Abstract Details

Title Augustin Morvan's Choree Fibrillaire (1890) and the Development of Autoimmune Neurology

Topic History of Neurology

Presentation(s) P05 - (-)

Poster/Presentation
Number 253

Objective

Background BACKGROUND: Autoimmune disorders of the nervous system are often considered to be discoveries of the 20th century. However, in 1890 Morvan published descriptions of patients suffering from disorders that he combined under the rubric of 'fibrillary chorea.'

Design/Methods DESIGN/METHODS: Examination of the original publication (in the Gazette hebdomadaire de medecine et de chirurgie, 1890) and subsequent research in the field.

Results RESULTS: In a series of papers published in April, 1890, Morvan described six patients (five humans and one horse) suffering from what he considered to be fibrillary chorea. The first of these patients has what is now termed Morvan's syndrome, a combination of myotonia with autonomic and central nervous system dysfunctions (including encephalopathy and insomnia, which he termed agrypnia excitant) along with pruritus. This condition is now recognized as one of the anti-voltage gated potassium channel (VGKC) disorders. One hundred years after the initial description, Vincent's group discovered the connection of this syndrome to anti-VGKC antibodies. Subsequent studies have distinguished the typical immune target in Morvan's syndrome, contacting-associated protein (CASPR2), from the more common syndrome of limbic encephalitis due to antibodies again the leucine-rich glioma inactivated protein (LGi1), and a number of anti-VGKC antibodies associated with Isaac's syndrome. The other patients in Morvan's series do not appear to have suffered from what we now call Morvan's syndrome; their descriptions will be discussed briefly.

Conclusions CONCLUSIONS: Only one of patients in Morvan's series appears to have had his eponymous syndrome, but his description contributed to the later development of autoimmune neurology.

Authors/Disclosures
Thomas P. Bleck, MD, FAAN (Northwestern University Department of Neurology) PRESENTER	Dr. Bleck has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ceribell. Dr. Bleck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Marinus Pharmaceuticals. Dr. Bleck has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for iECURE. Dr. Bleck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acasti. Dr. Bleck has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Society of Critical Care Medicine. Dr. Bleck has received research support from NIH.
Mario Zappia, MD (Università Degli Studi Di Catania- Dept of Medical & Surgical Sciences)	No disclosure on file

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