[Two cases of Dyke-Davidoff-Masson syndrome and adult cerebral hemiatrophy].

BACKGROUND

cerebral hemiatrophy (CHA) can present in childhood or adulthood, if it presents before or after two years of age. This two entities differ in etiology, clinical presentation and imaging characteristics. The CHA of childhood or Dyke-Davidoff-Masson syndrome, is originated by intrauterine or perinatal insults that affect the perfusion of a single cerebral hemisphere, manifesting clinically by variable mental retardation, refractory epilepsy, facial asymmetry, hemiplegia/hemiparesis or abnormal movements of the contralateral extremities and by imaging studies, loss of volume in one cerebral hemisphere and ipsilateral compensatory cranial changes such as skull vault thickening, elevation of the orbital roof and petreous ridge, also hyperpneumatization of the frontal sinus and mastoid cells. Instead, the CHA of the adult is multifactorial and by imaging it only manifests as loss of volume in one cerebral hemisphere, without compensatory changes in the skull.

CASES REPORT: we present a classic clinical case of CHA secondary to perinatal insult and another case of CHA of adulthood secondary to multiple embolic strokes in a patient with inactive rheumatic heart disease, commenting the imaging differences of these rare clinical entities.