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Seizures and epilepsy in Sotos syndrome: Analysis of 19 Caucasian patients with long-term follow-up

  1. Francesco Nicita1,
  2. Martino Ruggieri2,
  3. Agata Polizzi3,
  4. Laura Mauceri4,
  5. Vincenzo Salpietro5,
  6. Silvana Briuglia5,
  7. Laura Papetti1,
  8. Fabiana Ursitti1,
  9. Salvatore Grosso6,
  10. Luigi Tarani7,
  11. Maria Segni7,
  12. Salvatore Savasta8,
  13. Pasquale Parisi9,
  14. Alberto Verrotti10,
  15. Alberto Spalice1

Article first published online: 21 FEB 2012

DOI: 10.1111/j.1528-1167.2012.03418.x

Issue

Epilepsia

Epilepsia

Volume 53, Issue 6, pages e102–e105, June 2012

Additional Information

How to Cite

Nicita, F., Ruggieri, M., Polizzi, A., Mauceri, L., Salpietro, V., Briuglia, S., Papetti, L., Ursitti, F., Grosso, S., Tarani, L., Segni, M., Savasta, S., Parisi, P., Verrotti, A. and Spalice, A. (2012), Seizures and epilepsy in Sotos syndrome: Analysis of 19 Caucasian patients with long-term follow-up. Epilepsia, 53: e102–e105. doi: 10.1111/j.1528-1167.2012.03418.x

Author Information

  1. 1

    Child Neurology Division, Department of Pediatrics, Policlinico Umberto I, Sapienza University of Rome, Roma, Italy

  2. 2

    Department of Formative Processes, University of Catania, Catania, Italy

  3. 3

    National Center for Rare Diseases, Superior Institute of Health, Rome, Italy

  4. 4

    Health Care Center for Obstetrics and Gynecology “Lucina,” Catania, Italy

  5. 5

    Unit of Genetics and Immunology, Department of Pediatrics, University of Messina, Messina, Italy

  6. 6

    Department of Pediatrics, Gynecology, Obstetrics, and Reproductive Medicine, Pediatric Neurology Section, University of Siena, Siena, Italy

  7. 7

    Department of Pediatrics, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy

  8. 8

    Department of Pediatrics, University of Pavia, Pavia, Italy

  9. 9

    Department of Pediatrics, Sant’Andrea Hospital, Sapienza University of Rome, Rome, Italy;

  10. 10

    Department of Pediatrics, University of Chieti, Chieti, Italy

*Address correspondence to Alberto Spalice, Child Neurology Division, Department of Pediatrics, Policlinico Umberto I, Sapienza University of Rome, Viale Regina Elena 324, 00161, Roma, Italy. E-mail: childneurology.sapienzaroma@live.it

Publication History

  1. Issue published online: 1 JUN 2012
  2. Article first published online: 21 FEB 2012
  3. Accepted January 10, 2012; Early View publication February 21, 2012.

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Keywords:

  • Sotos syndrome;
  • Seizures;
  • Epilepsy;
  • Cerebral gigantism;
  • Overgrowth syndrome

Summary

Sotos syndrome (SS) is an overgrowth syndrome characterized by typical facial appearance, learning disability, and macrocephaly as cardinal diagnostic features. Febrile (FS) and afebrile seizures are reported in 9–50% of cases. There is no evidence that patients with SS and FS later develop epilepsy, and no studies have investigated the electroclinical features and the long-term outcome in epileptic SS patients. The authors report a series of 19 SS patients with FS and/or epilepsy during childhood and a long-term follow-up. More than half of FS evolved to epilepsy. Temporal lobe seizures were recorded in 40% of patients with SS. Seizures were easy to control with common antiepileptic drugs in almost all patients. A careful neurologic evaluation is useful for SS patients, since seizures are an important finding among people with this overgrowth syndrome.

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