Case Of the Week (COW) 08 June 2014
Answer:
Dyke-Davidoff-Mason Syndrome.
Findings:
Left cerebral hemiatrophy, ipsilateral thickening of skull vault and enlargement of frontal sinus.
Discussion:
Dyke, Davidoff, and Masson described the plain skull radiographic and pneumatoencephalographic changes in apatients characterized clinically by hemiparesis, seizures, facial-asymmetry, and mental retardation in 1933. The plain skull radiographic changes included thickening of calvarium and dilatation of ipsilateral frontal and ethmoid sinuses. Also, there was elevation of the greater wing of sphenoid and petrous ridge elevation of the greater wing of sphenoid and petrous ridge and upward tilting of planum- sphenoidale. In some sources it is equated to hemispheric infarction, whereas in other sources any cause of cerebral hemiatrophy are included. Its main differential considerations are Rasmussen encephalitis, where the calvarial changes are absent and Sturge Weber syndrome, which can also be an association.
Contributed By:
Dr. Iyappan Ponnuswamy, Dr. Ramesh Mohandoss
Kauvery Hospital, Chennai
Answer:
Dyke-Davidoff-Mason Syndrome.
Findings:
Left cerebral hemiatrophy, ipsilateral thickening of skull vault and enlargement of frontal sinus.
Discussion:
Dyke, Davidoff, and Masson described the plain skull radiographic and pneumatoencephalographic changes in apatients characterized clinically by hemiparesis, seizures, facial-asymmetry, and mental retardation in 1933. The plain skull radiographic changes included thickening of calvarium and dilatation of ipsilateral frontal and ethmoid sinuses. Also, there was elevation of the greater wing of sphenoid and petrous ridge elevation of the greater wing of sphenoid and petrous ridge and upward tilting of planum- sphenoidale. In some sources it is equated to hemispheric infarction, whereas in other sources any cause of cerebral hemiatrophy are included. Its main differential considerations are Rasmussen encephalitis, where the calvarial changes are absent and Sturge Weber syndrome, which can also be an association.
Contributed By:
Dr. Iyappan Ponnuswamy, Dr. Ramesh Mohandoss
Kauvery Hospital, Chennai