13 years old female patient with right-sided hemiparesis and convulsions.
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- atrophy of the left cerebral hemisphere with fronto-parietal cystic encephalomalacia and exvacuuo-dilatation of the left lateral ventricle.
- slight enlargement of the left frontal and sphenoidal sinuses is 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 their 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.
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 naevus (clinically).
- Piro E, Piccione M, Marrone G, Giuffrè M, Corsello G. Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery. Ital J Pediatr. 2013 May 14;39:32. doi: 10.1186/1824-7288-39-32.
- Singh P, Saggar K, Ahluwalia A. Dyke-Davidoff-Masson syndrome: Classical imaging findings. J Pediatr Neurosci. 2010 Jul;5(2):124-5. doi: 10.4103/1817-1745.76108.