Answer for BIR CoW 24 Apr 2022
Sturge Weber Syndrome
Findings
Left cerebral and cerebellar hemispheres appears relatively atrophic. Hyperpneumatisation of left frontal sinus. Gradient blooming noted in left parietal gyrus reflecting subpial angiomatosis. Left choroid plexus appears bulky.
Discussion
Sturge weber syndrome is a phakomatosis characterised by facial port wine stains and pial angiomas. Imaging findings: CT: 1.Subcortical calcification at an earlier age than plain study and can also demonstrate parenchymal loss. 2.Calvarial and regional sinus enlargement may be evident 3.Ipsilateral choroid plexus may be enlarged. 4.Tram track sign of cortical and subcortical calcification 5.Orbital choroidal hemangioma may be present. MR: T1:Signal of affected region largely normal with anatomic volume loss evident at older age T2: Low signal in white matter subjacent to angioma representing-postulated accelerated myelination in neonate,calcification later in life. SWI:Sensitive to calcification,seen as regions of signal drop.
REFERENCES:
1.Elster AD, Chen MY. MR imaging of Sturge-Weber syndrome: Role of gadopentetate dimeglumine and gradient-echo techniques. AJNR Am J Neuroradiol.1990;11:685-689. 2.Benedikt RA, Brown DC, Walker R, et al. Sturge-Weber syndrome: Cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol. 1993;14:409-415. 3.Martí-Bonmatí L, Menor F, Poyatos C, Cortina H. Diagnosis of Sturge-Weber syndrome: Comparison of the efficacy of CT and MR imaging in 14 cases. AJR Am J Roentgenol. 1992;158:867-871.
Findings
Left cerebral and cerebellar hemispheres appears relatively atrophic. Hyperpneumatisation of left frontal sinus. Gradient blooming noted in left parietal gyrus reflecting subpial angiomatosis. Left choroid plexus appears bulky.
Discussion
Sturge weber syndrome is a phakomatosis characterised by facial port wine stains and pial angiomas. Imaging findings: CT: 1.Subcortical calcification at an earlier age than plain study and can also demonstrate parenchymal loss. 2.Calvarial and regional sinus enlargement may be evident 3.Ipsilateral choroid plexus may be enlarged. 4.Tram track sign of cortical and subcortical calcification 5.Orbital choroidal hemangioma may be present. MR: T1:Signal of affected region largely normal with anatomic volume loss evident at older age T2: Low signal in white matter subjacent to angioma representing-postulated accelerated myelination in neonate,calcification later in life. SWI:Sensitive to calcification,seen as regions of signal drop.
REFERENCES:
1.Elster AD, Chen MY. MR imaging of Sturge-Weber syndrome: Role of gadopentetate dimeglumine and gradient-echo techniques. AJNR Am J Neuroradiol.1990;11:685-689. 2.Benedikt RA, Brown DC, Walker R, et al. Sturge-Weber syndrome: Cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol. 1993;14:409-415. 3.Martí-Bonmatí L, Menor F, Poyatos C, Cortina H. Diagnosis of Sturge-Weber syndrome: Comparison of the efficacy of CT and MR imaging in 14 cases. AJR Am J Roentgenol. 1992;158:867-871.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!