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Dyke-Davidoff-Masson syndrome is a condition characterized by hemicerebral atrophy/hypoplasia secondary to brain insult usually in fetal or early childhood period and is accompanied by ipsilateral compensatory osseous hypertrophy and contralateral hemiparesis.
It is characterized by:
thickening of the skull vault (compensatory)
enlargement of the frontal sinus (also ethmoidal and mastoid air-cells)
elevation of the petrous ridge
ipsilateral falcine displacement
capillary malformations (are a novel finding for children with Dyke-Davidoff-Masson syndrome) 6
In some sources, it is equated to hemispheric infarction, whereas in other sources any cause of cerebral hemiatrophy is included.
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intellectual disability 7
Some authors divide the condition into two types mainly dependent on clinical presentation age 12
results from various etiologies such as infection, neonatal or gestational vascular occlusion involving the middle cerebral artery, unilateral cerebral arterial circulation anomalies, and coarctation of the mid aortic arch.
patient becomes symptomatic in the perinatal period or infancy.
main causes of acquired type are trauma, tumor, infection, ischemia, hemorrhage, and prolonged febrile seizures
Imaging spectrum includes varying degrees of cerebral hemiatrophy of the affected hemisphere (with dilatation of the ipsilateral lateral ventricle and ipsilateral sulcal prominence) accompanied by homolateral hypertrophy of the skull and sinuses. Elevation of the petrous ridge and ipsilateral falcine displacement may also be present.
The spectrum of findings include
Wallerian degeneration of the mesencephalon and middle fossa hypoplasia
atrophy in basal ganglia
atrophy in the brainstem
capillary malformations: may be detected in some situations
calvarial thickening (affected side)
hyperpneumatisation of mastoid cells (affected side)
History and etymology
It was initially described as changes in the skull seen on skull x-ray in patients with cerebral hemiatrophy but is now applied more broadly to cross-sectional imaging. It was initially described by C G Dyke, L M Davidoff and C B Masson in 1933 5.
General imaging differential considerations include:
can also be an association
almost always have facial port-wine stain (cutaneous nevus) in the territory of ophthalmic division (V1) of the trigeminal nerve 13
Rasmussen encephalitis: tends not to have calvarial changes
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