Caspr 2 antibody associated disease–An unusual presentation

Abstract

Introduction: Antibodies to Voltage Gated Potassium Channels (VGKC) are directed against Leucine-rich Glioma- Inactivated1 (LGI1) and Contactin Associated Protein like 2 (Caspr2). Common presentations of Caspr2 antibody associated diseases include limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. New clinical phenotypes are being considered to the spectrum of Caspr2 antibody associated disease. Here we report a case of Caspr2 antibody associated disease with an unusual clinical presentation. Case Report: 40 year old male presented with distal symmetrical sensorimotor neuropathy of 4 months duration. Subsequently he had developed neurobehavioral symptoms in the form of irritability, episodic memory impairment and seizures. After medical evaluation he was diagnosed to have anti Caspr2 antibody associated disease. Patient showed improvement with an immunosuppressive therapy, but had recurrence of autoimmune encephalitis 1 month later. Along with that, he had also developed right vocal cord palsy, for which no other cause was identified. Patient improved with second line immunosuppressive therapy. He had no further recurrence and is under follow up. Keywords: Voltage gated potassium channels, Leucine-rich Glioma-inactivated 1, Contactin associated protein, Limbic encephalitis, Neuromyotonia.