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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