Anesthesia recommendations for patients suffering from Angelman syndrome - May 2012

Disease name: Angelman syndrome ICD 10: Q93.5 Synonyms: (Happy) puppet syndrome Angelman syndrome (AS) is a neuro-genetic disorder consisting of severe developmental delay, movement or balance dysfunction, a “happy demeanor” behavioral phenotype (frequent laughter/smiling, hand-flapping, etc.) and minimal or absent speech (with receptive and non-verbal communication skills more pronounced than verbal ones). Frequently (more than 80% of the time), AS is associated with microcephaly, seizures and an abnormal electroencephalogram (large amplitudes, slow spike waves, triphasic waves). Twenty to 80% of AS patients demonstrate clinical features such as tongue thrusting, prognathia, widespaced teeth, strabismus, scoliosis and fascination with water.