Presentation
Long-standing seizures not responding to antiepileptics. learning difficulties and progressive left-sided weakness.
Patient Data
Generalized right-sided cerebral hemiatrophy associated with extensive changes of cystic encephalomalacia involving the right frontotemporoparietal region along with ex vacuo dilatation of the right lateral ventricle, with atrophic changes affecting the right basal ganglia and right cerebral peduncle and ipsilateral falcine shift. Significant asymmetrical thickening involving the right side of the calvarium, along with ipsilateral sphenoid sinus enlargement, petrous ridge elevation and mastoid air cell hyperpneumatization.
Case Discussion
Dyke-Davidoff-Masson syndrome refers to cerebral hemiatrophy of varying degrees that could be congenital or acquired secondary to trauma, vascular conditions, intracranial hemorrhage or infection.
Patients may present with progressive hemiplegia/hemiparesis, refractory seizures, mental retardation, learning difficulties and facial asymmetry.
On imaging, common findings include cerebral hemiatrophy along with compensatory calvarial thickening, ipsilateral lateral ventricle enlargement, paranasal sinus enlargement and mastoid air cell hyperpneumatization, as well as elevation of the petrous ridge and ipsilateral falcine displacement.
Differential diagnoses may include chronic Rasmussen encephalitis or Sturge-Weber syndrome.
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