Dr. Md. Rashidul Haque, MBBS, FCPS (Psychiatry)
Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease comprising hemiparesis, seizures, facial asymmetry, and mental retardation.
This rare condition derives its name from the researchers Dyke, Davidoff, and Masson who first reported the condition way back in 1933.
They described plain skull radiographic changes in 9 patients who presented with seizures, facial asymmetry, hemiparesis, and mental retardation.
Clinical Features:
- seizures
- facial asymmetry
- contralateral hemiparesis
- intellectual disability
Some authors divide the condition into two types mainly dependent on clinical presentation age.
- Infantile (congenital):
- 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.
- Acquired
- Main causes of acquired type are trauma, tumor, infection, ischemia, hemorrhage, and prolonged febrile seizures
Radiographic features:
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)
Differential diagnosis:
General imaging differential considerations include:
- hemimegalencephaly
- Sturge-Weber syndrome:
- 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.
- Rasmussen encephalitis: tends not to have calvarial changes
