Labrune syndrome
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 edematous 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:
- leukoencephalopathy
- T2/FLAIR: diffuse hyperintensity in the white matter, especially prominent around cysts (due to vasogenic edema).
- 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 artefact
- 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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