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Absence of septum pellucidum and polymicrogyria: a forme fruste of the porencephalic syndrome.

Abstract
Two cases are presented of absence of the septum pellucidum associated with bilateral polymicrogyria. In one case a circumscribed, completely enclosed cavity was present in the white matter of one cerebral hemisphere, different in structure from typical prenatal porencephaly. It is suggested that these cases represent a "forme fruste" of the syndrome of absent septum, bilateral porencephaly, polymicrogyria and heterotopia and may be ascribed to a similar, if less severe, encephaloclastic process of debatable etiology, operating around the midterm of pregnancy.
AuthorsS Siejka, A M Strefling, H Urich
JournalClinical neuropathology (Clin Neuropathol) Vol. 8 Issue 4 Pg. 174-8 ( 1989) ISSN: 0722-5091 [Print] Germany
PMID2776383 (Publication Type: Case Reports, Journal Article)
Topics
  • Adolescent
  • Adult
  • Brain (abnormalities)
  • Female
  • Hemiplegia (pathology)
  • Humans
  • Intellectual Disability (pathology)
  • Male
  • Septum Pellucidum (abnormalities)
  • Syndrome

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