To explore the association of Hodgkin Lymphoma (HL) and CNS Angiitis. In this case, a male in his late 20s in remission from HL presented with new neurologic deficits due to granulomatous angiitis of CNS (GANS).

There are twenty previously reported cases of patients with HL and GANS, however, with no causal mechanism established. Proposed etiologies include paraneoplastic mechanisms, hypersensitivity reactions, or parainfectious processes particularly with varicella zoster virus [3]. In most patients, presenting symptoms include ataxia, headache, and confusion [2]. The diagnosis is difficult to establish as most diagnostic studies are nonspecific and brain biopsy is the only confirmatory study [1]. GANS associated with HL is known to respond adequately to prompt targeted chemotherapy of HL and steroids [1].

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We report a case of a male in his late 20s who presented with diplopia. Brain MRI showed enhancement of the leptomeninges, left cerebellum, and left occipital lobe. He was later diagnosed with HL by lymph node biopsy and treated successfully with rituximab. Two years later, he developed dysarthria and gait imbalance. A repeat brain MRI showed perivascular enhancement and left temporal lobe hemorrhage. A brain biopsy confirmed GANS with a negative paraneoplastic panel for relation to lymphoma. Treatment with dexamethasone and methotrexate resolved above symptoms, however, he developed new onset GTC seizures. CT head showed right frontal intraparenchymal and subfalcine subdural hematoma. A MRA showed posterior cerebral artery narrowing suggestive of vasculitis. Patient was discharged on high dose prednisone and methotrexate. Later he developed cognitive dysfunction that improved after treatment with rituximab vasculitis protocol. 

This case highlights the rare presentation of GANS in a patient who has HL with CNS involvement. Further studies may be needed to evaluate for any association of GANS with HL.