[Septo-optic dysplasia (de Morsier syndrome)].
Septo-optic dysplasia (or de Morsier syndrome) is a congenital disorder characterised by anomalies in cerebral midline structures, optic nerve hypoplasia, and hormonal deficiencies. Diagnosis should be made early, due to the possibility of treating the hormonal disturbances. We describe here a case with decreased visual acuity, one-sided hemianopia, nystagmus und agenesis of the septum pellucidum and discuss the heterogeneous appearance of this syndrome. There are two theories regarding its pathogenesis. The first postulates simultaneous damage to both cerebral structures and optic nerve development around the 6th week of gestation, while the other favours secondary degeneration of optic nerve fibres due to a cerebral lesion.
Stangel
M
M
Abteilung für Neurologie, Universitätsklinikum Benjamin Franklin, Freie Universität Berlin.
Vogeley
K T
KT
Jandeck
C
C
Boegner
F
F
Marx
P
P
Koch
H C
HC
D000293:Adolescent; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D009900:Optic Nerve / Q000002:abnormalities*; D012688:Septum Pellucidum / Q000002:abnormalities*; D013577:Syndrome; D015354:Vision, Low / Q000151:congenital*
Septooptische Dysplasie (de-Morsier-Syndrom).