IJAR.2019.216

Type of Article:  Case Report

Volume 7; Issue 3.1 (July 2019)

Page No.: 6748-6750

DOI: https://dx.doi.org/10.16965/ijar.2019.216

BILATERAL SYMMETRICAL LISSENCEPHALY WITH PACHYGYRIA: A CASE REPORT

Bishwajeet Saikia *1, Bipul Kumar Das 2, Pranjal Phukan 3.

*1 Assistant Professor, Department of Anatomy, North Eastern Indira Gandhi Regional Institute of Health and Medical   Sciences, Shillong, Meghalaya, India

2 Registrar, Department of Pediatrics, Guwahati Medical College and Hospital, Guwahati, Assam, India.

3 Associate Professor, Department of Radiology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India.

Corresponding Author: Dr. Bishwajeet Saikia, Assistant Professor, Department of Anatomy North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong–793018, Meghalaya, India (Phone: 91-9401059365) E-Mail: bishwajeetsaikia3@gmail.com

ABSTRACT:

Background: Lissencephaly is a rare developmental disorder characterized by absence of cerebral convolutions. Pachygyria (broad gyri) or agyria (no gyri) are terms used to describe appearance of cerebral surface. Together these associated conditions are a part of congenital cortical malformations and may result due to arrest of brain development before third or fourth month of gestation. Patients suffering from these conditions presents with significant developmental delays which further depends on the degree of malformation. Results: We report a case of bilateral symmetrical extensive lissencephaly with pachygyria. The major MRI findings during evaluation of our case were smooth gyral pattern with thickened cortex, thinning of periventricular white matter and prominent VR (Ventricular) spaces.

Conclusion: These defects can be idiopathic, associated with chromosomal abnormalities LIS 1 (chromosome 17) or can be to environmental factors (prenatal drugs or intrauterine perfusion failures). In our case Chromosome 17 defect was suspected as the parieto-occipital regions were more involved.

Key words: Lissencephaly, Pachygyria, Smooth gyral pattern, LIS 1.

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Cite this article: Bishwajeet Saikia, Bipul Kumar Das, Pranjal Phukan. BILATERAL SYMMETRICAL LISSENCEPHALY WITH PACHYGYRIA: A CASE REPORT. Int J Anat Res 2019;7(3.1):6748-6750. DOI: 10.16965/ijar.2019.216