Abstracts

Rationale: Septo-optic dysplasia plus (SOD- plus ) is a rare congenital disorder of midline prosencephalic structures with optic nerve hypoplasia, dysgenesis of corpus callosum, pituitary-hypothalamic dysfunction and malformations of cortical development. We present our experience in the presurgical evaluation of patients with this condition. Methods: Our database was interrogated for cases of SOD-plus. All patients underwent full clinical examination, including neurological exam, video-EEG, and MRI of the brain. When indicated patients underwent investigation with intracranially placed electrodes. Results: Three patients were identified in our database (2 female). Mean age was 31 years (range 22-46), 2 were right handed, all with long-history of complex partial seizures (one patient), multiple seizure type (one patient), and generalized tonic-clonic seizures (2 patients). In one patient there was total anosmia, while hemiplegia was seen in the other two cases. One patient also had left optic atrophy and congenital nystagmus. In two cases, the interictal EEG revealed normal background and temporal abnormalities (spikes and/or slowing), and in the other case background slowing and multifocal spikes were seen. Ictal recordings revealed a focal onset in two patients, one of them with left temporal onset, but with complex propagation on subdural recordings; and the other one with right temporal onset. The latter case underwent anterior temporal neocortical resection, but seizure recurrence was seen and has continued intractable to medical intervention. One case also had non-epileptic events. MRI findings were similar in all cases, the SOD was found to be associated with other malformations of cortical development (focal cortical dysplasia, subcortical band heterotopia, schizencephaly, and polymicrogyria). Conclusions: SOD-plus is a rare heterogenous syndrome with variable initial course and clinical presentation, from mild neurodevelopmental problems to intractable epilepsy. The epileptic spectrum comprises of both focal and generalised seizures, with variable age of onset with poor response to medical therapy.