Hodgkin Lymphoma Associated with Paraneoplastic Cerebellar Degeneration with Anti-Tr (DNER) Antibodies: A Case Report

Introduction: Paraneoplastic cerebellar degeneration (PCD) is a rare immune-mediated neurological syndrome associated with Hodgkin lymphoma (HL). It typically presents with subacute cerebellar dysfunction and is strongly linked to anti-Tr/delta/notch-like epidermal growth factor-related receptor (DNER) antibodies directed against cerebellar Purkinje cells. Objective: To describe a case of HL presenting with PCD and anti-Tr/DNER antibodies, highlighting the clinical, radiological, and immunological features, and to contextualise the findings within the existing literature. Case report: This article reports the case of a woman in her early 50s who presented with rapidly progressive cerebellar syndrome characterised by gait ataxia, severe dysarthria, and cerebellar signs. Cerebrospinal fluid analysis showed mononuclear pleocytosis with normal protein and glucose levels. Brain MRI demonstrated subtle bilateral cerebellar cortical hyperintensities. Anti-Tr/DNER antibodies were detected in serum and cerebrospinal fluid. Subsequent imaging revealed widespread lymphadenopathy, and lymph node biopsy confirmed classical HL (Ann Arbor Stage IIA). The patient received four cycles of ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy alongside immunomodulatory treatment for PCD, including corticosteroids, plasmapheresis, and rituximab. Results: Complete oncological remission was achieved; however, anti-Tr antibodies persisted and no significant neurological recovery occurred, with ongoing ataxia, dysarthria, and oculo-vestibular symptoms. Conclusion: This case underscores the role of anti-Tr/DNER-associated PCD as an early indicator of HL and highlights the striking dissociation between excellent oncological outcomes and poor neurological prognosis. Early recognition and prompt treatment are essential, as irreversible cerebellar damage may occur despite successful tumour control.