MORVAN'S SYNDROME : A RARE CASE REPORT

Abstract

Morvan's syndrome is an autoimmune disorder characterized by central, autonomic, and peripheral hyperactivity with associated thymoma in majority. The clinical features include insomnia, hyperhidrosis, myokymia, dysautonomia and hallucinations. Voltage gated potassium channel (VGKC) namely, leucine-rich glioma inactivated 1 (LGI-1) antibody and contactin-associated protein-like 2 (CASPR-2) antibody are thought to play a pathogenic role in peripheral and central nervous system symptoms in Morvan's syndrome. Electromyography (EMG) is useful in diagnosis of Morvan's syndrome characterized by spontaneous myober activity with various denervation potentials. Computed tomography (CT) scan and Magnetic resonance imaging (MRI) brain is usually normal. Immunosuppressive therapies, encompassing corticosteroids, azathioprine, methotrexate and recently, rituximab, are the mainstay of therapy. Other treatments include PE, IVIG, and thymectomy. We report a rare case of morvan syndrome with typical clinical features along with leucine-rich glioma inactivated 1 (LGI-1) antibody and contactin-associated proteinlike 2 (CASPR-2) antibody positivity successfully treated with intravenous immunoglobulin (IVIG) therapy.