[Tuberous sclerosis: neuropsychological implications]

The role of neurosurgical therapy in tuberous sclerosis is still controversial; generally, a surgical procedure is performed only to treat an increase in intracranial pressure secondary to an obstructive hydrocephalus caused by a neoplasm in the region of the foramen of Monro. The role of surgical treatment in the control of epileptic fits is much more limited because of multifocal EEG epileptogenic foci due to multiple cortical lesions. Recently some authors, however, have suggested that the surgical treatment may be useful in selected patients. In the present report 9 children affected by tuberous sclerosis who were operated on in the period 1982-1992, because of increased intracranial pressure are described. The neuroradiological examination showed tumors in the region of the foramen of Monro in all the patients, with secondary obstruction in the CSF shunt circulation. Operative procedures included CSF shunts and/or giant cell astrocytoma removal. The follow-up period ranged from 1 to 14 years (mean 6 years). According to our experience, tumor excision is the most satisfying therapy, allowing the removal of the cause of the hydrocephalus as well as the possibility of histological diagnosis. A CSF shunt procedure may resolve the symptomatology, but it is often weighted by frequent malfunctioning of the CSF shunt system due to catheter obstruction secondary to abnormally high CSF protein concentration; furthermore, in some patients the insertion of the ventricular catheter may induce intraventricular bleeding when the highly vascularized tumor is accidentally punctured during the procedure of shunt insertion or in cases in which the presence of the ventricular catheter causes chronic trauma on the tumoral surface.