Dyke-Davidoff-Masson syndrome

Case contributed by Tariq Walizai
Diagnosis probable

Presentation

The patient has been suffering from fits since childhood, followed by right-sided weakness.

Patient Data

Age: 7 years
Gender: Female

There is marked atrophy of the left cerebral hemisphere with evidence of fronto-parietal cystic encephalomalacia and ex-vacuo dilatation of the ipsilateral lateral ventricle.

Remodeling of the overlying calvarium is seen in left side.

The right-sided cerebral hemisphere is grossly unremarkable.

Note, however, that the images have not been obtained in the true axial plane but rather significantly tilted. This markedly accentuates the apparent asymmetry of the skull vault.

Case Discussion

CT findings are most likely suggestive of Dyke-Davidoff-Masson syndromeas described above, although the positioning of this scan makes this much harder to confirm.

Co-contributor: Dr. Anwar-ul-Haq Zadran.

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