ABSTRACT:
Morvan’s syndrome is a rare autoimmune disorder showing peripheral nervous system hyperexcitability accompanied by autonomic and central nervous system (CNS) hyperactivity. Peripheral hyperactivity includes clinical and electrophysiological evidence of painful cramps, myokymia and neuromyotonia. Acquired neuromyotonia manifests clinically in cramps, stiffness and fasciculation. Autonomic symptoms include hyperhidrosis, fluctuations in blood pressure, tachycardia. CNS hyperactivity include insomnia, agitation, hallucination, confusion, anxiety. We present a case of 48 year old female with morvan’s syndrome with central, peripheral and autonomic hyperexcitability. Admitted with alleged history of seizure and was having memory disturbances, paraesthesia and perfused perspiration with palpitation. She tested strongly positive for CASPR2 antibodies and LHI1 antibodies. On the basis of symptoms like neuromyotonia, hyperhidrosis and insomnia with features of encephalopathy and indirect immunofluorescence the patient was diagnosed to have morvan’s syndrome. Treatment was started Inj. Methylprednisolone 1gm IV, during this treatment the patient restarted dysautonomia features then intravenous immunoglobulin (IVIG) and T. Cellcept (mycophenolate mofetil) 1mg BD was given. She was managed symptomatically over time.