LABRUNE SYNDROME (LEUKOENCEPHALOPATHY,CEREBRAL CALCIFICATIONS AND CYSTS) A CASE REPORT

Abstract

Triad of leukoencephalopathy, cerebral calcifications and cysts (LCC) is a recently
reported rare disease named Labrune syndrome after the first case was reported in 1996 by Labrune
et al. Herein, we report a 22-year-old female with LCC who had cognitive impairment presenting in
late adolescence.Clinically she had rare convulsive seizures, slowly progressive cognition impairment
and normal neurological status.On radiological examination, there were extensive calcifications in the
basal ganglia and the deep cerebral white matter, as well as parenchymal cysts and diffuse
hyperintensities of the white matter on T2-weighted sequences on MR imaging.The association of
leukoencephalopathy, cerebral calcification, and cysts (LCC) is a very rare entity, which was originally
described in 3 patients by Labrune et al 1996.1 The clinical presentation of this entity includes decline
of cognitive performance, rare convulsive seizures, and a mixture of extrapyramidal, cerebellar, and
pyramidal signs.1,2 Progressive calcifications in the basal and cerebellar gray nuclei and the central
white matter are found on CT. MR imaging reveals diffuse abnormal signals of the white matter on
T2-weighted sequences.1,2 A special feature is the development of parenchymal cysts in the
supratentorial compartment and cerebellum, leading to compressive complications and surgical
considerations.1,2In this report, a new patient with LCC is presented, and clinical, neurological and
radiological findings of this rare and recently described entity are discussed in view of the relevant
literature.