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337 SHORT COMMUNICATION Paraneoplastic Opsoclonus-Myoclonus Syndrome Preceding a Mucosal Malignant Melanoma Flora DRESCO 1 , François AUBIN 1 *, Elise DEVEZA 1 , Eugeniu REVENCO 2 , Laurent TAVERNIER 3 and Eve PUZENAT 1 Department of Dermatology, University Hospital and University of Franche Comté, EA3181, 3 Bd Fleming, FR-25030 Besançon, 2 Department of Neurology, and 3 Department of Ear, Nose and Throat, University Hospital, Besançon, France. E-mail: [email protected] 1 Accepted Oct 3, 2018; E-published Oct 3, 2018 Paraneoplastic opsoclonus-myoclonus syndrome (POMS) is most commonly associated with neuroblas- toma in children, but it also occurs with a variety of neoplasms in adults, particularly small-cell lung cancer and breast cancer (1). We report here a case of POMS that led to the finding of a non-metastatic mucosal melanoma. examination confirmed a locally advanced malignant melanoma. Surgical resection was performed, but was incomplete. The patient progressed to coma within 2 weeks and died 8 months after the onset of neurological symptoms due to complications associated with the POMS (aspiration pneumonia). CASE REPORT DISCUSSION Neurological paraneoplastic syndromes, including A 69-year-old man was admitted to the emergency unit melanoma-associated retinopathy (1), dermatomyositis for subacute and intense dizziness, associated with nau- (2) and POMS are uncommon in patients with melanoma. sea and vomiting. Physical examination revealed vertigo POMS is most commonly associated with neuroblastoma with truncal and limb ataxia, in relation to a cerebellar in children, but it also occurs with a variety of neoplasms syndrome, orthostatic myoclonic jerks and characterized in adults, particularly small-cell lung cancer and breast arrhythmic, multidirectional, continuous involuntary cancer (3). To our knowledge, only 3 melanoma-associ- eye movements (even during sleeping) consistent with ated POMS have been reported (4–6). opsoclonus. Further examinations, including laboratory Opsoclonus is defined by the presence of spontaneous, investigations (autoimmune tests), cerebrospinal fluid arrhythmic and large amplitude conjugate saccades oc- analysis, serological tests for HIV, hepatitis B, C and curring in all directions of gaze, without saccadic interval. Lyme’s disease, neuronal antibodies in blood and cere- Opsoclonus is usually associated with myoclonus of the brospinal fluid, radiological investigations (chest X-ray, limbs and trunk, and sometimes, with encephalopathy. abdominal and pelvis computed tomography (CT) scan, POMS is observed in 3 clinical settings (3): (i) paediatric positron emission tomography (PET) scan, brain magne- tic resonance imaging (MRI)) and functional tests (videonystagmography, audiometry) were normal. Based on these data, the diagno- sis of post-infectious opsoclonus-myoclonus syndrome was initially made and the patient was treated with monthly intravenous im- munoglobulins (IVIG). No improvement was observed after 4 months, therefore the investigations were repeated. Although the nasal endoscopy was normal, the PET scan now showed a positive uptake in the left nasal cavity. Brain MRI was normal. Systemic cor- ticosteroids were added to IVIG. Two months later, the patient presented with worsening of the neurological syndrome, including an increase in opsoclonus and cerebellar ataxia, and the appearance of a confusional state and dysarthria, responsible for a major loss of autonomy. A new PET scan was performed and confirmed an increase in the uptake pre- viously observed in the left nasal cavity (Fig. 1a). Brain scan was normal. Nasal endoscopy finally demonstrated a friable bleeding lesion Fig. 1. (a) Increase in uptake in repeated fluorodeoxyglucose postitron emission under the left inferior turbinate and inside tomography/ computed tomography (FDG PET/CT) during follow-up. (b) Nasal endoscopy: the middle turbinate (Fig. 1b). Pathological mucosal melanoma in the left nasal cavity. This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3062 Acta Derm Venereol 2019; 99: 337–338