Presentation
Left-sided hemiparesis and convulsions.
Patient Data
Age: 20 years
Gender: Male
From the case:
Dyke-Davidoff-Masson syndrome
{"current_user":null,"step_through_annotations":true,"access":{"can_edit":false,"can_download":true,"can_toggle_annotations":true,"can_feature":false,"can_examine_pipeline_reports":false,"can_pin":false},"extraPropsURL":"/studies/102095/annotated_viewer_json?lang=us"}
Atrophy of the right cerebral hemisphere with frontoparietal and occipital cystic encephalomalacia surrounded by gliosis and ex-vacue dilatation of the right lateral ventricle.
Slight enlargement of the right frontal and sphenoidal sinuses.
Thickening of the skull vault in the right side with relatively much bone marrow compared to the left side.
No mass lesion. Normal sellar region. Normal posterior fossa.
Case Discussion
The imaging findings are consistent with Dyke-Davidoff-Masson syndrome (hemispheric infarction) with right cerebral hemiatrophy and encephalomalacia, likely due to in-utero vascular insult.
The differential diagnosis includes:
- 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.
- Sturge-Weber syndrome (progressive hemispheric atrophy and enhancement of pial angiomas, cortical calcifications, port-wine facial nevus).
Unable to process the form. Check for errors and try again.