Dyke-Davidoff-Masson syndrome

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

History of left hemiplegia since the age of 4 months with right hemiparesis.

Patient Data

Age: 8 years
Gender: Male
mri

The MRI sequences demonstrate:

  • atrophy of the right cerebral hemisphere with temporofrontal cystic encephalomalacia, surrounding gliosis and ex-vacuo dilatation of the right lateral ventricle
  • compensatory thickening of the ipsilateral skull vault
  • moderate enlargement of the ipsilateral sphenoid sinus and mastoid air-cells

Case Discussion

The MRI features most consistent with Dyke-Davidoff-Masson syndrome with right cerebral hemiatrophy, cystic encephalomalacia with surrounding gliosis and compensatory thickening of the calvarium.

Dyke-Davidoff-Masson syndrome refers to hemicerebral atrophy/hypoplasia secondary to brain insult usually in the fetal or early childhood period and characterized by ipsilateral compensatory hypertrophy of the skull vault and contralateral hemiparesis.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.