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Neuroimage Clin. 2015 Oct 30;10:18-26. doi: 10.1016/j.nicl.2015.10.016. eCollection 2016.

Neural correlates of abnormal sensory discrimination in laryngeal dystonia.

Author information

1
Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, USA.
2
Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, USA ; Head and Neck Surgical Group, New York, USA.
3
Trinity Centre for Bioengineering, Trinity College Dublin, Ireland.
4
Department of Neurology, St. Vincent's University Hospital, Dublin, Ireland.
5
Department of Neurology, Massachusetts General Hospital, Charlestown, MA, USA.
6
Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, USA ; Otolaryngology, Icahn School of Medicine at Mount Sinai, New York, USA.

Abstract

Aberrant sensory processing plays a fundamental role in the pathophysiology of dystonia; however, its underpinning neural mechanisms in relation to dystonia phenotype and genotype remain unclear. We examined temporal and spatial discrimination thresholds in patients with isolated laryngeal form of dystonia (LD), who exhibited different clinical phenotypes (adductor vs. abductor forms) and potentially different genotypes (sporadic vs. familial forms). We correlated our behavioral findings with the brain gray matter volume and functional activity during resting and symptomatic speech production. We found that temporal but not spatial discrimination was significantly altered across all forms of LD, with higher frequency of abnormalities seen in familial than sporadic patients. Common neural correlates of abnormal temporal discrimination across all forms were found with structural and functional changes in the middle frontal and primary somatosensory cortices. In addition, patients with familial LD had greater cerebellar involvement in processing of altered temporal discrimination, whereas sporadic LD patients had greater recruitment of the putamen and sensorimotor cortex. Based on the clinical phenotype, adductor form-specific correlations between abnormal discrimination and brain changes were found in the frontal cortex, whereas abductor form-specific correlations were observed in the cerebellum and putamen. Our behavioral and neuroimaging findings outline the relationship of abnormal sensory discrimination with the phenotype and genotype of isolated LD, suggesting the presence of potentially divergent pathophysiological pathways underlying different manifestations of this disorder.

KEYWORDS:

Brain imaging; Endophenotype; Sensory processing

PMID:
26693398
PMCID:
PMC4660380
DOI:
10.1016/j.nicl.2015.10.016
[Indexed for MEDLINE]
Free PMC Article

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