Journal of Pediatrics & Child Care
Research Article
Progressive Cavitational Leukoencephalopathy: An Enigma
Divahia G1, Natalia MC2*, Sara L1 and Eugenia E3
1Pediatric Neurologist, Universidad Militar Nueva Granada,Colombia
2General Practitioner, Universidad Militar Nueva Granada,Colombia
3Pediatric Neurologist at Hospital Militar Central, Universidad Militar Nueva Granada, Colombia
*Address for Correspondence: Natalia MC, Universidad Militar Nueva Granada, Transversal 3 No.49-00, Bogotá, Colombia; E-mail: nataliamartinezc@hotmail.com
Submission: 27 January, 2021;
Accepted: 1 March, 2021;
Published: 5 March, 2021
Copyright: © 2021 Divahia G, et al. This is an open access article
distributed under the Creative Commons Attribution License, which
permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
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.
Patients and methods: A case of a female infant with regression
of motor skills and speech is described at 27 months, followed by
progressive neurological deterioration during the following year.
Results: In cranial MR findings, white matter compromise and cystic
degeneration with contrast enhancement were observed, along with
a brain MRS with double negative lactate spikes. Enzymatic study of
leukodystrophies, genetic panel for leukodystrophies and negative
mitochondrial Exome.
Conclusion: Due to the progressive form of the disease and paraclinical criteria, it is concluded that the patient meets the diagnostic criteria for PCL. This entity does not have treatment; only supportive care is available. The prognosis is fatal, with an average life of up to 14 years in the case studies described.