Cerebral malformation associated with metabolic disorder - A report of 2 cases

Magda Erdohazi, N. D. Barnes, M. J. Robinson, B. D. Lake

Research output: Contribution to journalArticle

19 Scopus citations


A clinical and neuropathological study is presented of two cases each of which showed neuronal heterotopia. Microgyria was also present in one case. One patient was suffering from a degenerative disorder affecting the white matter. The other was a case of Menkes' disease. It is suggested that the antenatal damage may have been caused by an imbalance of the maternal metabolism, the predisposing factor being the mother's carrier state for a metabolic defect. This is the first report of teratogenesis in a case of Menkes' disease. It is also noted that in this case there is interference with the postnatal as well as the antenatal development of the brain.

Original languageEnglish (US)
Pages (from-to)315-325
Number of pages11
JournalActa neuropathologica
Issue number4
StatePublished - Dec 1 1976



  • Maternal metabolism
  • Menkes' disease
  • Metabolic disorder
  • Microgyria
  • Neuronal heterotopia
  • Teratogenesis

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology

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