Abstract Details

Title Anti-Yo associated non-paraneoplastic autoimmune neuropathy: Report of 2 cases

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (12:00 PM-1:00 PM)

Poster/Presentation
Number 15-001

Objective

Report two patients with probable autoimmune sensorimotor neuropathy positive for serum anti-Yo antibodies but without malignancy.

Background

Anti-Yo antibodies are associated with paraneoplastic cerebellar ataxia and denote an underlying malignancy, usually gynecological tumors. Anti-Yo antibodies are directed against CDR proteins which, in the CNS, are primarily expressed on cerebellar Purkinje cells. In spite of immunotherapies and tumor removal, the neurologic outcome of these patients is often poor probably due to ongoing cellular autoimmunity.

Design/Methods Clinical studies, electrophysiology, testing for anti-Yo antibody specificity with Western blot (WB) and tissue immunostaining in cerebellum and peripheral nerves.

Results The first patient was a 68 year-old male with progressive burning paresthesias, proximal leg weakness and foot-drop. Work-up showed axonal sensorimotor neuropathy and positive anti-Yo antibodies. Search for tumors up to a 3-year follow-up, including PET scan, was negative. He responded favorably to IVIg treatment. The second patient was a 74 year-old male developed gradually progressive distal leg paresthesias and unsteady gait. Work-up revealed sensorimotor, predominantly large fiber axonal neuropathy and impaired distal proprioception. He was positive for anti-Yo antibodies by WB and Cell-Based Assay. Extensive work-up for malignancy was negative. Serum from both patients applied to primate sural nerve revealed distinct staining pattern. Of interest, both patients’ sera did not reveal the typical Purkinje cell staining pattern seen in paraneoplastic anti-Yo-positive patients.

Conclusions This is the first report of a non-paraneoplastic anti-Yo antibody-associated neuropathy. It appears that in neuropathy patients anti-Yo antibodies recognize different epitopes compared to paraneoplastic patients. Ongoing work is in progress to identify the precise peripheral nerve antigenic target recognized by these antibodies and determine their pathogenicity.

Authors/Disclosures
MICHAIL KOSMIDIS PRESENTER	No disclosure on file
Harry Alexopoulos	No disclosure on file
	No disclosure on file
Marinos C. Dalakas, MD, F�鶹��ýӳ�� (Thomas Jefferson University)	Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols, . Dr. Dalakas has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Dysimmune Diseases Foundation. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Octapharma. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ARGENX. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for �鶹��ýӳ��. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Therapeutic Advances in Neurology (TAND). Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Medlink.

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