Anti-Tr/DNER Paraneoplastic Cerebellar Degeneration with Marked Cerebellar and Psychological Symptoms Responsive to Plasma Exchange

Objective We present a patient who developed cerebellar degeneration and severe psychological symptoms leading to the diagnosis of Hodgkin's disease detection anti-Tr/DNER antibodies. The failed respond methylprednisolone intravenous immunoglobulin G, rituximab, tumor treatment but had significant improvement with plasma exchange (PLEX). Background Paraneoplastic accompanying may have its onset prior underlying malignancy, during course, or following treatment. associated autoantibody, anti-Tr, is reactive neuronal delta/notch-like epidermal growth factor-related receptors (DNER), an autoantibody not included in all paraneoplastic testing screens. condition characterized by progressive injury, response immunosuppressive therapy generally poor. Design/Methods Case Presentation: A 60-year-old male presented diplopia, loss balance, ataxia, impaired short-term memory, confusion, anger outbursts. Initial commercial screen for autoantibodies was negative. Two months his initial presentation he inguinal lymphadenopathy. He diagnosed as having Lymphoma Stage 1B found second laboratory anti-TR/DNER antibodies (Titer 1:3480; Reference range <1:240), antibody panel. CSF analysis notable protein 92 mg/dL. MRI demonstrated normal findings age. Results Treatment Doxorubicin-Bleomycin-Vinblastine-Dacarbazine (ABVD), pulse methylprednisolone, did affect progression. Plasma PLEX resulted marked improvement. Symptoms worsened subsequent immunoglobulins rituximab improved further exchange. Conclusions Although important malignancy degeneration, necessarily screens, potentially delay diagnosis. Our patient's dramatic suggests that should be considered early treatment, where there poor other modalities.