Septo-optic dysplasia plus: case report
Arq. neuropsiquiatr
;
61(3A): 671-676, Sept. 2003. ilus
Article
Dans Anglais
| LILACS
| ID: lil-345793
ABSTRACT
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
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Nerf optique
/
Septum pellucidum
/
Dysplasie septo-optique
Limites du sujet:
Femelle
/
Humains
/
Bébé
langue:
Anglais
Texte intégral:
Arq. neuropsiquiatr
Thème du journal:
Neurologie
/
Psychiatrie
Année:
2003
Type:
Article
Pays d'affiliation:
Brésil
Institution/Pays d'affiliation:
University of Paraná/BR
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