title: Cardiovascular and neuroendocrine features of Panayiotopoulos syndrome in three siblings.
abstract: OBJECTIVE
  Panayiotopoulos syndrome is a benign idiopathic childhood epilepsy characterized by altered autonomic activity at seizure onset.
  
  
  METHODS
  Three siblings with Panayiotopoulos syndrome underwent 24-hour EEG recording and head-up tilt testing with continuous blood pressure and RR interval monitoring. Plasma catecholamines and vasopressin were measured while supine, upright, and during a typical seizure.
  
  
  RESULTS
  Patient 1, a 12-year-old girl, had a history of involuntary lacrimation, abdominal pain, and recurrent episodes of loss of muscle tone and unresponsiveness followed by somnolence. Her EEG revealed bilateral frontotemporal spikes. Patient 2, a 10-year-old boy, had episodic headaches with pinpoint pupils, skin flushing of the face, trunk, and extremities, purple discoloration of hands and feet, diaphoresis, nausea, and vomiting. Tilt testing triggered a typical seizure after 9 minutes; there was a small increase in blood pressure (+5/4 mm Hg, systolic/diastolic) and pronounced increases in heart rate (+59 bpm) and norepinephrine (+242 pg/mL), epinephrine (+175 pg/mL), and vasopressin (+22.1 pg/mL) plasma concentrations. Serum glucose was elevated (206 mg/dL). His EEG revealed right temporal and parietal spikes. Patient 3, an 8-year-old boy, had a history of restless legs at night, enuresis, night terrors, visual hallucinations, cyclic abdominal pain, and nausea. His EEG showed bitemporal spikes.
  
  
  CONCLUSIONS
  Hypertension, tachycardia, and the release of vasopressin suggest activation of the central autonomic network during seizures in familial Panayiotopoulos syndrome. These autonomic and neuroendocrine features may be useful in the diagnosis and may have therapeutic implications.
authors.affiliation: Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. gonzalezduarte@aol.com
authors.forename: Alejandra
authors.initials: A
authors.lastname: Gonzalez-Duarte
authors.forename: Lucy
authors.initials: L
authors.lastname: Norcliffe-Kaufmann
authors.forename: Jose
authors.initials: J
authors.lastname: Martinez
authors.forename: Alcibiades J
authors.initials: AJ
authors.lastname: Rodriguez
authors.forename: Ruben
authors.initials: R
authors.lastname: Kuzniecky
authors.forename: Felicia
authors.initials: F
authors.lastname: Axelrod
authors.forename: Horacio
authors.initials: H
authors.lastname: Kaufmann
mesh_terms: D002318:Cardiovascular Diseases / Q000175:diagnosis / Q000209:etiology*; D002395:Catecholamines / Q000097:blood; D002648:Child; D002851:Chromatography, High Pressure Liquid; D004569:Electroencephalography; D004700:Endocrine System Diseases / Q000175:diagnosis / Q000209:etiology*; D020936:Epilepsy, Benign Neonatal / Q000150:complications*; D019305:Epilepsy, Rolandic / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D010991:Plethysmography; D035781:Siblings; D013057:Spectrum Analysis; D014667:Vasopressins / Q000097:blood
chemical_list: D002395:Catecholamines; D014667:Vasopressins