Asystole during outbursts of laughing in a child with Angelman syndrome.
A girl with Angelman syndrome had recurrent episodes of ventricular asystole and syncope caused by severe vagal hypertonia during outbursts of laughing. After intravenous administration of atropine, laughing no longer induced asystole or syncope. The vast majority of patients with Angelman syndrome have seizures. Since hypoxia associated with asystole can provoke convulsions, we suggest electrocardiographic evaluation of Angelman patients with symptomatic bradycardia, loss of consciousness, or convulsions related to laughing.
Vanagt
W Y
WY
Department of Pediatrics, Division of Pediatric Cardiology, University Hospital Maastricht, P.O. Box 5800, Maastricht, AZ, 6202, The Netherlands. ward.vanagt@fys.unimaas.nl
Pulles-Heintzberger
C F
CF
Vernooy
K
K
Cornelussen
R N
RN
Delhaas
T
T
D000293:Adolescent; D017204:Angelman Syndrome / Q000503:physiopathology* / Q000628:therapy; D001285:Atropine / Q000008:administration & dosage; D004562:Electrocardiography / Q000187:drug effects; D005260:Female; D006323:Heart Arrest / Q000503:physiopathology* / Q000517:prevention & control; D006801:Humans; D007845:Laughter / Q000502:physiology*; D010276:Parasympatholytics / Q000008:administration & dosage / Q000627:therapeutic use; D014630:Vagus Nerve / Q000187:drug effects / Q000503:physiopathology*; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use
D010276:Parasympatholytics; D014665:Vasodilator Agents; D001285:Atropine