Altered activation and functional asymmetry of exner's area but not the visual word form area in a child with sudden-onset, persistent mirror writing

Abstract

Mirror writing is often produced by healthy children during early acquisition of literacy, and has been observed in adults following neurological disorders or insults. The neural mechanisms responsible for involuntary mirror writing remain debated, but in healthy children, it is typically attributed to the delayed development of a process of overcoming mirror invariance while learning to read and write. We present an unusual case of sudden-onset, persistent mirror writing in a previously typical seven-year-old girl. Using her dominant right hand only, she copied and spontaneously produced all letters, words and sentences, as well as some numbers and objects, in mirror image. Additionally, she frequently misidentified letter orientations in perceptual assessments. Clinical, neuropsychological, and functional neuroimaging studies were carried out over sixteen months. Neurologic and ophthalmologic examinations and a standard clinical MRI scan of the head were normal. Neuropsychological testing revealed average scores on most tests of intellectual function, language function, verbal learning and memory. Visual perception and visual reasoning were average, with the exception of below average form constancy, and mild difficulties on some visual memory tests. Activation and functional connectivity of the reading and writing network was assessed with fMRI. During a reading task, the VWFA showed a strong response to words in mirror but not in normal letter orientation – similar to what has been observed in typically developing children previously – but activation was atypically reduced in right primary visual cortex and Exner's Area. Resting-state connectivity within the reading and writing network was similar to that of age-matched controls, but hemispheric asymmetry between the balance of motor-to-visual input was found for Exner's Area. In summary, this unusual case suggests that a disruption to visual-motor integration rather than to the VWFA can contribute to sudden-onset, persistent mirror writing in the absence of clinically detectable neurological insult.