Dyke-Davidoff-Masson syndrome (DDMS) 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
- facial asymmetry
- contralateral hemiparesis
- 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 brain stem
- 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:
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- 11. Ayas ZÖ, Asil K, Öcal R. The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults. (2017) Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 38 (10): 1823-1828. doi:10.1007/s10072-017-3074-7 - Pubmed
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