Anti-CV2-Associated Paraneoplastic Hemichorea Secondary to Diffuse Large B-cell Lymphoma

Patients with paraneoplastic chorea associated with anti-CV2 antibodies present with characteristic movement patterns.1 This paraneoplastic syndrome is most often in association with smallcell lung cancer although there are cases of patients diagnosed with adenocarcinomas, and rarely non-Hodgkin's lymphoma.[1][2][3] We report paraneoplastic hemichorea in a 71-year-old man diagnosed with diffuse large B-cell lymphoma, a unique case given the rare association with this malignancy.A 71-year-old man with a past medical history of essential thrombocytosis (platelet count in the normal range with hydroxyurea), well-controlled type II diabetes mellitus (HbA1c of 6.6% and point of care glucose of 6.2 on the day of presentation) and hypertension, presented to the emergency department with newonset left-sided movement abnormalities.The involuntary movements initially progressed from the left lower limb to left face and then left upper limb, worsening in amplitude and frequency over a period of days.Examination revealed left-sided chorea and dysarthria.The rest of the neurological examination was normal.The patient had no known family history of Huntington's disease and four siblings without neurological conditions.CT angiogram was negative for neurovascular findings; however, it demonstrated a 1.5 cm left anterolateral pharyngeal soft tissue mass.Brain MRI showed only mild generalised atrophic changes, typical for age, without signal abnormalities or diffusion restriction.There was no abnormal enhancement with gadolinium-enhanced MRI.CT of the chest, abdomen and pelvis was unremarkable except for calcified coronary arteries.Biopsy of the vallecular lesion showed a monoclonal B-cell population, in keeping with serum protein electrophoresis results which demonstrated an IgG kappa monoclonal band.An autoimmune work-up, including ANA and ENA antibodies, ANCA and cardiolipin, was negative and a paraneoplastic antibody panel was positive only for anti-CV2 (CRMP-5) antibodies in serum (concentration 1:200 with reference <1:100; the method used was Nanoliter Scale Immunoassay from Athena Diagnostics).The patient was diagnosed with diffuse large B-cell lymphoma and received three cycles of cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab, followed by radiation with no evidence of disease recurrence post-treatment.During chemotherapy, hydroxyurea was discontinued resulting in a slightly elevated platelet count of 437 × 10^9/L (reference range 150-400 × 10^9/L).The patient's chorea was successfully controlled with haloperidol and tetrabenazine, medications that were subsequently discontinued without re-emergence of the chorea even after 6 months of follow-up, and his neurological examination was normal.Paraneoplastic chorea is a rare clinical entity, most often presenting in the context of small-cell lung cancer and various adenocarcinomas.[1][2][3] There are only four reported cases of Dr. PR reports grants and personal fees from Allergan, grants and personal fees from Abbvie, personal fees from Merz, UCB

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