ATYPICAL PRESENTATION OF SPINAL CORD TUMORS IN CHILDREN

Objectives: Intramedullary spinal cord tumors account for 25–35% of spinal cord tumors in children. Diagnosis is often delayed because symptoms progress insidiously reflecting the generally benign pathology. The most common presenting symptoms are back pain, gait disturbance and scoliosis. We describe two children with intramedullary spinal cord tumors who had atypical presentations of focal dystonia and status epilepticus. Methods: Case 1: An 18 month old boy presented with early right hand preference at 10 months and dystonic posturing of the left fingers of one month duration. CT and MRI scans of the brain demonstrated a left subarachnoid cyst which did not explain the dystonia of the left hand. Spinal MRI demonstrated an intramedullary mass extending from C1 to T2. Case 2: A 4.5 year old developmentally normal boy presented with generalized tonic-clonic status epilepticus during a febrile illness. A CT demonstrated ventriculomegaly. The CSF protein was elevated. MRI of the brain and spine demonstrated an intramedullary spinal mass at T9-T10 with diffuse intracranial meningeal enhancement. Results: Case 1: Laminectomy and subtotal resection were performed. Histology revealed a Grade II Astrocytoma. There was no tumor progression at 24 months follow-up. Case 2: Three leptomeningeal biopsies suggested glioma not otherwise specified. A ventriculoperitoneal shunt was inserted and the child received chemotherapy. He progressed with a supratentorial mass 16 months later which was subtotally resected. The final diagnosis was primitive neuroectodermal tumor (PNET) with glial and neuronal differentiation infiltrating into leptomeninges and dura. Despite adjuvant chemotherapy and craniospinal radiation, the patient died 2.5 years after initial presentation. Conclusion: Focal dystonia is an unusual presentation of intramedullary spinal cord tumor. Seizures may indicate leptomeningeal disease, especially with elevated CSF protein and unexplained hydrocephalus. The primary spinal tumor may be asymptomatic. Spinal imaging should be considered when such clinical features cannot be explained by intracranial pathology.