Download PDF
Neuropediatrics 1998; 29(3): 113-119
DOI: 10.1055/s-2007-973545
Original articles

© Hippokrates Verlag GmbH Stuttgart

Microlissencephaly: A Heterogeneous Malformation of Cortical Development

A. J. Barkovich1 , 2 , 3 , Donna M. Ferriero2 , 3 , R. M. Barr1 , 4 , P. Gressens5 , W. B. Dobyns6 , Ch. L. Truwit7 , Ph. Evrard5
  • 1Neuroradiology, Room L-371, Mecklenburg Radiology Associates, Charlotte, NC 28222, USA
  • 2Department of Neurology, Mecklenburg Radiology Associates, Charlotte, NC 28222, USA
  • 3Department of Pediatrics, Mecklenburg Radiology Associates, Charlotte, NC 28222, USA
  • 4Present Address: Mecklenburg Radiology Associates, Charlotte, NC 28222, USA
  • 5Faculté de Médicine Xavier-Bichat (Université de Paris VII), Hôpital Robert-Debré, 48 Boulevard Serurier, Paris, France,
  • 6Department of Neurology and Pediatrics University of Minnesota School of Medicine, USA
  • 7Department of Radiology, University of Minnesota School of Medicine, USA
Further Information

Publication History

Publication Date:
12 March 2007 (online)

  PDF Download  Permissions and Reprints

Abstract

We report the neonatal courses, early postnatal development, and neuroimaging findings of 17 patients with marked microcephaly and simplified cerebral gyral patterns, a condition that we call microlissencephaly. Retrospective analyses of the clinicoradiologic features of these patients allowed segregation of the patients into 5 distinct groups with varying outcomes. The apparent discreteness of these groups suggests multiple etiologies of this malformation, although there appears to be a strong genetic component with probable autosomal recessive inheritance. Utilizing the neonatal course and neuroradiologic features of these infants allows classification of specific subsets, which may be useful to predict outcome.

Key words

Microcephaly - Magnetic resonance imaging - Malformations of cortical development - Microlissencephaly

      >