Presentation
History of left hemiplegia since the age of 4 months with right hemiparesis.
Patient Data
Age: 8 years
Gender: Male
From the case:
Dyke-Davidoff-Masson syndrome
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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.