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Development of a standard of care for patients with valosin-containing protein associated multisystem proteinopathy.
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In: Orphanet journal of rare diseases, vol 17, iss 1 (2022)
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Family history of FXTAS is associated with age-related cognitive-linguistic decline among mothers with the FMR1 premutation.
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In: Journal of neurodevelopmental disorders, vol 14, iss 1 (2022)
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Cortical microstructure in primary progressive aphasia: a multicenter study.
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In: Alzheimer's research & therapy, vol 14, iss 1 (2022)
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Hippocampal ensembles represent sequential relationships among an extended sequence of nonspatial events.
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In: Nature communications, vol 13, iss 1 (2022)
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Neural dynamics of semantic categorization in semantic variant of primary progressive aphasia.
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Resting functional connectivity in the semantic appraisal network predicts accuracy of emotion identification.
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| 7 |
Development of thalamus mediates paternal age effect on offspring reading: A preliminary investigation.
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In: Human brain mapping, vol 42, iss 14 (2021)
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B Cell-Restricted Depletion of Dnmt3a Activates Notch Signaling and Causes Chronic Lymphocytic Leukemia
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In: Blood, vol 138, iss Supplement 1 (2021)
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Comparing two facets of emotion perception across multiple neurodegenerative diseases.
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In: Social cognitive and affective neuroscience, vol 15, iss 5 (2020)
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Amount and delay insensitivity during intertemporal choice in three neurodegenerative diseases reflects dorsomedial prefrontal atrophy.
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Depressive Symptom Profiles Predict Specific Neurodegenerative Disease Syndromes in Early Stages.
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Depressive Symptom Profiles Predict Specific Neurodegenerative Disease Syndromes in Early Stages.
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Deformation-based shape analysis of the hippocampus in the semantic variant of primary progressive aphasia and Alzheimer's disease.
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| 14 |
Graded, multidimensional intra- and intergroup variations in primary progressive aphasia and post-stroke aphasia.
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In: Brain : a journal of neurology, vol 143, iss 10 (2020)
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Regional and hemispheric susceptibility of the temporal lobe to FTLD-TDP type C pathology.
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| 16 |
Amount and delay insensitivity during intertemporal choice in three neurodegenerative diseases reflects dorsomedial prefrontal atrophy.
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| 17 |
A neurophysiological model of speech production deficits in fragile X syndrome.
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In: Brain communications, vol 2, iss 1 (2020)
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Abstract:
Fragile X syndrome is the most common inherited intellectual disability and monogenic cause of autism spectrum disorder. Expressive language deficits, especially in speech production, are nearly ubiquitous among individuals with fragile X, but understanding of the neurological bases for these deficits remains limited. Speech production depends on feedforward control and the synchronization of neural oscillations between speech-related areas of frontal cortex and auditory areas of temporal cortex. Interaction in this circuitry allows the corollary discharge of intended speech generated from an efference copy of speech commands to be compared against actual speech sounds, which is critical for making adaptive adjustments to optimize future speech. We aimed to determine whether alterations in coherence between frontal and temporal cortices prior to speech production are present in individuals with fragile X and whether they relate to expressive language dysfunction. Twenty-one participants with full-mutation fragile X syndrome (aged 7-55 years, eight females) and 20 healthy controls (matched on age and sex) completed a talk/listen paradigm during high-density EEG recordings. During the talk task, participants repeated pronounced short vocalizations of 'Ah' every 1-2 s for a total of 180 s. During the listen task, participants passively listened to their recordings from the talk task. We compared pre-speech event-related potential activity, N1 suppression to speech sounds, single trial gamma power and fronto-temporal coherence between groups during these tasks and examined their relation to performance during a naturalistic language task. Prior to speech production, fragile X participants showed reduced pre-speech negativity, reduced fronto-temporal connectivity and greater frontal gamma power compared to controls. N1 suppression during self-generated speech did not differ between groups. Reduced pre-speech activity and increased frontal gamma power prior to speech production were related to less intelligible speech as well as broader social communication deficits in fragile X syndrome. Our findings indicate that coordinated pre-speech activity between frontal and temporal cortices is disrupted in individuals with fragile X in a clinically relevant way and represents a mechanism contributing to prominent speech production problems in the disorder.
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Keyword:
2.1 Biological and endogenous factors; Behavioral and Social Science; Brain Disorders; Clinical Research; EEG; event-related potential; fragile X syndrome; Intellectual and Developmental Disabilities (IDD); Mental Health; Neurological; neurophysiology; Neurosciences; Pediatric; speech production
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URL: https://escholarship.org/uc/item/06m5m363
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| 18 |
The Relationship between Expressive Language Sampling and Clinical Measures in Fragile X Syndrome and Typical Development.
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In: Brain sciences, vol 10, iss 2 (2020)
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Contributions of common genetic variants to risk of schizophrenia among individuals of African and Latino ancestry.
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In: Molecular psychiatry, vol 25, iss 10 (2020)
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Contributions of common genetic variants to risk of schizophrenia among individuals of African and Latino ancestry.
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In: Molecular psychiatry, vol 25, iss 10 (2020)
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