Septo-optic dysplasia plus: case report

Septo-optic dysplasia (SOD) is a syndrome composed by optic nerve and septum pellucidum dysgenesis. It has been classified into two subsets according to the embryogenesis and the neuropathological findings. Basically, the difference between these two groups is the presence or not of schizencephaly....

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Bibliographic Details
Published in:Arquivos de neuro-psiquiatria 2003-09, Vol.61 (3A), p.671-676
Main Authors: Gasparetto, Emerson L, Warszawiak, Danny, de Carvalho Neto, Arnolfo de, Benites Filho, Paulo R, Bruck, Isac, Antoniuk, Sérgio
Format: Article
Language:English
Subjects:
Algorithms
computed tomography
Evoked Potentials
Female
Humans
Infant
Magnetic Resonance Imaging
NEUROSCIENCES
Optic Nerve - abnormalities
PSYCHIATRY
septo-optic dysplasia
Septo-Optic Dysplasia - diagnostic imaging
Septo-Optic Dysplasia - pathology
septum pellucidum
Septum Pellucidum - abnormalities
Tomography, X-Ray Computed
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Summary:Septo-optic dysplasia (SOD) is a syndrome composed by optic nerve and septum pellucidum dysgenesis. It has been classified into two subsets according to the embryogenesis and the neuropathological findings. Basically, the difference between these two groups is the presence or not of schizencephaly. The term SOD-Plus was recently proposed to describe SOD associated with cortical dysplasia. We report a 6-month-old female patient who presented absent visual fixation since 4 months of age and delayed psychomotor development. Neurological examination demonstrated spastic left hemiparesis and ophtalmological evaluation revealed bilateral optic disc hypoplasia. The head computed tomography (CT) scan showed absence of the septum pellucidum, ventricular asymmetry and schizencephaly. The magnetic resonance imaging (MRI) showed complete absence of the septum pellucidum associated to optic nerves and chiasma atrophy, schizencephaly and cortical dysplasia. The patient underwent an evoked potential examination with flash stimulation, which revealed bilateral absence of cortical evoked potential. She was referred to visual stimulation and physiotherapy. We emphasize the neuroimaging of this syndrome and stress the importance of the clinical investigation for patients with septum pellucidum dysgenesis on MRI or CT scans.
ISSN:0004-282X
1678-4227
0004-282X
1678-4227
DOI:10.1590/s0004-282x2003000400028