Septo-optic dysplasia plus: case report

Arquivos de Neuro-Psiquiatria

Emerson L. Gasparetto¹  Danny Warszawiak¹  Arnolfo De Carvalho Neto²  Paulo R. Benites Filho¹  Isac Bruck¹  Sérgio Antoniuk¹ 

[1] ,University of Paraná School of Medicine Department of Internal Medicine

关键词: magnetic resonance imaging;    computed tomography;    septo-optic dysplasia;    septum pellucidum;    ressonância magnética;    tomografia computadorizada;    displasia septo-óptica;    septo pelúcido;   

DOI  :  10.1590/S0004-282X2003000400028

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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