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Old 06-06-2012
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Question Skull X Ray Diagnosis?

4 year old girl has history of hemiplegia, seizures and early onset glaucoma.
skull radiograph shows the following:

Skull X Ray Diagnosis?-image1.gif
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what it the likely diagnosis:

a. Churg Straus Syndrome
b. Encephalotrigeminal angiomatosis
c. Adrenoleukodystrophy
d. Krabbe's Disease
e. Werding Hoffmann Disease
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Old 06-06-2012
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Answer is B.....TRAM TRACK calcification in sturge-weber syndrome......
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Matched!!!
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Quote:
Originally Posted by anomali View Post
Answer is B.....TRAM TRACK calcification in sturge-weber syndrome......
whrz the tram track calcification? i dnt see anything whr the arrow is pointing ... n i never knew abt encephalotrigeminal angiomatosis.

whrz the question frm? its hard!
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says B too...
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Quote:
Originally Posted by numbndumb View Post
whrz the tram track calcification? i dnt see anything whr the arrow is pointing ... n i never knew abt encephalotrigeminal angiomatosis.
all the double lined markings are the calcifications in the arteries.... not just near the arrow but all over the hemispheres....
encephalotrigeminal angiomatosis is just a fancy name i guess... break it down.... it just means angiomas over the trigeminal supply of face a.k.a portwine stain, and angiomas in the brain...
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Correct Sturge Weber Syndrome is the answer.

The tram-track sign is produced by cortical calcifications that result from leptomeningeal vascular malformations found in patients with Sturge-Weber syndrome. The malformations consist of simple vascular structures situated along the space between the pia mater and the arachnoid membrane. A simpler theory suggests that the primary defect in Sturge-Weber syndrome occurs early during lamination, when there should normally be a persistent connection between the developing cortical veins (cerebral circulation) and the superior sagittal sinus (dural and calvarial circulation). If this communication is not maintained during the differentiation and separation of these two areas of circulation, the venous outflow from the cerebral cortex will be impaired. Because of poor venous drainage, the cerebral circulation in the affected areas becomes metabolically insufficient over time. The cerebral cortex underlying the areas of leptomeningeal vascular malformation usually becomes dysfunctional, progressively atrophic, and calcified


For further info check : http://radiology.rsna.org/content/231/2/515.full
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Nervous-System-, Pathology-, Radiographs-, Step-1-Questions, Syndromes-Acronyms

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