Focal subcortical heterotopias

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Focal subcortical heterotopias are a form of neuronal heterotopia and belong to the group of neuronal migration disorders .

pathology

For the pathology, see Neuronal Heterotopia Pathology

In focal subcortical heterotopias, the neurons have already emigrated from the germinal matrix zone and migrated towards the cerebral cortex, but they never reached it.

Classification

For the classification see neuronal heterotopia classification

In the case of subcortical heterotopias, a distinction can be made between nodular , curvilinear and mixed forms.

In the nodular form , gray matter nodules lie in the white matter and merge into the overlying cerebral cortex or the underlying lateral ventricle .

Clinical manifestations

With subcortical heterotopias, epilepsy very often occurs in childhood. It is often associated with varying degrees of developmental delay .

There are usually additional malformations such as

  • diffuse reduction in size of the affected hemisphere
  • deformed brain ventricle
  • decreased and abnormal white matter
  • thinned overlying cerebral cortex with flattened sulci
  • deformed basal ganglia

Depending on the size of the heterotopia and its effect on the overlying cerebral cortex, the extent of a motor disorder and development delay varies. Smaller or thinner heterotopias can be clinically normal.

diagnosis

As with the other heterotopias, magnetic resonance imaging is used here to examine and then the diagnosis is made. Focal subcortical heterotopias in particular are often mistaken for a tumor. In addition, other brain malformations are often diagnosed with this heterotopia.

literature

  • AJ Barkovich: Morphologic characteristics of subcortical heterotopia: MR imaging study. In: AJNR. American journal of neuroradiology. Volume 21, Number 2, February 2000, pp. 290-295, PMID 10696010 .

Individual evidence

  1. a b c Birgit Ertl-Wagner: Pediatric Neuroradiology . Springer, Berlin / Heidelberg 2007, ISBN 978-3-642-51767-9 .
  2. a b c Radiopaedia
  3. AJ Barkovich, BO Kjos: Gray matter heterotopias: MR characteristics and correlation with developmental and neurologic manifestations. In: Radiology. Volume 182, Number 2, February 1992, pp. 493-499, doi: 10.1148 / radiology.182.2.1732969 , PMID 1732969 .
  4. AJ Barkovich: Pediatric Neuroimaging. 2nd edition, Raven 1995, ISBN 0-7817-0179-1
  5. ^ S. Fukumura, T. Watanabe, S. Kimura, S. Ochi, K. Yoshifuji, H. Tsutsumi: Subcortical heterotopia appearing as huge midline mass in the newborn brain. In: Child's nervous system: ChNS: official journal of the International Society for Pediatric Neurosurgery. Volume 32, Number 2, February 2016, pp. 377-380, doi: 10.1007 / s00381-015-2841-0 , PMID 26231566 .