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

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.