Altered organization of the visual cortex in FHONDA syndrome

Khazar Ahmadi, Alessio Fracasso, Jelle A. van Dijk, Charlotte Kruijt, Maria van Genderen, Serge O. Dumoulin, Michael B. Hoffmann*

*Corresponding author for this work

Research output: Contribution to JournalArticleAcademicpeer-review

12 Downloads (Pure)


A fundamental scheme in the organization of the early visual cortex is the retinotopic representation of the contralateral visual hemifield on each hemisphere. We determined the cortical organization in a novel congenital visual pathway disorder, FHONDA-syndrome, where the axons from the temporal retina abnormally cross to the contralateral hemisphere. Using ultra-high field fMRI at 7 T, the population receptive field (pRF) properties of the primary visual cortex were modeled for two affected individuals and two controls. The cortical activation in FHONDA was confined to the hemisphere contralateral to the stimulated eye. Each cortical location was found to contain a pRF in each visual hemifeld and opposing hemifields were represented as retinotopic cortical overlays of mirror-symmetrical locations across the vertical meridian. Since, the enhanced crossing of the retinal fibers at the optic chiasm observed in FHONDA has been previously assumed to be exclusive to the pigment-deficiency in albinism, our direct evidence of abnormal mapping in FHONDA highlights the independence of pigmentation and development of the visual cortex. These findings thus provide fundamental insights into the developmental mechanisms of the human visual system and underline the general relevance of the interplay of subcortical stability and cortical plasticity.

Original languageEnglish
Pages (from-to)224-231
Number of pages8
Early online date8 Mar 2018
Publication statusPublished - 15 Apr 2019

Bibliographical note

Part of special issue: Mapping diseased brains


This work was supported by European Union’s Horizon 2020 research and innovation programme under the Marie Sklodowska-Curie grant agreement (No 641805 ) to M.B.H and S.O.D. and the German Research Foundation (DFG: HO2002/10-2 ) to M.B.H. The authors are grateful for cooperation of the participants and the insightful feedback from the reviewers on an earlier version of the manuscript. Appendix A

FundersFunder number
Marie Sklodowska-Curie
Horizon 2020 Framework Programme
H2020 Marie Skłodowska-Curie Actions641805
Deutsche ForschungsgemeinschaftHO2002/10-2
Horizon 2020


    • FHONDA
    • fMRI
    • Plasticity
    • pRF
    • Visual cortex


    Dive into the research topics of 'Altered organization of the visual cortex in FHONDA syndrome'. Together they form a unique fingerprint.

    Cite this