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KEEP YOUR HEAD DOWN AND SHAKE
  1. Girija Sadalage,
  2. Muhammad Yousaf,
  3. Saiju Jacob
  1. Queen Elizabeth Hospital Birmingham

Abstract

Introduction Camptocormia is due to weakness of neck extensors or increased tone of neck flexors. Spinal myoclonus is a frequent occurrence in different neurological conditions but rarely documented Potassium channel antibodies. CASPR2 antibodies were originally reported in association with classical Limbic encephalitis, peripheral nerve hyper-excitability (PNH) or Morvan's Syndrome.

Case Report: We report a case of a 75 year old gentleman who presented with involuntary movements of his upper spine and head and progressive camptocormia with mild memory problems. On examination he had a stooped posture, proximal lower limb weakness and mild distal upper limb weakness (Video is available). His investigations were normal other than VGKC antibodies which were very high on three occasions (2794, 2743 and 2306 pmol/L) and CASPR2 antibodies were strongly positive.

He was given two courses of iv immunoglobulins at two monthly intervals and started on levetiracetam which resulted in an improvement in symptoms. He is currently on methotrexate 7.5 mg once a week and has responded very well to this with improvement in both the myoclonic jerks and camptocormia.

Conclusion The phenotype of neurological disorders occurring in patients with autoantibodies against subunits of VGKCs is rapidly expanding. Our patient presented with severe camptocormia, myoclonus and head drop with memory impairment indicating that the clinical spectrum of CASPR2 autoimmunity is larger than formerly reported.

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