Paraneoplastic opsoclonus-myoclonus syndrome revealing T-cell lymphoma

BACKGROUND: Nonrhythmic involuntary ocular oscillations and axial and segme ntary myoclonia are associated in the opsoclonus-myoclonus syndrome. In adu lts, a paraneoplastic origin is generally found. We report the first of ops oclonus-myoclonus associated with non-Hodgkin's lymphoma. CASE REPORT: A 66-year-old woman rapidly developed a typical opsoclonus-myo clonus syndrome within a few hours, presenting vertigo, cerebellous ataxia, multidirectional involuntary ocular movements and nonrhythmic axial and se gmentary myoclonia. Brain computed tomography and magnetic resonance imagin g demonstrated discrete diffuse anomalies of the white substance predominat ing in the pens. The cerebrospinal fluid showed discrete lymphocytosis. Ant ineuron antibodies were negative. No cause could be identified until the de velopment 11 months later of pleomorphic T-cell mediastino-cervical lymphom a. The patient responded moderately to a CHOP regimen which had no effect o n the opsoclonus-myoclonus syndrome. Death occurred after a 16-month course due to pulmonary complications. DISCUSSION: Neuroblastoma and infectious causes predominate in opsoclonus-m yoclonus syndromes observed in children; in adults, the predominant cause i s cancer. Antineuron, anti-Ri and anti-Hu antibodies can be evidenced in so me cases, arguing in favor of a paraneoplastic mechanism. Recent reports ha ve evidenced MRI anomalies in the pens and the cerebellum, anatomically wel l correlated with the opsoclonus-myoclonus syndrome. Besides small-cell bro nchogenic anaplastic cancer, the possibility of cancer of the breast and ut erus, and both non-Hodgkin and Hodgkin lymphoma should be explored, knowing that cancer develops several months after the opsoclonus-myoclonus syndrom e.