Acquired cerebral hemiatrophy: Dyke-Davidoff-Masson Syndrome - a case report.

A rare syndrome, Dyke-Davidoff-Masson Syndrome (DDMS), with a diagnostic conundrum, and the way it was solved is presented. A 13-year-old boy presented with recurrent seizures for the past 10 years. He had been treated with anticonvulsant medication which was satisfactory at first but later the seizures recurred. Recently, the frequency of the seizures increased with preictal dizziness and postictal drowsiness. Physical examination revealed mild left hemiparesis and left deviated gait irregularity. He was mentally alert but had not achieved all the developmental milestones as compared to normal child of his age. CT and MRI scan of the head showed hemiatrophic cerebral parenchyma with prominent sulci and encephalomalacia. 24-hour intensive video EEG monitoring revealed suppression of alpha rhythm and local slow wave activity on the side of the atrophic hemisphere. PET-CT showed highly functional left cerebral hemisphere and less functional right cerebral hemisphere. The patient underwent functional hemispherectomy under neurophysiological monitoring and the nonfunctional brain tissues were resected while selectively preserving the functional areas detected by fMRI and PET-CT scan. During follow up, the patient was seizure free as well as without difficulties in performing his daily activities and communications. Functional hemispherectomy for DDMS patient has a good prognosis.

Gamma knife treatment of an endolymphatic sac tumor: unique features of a case and review of the literature.

Endolymphatic sac tumors are rare, histologically non-aggressive lesions, but often become locally invasive and lead to erosion of the petrous bone. We report a rare case with multiple endolymphatic sac tumors located at bilateral cerebellopontine angles and vermis of the cerebellum. One of the lesions was surgically resected and others were treated using gamma knife radiosurgery. Local recurrence and metastasis occurred, which was treated with gamma knife radiosurgery. With an appropriate prescription dose and isodose, gamma knife radiosurgery may have an important role in the treatment of endolymphatic sac tumor.

Intracranial clear-cell meningioma.

Intracranial clear-cell meningioma (CCM) is rarely reported in the literature since it has to be distinguished from other subtypes of meningioma. Most of the CCMs are intraspinal, according to the related literature. We report a case of occipital parietal CCM in a 6-year-old child, review all the 35 intracranial CCMs that have been reported since 1995 to present and discuss their clinical, radiological and histopathologic characteristics.