Season 8 Case 31

Hx: altered mental status

Season 8 Case 31

History: altered mental status. Weakness

image
image
image
image












Answer: Dyke-Davidoff-Masson Syndrome (DDMS) 

Early infection/infarction/insult to one of the cerebral hemispheres  -> hemi-cerebral atrophy + homolateral hypertrophy of the skull and sinuses (to fill the space) 

CT shows large area of encephalomalacia of the R MCA distribution + R calvarial thickening and hyperpneumatization of the frontal sinuses 

Bony findings are apparent on the X-ray, just a little more difficult to appreciate (in fact originally described on plain films I believe)

Clinically patients are young presenting with hemiparesis (contralateral of course), seizures and intellectual disability