Morvan's syndrome

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Morvan’s Syndrome, or Morvan’s fibrillary chorea (MFC), is a rare autoimmune disease named after nineteenth century French physician Augustin Marie Morvan. “La chorée fibrillaire” was first coined by Morvan in 1890 when describing patients with multiple, irregular contractions of the long muscles, cramping, weakness, pruritis, hyperhidrosis, insomnia, and delirium.[1] It normally presents with a slow insidious onset over months to years.[2] 90% of cases spontaneously go into remission, while the other 10% of cases lead to death.[3]

Contents

Overview

In 1890, Morvan described a patient with myokymia (muscle twitching) associated with muscle pain, excessive sweating, and disordered sleep.[4] This rare disorder is characterized by severe insomnia, amounting to no less than complete lack of sleep (agrypnia) for weeks or months in a row, and associated with autonomic alterations consisting of profuse perspiration with characteristic skin Miliaria (miliaria rubra, sweat rash or prickly heat), tachycardia, increased body temperature, and hypertension. Patients display a remarkable hallucinatory behavior, and peculiar motor disturbances, which Morvan reported under the term “fibrillary chorea” but which are best described nowadays as neuromyotonic discharges.[5] The association of the disease with thymoma, tumour, autoimmune diseases, and autoantibodies suggests an autoimmune or paraneoplastic aetiology.[1] Besides an immune-mediated etiology, it is also believed to occur in gold, mercury, or manganese poisoning.[6]

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