Mid-gestation right basal ganglia lesion

DeLong, G. Robert

doi:10.1212/wnl.59.1.54

Cited by 14 publications

(1 citation statement)

References 28 publications

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“…58 Our group has suggested that disruption to the caudate may disrupt anxiety and socioemotional behavior in FraX. 4 Evidence from lesion studies have implicated the caudate in dyscontrol of emotion, 59 depression, inattention, high distractibility, and frequent expressions of fear 60 —all symptoms commonly occurring in people with FraX. Taken together, the FraX group may be less able to inhibit emotional responses, particularly toward faces.…”

Section: Discussionmentioning

confidence: 99%

Aberrant Neural Function During Emotion Attribution in Female Subjects With Fragile X Syndrome

Hagan

Hoeft

Mackey

et al. 2008

Journal of the American Academy of Child & Adolescent Psych

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Objective Fragile X (FraX) syndrome is caused by mutations of the FraX mental retardation–1 gene—a gene responsible for producing FraX mental retardation protein. The neurocognitive phenotype associated with FraX in female subjects includes increased risk for emotional disorders including social anxiety, depression, and attention deficit. Here, the authors investigated the neurobiological systems underlying emotion attribution in female subjects with FraX syndrome. Method While undergoing functional magnetic resonance imaging, 10 high-functioning female subjects with FraX syndrome and 10 typically developing (TD) female subjects were presented with photographs of happy, sad, and neutral faces and instructed to determine the facial emotion. Results No significant group differences were found for the recognition of happy faces, although the FraX group showed a trend toward a significant difference for the recognition of sad faces and significantly poorer recognition of neutral faces. Controlling for between-group differences in IQ and performance accuracy, the TD group had greater activation than the FraX group in the anterior cingulate cortex (ACC) for neutral faces compared with scrambled faces and the caudate for sad faces compared with scrambled faces (but not for sad faces compared with neutral faces). In the FraX group, FraX mental retardation protein levels positively correlated with activation in the dorsal ACC for neutral, happy, and sad faces when independently compared with scrambled faces. Significantly greater negative correlation between IQ and insula activation for neutral faces relative to scrambled faces was observed in the FraX group compared with the TD group. Significantly greater positive correlation between IQ and ACC activation for neutral faces relative to scrambled faces was observed in the TD group compared with the FraX group. Conclusions Although emotion recognition is generally spared in FraX syndrome, the emotion circuit (i.e., ACC, caudate, insula) that modulates emotional responses to facial stimuli may be disrupted.

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