Paraneoplastic Opsoclonus-Myoclonus-Ataxia (OMA) syndrome in an adult patient with esthesioneuroblastoma

Abstract

JO N 2 74 1 that occur in all directions and brief multifocal myoclonic muscle jerks usually accompanied by cerebellar dysfunction with dysarthria and ataxia [1, 2]. OMA is associated with multiple etiologies: paraneoplastic, post-infectious [3], celiac disease [4], pregnancy [5], postvaccination [6] and idiopathic [1]. Paraneoplastic OMA in adults has been associated with a variety of tumors but most often with small cell lung cancer and breast cancer, while in children OMA is usually associated with neuroblastoma [1]. We report the history of a 51 year-old female who presented with a subacute onset of opsoclonus, vertigo, vomiting, severe ataxia of the four limbs, dysarthria and sporadic myoclonic muscle jerks. MRI of the brain revealed a tumor of the nasal septum and the ethmoidal sinuses. Two weeks prior to admission, the patient underwent a biopsy of a polypoid lesion in the right nasal cavity. Pathology revealed a grade 4 esthesioneuroblastoma, with a high degree of lymphoid infiltration. Some of the tumor cells were grouped in rosettes. Diagnosis was confirmed by immunohistochemistry: tumor cells showed a nuclear immunoreactivity for Ki 67, for synaptofysine, Leu-7 pancytokeratine and neurofilamen protein. They showed no immunoreactivity for chromogranine, TTF1, CD45, CD34 and CD 99. The tumor cells were surrounded by S100 immunoreactive sustenticular cells. We determined after onconeural antibodies in the peripheral blood: anti-Hu, anti-Yo and anti-Ri were absent. The patient had no oncologic antecedents and PET scan demonstrated no other tumor locations. Based on a case report of a child with neuroblastoma linked OMA [7] we treated our patient with high dose methylprednisolone (1 g/d) for 10 days. The patient showed a progressive positive neuro logical Dominique Van Diest Hendrik De Raeve Jozef Claes Paul M. Parizel Dirk De Ridder Patrick Cras