Sign Up for a Free Account
  • Updated 12.14.2020
  • Released 12.14.2020
  • Expires For CME 12.14.2023

Morvan syndrome and related disorders associated with CASPR2 antibodies

Introduction

Overview

Morvan syndrome is a rare, life-threatening autoimmune disorder associated with anti-CASPR2 autoantibodies (CASPR2-Abs) (28). Patients develop severe peripheral nerve hyperexcitability symptoms along with agrypnia excitata, a specific sleep disorder accompanied by behavioral disturbances and hallucinations. Clinical categorization is difficult because CASPR2-Abs are also found in autoimmune limbic encephalitis, which may feature behavioral disturbances and insomnia, as well as in acquired neuromyotonia (another peripheral nerve hyperexcitability syndrome) (09). In fact, Morvan syndrome appears to have distinct clinical and immunological specificities (21). Although the biology of Morvan syndrome remains poorly elucidated, progress has been made in its understanding.

Key points

• Morvan syndrome is a life-threatening disease characterized by severe peripheral nerve hyperexcitability, severe insomnia with encephalopathy and hallucinations (agrypnia excitata), and CASPR2-Abs.

• Morvan syndrome frequently associates with a malignant thymoma.

• Autoimmune accompaniments such as myasthenia gravis or autoimmune cytopenias are frequent.

• Immunomodulatory agents such as steroids and rituximab are usually efficient.

• Relapses may occur and generally herald malignant thymoma recurrence.

Historical note and terminology

The term “Morvan syndrome” comes from a publication in the late 19th century by French physician Augustin Morvan describing 6 patients with generalized "fibrillar contractions of the muscles," accompanied in 1 case by anxiety, sudation, and pain (20). In the mid-20th century, Roger and colleagues reported a series of 70 patients with a combination of confusion, visual hallucinations, sleep disorders, and severe peripheral nerve hyperexcitability symptoms, which they referred to as "syndrome de Morvan" (27). A specific sleep disorder, agrypnia excitata, was later linked to this syndrome (18). Finally, Irani and colleagues described CASPR2-Abs in Morvan syndrome, acquired neuromyotonia, and autoimmune limbic encephalitis (09).

Start a MedLink Neurology trial
to unlock 3 free articles.

  • Nearly 3,000 illustrations, 
including video clips of 
neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of 
neurology in 1,200 
comprehensive articles.

Questions or Comment?

MedLink, LLC

10393 San Diego Mission Rd, Suite 120

San Diego, CA 92108-2134

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com