Vertex extradural hematoma in association with Paget's disease of the skull.

Paget's disease of skull usually causes neurological complications such as basilar invagination, cranial neuropathies, etc. Occurrence of extradural hematoma in association with Paget's disease of skull is rare. A 48 year old man presented with headache and right upper limb weakness, two days after a fall from scooter. CT Scan of brain showed a large extradural hematoma at the vertex with the cranial vault showing features of Paget's disease. At surgery, no skull fracture or injury to the superior sagittal sinus was evident. There was diffuse oozing from the inner table of the skull, which showed features of Paget's disease. The extradural hematoma was evacuated and the patient made good recovery. He deteriorated a few hours after surgery. Follow up CT Scan showed diffuse brain swelling with minimal recollection of hematoma. Re-exploration showed a small recollection which was evacuated and the part of the oozing diseased skull was excised. The patient recovered completely. This is the first reported case of vertex extradural hematoma in association with the Paget's disease of skull. The unusual features are the absence of skull fracture and injury to the superior sagittal sinus. The increased vascularity of the skull due to Paget's disease has caused the hematoma by diffuse oozing from the inner table.

Desmoplastic infantile ganglioglioma -- a case report.

Desmoplastic infantile ganglioglioma is a very rare supratentorial tumor occurring in the first two years of life. A five-month-old female infant presented with recurrent seizures, large head and loss of acquired milestones. Computerized Tomographic Scan of brain showed a large subarachnoid cyst with a solid intensely contrast enhancing tumor in the right temporoparietal region with severe degree of mass effect. Craniotomy and total excision of the tumor followed subsequently by subduro-peritoneal shunt for the extracerebral fluid collection was done. The child made good recovery. Histopathology revealed features of desmoplastic infantile ganglioglioma, viz., marked desmoplastic component with glial and neuronal elements. Immunohistochemistry showed positive staining for glial fibrillary acidic protein (GFAP) with areas of synaptophysin and chromogranin positivity. Desmoplastic infantile ganglioglioma is a rare tumor of infancy, which has excellent prognosis after total excision. No adjuvant therapy is required. This is the first Indian report of desmoplastic infantile ganglioglioma out of less than fifty cases reported worldwide.