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Year : 2015  |  Volume : 10  |  Issue : 2  |  Page : 125-126

A pediatric epilepsy classic: "Double cortex" syndrome

1 Department of Radiology, Indira Gandhi Medical College, Shimla, India
2 Department of Neuroradiology, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication22-Jun-2015

Correspondence Address:
Mandeep Singh Ghuman
Department of Neuroradiology, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.159201

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How to cite this article:
Kaur S, Ghuman MS, Devarajan LJ. A pediatric epilepsy classic: "Double cortex" syndrome. J Pediatr Neurosci 2015;10:125-6

How to cite this URL:
Kaur S, Ghuman MS, Devarajan LJ. A pediatric epilepsy classic: "Double cortex" syndrome. J Pediatr Neurosci [serial online] 2015 [cited 2022 Jun 27];10:125-6. Available from: https://www.pediatricneurosciences.com/text.asp?2015/10/2/125/159201

A 13-year-old female presented with brief dyscognitive focal seizures occurring a number of times in a day, since last few years. She also has a history of intellectual subnormality with poor scholastic performance. Her birth history was unremarkable. No similar illness was seen in the family. Computed tomography [Figure 1] and magnetic resonance imaging [Figure 2] revealed a heterotopic band of grey matter interposed between cerebral white matter and cortex with a thin rim of intervening white matter.
Figure 1: Axial computed tomography sections show smooth, thick rim of abnormal tissue in the white matter, isodense and parallel to cortical grey matter

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Figure 2: Axial T2-weighted (a and b), coronal inversion recovery (c) and double inversion recovery (d) images demonstrating the smooth, laminar tissue, with same signal intensity as that of the grey matter, located between the white matter and cortical grey matter with thin rim of subcortical white matter separating the two layers of grey matter ("double cortex")

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Subcortical band heterotopias or "double cortex" syndrome is a rare neuronal migration disorder, classically present with seizures and intellectual impairment and is seen almost exclusively in females. [1],[2],[3] It is an X-linked genetic disorder with doublecortin (DCX) gene mutation being the causative factor in most of the cases. [1] It is the same gene mutation, which is also responsible for X-linked lissencephaly in males. [2] A microtubule-associated protein is encoded by DCX gene, which is essential for neuronal migration in the in-utero cerebral embryogenesis. Most cases are sporadic, familial form has also been reported with X-linked dominant inheritance. [1]

Laminar or band heterotopias is characterized by bilateral presence of smooth ribbons of grey matter interposed in the white matter between the cerebral cortex and the ventricular surface with normally myelinated white matter seen in-between the heterotopic grey matter ribbon and the cortex. [1],[2]

Epilepsy and cognitive disability are the most common clinical presentations with seizures, any seizure type can be seen, generally starts in the first decade and more often evolves to drug refractory state. [3] Symptom severity positively correlates with the thickness of the heterotopic band of grey matter, the presence of associated cortical abnormities like pachygyria and increased ventricular size. [1]

   References Top

Bahi-Buisson N, Souville I, Fourniol FJ, Toussaint A, Moores CA, Houdusse A, et al. New insights into genotype-phenotype correlations for the doublecortin-related lissencephaly spectrum. Brain 2013;136:223-44.  Back to cited text no. 1
Parisi P, Miano S, Mei D, Paolino MC, Castaldo R, Villa MP. Diffuse subcortical band heterotopia, periodic limb movements during sleep and a novel "de novo" mutation in the DCX gene. Brain Dev 2010;32:511-5.  Back to cited text no. 2
Dericioglu N, Oguz KK, Ergun EL, Tezer FI, Saygi S. Ictal/interictal EEG patterns and functional neuroimaging findings in subcortical band heterotopia: Report of three cases and review of the literature. Clin EEG Neurosci 2008;39:43-9.  Back to cited text no. 3


  [Figure 1], [Figure 2]


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