Abstract
An EEG study has been carried out on 19 children (including siblings in 3 families) with clinical features of Angelman syndrome. The age at time of the first EEG ranged from 11 months to 11 years with the majority under 5 years. Six children had no history of seizures at the time of the first EEG. One or more of the following EEG abnormalities were seen in all patients: 1. Persistent rhythmic 4–6/s activities reaching more than 200 μV not associated with drowsiness. 2. Prolonged runs of rhythmic 2–3/s activity (200–500 μV) often more prominent anteriorly, sometimes associated with discharges (ill-defined spike/wave complexes). 3. Spikes mixed with 3–4/s components usually more than 200 μV mainly posteriorly and facilitated by, or only seen with, eye closure. Two and sometimes three of these EEG features could be present in the same record particularly at a young age. The appearance of discharges mixed with slow components on eye closure was the commonest finding seen at some stage in 17 patients (aged from 11 months to over 12 years). The EEG features of Angelman syndrome appear to be sufficiently characteristic to help identify patients at an early age before the clinical features become obvious and at a time when genetic counselling may be particularly important.
Similar content being viewed by others
References
Al-Mateen M, Philippart M, Shields WD (1986) Rett syndrome. A commonly overlooked progressive encephalopathy in girls. AJDC 140:761–765
Angelman H (1965) “Puppet” Children. A report of three cases. Dev Med Child Neurol 7:681–687
Baraitser M, Patton M, Lam S, Brett E, Wilson J (1987) The Angelman (Happy puppet) syndrome. It is autosomal recessive? Clin Genet 31:323–330
Bower BD, Jeavons PM (1967) The “Happy Puppet” syndrome. Arch Dis Child 42:298–302
Dooley JM, Berg JM, Pakula Z, MacGregor DL (1981) The Puppet-like syndrome of Angelman. Am J Dis Child 135:621–624
Elian M (1975) Fourteen Happy Puppets. Two new cases and a review. Clin Pediatr 14:902–908
Hagberg B, Aicardi J, Dias K, Ramos O (1983) A progressive syndrome of autism, dementia, ataxia and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases. Ann Neurol 14: 471–479
Kuroki Y, Matsui I, Yamamoto Y, Ieshima A (1980) The “Happy Puppet” syndrome in two siblings. Hum Genet 56:227–229
Mayo O, Nelson MM, Townsend HRA (1973) Three more “Happy Puppets”. Dev Med Child Neurol 15:63–74
Moore JR, Jeavons PM (1973) The “Happy Puppet” syndrome: two new cases and a review of five previous cases. Neuropädiatrie 4:172–179
Niedermeyer E, Rett A, Renner H, Murphy M, Naidu S (1986) Rett syndrome and the Electroencephalogram. Am J Med Genet 24:195–199
Pampiglione G (1977) Development of rhythmic EEG activities in infancy (waking state). Rev EEG Neurophysiol 7:327–334
Pampiglione G, Martinez A (1983) Evolution of Angelman syndrome. Follow up of 3 new cases. Electroencephalogr Clin Neurophysiol 56:72P
Pashayan HM, Singer W, Bove C, Eisenberg E, Seto B (1982) The Angelman syndrome in two brothers. Am J Med Genet 13: 295–298
Rett A (1966) Über ein eigenartiges hirnatrophisches Syndrom bei Hyperammonämie in Kindesalter. Wien Med Wochenschr 116:723–726
Verma NP, Chheda RL, Nigro M, Hart ZH (1986) Electroencephalographic findings in Rett syndrome. Electroencephalogr Clin Neurophysiol 64:394–401
Williams CA, Frias JL (1982) The Angelman (“Happy Puppet”) Syndrome. Am J Med Genet 11:453–460
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Boyd, S.G., Harden, A. & Patton, M.A. The EEG in early diagnosis of the Angelman (Happy Puppet) syndrome. Eur J Pediatr 147, 508–513 (1988). https://doi.org/10.1007/BF00441976
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00441976