Presentation
Headache and numbness.
Patient Data
Three main findings in this case including:
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leukoencephalopathy
T2/FLAIR: diffuse hyperintensity in the white matter, especially prominent around cysts (due to vasogenic oedema).
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Calcifications
T1: multiple scattered calcific foci of variable intensities, some appear hypointense and others are hyperintense, prominent at the frontal periventricular regions
T2/FLAIR: they appear hypointense
SWI: they appear hypointense with blooming artifact
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Cysts
multiple cysts are seen scattered at the supratentorial white matter: centrum semiovale and cerebellum
They elicit low T1 and high T2 WI with no post-contrast enhancement
CT scan of the brain showed scattered calcifications within both cerebral hemispheres and cerebellum.
Bilateral cerebral white matter hypodensity denoting leukoencephalopathy with scattered cerebral and cerebellar cysts.
Case Discussion
Leukoencephalopathy with calcification and cysts, also known as Labrune syndrome, is a rare condition that consists of a triad of leukoencephalopathy, cerebral calcification, and oedematous cysts.
Here, in this case, the patient had a normal ophthalmological examination which excluded Coats plus syndrome.
Labrune syndrome is an extremely rare condition, with only a small number of cases reported in the literature.
The radiological triad of Labrune syndrome includes:
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leukoencephalopathy
T2/FLAIR: diffuse hyperintensity in the white matter, especially prominent around cysts (due to vasogenic oedema).
-
Calcifications
T1: multiple scattered calcific foci of variable intensities, some appear hypointense and others are hyperintense, prominent at the frontal periventricular regions
T2/FLAIR: hypointense
SWI: hypointense with blooming artifact
-
Cysts
multiple cysts are seen scattered at the supratentorial white matter: centrum semiovale, cerebellum, brainstem, and basal ganglia
They elicit a low T1 high T2 signal with possible ring enhancement.
This case is courtesy of Dr Ayda Yosif, Assistant Prof. of Diagnostic Radiology, Cairo University Children Cancer Hospital.
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