Paraneoplastic Neurologic Syndromes in Children: A Review

Paraneoplastic neurological syndromes (PNS) were initially defined as neurological syndromes with unknown etiology that often associate with cancer. This broad definition may lead to misconception that any neurological syndrome, which coincides with a cancer might be considered as PNS. In the last two decades it has been suggested that PNSs are mainly immune-mediated. The detection of onconeural antibodies has been very helpful in indicating the existence of a tumor and defining a given neurological syndrome as paraneoplastic. However, PNS may occur without onconeural antibodies, and the antibodies can occur with no neurological syndrome; thus, their presence should not be the only condition to define a neurological syndrome as paraneoplastic. Diagnosis of paraneoplastic syndromes in children may result in early detection and treatment of the pediatric cancer and can reduce the neurological damage that is the major source of morbidity in children with successfully treated tumors. This study reviews the presenting symptoms, immunology, and management options for paraneoplastic syndromes, focusing on those most commonly reported in children.