Neural heterotopia

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Classification according to ICD-10
Q04.8 Other specified congenital malformations of the brain
ICD-10 online (WHO version 2019)

A neural heterotopia , also cerebral heterotopia , is a form of a heterotopy in the central nervous system . One or more groups of nerve cells are not located in the anatomically common location, the cerebral cortex .

MRI in a child with seizures . There are small areas of gray matter heterotopy in the corpus callosum , deep below a cortical dysplasia . (Double arrow)

pathology

In the germinal matrix zone , certain neurological cells are formed by mitosis . The neurons formed there migrate to the cerebral cortex after their formation . If these do not “arrive” there and are outside the cerebral cortex and the germinal matrix zone, one speaks of heterotopias . This creates a “bundle” of gray matter in the white matter .

Classification

Depending on the localization, different forms of heterotopias can be distinguished:

  • Subependymal or periventricular heterotopias (PVNH, PNH), each adjacent to the ventricular ependyma , including Ehlers-Danlos syndrome with periventricular heterotopia
  • Subcortical heterotopias , focal subcortical heterotopias
  • Band-shaped heterotopia , also known as subcortical band heterotopy, subcortical laminar heterotopia (SCLH)

Depending on the shape, a distinction can be made between nodular heterotopia and diffuse heterotopia . The most common form, the subependymal heterotype, belongs to the nodular forms as well as the subcortical heterotopy.

causes

In addition to genetic changes, intrauterine infections or circulatory disorders are possible causes or triggers .

Occurrence

Heterotopias occur more frequently in connection with other malformations of the central nervous system such as:

Clinical manifestations

Most of those affected have focal seizures in the second decade of life. Developmental delays or intellectual disabilities can occur in children and adolescents .

Diagnosis

The imaging procedure of choice is magnetic resonance imaging . The signaling of the heterotopic tissue corresponds to that of gray matter in all sequences. In fMRI , activation of the heterotopias can be shown with BOLD contrast .

literature

  • I. Pogledic: Migration and Gyration Disorders . In: The Radiologist. 58, 2018, p. 653, doi: 10.1007 / s00117-018-0400-x .
  • AM Coady: Cranial Abnormalities: In: AM Coady, S. Bowler (Ed.): Twining's Textbook of Fetal Abnormalities, 3rd ed., Pp. 223-263, 2015
  • W. Hirsch: Heterotopia and other migration disorders in the child's brain. In: RÖFo - Advances in the field of X-rays and imaging processes. 181, 2009, doi: 10.1055 / s-0029-1221190 .

Web links

Individual evidence

  1. ^ F. Ebinger: Malformations and damage to the CNS in early childhood. Thieme, Stuttgart 2007. ISBN 978-3-13-137591-9 . P. 28f.
  2. ↑ a b B. Ertl-Wagner: Pediatric Neuroradiology. Springer, Berlin / Heidelberg 2007, ISBN 978-3-642-51767-9 .
  3. ↑ heterotopia, nodular periventricular. In: Orphanet (Rare Disease Database).
  4. ↑ Band heterotopy, subcortical. In: Orphanet (Rare Disease Database).
  5. ↑ a b c d Radiopaedia
  6. ↑ Radiopaedia Subcortial
  7. ↑ N. Di Donato: Genetics of Cortical Malformations. In: medical genetics. 30, 2018, p. 21, doi: 10.1007 / s11825-017-0165-z .
  8. ↑ a b c A. A. Abdel Razek, AY Kandell, LG Elsorogy, A. Elmongy, AA Basett: Disorders of cortical formation: MR imaging features. In: AJNR. American journal of neuroradiology. Volume 30, Number 1, January 2009, pp. 4-11, doi: 10.3174 / ajnr.A1223 , PMID 18687750 (review).
  9. ↑ a b A. J. 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 .
  10. ↑ E. Kobayashi, AP Bagshaw, C. Grova, J. Gotman, F. Dubeau: Gray matter heterotopia: what EEG-fMRI can tell us about epileptogenicity of neuronal migration disorders. In: Brain: a journal of neurology. Volume 129, Pt 2 February 2006, pp. 366-374, doi: 10.1093 / brain / awh710 , PMID 16339793 .