Objective: To report a case of Anti-Ri Rhombencephalitis presenting with subacute ophthalmoplegia, jaw dystonia, and gait ataxia with minimal clinical improvement despite radiographic resolution after treatment.

Background: Antibodies targeting intracellular Ri antigen are classically associated with Opsoclonus-Myoclonus syndrome. Since the initial description, there have been established neurological presentations associated with this antibody, such as subacute cerebellar, brain stem, and oculomotor disturbances. It is now known to be highly associated with malignancy. Radiographic and clinical response to treatment — particularly their congruence — long term outcome, and appropriate cancer screening needs to be clarified.

Method: N/A

Results: A 75-year-old woman presented with subacute shortness of breath, gait instability, diplopia, and jaw dystonia. Her neurological exam was notable for complete ophthalmoparesis, jaw dystonia, ataxia, and neck flexion/extension weakness. MRI of the brain noted a T2 hyperintensity in the periaqueductal gray matter without enhancement. MRIs of her spine were normal. Her CSF studies were notable for lymphocytic pleocytosis, elevated protein, and greater than 5 oligoclonal bands. Her CSF showed a high titer of anti-Ri antibodies by immunofluorescence assay. She was empirically treated with intravenous steroids for 3 days with no clinical improvement. However, repeat imaging showed resolution in her initial periaqueductal gray matter lesion. She was treated with plasmapheresis for 5 sessions every other day with minimal further clinical improvement. No cancer was identified by pelvic ultrasound, mammogram, and CT of the chest, abdomen, and pelvis.

Conclusion: Subacute brainstem and cerebellar symptoms such as ophthalmoplegia and jaw dystonia should raise suspicion for Anti-Ri rhombencephalitis. Resolution in imaging abnormalities may not translate into clinical improvement, at least in the acute to subacute setting.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/radiographic-resolution-without-clinical-improvement-in-anti-ri-rhombencephalitis/