Presentation
Right-sided hemiparesis and convulsions.
Patient Data
Age: 13 years
Gender: Female
From the case:
Dyke-Davidoff-Masson syndrome
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- atrophy of the left cerebral hemisphere with fronto-parietal cystic encephalomalacia and ex-vacuo dilatation of the left lateral ventricle
- slight enlargement of the left frontal and sphenoidal sinuses are seen as well as relative thickening of the skull vault with relatively much bone marrow compared to the right side
- no mass lesion
- normal sellar region
- normal posterior fossa
-
MR angiography shows:
- mild attenuation of the M2 and M3 segments of the left MCA with decreased arborization
- otherwise, normal rest of the cerebral arteries as well as intra-cranial carotid arteries
- no A-V malformation or aneurysmal dilatation
- normal vertebro-basilar system
Case Discussion
The above described findings are consistent with Dyke-Davidoff-Masson syndrome (hemispheric infarction) with left cerebral hemiatrophy and fronto-parietal encephalomalacia, likely due to in-utero vascular insult.
The differential diagnosis includes:
Chronic Rasmussen encephalitis (chronic, progressive, unilateral inflammation of brain of uncertain etiology with hemispheric volume loss and intractable focal seizure activity ) however, Rasmussen encephalitis tends not to have calvarial changes.
- progressive hemispheric atrophy and enhancement of pial angiomas
- cortical calcifications
- port-wine facial nevus (clinically)
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