Absence of septum pellucidum and polymicrogyria: a forme fruste of the porencephalic syndrome.
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.
Siejka
S
S
Department of Neuropathology, Royal Perth Hospital, Western Australia.
Strefling
A M
AM
Urich
H
H
D000293:Adolescent; D000328:Adult; D001921:Brain / Q000002:abnormalities*; D005260:Female; D006429:Hemiplegia / Q000473:pathology; D006801:Humans; D008607:Intellectual Disability / Q000473:pathology; D008297:Male; D012688:Septum Pellucidum / Q000002:abnormalities*; D013577:Syndrome