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 Citations (Scopus)

Abstract

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
Pages (from-to)315-325
Number of pages11
JournalActa Neuropathologica
Volume36
Issue number4
DOIs
StatePublished - Dec 1 1976
Externally publishedYes

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Menkes Kinky Hair Syndrome
Mothers
Teratogenesis
Carrier State
Causality
Brain

Keywords

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

ASJC Scopus subject areas

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

Cite this

Cerebral malformation associated with metabolic disorder - A report of 2 cases. / Erdohazi, Magda; Barnes, N. D.; Robinson, M. J.; Lake, B. D.

In: Acta Neuropathologica, Vol. 36, No. 4, 01.12.1976, p. 315-325.

Research output: Contribution to journalArticle

Erdohazi, M, Barnes, ND, Robinson, MJ & Lake, BD 1976, 'Cerebral malformation associated with metabolic disorder - A report of 2 cases', Acta Neuropathologica, vol. 36, no. 4, pp. 315-325. https://doi.org/10.1007/BF00699637
Erdohazi, Magda ; Barnes, N. D. ; Robinson, M. J. ; Lake, B. D. / Cerebral malformation associated with metabolic disorder - A report of 2 cases. In: Acta Neuropathologica. 1976 ; Vol. 36, No. 4. pp. 315-325.
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