Visual Agnosia

The Argentinian author Silvina Ocampo (1903-1993) left us a vast body of works which are considered outstanding in many ways. In 1960, she published a short story, entitled “El médico encantador" (The Charming Physician), in the renowned literary magazine Sur. The central character of this piece is a family doctor named Albino Morgan, who had a secret truth: in any house he visited, all variety of disease also entered. He brought with him the viruses he disseminated. The narrator of this short story—one of his patients—describes four of Morgan's diseases. These imaginary neurological conditions allowed Ocampo to explore improbable situations in everyday life.

We describe the performance of a brain-damaged subject who suffered from visual agnosia leading to major difficulties in generating and exploiting visual representations from long-term memory. His performance in a physical judgement task in which he was required to answer questions about the visual shapes of Arabic numerals reflected his agnosic problems. However, he showed no impairment in usual number processing and calculation tasks. This case shows that, despite some commonalities in number and object processing, actual numerical processes are not affected by visual agnosia and can be preserved even when fine visual processes are impaired.

In this paper we describe a case of severe visual agnosia in a child with an electrophysiological pattern of continuous spike-wave discharges in slow sleep (CSWS) in the occipito-temporal regions. The neuropsychological spectrum related to this phenomenon is discussed. Published paediatric reports associate visual agnosia (i.e. an inability to recognize objects without impairment of visual acuity) mainly with symptomatic occipito-temporal aetiology (e.g. cortical dysplasia, vascular insults) and other neurological symptoms (e.g. autism). We describe a detailed 2 year electrophysiological and neuropsychological follow-up of an 8-year-old boy with sporadic seizures, occipito-temporal CSWS and visual agnosia. The growth and neurological development of the child had been considered as normal, neurological examination did not reveal any focal signs, visual acuity was intact and MRI was normal. First EEG and six consecutive 24 h video EEG recordings during the follow-up of 22 months showed continuous spike-and-wave activity covering over 85% of the non-REM sleep. According to structured neuropsychological tests (Wechsler Intelligence Scale for Children -Third Edition, A Developmental Neuropsychological Assessment (NEPSY), Test of Visual-Perceptual Skills, Corsi block, Hooper Visual Organization Test) the boy had normal verbal intelligence but major deficits in visual perception, especially in object recognition, impaired shape discrimination and detection, and poor copying skills. Attention and executive functions were intact. There were no difficulties in short-or long-term memory. Verbal cues and naming the objects improved visual memory. Tracing the objects with a finger or by moving the head improved object recognition. Currently the boy attends a special school with a rehabilitation plan including neuropsychological and occupational therapies. This case adds a new facet to the spectrum of neuropsychological deficits in children with CSWS. Sleep EEG should be included in the etiological studies of children with specific neuropsychological problems and detailed neuropsychological assessment is needed for diagnostic and rehabilitation purposes. q