Microcephaly associated with abnormal gyral pattern

L. Sztriha, A. Dawodu, A. Gururaj, J. G. Johansen

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

Primary microcephaly is a heterogeneous group of cerebral malformations either with a relatively well-preserved or an abnormal gyral pattern. We describe the MRI findings and clinical features of 14 children with the combination of microcephaly and an abnormal gyral pattern. All children except one were Arabs and nine out of the 14 patients were born to consanguineous parents. Seven patients showed features of a simplified gyral pattern with relatively preserved posterior fossa structures. Two boys had a cortical malformation in the agyria-pachygyria spectrum; one of these two patients showed agenesis of the corpus callosum and severe cerebellar hypoplasia as well. The microcephaly was associated with polymicrogyria and leukoencephalopathy in two patients, with cortical dysplasia and hypogenesis of the corpus callosum in one patient, with agyria-pachygyria with callosal and pontocerebellar dysplasia in one patient, and a simplified gyral pattern with severe cerebellar hypoplasia in one case. One patient died in the neonatal period and three in infancy. All patients, who survived the neonatal period, had developmental delay, intellectual disability, and neurological deficits, and nine suffered from epilepsy.

Original languageEnglish
Pages (from-to)346-352
Number of pages7
JournalNeuropediatrics
Volume35
Issue number6
DOIs
Publication statusPublished - Dec 2004

Fingerprint

Microcephaly
Lissencephaly
Agenesis of Corpus Callosum
Leukoencephalopathies
Malformations of Cortical Development
Corpus Callosum
Intellectual Disability
Epilepsy
Parents

Keywords

  • Abnormal gyral pattern
  • Cerebral malformations
  • Cortical dysplasia
  • Microcephaly

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Microcephaly associated with abnormal gyral pattern. / Sztriha, L.; Dawodu, A.; Gururaj, A.; Johansen, J. G.

In: Neuropediatrics, Vol. 35, No. 6, 12.2004, p. 346-352.

Research output: Contribution to journalArticle

Sztriha, L, Dawodu, A, Gururaj, A & Johansen, JG 2004, 'Microcephaly associated with abnormal gyral pattern', Neuropediatrics, vol. 35, no. 6, pp. 346-352. https://doi.org/10.1055/s-2004-830430
Sztriha, L. ; Dawodu, A. ; Gururaj, A. ; Johansen, J. G. / Microcephaly associated with abnormal gyral pattern. In: Neuropediatrics. 2004 ; Vol. 35, No. 6. pp. 346-352.
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