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Impaired growth and abnormal microstructure of supratentorial gray and white matter regions in a child with Joubert syndrome


Bosemani, T; Baum, J; Meoded, A; Boltshauser, E; Huisman, T A; Poretti, A (2015). Impaired growth and abnormal microstructure of supratentorial gray and white matter regions in a child with Joubert syndrome. Neurographics, 5(5):209-213.

Abstract

The interaction between the cerebellum and cerebrum forms the anatomic basis of the cerebellar role in neurocognitive functions. We performed whole-brain atlas-based analysis of volumetric and DTI data in a 16-month-old boy with Joubert syndrome and 5 age-matched controls. In our patient, we found a decrease in volume of multiple gray and white matter regions, and reduced fractional anisotropy in selected white matter regions. These findings result from trophic trans-synaptic effects and disconnection of the cerebellar-cerebral pathway secondary to a malformed cerebellum. In addition, we found an increase in fractional anisotropy in multiple cortical gray matter regions, which may reflect disrupted cortical neuronal radial glial cell scaffolding and neuronal laminar organization.

Abstract

The interaction between the cerebellum and cerebrum forms the anatomic basis of the cerebellar role in neurocognitive functions. We performed whole-brain atlas-based analysis of volumetric and DTI data in a 16-month-old boy with Joubert syndrome and 5 age-matched controls. In our patient, we found a decrease in volume of multiple gray and white matter regions, and reduced fractional anisotropy in selected white matter regions. These findings result from trophic trans-synaptic effects and disconnection of the cerebellar-cerebral pathway secondary to a malformed cerebellum. In addition, we found an increase in fractional anisotropy in multiple cortical gray matter regions, which may reflect disrupted cortical neuronal radial glial cell scaffolding and neuronal laminar organization.

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Additional indexing

Item Type:Journal Article, not refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2015
Deposited On:29 Jan 2016 13:36
Last Modified:22 Nov 2017 13:39
Publisher:American Society of Neuroradiology
ISSN:1541-6593
Publisher DOI:https://doi.org/10.3174/ng.5150126
Related URLs:http://www.ingentaconnect.com/content/asnr/ng/2015/00000005/00000005/art00004 (Publisher)

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