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J Neurodev Disord. 2017 May 8;9:17. doi: 10.1186/s11689-017-9195-8. eCollection 2017.

Delta rhythmicity is a reliable EEG biomarker in Angelman syndrome: a parallel mouse and human analysis.

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Department of Cell Biology and Physiology, University of North Carolina, Chapel Hill, NC 27599 USA.
Carolina Institute for Developmental Disabilities, University of North Carolina, Chapel Hill, NC 27599 USA.
Neuroscience Center, University of North Carolina, Chapel Hill, NC 27599 USA.
Department of Neurology, Massachusetts General Hospital, Boston, MA 02114 USA.
Harvard Medical School, Boston, MA 02215 USA.
Present Address: The Neurology Foundation, Rhode Island Hospital and Warren Alpert School of Medicine at Brown University, Providence, RI 02903 USA.
Department of Pediatrics, University of California, San Diego, CA USA.
Division of Dysmorphology/Genetics, Rady Children's Hospital, San Diego, CA USA.



Clinicians have qualitatively described rhythmic delta activity as a prominent EEG abnormality in individuals with Angelman syndrome, but this phenotype has yet to be rigorously quantified in the clinical population or validated in a preclinical model. Here, we sought to quantitatively measure delta rhythmicity and evaluate its fidelity as a biomarker.


We quantified delta oscillations in mouse and human using parallel spectral analysis methods and measured regional, state-specific, and developmental changes in delta rhythms in a patient population.


Delta power was broadly increased and more dynamic in both the Angelman syndrome mouse model, relative to wild-type littermates, and in children with Angelman syndrome, relative to age-matched neurotypical controls. Enhanced delta oscillations in children with Angelman syndrome were present during wakefulness and sleep, were generalized across the neocortex, and were more pronounced at earlier ages.


Delta rhythmicity phenotypes can serve as reliable biomarkers for Angelman syndrome in both preclinical and clinical settings.


Angelman syndrome; Biomarker; Delta; EEG; Mouse model; Outcome measure; UBE3A

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