Clinical features and evolution of the gelastic seizures-hypothalamic hamartoma syndrome

Salvatore Striano, Pasquale Striano

Research output: Contribution to journalArticle

Abstract

Gelastic seizures, usually with onset in early infancy, are the hallmark manifestation of hypothalamic hamartoma. This seizure type is directly generated by hamartoma itself, intrinsically epileptogenic because of its anatomofunctional organization. Other types of seizures, focal or generalized, may appear during the evolution, probably resulting from mechanisms of secondary epileptogenesis. Nevertheless, the clinical expression and the severity of the syndrome, ranging from a focal drug-resistant epilepsy to a catastrophic generalized encephalopathy with severe cognitive and behavioral impairments, depends on the size and the site of attachment of the hamartoma. Early suspicion, timely diagnosis, and appropriate treatment are mandatory to reverse a potential catastrophic evolution of this condition.

Original languageEnglish
Pages (from-to)12-15
Number of pages4
JournalEpilepsia
Volume58 Suppl 2
DOIs
Publication statusPublished - Jun 2017

Keywords

  • Child
  • Child Behavior Disorders
  • Child, Preschool
  • Cognition Disorders
  • Disease Progression
  • Drug Resistant Epilepsy
  • Early Diagnosis
  • Early Medical Intervention
  • Electroencephalography
  • Epilepsies, Partial
  • Epilepsy, Generalized
  • Hamartoma
  • Humans
  • Hypothalamic Diseases
  • Hypothalamus
  • Infant
  • Kindling, Neurologic
  • Positron-Emission Tomography
  • Prognosis
  • Radiosurgery
  • Signal Processing, Computer-Assisted
  • Syndrome
  • Journal Article

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