Whenever you see foci of cortical and subcortical increased T2 signal with intracranial calcification, tuberous sclerosis is a good thought. In this case however there are no subependymal calcifications which are more characteristic of tuberous sclerosis. Subcortical calcifications may be present in tuberous sclerosis however. These represent calcified hamartomas. Furthermore, enhancement and confluent deep white matter of T2 signal abnormality are not characteristic of tuberous sclerosis. Demyelination may be considered; however, these lesions would not be typical and this diagnosis will not explain the intracranial calcifications. Given the presumed pathogenesis of the disease including a vasculopathy with meningoencephalitis, the findings of enhancing lesions with confluent signal abnormality would be compatible those processes. Related Cases
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Terstegge K., Kunath B, Felber S, Speciali JG, Henkes H, Hosten N. MR of brain involvement in progressive facial hemiatrophy (Romberg disease): reconsideration of a syndrome. AJNR, Jan 1994; 15(1):145-50.
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|Tuberous sclerosis||CNS vasculitis||Tuberous sclerosis|