An Adult With Dyke-Davidoff-Masson Syndrome: A Case Report

Abstract

Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease affecting the brain with almost 100 cases previously reported, with only 21 cases among adults. Due to the intricacy of clinical manifestations and radiological findings, it is difficult to reach the diagnosis. It usually includes atrophy of the cerebral hemisphere, dilation of the lateral ventricle, hypertrophy of skull bones, and hyperpneumatization of air sinuses. Herein, we present a case of a 55-year-old female patient who presented with a new-onset seizure. This case emphasizes the importance of considering DDMS in the differential diagnosis of adult-onset seizures, especially in patients with a previous history of brain insult, and demonstrates the possibility of developing this condition despite the lack of childhood symptoms. To our knowledge, this is the first case reported in Jordan.

Keywords: adult presentation; case report; cerebral hemiatrophy; dyke–davidoff–masson syndrome; seizure.

Figure 1. A: Encephalomalacia of the left frontoparietal lobes corresponding to a previous infarction of the left MCA territory. B: Associated skull vault thickening.

Figure 2. Mild gliosis associated with encephalomalacia (arrow).

Figure 3. Ex vacuo dilatation of the occipital horn of the lateral ventricle (arrow).

Figure 4. A: Hypertrophied left frontal sinus. B: Hypertrophied left mastoid air sinus.

Figure 5. Elevation of the left petrous bone (arrow).