A 40-year-old man presented with respiratory compromise and was intubated. After tracheostomy, he was found to have ophthalmoplegia, severe limb rigidity, stimulus-sensitive myoclonus and autonomic dysfunction. For 1 week before admission, there had been a prodromal illness with low mood, hallucinations and limb myoclonus. Serum glycine receptor antibodies were strongly positive: we diagnosed progressive encephalomyelitis with rigidity and myoclonus. Despite a relapse, he has done well following immunotherapies. The clinical syndrome of encephalomyelitis with rigidity, described in 1976, often has a severe progressive course. A minority of patients have glutamic acid decarboxylase antibodies. The association with glycine receptor antibody was first reported in 2008, and we briefly review subsequent case reports to illustrate the range of clinical features. The antibody is likely to be disease mediating, although this remains unproven. The spectrum of diagnosable and treatable antibody mediated neurological syndromes is expanding. It is vital to recognise these conditions early to reduce morbidity and mortality.

A 40-year-old man was intubated after presenting with respiratory compromise. After tracheostomy, he was found to have an ophthalmoplegia, severe limb rigidity, stimulus sensitive myoclonus, and autonomic dysfunction. A history of prodromal illness with low mood, hallucinations and limb myoclonus preceded admission by a week. He remained ventilator dependent with marginal neurological improvement after 2 months before a diagnosis was made. Treatment with intravenous immunoglobulins was commenced. Neuroaxis MRI and whole body Computer Tomography was unremarkable. Cerebrospinal fluid analysis showed 10 lymphocytes, with normal protein and glucose, and negative oligoclonal bands. Antibodies against voltage gated potassium channels, glutamic acid decarboxylase, N-methyl-D-aspartic acid receptors, thyroid peroxidase, basal ganglia, and GQ1B were negative, as were vasculitic screen, tumour markers, antineuronal antibodies, HIV and syphilis serolgy. Anti-glycine receptor antibodies were strongly positive. A diagnosis of Progressive Encephalomyelitis with Rigidity and Myoclonus was made. The antibody is thought to be disease mediating. Although the clinical syndrome of encephalomyelitis with rigidity was described as early as 1976, the association with anti glycine receptor antibody was first reported in 2008. A brief review of the literature will be described. The spectrum of diagnosable and treatable antibody mediated neurological syndromes is expanding. It is vital to keep up to date and recognise these conditions early to reduce morbidity and mortality.