DYKE-DAVIDOFF-MASSON SYNDROME SECONDARY TO VASCULAR COMPROMISE: A RARE CAUSE OF CEREBRAL HEMIATROPHY

Abstract

Dyke-Davidoff-Masson syndrome is a rare neurological condition resulting from brain insult during intrauterine life or in early childhood. Clinical presentation includes seizures, mental retardation, contralateral hemiparesis, and facial asymmetry.1 Crossectional radiology may show unilateral cerebral hemiatrophy/hypoplasia, ipsilateral ventricular enlargement, ipsilateral hyperpneumatization of the sinuses, and compensatory calvarial thickening.1 Being a rare case according to literature review,1 it may be easily missed clinically and radiologically. Here we report a case of an 8 year old male patient who preseted with right sided body weakness, generalized tonic clonic fits and mental retardation. MRI of the patient showed atrophy of left cerebral hemisphere along with dialatation of left lateral ventricle, enlargement of left frontal sinus and compensatory hypertrophy of left side of the clavarium, Wallerian degeneration of left hemi mid brain. The findings were suggestive of Dyke-Davidoff-Masson syndrome and most likely cause in this patient was vascular compromise.
Key words: Cerebral hemiatrophy, Dyke–Davidoff–Masson syndrome, seizures, hemiparesis.