Many neurodevelopmental conditions imply absent or severely reduced language capacities at school age. Evidence from functional magnetic resonance imaging is highly limited. We selected a series of five cases scanned
with the same fMRI paradigm and the aim of relating individual language profiles onto underlying patterns of
functional connectivity (FC) across auditory language cortex: three with neurogenetic syndromes (Coffin-Siris,
Landau-Kleffner, and Fragile-X), one with idiopathic intellectual ...
Many neurodevelopmental conditions imply absent or severely reduced language capacities at school age. Evidence from functional magnetic resonance imaging is highly limited. We selected a series of five cases scanned
with the same fMRI paradigm and the aim of relating individual language profiles onto underlying patterns of
functional connectivity (FC) across auditory language cortex: three with neurogenetic syndromes (Coffin-Siris,
Landau-Kleffner, and Fragile-X), one with idiopathic intellectual disability, one with autism spectrum disorder
(ASD). Compared to both a group with typical development (TD) and a verbal ASD group (total N = 110), they all
showed interhemispheric FC below two standard deviations of the TD mean. Children with higher language
scores showed higher intrahemispheric FC between Heschl’s gyrus and other auditory language regions, as well
as an increase of FC during language stimulation compared to rest. An increase of FC in forward vs. reversed
speech in the posterior and middle temporal gyri was seen across all cases. The Coffin-Siris case, the most severe,
also had the most anomalous FC patterns and showed reduced myelin content, while the Landau-Kleffner case
showed reduced cortical thickness. These results suggest potential for neural markers and mechanisms of severe
language processing deficits under highly heterogeneous etiological conditions.
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