Opsoclonus-myoclonus syndrome in a patient with an anaplastic oligoastrocytoma.

To the Editor, A 35 year-old woman presented to our emergency department presenting with acute onset of dizziness, inability to stand, and ‘‘eye fluttering’’ that disrupted her vision. Her neurological exam revealed opsoclonus and action-induced myoclonus affecting her legs, leading to inability to stand with two-person assistance. A diagnosis of opsoclonusmyoclonus syndrome (OMS) was made, a rare condition characterized by unpredictable, multidirectional, involuntary saccades and myoclonic jerks in the limbs. Eleven years earlier she was diagnosed with a left frontal anaplastic oligoastrocytoma after presenting with a seizure. Molecular analysis demonstrated 1p19q co-deletion, and she underwent treatment with neo-adjuvant temozolomide, followed by treatment at recurrences that included radiation therapy, three additional resections and procarbazine, lomustine, and vincristine (PCV). Her 3rd and final cycle of PCV ended 1 month prior to presentation. Her concurrent medications at presentation included dexamethasone, ondansetron, lamotrigine, levetiracetam, clobazam, atovaquone and rare use of oxycodone-acetaminophen. On admission for OMS, brain MRI demonstrated stable tumor without any new abnormality. Lumbar puncture demonstrated normal intracranial pressure and CSF profile notable for 0 RBCs, 2 WBCs per lL, mildly elevated protein 58 mg/dL (upper limit of normal 45), and normal glucose at 66 mg/dL. Serum and CSF studies for reported parainfectious or autoimmune etiologies of OMS were all normal or negative, while transvaginal ultrasound and computed tomography of the chest, abdomen, and pelvis did not reveal other malignancy. To our knowledge, neither temozolomide nor PCV have been reported to cause OMS. Clonazepam provided mild symptomatic relief. Empiric treatment with high-dose methylprednisolone 9 5 days and intravenous immunoglobulin 9 5 days were started on hospital days 3 and 7, respectively. She dramatically improved after completing the course of IVIg by 11 days after presentation. Symptoms had resolved completely by 1 month, and yet to recur over 6 months later. However, less than 2 months later her tumor progressed, for which she was started on bevacizumab monotherapy. Her glioma remained confined to the left cerebral hemisphere without clear dissemination to the brainstem except for minor infiltrative tumor evident in the left cerebral peduncle. In adults, OMS has been associated with several malignancies, including small cell lung cancer, breast carcinoma, melanoma, and ovarian neoplasm [1]. Paraneoplastic antibodies have been detected in 33 % of adults with OMS and cancer [1]. Antineuronal nuclear antigen type 2 (ANNA-2; anti-Ri) represents the paraneoplastic antibody most commonly found in individuals with OMS [1]. All such evaluations were unrevealing in this case. In women particularly, ovarian teratomas are a common cause [2], but ruled out by transvaginal ultrasound in our case. To our knowledge, this is the first report of a patient with a primary brain tumor who developed any paraneoplastic neurological syndrome, including OMS, limbic encephalitis, stiff-person syndrome, or cerebellar degeneration [3]. Despite finding no alternative etiology, we acknowledge that it is possible that our patient’s OMS could be unrelated to her & Yazmin Odia yo2240@cumc.columbia.edu