https://www.ijmhr.org/ijar.htm editor_anatomy@ijmhr.org
ISSN:       2321-4281 (E) 2321-8967 (P)

IJAR.2019.216

DOWNLOAD PDF
Table of Contents

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.

REFERENCES

  1. Daube JR, Chou SM: Lissencephaly: Two cases. Neurology (Minneap) 1966 ; 16: 179-191.
  2. Grossman RI, Yousem DM: Congenital Disorders of the Brain and Spine Neuroradiology, 2nd edition. Elsevier Inc. USA; 2003: 411-467.
  3. Hanaway J, Lee SI, Netsky MG: Pachgyria; Relation of findings to modern embryologic concepts. Neurology (minneap) 1968 ; 18: 791-199.
  4. Allen MV, Clarrean SK: A spectrum of gyral anomalies in Miller-Dieker (lissencephaly) syndrome. Pediatrics 1983 ; 102: 559-563.
  5. Greco, M. Resta, A. Vimercati, F. Dicuonzo, G. Loverro, M. Vicino and L. Selvaggi: Antenatal diagnosis of isolated lissencephaly by ultrasound and magnetic resonance imaging. Ultrasound Obstet. Gynecol 1998; 12 : 276–279.
  6. Dobyns WB, Greenberg F. Syndromes with lissencephaly type I: Miller–Dieker and Norman–Roberts syndromes and iso-lated lissencephaly. Am J Med Genet1984; 18:509–26.
  7. Aicardi J. The agyria pachygyria complex: a spectrum of cortical malformations. Brain Dev1991; 13:1–8.
  8. Stewart RM, Richman DP, Caviness Jr. VS: Lissencephaly and pachygyria. Acta Neuropathologica 1975;  31 : 1-12.
  9. Kuzniecky RI. Magnetic resonance imaging of the develop-mental disorders of the cerebral cortex.Epilepsia1994; 35 (Suppl 6): 44–56.

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