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Aberrant supracallosal longitudinal bundle: MR features, pathogenesis and associated clinical phenotype

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Abstract

Objective

To describe the MRI and structural features of a peculiar malformation of the corpus callosum (CC) in a group of young patients with intellectual disability.

Methods

We studied with conventional MRI and DTI a group of subjects showing an aberrant supracallosal bundle, characterized by the presence of a triangle-shaped bulging above the dorsal surface of CC on the midline. Clinical evaluations, CGH-array and instrumental analysis were also collected.

Results

Among 85 patients with malformed CC, we identified 15 subjects that showed the supracallosal bundle. The CC was thickened in five cases, long and thinned in three cases, short and thinned in three cases and it had a “ribbon-like” appearance in four subjects. Additional brain anomalies were present in eight cases. DTI colour maps and tractography showed that the bundle had an antero-posterior longitudinal orientation and that the tract bifurcated posteriorly, ending in the posterior hippocampi. Patients had different combinations of neurological symptoms, but all showed mild or severe intellectual disability.

Conclusions

Combining radiological and genetic data with embryological knowledge of the development of cerebral commissures, we hypothesize that the supracallosal bundle represents a vestigial structure, the dorsal fornix, present during fetal life. Its persistence is associated with intellectual disability.

Key Points

• An aberrant longitudinal bundle can be detected above corpus callosum.

• The presence of the supracallosal bundle is associated with intellectual disability.

• The supracallosal bundle may represent a persistent dorsal fornix.

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Abbreviations

CC:

Corpus callosum

DTI:

Diffusion tensor imaging

VF:

Volume fraction

FA:

Fractional anisotropy

AD:

Axial diffusivity

RD:

Radial diffusivity

MD:

Mean diffusivity

ROI:

Region of interest

IG:

Indusium griseum

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Acknowledgments

The scientific guarantor of this publication is Dr. Fabio Triulzi. The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article. This study has received funding by the Italian Ministry of Health to Dr. Arrigoni (RC2012) and Dr. Borgatti. No complex statistical methods were necessary for this paper. Institutional Review Board approval was obtained. Written informed consent was obtained from all subjects (patients) in this study. Methodology: retrospective, cross sectional study, performed at one institution.

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Authors and Affiliations

  1. Neuroimaging Laboratory, Scientific Institute IRCCS Eugenio Medea, Via don Luigi Monza 20, 23842, Bosisio Parini, Lecco, Italy

    Filippo Arrigoni & Denis Peruzzo

  2. Neuropsychiatry and Neurorehabilitation Unit, Scientific Institute IRCCS Eugenio Medea, Bosisio Parini, Lecco, Italy

    Romina Romaniello & Renato Borgatti

  3. Department of Pediatric Radiology and Neuroradiology, Children Hospital V. Buzzi, Milano, Italy

    Andrea Righini & Cecilia Parazzini

  4. Child Psychopathology Unit, Scientific Institute IRCCS Eugenio Medea, Bosisio Parini, Italy

    Paola Colombo

  5. Laboratory of Molecular Biology, Scientific Institute IRCCS Eugenio Medea, Bosisio Parini, Lecco, Italy

    Maria Teresa Bassi

  6. Neuroradiology Department, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy

    Fabio Triulzi

Authors
  1. Filippo Arrigoni
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  2. Romina Romaniello
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  3. Denis Peruzzo
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  4. Andrea Righini
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  7. Maria Teresa Bassi
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  8. Fabio Triulzi
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  9. Renato Borgatti
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Corresponding author

Correspondence to Filippo Arrigoni.

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Arrigoni, F., Romaniello, R., Peruzzo, D. et al. Aberrant supracallosal longitudinal bundle: MR features, pathogenesis and associated clinical phenotype. Eur Radiol 26, 2587–2596 (2016). https://doi.org/10.1007/s00330-015-4084-6

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  • DOI: https://doi.org/10.1007/s00330-015-4084-6

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