Parainfectious opsoclonus-myoclonus syndrome: high dose intravenous immunoglobulins are effective

Opsoclonus-myoclonus syndrome (OMS) is a rare neuro-ophthalmical disorder that affects children more often than adults. Opsoclonus is characterised by involuntary, irregular, but conjugate saccadic eye movements either multidirectional or horizontal (“ocular flutter”) precipitated by change of fixation.1 Pathophysiologically, a disordered interaction of “burst” and “omnipause” cells located in the brain stem has been suggested.2 The associated myoclonus is typically exacerbated by muscle activation and predominantly involves the face, limbs, and trunk. Among various aetiologies of OMS, paraneoplastic, paraviral or idiopathic encephalitis are the most common causes and an autoimmune mediated brain stem dysfunction is the suggested underlying pathomechanism.3 Tumour related childhood OMS occurs predominantly in association with neuroblastoma and ganglionneuroblastoma. Paraneoplastic OMS in adults may evolve with lung, breast or uterus cancer, or neuroblastoma.2 Parainfectious and idiopathic forms account for about 50% of cases.1 As OMS is rare there is no standard treatment recommendation. Some cases resolve spontaneously or with symptomatic treatment including clonazepam, valproic acid, piracetam, thiamine, reserpine, chlormethiazole. ACTH seems to be the treatment of choice in children with paraneoplastic OMS whereas in adult onset OMS the role of immunotherapy is less well established.2 Here we report a patient with parainfectious OMS whose symptoms were well controlled with repeated administration of high dose intravenous immunoglobulin (Ig). A few …