Presentation
Headache and numbness.
Patient Data
Three main findings in this case including:
- 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: they appear hypointense
- SWI: they appear hypointense with blooming artefact
- 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 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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