Abstract

Progressive Cavitational Leukoencephalopathy: An Enigma

Highlights

  • Progressive Cavitational Leukoencephalopathy (PCL) is a recently described pathology, with few cases reported in the world literature

  • In 2005 Naidu et al [2] characterized an infantile neurodegenerative syndrome with pictures of acute clinical deterioration associated with asymmetric patchy leukoencephalopathy in neuroimaging studies along with cavities and vascular permeability, found initially on corpus callosum and centrum semiovale, and developing cystic degeneration; This entity was called Progressive Cavitational Leukoencephalopathy

  • The second patient was a female born to consanguineous parents, who showed rapid regression in motor and visual abilities later than 6 months, as well as evolutionary spastic quadriparesia, decreased visual acuityand pupillary reflex; her MRI showed extensive bilateral leukocyencephalopathy with corpus callosum and middle cerebellar peduncles involved, and large cavitaties in the deep white matter

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Summary

Introduction

Progressive Cavitational Leukoencephalopathy (PCL) is a recently described pathology, with few cases reported in the world literature. It is characterized by progressive neurological deterioration and characteristic neuroimaging findings, which distinguish this disorder as a unique entity given the massive cystic degeneration. In 2005 Naidu et al [2] characterized an infantile neurodegenerative syndrome with pictures of acute clinical deterioration associated with asymmetric patchy leukoencephalopathy in neuroimaging studies along with cavities and vascular permeability, found initially on corpus callosum and centrum semiovale, and developing cystic degeneration; This entity was called Progressive Cavitational Leukoencephalopathy Open Access Research Article. Contrast enhancement and involvement of the corpus callosum makes it different from other pathologies, such as Vanishing White Matter Disease (VWMD) [3]. An autosomal recessive inheritance related to a history of first degree consanguinity has been suggested; even though its pathophysiology is not yet clear, mitochondrial DNA mutations may be involved [1]

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