Abstracts

Rationale: Describe a case of Desmoplastic Infantile Ganglioglioma presenting as partial status epilepticus in a newborn with review of the literature. Methods: We describe the presentation and clinical management of a newborn girl who presented on day of life one with focal intractable seizures. Video EEG showed nearly continuous left central electrographic seizures, unresponsive to Phenobarbital , Phenytoin necessitating midazolam drip to control the seizures. MRI showed a rounded lesion in the left posterior frontal-parietal peri-rolandic region, abutting the ependymal margin of the body of the left lateral ventricle. Biopsy of the lesion was performed and showed a low grade neuronal-glial tumor, consistent with ganglioglioma. Left frontal craniotomy was performed with complete resection of the lesion. The patient who is now 5 months old has remained seizure free on one antiepileptic medication with mild right hemiparesis and normal development. Review of literature was performed on this rare condition to compare clinical presentation, neuroradiological and histological findings and management. Results: Desmoplastic Infantile Gangliogliomas are rare supratentorial tumors, with the large majority diagnosed in the first 24 months. Boys are affected more commonly than girls. Multiple lobe involvement is common, with a predilection for frontal and parietal lobes. Symptomatology usually involves macrocephaly, bulging fontanelle, lethargy and frequent seizures. Total resection is curative and disease-free survival has been reported to be 8-20 years. Conclusions: Desmoplastic Infantile Ganglioglioma is a rare desmoplastic supratentorial neuroepithelial tumor that accounts for 0.5%-1% of all intracranial tumors. Most patients present with new onset seizures and the large majority present within the first year of life. An accurate diagnosis and intervention is paramount because it carries a different prognosis from other malignant brain tumors with similar neuroradiological and histological findings.