Dyke-Davidoff-Masson syndrome

Case contributed by Dr Ammar Haouimi

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.

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