce pathogenic autoantibodies; late tumour removal or immunotherapy worsens prognosis.}, issn = {0022-3050}, URL = {https://jnnp.bmj.com/content/90/12/e8.2}, eprint = {https://jnnp.bmj.com/content/90/12/e8.2.full.pdf}, journal = {Journal of Neurology, Neurosurgery \& Psychiatry} }