Dyke-Davidoff-Masson syndrome

Case contributed by Dr Mohammad A. ElBeialy

Presentation

13 years old female patient with right-sided hemiparesis and convulsions.

Patient Data

Age: 13 years
Gender: Female
MRI
  • atrophy of the left cerebral hemisphere with fronto-parietal cystic encephalomalacia and exvacuuo-dilatation of the left lateral ventricle.
  • slight enlargement of the left frontal and sphenoidal sinuses is seen as well as relative thickening of the skull vault with relatively much bone marrow compared to the right side.
  • no mass lesion.
  • normal sellar region.
  • normal posterior fossa.
  • MR angiography shows:
    • mild attenuation of the M2 and M3 segments of the left MCA with decreased their arborization.
    • otherwise, normal rest of the cerebral arteries as well as intra-cranial carotid arteries.
    • no A-V malformation or aneurysmal dilatation.
    • normal vertebro-basilar system.

Case Discussion

The above described findings are consistent with Dyke-Davidoff-Masson syndrome (hemispheric infarction) with left cerebral hemiatrophy and fronto-parietal encephalomalacia, likely due to in-utero vascular insult.

The differential diagnosis includes:

Chronic 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 naevus (clinically).
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Case information

rID: 23563
Case created: 23rd Jun 2013
Last edited: 20th Nov 2015
Inclusion in quiz mode: Included

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