Abstracts

RATIONALE: To describe a case of severe visual agnosia in otherwise neurologically normal child with persistent continuous spike-and-wave activity in the occipital regions during slow wave sleep (CSWS). METHODS: Published reports associate visual agnosia (i.e. inability to recognize objects without impairment of visual acuity) with localized cortical lesions in the occipito-temporal regions (cortical dysplasia, SSPE, meningitis, tumors, vascular insults, traumas etc.) in children with often also other neurological symptoms (e.g. autism, mental retardation). 7-year old boy was admitted to our hospital due to generalized tonic-clonic seizure during a febrile respiratory infection without any previous neurological history. RESULTS: First EEG and consecutive five 24 hour video-EEG recordings during the following 16 months showed continuous spike-and-wave activity covering approximately 80% of the nonREM sleep with the maximal topographic distribution bilaterally on the occipito-temporo-parietal regions with the spike initiation on the left hemisphere. This was not affected by adequate doses of sodium valproate, ethosuximide and lamotrigine in mono- or polytherapy. According to structured neuropsychological tests the boy had normal verbal intelligence but major problems in object recognition, both shape discrimination and detection, and poor coping ability. There were no difficulties in short- or long-term memory. Naming the objects improved visual memory and verbal hints also increased visual object recognition. Currently the boy attends special school with rehabilitation plan including neuropsychological and occupational therapies. CONCLUSIONS: If a failure of auditory impulses to reach consciousness can be due to epileptiform discharges as in the well-defined epileptic syndrome of Landau-Kleffner, it is highly likely that visual pathways can be similarly affected - of which our case offers preliminary evidence. It is probable that also other regions of brain can be affected by the same way and specific but profound neuropsychological problems may be produced -but easily overlooked- in children without manifest motor seizures.