Abstract
Background and Objectives: The opsoclonus-myoclonus syndrome (OMS) is characterised by opsoclons, myoclons and impaired balance, often concomitant with sleep disorder and behavioural difficulties. The symptoms develop as a result of autoimmune response triggered by a neuroblastic tumour (NT). OMS can also develop secondarily to a viral infection or as an immune response triggered by an unknown agent. This leads to the activation of B- and T-cells, which produce and release autoantibodies or cytokines directly within the central nervous system (CNS), thus damaging the neurons within the cerebellum and the brain stem. The available OMS treatments aim at decreasing lymphocyte, cytokine and autoantibody production or accelerating the utilisation of the latter. Another treatment option for OMS involves using cytostatic agents, which damage T- and B-cells causing their depletion and impaired function, which reduces their ability to produce antibodies and cytokines. Materials and Methods: We present a single-centre experience in treating OMS secondary to NT in 7 children. Results: The combined treatment with cyclophosphamide plus dexamethasone resulted in a complete resolution of OMS symptoms in 4 children, and a significant improvement in the 3 children. Two of them periodically present hyperactivity, and one girl requires an additional support at school due to special educational needs (SEN). Conclusions: NT resection does not resolve OMS in children with OMS secondary to NT. The combined treatment with dexamethasone plus cyclophosphamide seems to be an effective treatment of OMS.
Highlights
Opsoclonus-myoclonus syndrome (OMS) is a clinical syndrome of an undetermined, most likely autoimmune, aetiology [1,2,3,4,5]. It is characterised by opsoclony, myoclony and impaired balance, often concomitant with sleep disorder and behavioural difficulties
In 40–80% of paediatric patients with neuroblastic tumour, such as neuroblastoma, ganglioneuroblastoma or ganglioneuroma, opsoclonus-myoclonus syndrome could be the first sign of this type malignancy [6,7,9]
Prior to neuroblastic tumour (NT) diagnosis, all 7 children diagnosed with opsoclonus-myoclonus syndrome (OMS) reported presented neurological symptoms in keeping with OMS of variable severity
Summary
Opsoclonus-myoclonus syndrome (OMS) is a clinical syndrome of an undetermined, most likely autoimmune, aetiology [1,2,3,4,5] It is characterised by opsoclony, myoclony and impaired balance, often concomitant with sleep disorder and behavioural difficulties. The opsoclonus-myoclonus syndrome (OMS) is characterised by opsoclons, myoclons and impaired balance, often concomitant with sleep disorder and behavioural difficulties. OMS can develop secondarily to a viral infection or as an immune response triggered by an unknown agent. This leads to the activation of B- and T-cells, which produce and release autoantibodies or cytokines directly within the central nervous system (CNS), damaging the neurons within the cerebellum and the brain stem. Materials and Methods: We present a single-centre experience in treating
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