Neuroblastoma presenting as opsoclonus-myoclonus: A series of six cases and review of literature

Abstract

The opsoclonus-myoclonus ataxia syndrome (OMAS) also called "Kinsbourne syndrome" or "dancing eye syndrome" is a rare but serious disorder characterized by opsoclonus, myoclonus, and ataxia, along with extreme irritability and behavioural changes. Data on its epidemiology, clinical features, and outcome are limited worldwide. The aim of the study was to evaluate the clinical profile and outcome of children with OMAS. A retrospective data of all children presented to Pediatric oncology clinic with a diagnosis of opsoclonus-myoclonus from 2013 to 2016 were collected. 6 patients with a diagnosis of OMAS were presented over a 4-year period. All 6 cases had paraneoplastic etiology. All Children had good outcome without any relapse. Paraneoplastic opsoclonus myoclonus had a good outcome in our experience.

Keywords: Children; neuroblastoma; opsoclonus-myoclonus-ataxia syndrome; paraneoplastic syndrome.

Figure 1

(Case 2) (Original) Axial section showing well-defined enhancing soft-tissue mass lesion (3.5 cm × 2 cm × 1.4 cm) seen in the left upper psoas muscle extending into left neural foramen in between L2 and L3 vertebra up to dura mater

Figure 2

(a and b) (Case 3) Axial section and coronal section showing right paravertebral mass of size 1.5 cm × 0.6 cm × 2.4 cm at the level of D6 to D8 vertebral body

Figure 3

(a and b) (Case 5) Axial and coronal section showing well-defined left paravertebral mass lesion 2 cm × 1.8 cm × 2.9 cm at the level of D6 to D8 vertebral body