Steroid-Responsive Encephalopathy, Dropped Head Syndrome, and Hypertension in a Toddler: Is There a Clue?

BACKGROUND: Paraneoplastic manifestations may provide an early clue to underlying malignancies in children.

METHODS: We describe a 3-year-old girl with an unusual neurological paraneoplastic syndrome of steroid-responsive encephalopathy and dropped head syndrome associated with paraspinal neuroblastoma.

RESULTS: Cranial neuroimaging, metabolic screen, and thyroid functions were normal. Abdominal imaging identified a paraspinal neuroblastoma. Encephalopathy and neck extensor weakness responded to pulse corticosteroids followed by tumor resection.

CONCLUSIONS: This is a unique child with paraneoplastic steroid-responsive encephalopathy and dropped head syndrome in association with neuroblastoma.