Visual Inattention in West Syndrome: A Neuropsychological and Neurofunctional Imaging Study

Abstract

Visual behavior is frequently impaired at onset of West syndrome (WS). We studied the neuropsychological outcome of eight children who had cryptogenic WS and moderate to severe visual impairment at the onset of epilepsy. At the last examination, a regional cerebral blood flow study using SPECT (single photon emission computed tomography) was performed. The behavior abnormalities observed initially evolved to various defects of cognitive function. Three patients had severe mental retardation with autistic features. Two had marked speech disorders but one had global cognitive impairment. Three patients had specific visual-spatial deficits. SPECT showed perfusion defects involving the parieto-occipital areas in 6 of 8 patients. These abnormalities were restricted to parieto-occipital regions in the three patients with selective visual-spatial deficits, whereas they were associated with other perfusion defects in the three remaining patients. This study demonstrates that the visual inattention observed at the onset of WS is frequently associated with long-term cognitive and/or perfusion defects involving the parieto-occipital regions.

Neuronal Networks in West Syndrome as Revealed by Source Analysis and Renormalized Partial Directed Coherence

“…Focal posterior cortical hypometabolism has been found in a great proportion of children with the West syndrome (Chugani et al, 1990, Jambaque et al, 1993, Metsahonkala et al, 2002. There is a discrepancy, however, concerning the relation between the occipital cortex and pathological changes in hypsarrhythmia.…”

Is hypsarrhythmia a form of non-convulsive status epilepticus in infants?

“…It is well recognized that hypsarrhythmia occurring without any clinically striking ictus can be associated with developmental regression, with a particularly well-described pattern being loss of visual function (20). This idea might be more valid when there is no history of clinical ictus.…”

Autism and visual agnosia in a child with right occipital lobectomy

“…Children with delayed visual maturation may exhibit transient autistic behaviour 1415 Central visual disturbances also contribute largely to the autistic picture seen in children who develop infantile spasms 16. Futhermore, several cases of prosopagnosia in childhood suggest an association between autism and developmental visual agnosia17-20 although some cases are clearly non-autistic 21-23.…”