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GLYCINE RECEPTOR ANTIBODY MEDIATED PROGRESSIVE ENCEPHALOMYELITIS WITH RIGIDITY AND MYOCLONUS (PERM) PRESENTING WITH RESPIRATORY COMPROMISE IN A 40-YEAR-OLD MALE
  1. M Wickremaratchi1–5,*,
  2. RM Chalmers1–5,
  3. W Stern1–5,
  4. R Howard1–5,
  5. MR Woodhall1–5,
  6. A Vincent1–5
  1. 1Hurstwood Park Neurological Centre
  2. 2Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford
  3. 3The National Hospital for Neurology and Neurosurgery
  4. 4Western Sussex Hospitals NHS Trust
  5. 5Worthing Hospital

    Abstract

    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.

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