Abstract
The triad of leukoencephalopathy, cerebral calcifications, and ysts (LCC) represents a very rare, and distinct clinico-radiologic ntity of unknown etiology, first described in children by Labrune t al. [1] in 1996. The onset ranges from infancy to adults 1–5], with varying clinical presentations including a combinaion of mild cognitive dysfunction or normal intelligence, seizures, erebellar, pyramidal and extrapyramidal signs. The disorder s characterized by diffusely progressive basal ganglia, brain tem and subcortical white matter calcifications, cerebellar, and upratentorial parenchymal cysts, as well as diffusely abnormal 2-weighted sequences on MRI [1–4]. The white matter abnoralities were described as a leukoencephalopathy rather than demyelinating process (leukodystrophy) [2]. Histopathologic xamination reveals angiomatous-like blood vessels, gliosis, and eaded, corkscrew-shaped intracytoplasmic inclusions typically ssociated with highly gliotic tissue surrounding cysts and vascuar malformations (Rosenthal fiber formation) in the white matter 1,3,4]. Complications are typically a consequence of mass effect ncluding cerebral edema leading to intracranial hypertension, apilledema, syringomyelia and eventually tonsillar herniation [4].
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