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.
West China Hospital, West China School of Medicine, Sichuan University, Department of Neurosurgery, Chengdu, China. shresthabal2003@gmail.com
Balkrishna
B
Shrestha
D000293:Adolescent; D001284:Atrophy / Q000175:diagnosis / Q000601:surgery; D001927:Brain Diseases / Q000175:diagnosis* / Q000601:surgery; D019465:Craniofacial Abnormalities / Q000175:diagnosis* / Q000601:surgery; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D049268:Positron-Emission Tomography; D013577:Syndrome; D014057:Tomography, X-Ray Computed