Abstract Details

Title Anti- Tr antibodies in autoimmune cerebellar ataxia: Serial antibody testing and response to immunotherapy

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-039

Objective To present a 2-year follow-up of two patients with autoimmune cerebellar ataxia harboring anti-Tr antibodies.

Background Serum anti-Tr antibodies are most often associated with paraneoplastic cerebellar degeneration due to Hodgkin’s disease. These antibodies often become negative after successful chemotherapy and associated remission of cerebellar symptoms.

Design/Methods Patients’ sera were tested for anti-Tr using immunohistochemistry in mouse brain tissue and cell based assay (CBA) in serial serum samples. Patients’ cerebellar dysfunction was assessed using the Scale for the Assessment and Rating of Ataxia (SARA).

Results High titer anti-Tr antibodies were detected with immunohistochemistry and CBA in both patients. The first patient was a 46-year-old man with Hodgkin’s lymphoma and cerebellar ataxia (SARA scale=28 at diagnosis), diagnosed 3 months after symptoms onset. He was treated with lymphadenectomy and chemotherapy as well as IVIg and rituximab. In a 2-year follow-up, the cerebellar symptoms had clinically improved (SARA scale=17) but the anti-Tr antibodies remained low positive. The second patient was a 32-year-old woman who presented with headache, fever, confusion and photophobia, suggestive of encephalitis, along with cerebellar ataxia (SARA scale=24 at diagnosis); no malignancy was identified during the 2-year follow-up period. She was treated with IVIg and methylprednisolone with rapid improvement of cerebellar symptomatology. In the two-year follow-up, her SARA score was 2 and her serum anti-Tr antibodies undetectable.

Conclusions

Anti-Tr cerebellar ataxia, although considered a paraneoplastic autoimmune disease, can present without a tumor. The autoimmune, non-paraneoplastic disease responds better to immunotherapy with disappearance of the antibodies. In contrast, patients with the paraneoplastic form, although also improve with immunotherapy and tumor resection, may continue to be symptomatic with persistent low-titers of anti-Tr antibodies. Screening for anti-Tr should be within the routine work-up of patients with acquired cerebellar ataxia as these antibodies identify an autoimmune subset responding to immunotherapy, if initiated early.

Authors/Disclosures
Chrysanthi Barba PRESENTER	No disclosure on file
Harry Alexopoulos	No disclosure on file
Maria Dimitriadou	No disclosure on file
Sofia Akrivou	No disclosure on file
Popianna Tsiortou	Popianna Tsiortou has nothing to disclose.
Androniki Plomaritoglou, MD, PhD, MSc	No disclosure on file
Marinos C. Dalakas, MD, FAAN (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 AAN. 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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