EEG in Epilepsy Series: Angelman Syndrome
- Roya Tompkins
- 9 hours ago
- 2 min read
Angelman syndrome (AS) is a rare neuro‑developmental disorder that affects one in 15,000 people.
Angelman syndrome shares symptoms and characteristics with other disorders including autism and cerebral palsy so due to the common characteristics, misdiagnosis can occur.
These developmental concerns become noticeable by the age of 6 – 12 months.
What can we expect in the EEG?
The pattern most frequently seen both in children and adults are prolonged runs of high amplitude rhythmic 2-3 Hz activity predominantly over the frontal regions with superimposed interictal epileptiform discharges.
High amplitude rhythmic 4-6 Hz activity with spikes, prominent in the occipital regions. This pattern can be facilitated by eye closure.
These patterns can exist in Angleman Syndrome patients regardless of whether they have a history of seizures or not.
What can we expect clinically?
Angelman syndrome symptoms include:
Developmental delays, including no crawling or babbling at 6 to 12 months.
No speech or little speech.
Trouble walking, moving or balancing.
Smiling and laughing often and seeming happy.
Being easy to excite.
Trouble sucking or feeding.
Trouble going to sleep and staying asleep.
People who have Angelman syndrome also may have:
Seizures, often beginning between 2 and 3 years of age.
Stiff or jerky movements.
Small head size by age 2.
Tongue thrusting.
Hair, skin and eyes that are light in color.
Hand flapping and arms uplifted while walking.
Strabismus.
Scoliosis.
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Roya Tompkins, MS, REEG/EP T, RPSGT
Resources:
Anglican Syndrome Foundation
Mayo Clinic
National Institute of Health